Lower Extremity Length Discrepancies

6.1 Terminology side owing to the associated pelvic obliquity and the
uncovering of the femoral head associated with this, and
Lower extremity length discrepancies refer to differences in (iv) low back pain in association with the compensatory
length between the two extremities which can be due to lumbar scoliosis (Fig. 6.1). It is the general experience that
some or all of pelvic, femoral, tibial, and foot height dif- those with discrepancies >2.5 cm warrant treatment. In those
ferences. The total length differential in the standing position with projected discrepancies of <2.0 cm, there is no defini-
is not dependent solely on the height of the involved bones tive evidence that leaving these discrepancies untreated will
but can be altered further by unilateral joint dislocation or lead to long-term degenerative problems. The zone of
subluxation, particularly at the hip but also at the sacroiliac uncertainty concerning the need for limb equalization is
or knee joints; by asymmetric femoral–tibial angular defor- between 2.0 and 2.5 cm shortness. The extent of any dis-
mity; by asymmetric hip, knee, or ankle contractures; and by crepancy must also be considered in relation to the indi-
fixed pelvic obliquity. The term leg length discrepancy is vidual’s total height since the effect of the same discrepancy
still used commonly to refer to this broad entity but is is relatively more marked in someone 5 ft tall than in another
imprecise. In strict anatomic terminology, leg refers to the >6 ft tall.
segment between the knee and the ankle rather than to the
entire extremity. The terms lower limb or lower extremity
length discrepancy are more accurate [1, 2]. 6.2.2 Percentage of Individuals with Equal
Limb Lengths

6.2 Clinically Significant Length 6.2.2.1 Clinical-Radiological Data

Discrepancies Only 25–50 % of people have equal lower extremity lengths.
Hasse and Dehner over 100 years ago (1893) reported leg
6.2.1 General Guidelines Concerning Extent length differences in 68 % of 5,141 soldiers with the left
of Clinically Significant Length longer than the right in a proportion of 3.3 to 1 [3]. Lower
Discrepancies extremity lengths were equal in only 32 %. Edinger and
Biedermann documented lower extremity lengths in 351
There are no absolute numbers concerning the amount of individuals radiologically [4]. Both extremities were of equal
length differences which require treatment. For most prac- length in 178 (51 %), had <5 mm difference in 27 (8 %), and
titioners in this field any discrepancy projected at skeletal had discrepancies between 5 and 50 mm in 146 (42 %). The
maturity to be >2.0–2.5 cm warrants treatment whereas left was longer than the right in a proportion of 3.6 to 1.
anything under that cannot be expected definitively to have Rush and Steiner documented 1,000 healthy army recruits
serious negative long-term sequelae. For those wishing to referred to the radiology department because of a low back
“fine-tune” discrepancy management, there is some evidence complaint [5] and 100 soldiers without complaint. An
that discrepancies as little as 1.25 cm or ½ in. predispose to anteroposterior radiograph of the lumbosacral spine, pelvis,
negative sequelae but discrepancies less than that are rarely and proximal femurs in the standing position was made in
treated surgically by even the most ardent practitioners. The standardized fashion. The lower edge of the film was parallel
negative sequelae of a lower extremity length discrepancy to the standing platform such that any differences in the level
involve (i) an asymmetric appearance, (ii) an awkward gait, of the femoral heads indicated a difference in lower
(iii) the possibility of osteoarthritis of the hip on the longer extremity length. Length differences were measured in

© Springer International Publishing Switzerland 2016 613

F. Shapiro, Pediatric Orthopedic Deformities, Volume 1, DOI 10.1007/978-3-319-20529-8_6
614 6 Lower Extremity Length Discrepancies

combined, only 5 % had equal lengths in 727 individuals.

The left was longer in 54 % and the right was longer in 41 %
with a left/right predominance of 1.3 to 1. These studies
were similar to clinical measurements made by Haase and
Dehner [3]. Garson measured 70 skeletons noting that the
combined femur and tibia lengths were longer on the left in
54.3 % and longer on the right in 35.8 %, with equal lengths
being present in 10 % [7]. The combined femoral–tibial
differences when the left was longer averaged 4.8 mm and
when the right was longer averaged 3.3 mm. In 124 skele-
tons from Switzerland, Schwerz found the left femur longer
in 52 %, the right femur longer in 31 %, and both equal in
17 % [8]. Almost all studies show smaller discrepancies with
the tibia. In all racial groups and in both sexes asymmetries
favoring the left bone of the lower extremity, in particular in
relation to the femur, are much more frequent than those
Fig. 6.1 The clinical consequences of a lower extremity length favoring the right. The absolute amounts of discrepancy,
discrepancy are illustrated. Lower extremity length discrepancy with
however, are small with the series in adult man showing a
shortness on the left is associated with a compensatory lumbar scoliosis,
pelvic obliquity, and a relative adduction positioning of the hip on the general average of only 2.5 mm difference in the femurs. The
longer right side. Note the diminution of the CE angle on the longer humeral differences were greater, however, averaging
right side which can predispose to superolateral osteoarthritis 4.1 mm longer on the right. Munter compiled a detailed
analysis of 326 (233 male/93 female) adult Anglo-Saxon
millimeters. The limbs were equal in length in 23 % of the skeletons although all bones were not available from each
larger mildly symptomatic group and in 29 % of the smaller skeleton [9]. The mean values for femoral and tibial lengths
fully asymptomatic group. Based on this study, only 25 % of in his series were also greater on the left side for both males
individuals had equal lower extremity lengths. and females. The mean femoral lengths in the male were
2.4 mm greater on the left side for the femur and 4.5 mm
6.2.2.2 Anthropological Data longer on the left side for the tibia. In females, the left side
Anthropological data involving skeletal measurements of was longer for the femur by 4.8 mm and on the tibia the left
paired long bones in man also demonstrate a tendency to side was longer by a mean of 1.5 mm. Munter noted from
increased length of the left femur compared to the right with several other series that there was little to no racial variation
a left/right predominance also present but less marked in the in side differences or in proportional differences such that
tibia. Schultz reported on skeletal measurements from 753 data could be pooled for more statistical significance. There
human skeletons involving 232 white, 233 American black, is little presentation of absolute data in the Munter study
118 North American Indian, 122 Alaskan Eskimo, 41 Chi- since most of the assessments related to correlative values.
nese, and 7 Australian aboriginal skeletons [6]. On average, All the basic numerical data, however, are presented in the
the humerus and femur were approximately 8 % shorter in appendix such that the bilateral differences for each indi-
females than in males. The measurements were made to the vidual could be calculated.
nearest millimeter. In virtually all studies reported, the right
upper extremity is longer than the left, usually in the range of
75 % of cases. This is felt to reflect the right-handed dom- 6.2.3 Clinical Effects of Lower Extremity
inance in the human species. Virtually, all studies however, Length Discrepancies
also show the length of the femur and tibia to be longer on
the left in those where equal limb lengths are not present. Possible negative sequelae of lower extremity length dis-
When measurements in 744 individuals were averaged, in all crepancies have been studied in efforts to relate symptoms to
races the left femur was longer than the right in 50 %, the particular amounts of discrepancy (Fig. 6.1). Gurney has
right was longer than the left in 33 %, and both were equal in written an excellent overview of the subject concentrating on
only 17 %. The left/right predominance was therefore 1.5 to studies attempting to assess the clinical sequelae of various
1. Differences were less marked in the tibia. In 734 indi- amounts of discrepancy [10]. The major considerations
viduals, the left was greater than the right in 45 %, the right assessed below concern the relationship of lower extremity
was greater than the left in 40 %, and the right equaled the length discrepancies to hip pain and eventual osteoarthritis
left in only 15 %. When both femur and tibia were of the hip, osteoarthritis of the knee, scoliosis, low back pain
6.2 Clinically Significant Length Discrepancies 615

and sciatica, and gait disturbances with associated physio- >1.25 cm (½ in.). The differences in lower extremity lengths
logical sequelae. were determined radiographically with strict application of a
standardized standing orthoroentgenographic technique. It
6.2.3.1 Osteoarthritis of the Hip was the superolateral variant of osteoarthritis that was par-
Primary osteoarthritis (OA) of the hip is quite common as is ticularly related to the length discrepancy. The more global
osteoarthritis secondary to the major childhood hip abnor- or medial forms of OA were not assessed and none of the
malities of developmental dysplasia, Legg–Calve–Perthes patients had clear predisposing causes of OA such as hip
disease and slipped capital femoral epiphysis. It is thus very subluxation or dislocation or residual evidence of any of the
difficult to document with statistical certainty whether a other childhood hip disorders. When measurements alone
length discrepancy alone is the primary determinant of a hip were considered 4 patients had OA on the shorter side, 3 had
arthritic condition. Friberg reported that in 254 patients with OA but were level, and 29 had OA on the longer side. When
lower extremity length discrepancies complaining of chronic a correction was made to allow for an estimation of the
hip pain, 226 (88.9 %) had the pain on the longer extremity original prearthritic leg length discrepancy by adding 5 mm
side [11]. In 28 cases (11.1 %), pain was located in the hip or 3/16 of an inch to the side with the OA to represent the
on the shorter side. amount of articular surface collapse, even more patients
Morscher has clearly discussed the changes in hip joint were shifted to the group showing length discrepancy with
mechanics due to leg length discrepancies [12]. He pointed the longer side affected. They concluded that length dis-
out that studies by Pauwels had shown that a lesser amount crepancy was present in at least 33 of the 36 cases with the
of pressure is actually transmitted to the hip joint of the longer side developing the OA.
shorter leg owing to the pelvic tilt which serves to increase
the area of contact between the femoral head and the 6.2.3.2 Osteoarthritis of the Knee
acetabulum [13]. There is also truncal shift over the short In a study of 3,026 adults, 50–79 years of age with or at a
side further minimizing the effort needed for hip abduction. high risk for knee osteoarthritis, osteoarthritis in the shorter
Conversely, the weight-bearing characteristics of the longer leg was more commonly seen than in the longer [17]. This
side hip are worsened since there is a decrease in coverage of relationship became more common the greater the discrep-
the femoral head by the acetabulum on the longer side owing ancy. Inequality as little as 0.5–1.0 cm increased the risk for
to the nature of the pelvic tilt and increased load at the joint osteoarthritis primarily in the shorter leg. Inequality of 1 cm
on the longer side due both to diminution of the area of or more was associated with increased odds of having knee
contact between the femoral head and the acetabulum and radiographic osteoarthritis in the shorter leg (53 vs. 36 %)
also to the increase in tone necessitated for the hip abductor and inequality of 2 cm or more was associated with even
muscles with increased distance between origin and inser- greater odds of having radiographic knee osteoarthritis in the
tion. He likened the action of a lower extremity length dis- shorter leg (68 vs. 37 %).
crepancy on the longer side to a coxa valga deformity. The
extent of increased coverage of the femoral head on the short 6.2.3.3 Scoliosis, Low Back Pain, and Sciatica
side and decreased coverage on the long side has been The correlation of compensatory lumbar scoliosis with lower
documented by Krakovits using a trigonometric series of extremity length discrepancies has also been studied as have
calculations [14]. When the leg is shortened by 1 cm, the attempts to equate the shortness with increases in lumbar
diminution of the CE angle of Wiberg on the longer side is discomfort and sciatica. The relationship between shorten-
2.3°; 2 cm shortening = diminution of 4.6°; 3 cm = 6.8°; ing, compensatory scoliosis, and discomfort, however, has
4 cm = 9.1°; 5 cm = 11.3°; 6 cm = 13.5°; 7 cm = 15.6°; been difficult to document. Morscher describes the attempts
8 cm = 17.7°; 9 cm = 19.8°; and 10 cm = 21.8°. at correlations well [12]. He noted that Hult found that
Gofton also supported the contention that stresses almost 54 % of patients with lower extremity length dis-
imposed on the longer side hip are greater than normal with crepancies complained of lumbar pain but that 60 % of
those on the shorter side reduced [15]. Acetabular pressure patients without such discrepancies had similar complaints
on the longer side was concentrated laterally due to the [18]. Electromyographic studies by Taillard and Morscher
adducted position of the proximal femur leading to supero- showed that relatively small leg length discrepancies
lateral femoral head OA. Gofton and Trueman detected a between 1 and 2 cm could lead to a remarkable increase in
clear association between the idiopathic superolateral muscle activity in several muscle groups [19]. The possi-
osteoarthritis of the hip and the lower extremity length dis- bility remains therefore that even small discrepancies make it
crepancy with the hip on the longer side involved in 33 of 36 difficult to maintain a complete resting position owing to
instances [16]. There were 31 of the 36 cases showing OA secondary muscle activations.
on the longer side with discrepancies from 5 mm (3/16 of an Scoliosis: A lower extremity length discrepancy leads to a
inch) and greater with 16 of the 36 having a discrepancy clinically detectable compensatory lumbar scoliosis in the
616 6 Lower Extremity Length Discrepancies

standing position with pelvic tilt, low on the shorter side, and recruits with low back pain and a smaller group of 100
the curve convex to the shorter side. With the patient sitting without pain, the percentages of lower extremity length
or the short side elevated by standing on blocks, the pelvis differences in the larger/smaller groups were as follows: 1–
levels and the scoliosis corrects. Forward bend in the 5 mm 39.5/38 %; 6–10 mm 22.5/29 %; 11–20 mm 13.3/4 %;
standing position also eliminates compensatory scoliosis. and 21 mm +1.7/0 % [5]. The combined patients with length
The relationship between lumbar scoliosis and lower discrepancies 5 mm or less were 62.5/67 % and with length
extremity length discrepancies is not invariably the same. In discrepancies 10 mm (1 cm) or less were 85/96 %. It was
the large majority of cases, the convexity of the lumbar only beyond the 11 mm discrepancy level that the percent-
scoliosis is directed toward the shorter side but in perhaps age incidence of back symptoms increased beyond the
10–15 % of cases, the scoliosis is contralateral to that control range. The length discrepancies were then correlated
expected on a purely mechanical basis. Morscher indicates with the pathological conditions of the spine seen on
that the development of a lumbar scoliosis may be due more anteroposterior and lateral radiographs. The radiographic
to dynamic forces in association with walking rather than abnormalities were present in the same percentage of
static forces as demonstrated in the standing position [12]. patients with equal limb lengths and in those with length
Low back pain: The findings in scoliosis due to leg length discrepancies with 25 % of each group showing changes.
inequality (discrepancy) have been assessed and are depen- The authors concluded that in the symptomatic group it
dent on the age of the patient as well as the extent of the could have been length discrepancy itself with the associated
discrepancy and the length of time it remains uncorrected. compensatory scoliosis rather than the radiographic abnor-
Papaioannou et al. studied 23 young adults with untreated malities that was responsible for the symptoms.
limb length inequality (1.2–5.2 cm) present since childhood Nichols used clinical tape measurements from anterior
[20]. They used anteroposterior radiographs in the standing superior iliac spine to the tip of the medial malleolus to
position without correction of the discrepancy and after document that 7 % of 1,007 patients without back pain had a
correction with a wooden block. They assessed the Cobb length discrepancy of 1.25 cm (½ in.) or more while a limb
angle, axial rotation of the vertebrae, and pelvic asymmetry. length discrepancy of 1.25 cm or more was seen in 22 % of
There was no significant pelvic asymmetry. The lateral curve 180 airmen complaining of low back pain [22]. In his review
was always convex to the short side (without limb equal- of the Rush-Steiner data, he also concluded that a significant
ization), almost invariably from L1 to L5, and associated difference in the incidence of back pain and a shortened
with a definite rotatory deformity. After neutralization of the lower extremity could only be seen when the discrepancy
inequality, the Cobb angle was decreased but not always was 11 mm or more.
totally corrected. The lumbar scoliosis with limb length Giles and Taylor studied the relationship of lower
inequality was compensatory and nonprogressive but extremity length inequality and low back pain in 1,309
abnormalities of the Cobb angle and axial rotation are rarely patients and a small control group of 50 [23]. The prevalence
corrected fully with neutralization of the discrepancy. of length discrepancy of 1 cm or more was more common in
In a slightly older group of patients with limb inequality patients suffering from low back pain (18.3 % of 1,309) than
(mean age 37 years), the compensatory lumbar scoliosis in the normal population (8 % of 50 controls). Friberg
showed not only lateral bend but also axial rotation of the studied the correlation between lower extremity length dis-
lumbar vertebrae, compression of the intervertebral disc crepancy, low back pain and chronic unilateral hip dis-
spaces on the concavity, and pelvic rotation [11]. All of comfort [11]. The study comprised 1,157 subjects; 653
these eventually led to pain with disc and nerve root patients with chronic low back pain with or without sciatica,
irritation. 254 with chronic unilateral hip pain, and 359 symptom-free
In a comparison of spinal radiographs of patients with army conscripts. In the total series, the lower extremities
chronic low back pain comparing 50 with 1 cm or more of were of equal length in only 8 % of patients. The left lower
length discrepancy (compensatory scoliosis) and 50 with extremity was longer than the right by a ratio of 1.4 to 1.
straight spines (no compensatory scoliosis), asymmetric There was excellent correlation between an increase in the
structural changes in the former group included asymmetric amount of lower extremity length discrepancy and both back
end-plate contours of the lumbar vertebral bodies, wedging pain and hip discomfort. The incidence of low back pain in
of the fifth lumbar vertebra, and lateral traction spurs and the lower extremity length groupings 5.0–9.0 mm, 10.0–
osteophytes (especially in those over 40 years of age) [21]. 14.0 mm, and 15.0 mm or more was 45.3, 18.4, and 11.7 %,
Low back pain itself is quite common and relatively large respectively, with the numbers of the symptom-free group at
numbers of patients very carefully studied would be needed the same length discrepancies much less at 27.9, 13.4, and
to determine whether or not there was an increased preva- 2.2 %. The ratio of symptomatic to nonsymptomatic back
lence in those with lower extremity length discrepancies pain patients with limb lengths of 5.0 mm or more was 1.73
alone. In the study by Rush and Steiner involving 1000 army to 1; 10.0 mm or more, 1.93 to 1; and 15.0 mm or more,
6.2 Clinically Significant Length Discrepancies 617

5.32–1. A discrepancy of 1.5 cm or more therefore clearly pain was not a significant problem” [30]. Hoikka et al. also
appeared to predispose the individual to a relatively high noted that leg length inequality and lumbar scoliosis have a
likelihood of back discomfort; the presence of leg length poor correlation with low back pain in 100 young or
inequality of 1.5 cm or more was 5.32 times more likely in middle-aged adults suffering from chronic low back pain
653 patients with chronic low back pain than in 359 [31]. Gibson et al. found that otherwise healthy young adults
symptom-free soldiers. Friberg also refers to several studies with an average of 3 cm of limb length discrepancy per-
where “the high prevalence of LLI (leg length inequality) in ceived no functional effect [32], while Gross evaluated 35
patients with low-back symptoms has been established.” marathon runners and found 7 with limb length discrepan-
Sciatica: Sciatica predisposed to radiate into the longer cies ≥ 1 cm who reported no effects upon their performance
side by a ratio of 3.7 to 1 [11], similar to findings in the [33].
small series of Redler [24] where in 15 cases of sciatica, it
was present on the longer side by a 2 to 1 ratio. 6.2.3.4 Gait Asymmetry
Ten Brinke et al. found that 62 % of 104 patients with a Kaufman et al. performed detailed gait studies on 20 subjects
length discrepancy of 1 mm or more had pain radiating to the to determine the magnitude of discrepancies that resulted in
shorter leg [25]. gait abnormalities [34]. A limb length inequality >2 cm
Back Pain Before and After Length Discrepancy Treat- (3.7 % difference) resulted in gait asymmetry that was
ment: The review by Gurney refers to four studies that greater than that observed in the normal population. Com-
indicated substantial to complete relief of low back pain in pensatory mechanisms to lengthen the shorter side include
mild-to-moderate length discrepancies with use of a shoe lift downward pelvic obliquity, increasing knee extension in
on the short side [10]. Friberg also refers to several reports, midstance, vaulting, toe walking, and combinations of these.
including his own, of “positive results of the simple and On the longer side, mechanisms include increasing pelvic
non-invasive shoe-lift under the foot of the short leg” in obliquity, circumduction of the limb, increasing hip or knee
relieving back pain [11]. flexion, and increasing ankle dorsiflexion. For minimal dis-
Rossvoll et al. assessed back pain in young adult patients crepancies, the foot can also participate with the shorter side
before and after subtrochanteric shortening osteotomies of foot-supinating (inversion) and the longer side pronating
the femur performed after skeletal maturity [26]. There were (eversion). This number correlates well with the generally
22 patients followed for an average of 5 years. The mean accepted clinical guideline beyond which length discrepancy
preoperative length discrepancy was 3.2 cm with follow-up correction is warranted.
discrepancy diminished to 0.43 cm. Approximately, half of Goel et al. performed gait analysis for discrepancies
the patients had relatively serious low back pain prior to <2 cm to determine the maximum moments at the hip, knee,
surgery with the other half having minimal to no low back and ankle joints [35]. They concluded that a minor length
pain. The mean ages at operation in the two groups were discrepancy of 1.2 cm did not produce meaningful biome-
25.9 and 20.2 years. The degree of low back pain was felt to chanical changes and that the body was well able to com-
be significantly reduced after the operation. Tjernstrom and pensate for minor lower extremity length discrepancies up to
Rehnberg studied back pain before and after leg lengthening 2 cm. They studied 10 healthy subjects with equal limb
procedures [27]. Before their 100 lengthenings, 18 patients lengths, simulated minor limb length discrepancies using a
in the age range 15–36 years experienced low back pain but shoe lift of 1.25 cm, and an additional 10 asymptomatic
after an average 6 years postoperative follow-up only 6 patients with limb length discrepancies ranging from 1 to
experienced pain. Bhave et al. also noted improvement of 2 cm. Their study “did not find an association between minor
lumbosacral pain after lengthening [28]. Eleven of 18 limb length discrepancies and predictable changes in lower
patients had pain prelengthening for a mean discrepancy of extremity joint kinetics that might potentially lead to joint
4.9 cm with none having pain at follow-up after correction to abnormalities.”
within 1 cm discrepancy. Gurney et al. found a breakpoint in induced artificial limb
Asymptomatic Regarding Back Pain with Limb Leg length discrepancy between 2 and 3 cm studying several
Length Discrepancies: Some studies failed to identify any parameters in adults on a treadmill [36]. There was a sig-
relationship between low back pain and leg length inequal- nificant increase in oxygen consumption and perceived
ity. Soukka et al. studied 247 males and females 35–54 years exertion with 2, 3, and 4 cm discrepancies; a significant
of age [29]. There were 53 symptom-free individuals found increase in heart rate, minute ventilation, and quadriceps
to have a mean 5.5 ± 4.1 mm shortness, while 78 persons activity in the longer limb with 3 and 4 cm discrepancies;
with severe low back pain had a similar mean of and a significant increase in plantar flexor activity in the
5.3 ± 4.0 mm shortness. Yrjonen et al. followed 96 patients shorter limb with a 4 cm discrepancy.
with Legg–Perthes for several decades noting that White et al. noted that the shorter limb sustained a greater
“leg-length inequality was a common finding but low-back proportion of load and loading rates in those with 1–3 cm
618 6 Lower Extremity Length Discrepancies

discrepancy [37]. Walsh et al. found that pelvic obliquity walking with the foot in an equinus position on the shorter
was the most common mechanism to compensate for dis- side. Assessment of a patient with a lower extremity length
crepancies up to 2.2 cm with larger discrepancies leading to discrepancy should check for the range of motion of the
knee flexion on the longer side for compensation [38]. ankle as in some of the larger longstanding discrepancies,
Bhave et al. studied objective gait parameters in 18 shorter this equinus posturing can become partially fixed with active
and longer limbs before and after limb lengthening and dorsiflexion decreased compared to the normal longer side
compared to findings to 20 subjects with no limb length ankle.
discrepancy [28]. The difference in mean stance times To measure the extent of discrepancy, rectangular blocks
between shorter and longer limbs was 12 % before length- of known height are placed under the foot on the shortened
ening and only 2.4 % after lengthening. Length differences side with the patient standing in bare feet with hips and
were corrected to within 1 cm. The differences between the knees fully extended. The blocks are positioned until the
two limbs in those without discrepancy were 2 %. compensatory scoliosis disappears and the iliac crests can be
Song et al. studied mechanical work during gait of 35 palpated at the same level. This clinical assessment is
children with true limb length discrepancies by assessing essential as it accurately denotes the entire extent of any
compensatory mechanisms on frontal and sagittal plane discrepancy including pelvic, thigh, leg, and foot
videotape [39]. The average discrepancy for the 7 patients components.
with no observable compensatory strategy was Another characteristic clinical measurement of lower
1.64 ± 2.83 cm (2.2 % of the length of the long extremity). extremity length discrepancy, done with a tape measure and
For this range of deformity they felt no treatment was nec- the patient lying supine, measures the distance from the
essary. The average discrepancy for the children who used inferior tip of the anterior superior iliac spine to the inferior
toe-walking as a compensatory strategy was 6.54 ± 2.83 cm tip of the medial malleolus on each side. Jamaluddin et al.
(10.4 %). They also found no correlation between limb have shown in a study of 48 patients that the tape mea-
length discrepancy and pelvic obliquity or abductor muscle surement method with a nearest reading of 5 mm is as
strength. The compensatory gait strategies they observed accurate as computerized tomography (CT) scanograms.
were equinus of the ankle (toe-walking) on the short side, [40]. It is important to check that there is no pelvic obliquity,
vaulting over the longer limb (at knee and hip), and cir- hip or knee contractures, or femoral–tibial angular defor-
cumduction of the longer limb. mities in the patients being assessed in this way. If these
conditions are present, measurement from the umbilicus to
the medial malleolus can also be performed to register what
6.3 Limb Length Determination is referred to as the apparent limb length discrepancy. Smith
has reported a clinical method for determining lower
6.3.1 Clinical Measurements extremity length discrepancy which is particularly valuable
in those with hip or knee flexion contractures [41]. The
Clinical examination of a patient with a lower extremity method, referred to as the thigh-leg inspection test, is per-
length discrepancy remains the basic form of assessment. formed by placing the patient in the supine position with the
When viewed from the back in the standing position, one hips and knees flexed 90°. Determination of the difference in
looks for a compensatory scoliosis, palpates the levels of the height of the thighs is made by using a flat protractor on the
iliac crests, and examines for the levels of the buttock and most superior aspect of the knee joint on the longer side and
popliteal creases, the presence of a plantigrade foot, and the measuring the difference between the protractor and the
thigh and calf circumferences. Children with lower extremity shortened thigh at the knee. The shortened leg is then
length discrepancies use compensatory mechanisms to measured as the thighs are held parallel. The difference in
maintain an upright alignment. In the standing position with length of the longer side heel and the shortened heel is
the feet flat and the knees fully extended, a compensatory measured by placing the protractor on the plantar surface of
lumbar scoliosis is seen with the curve convex on the the longer heel and measuring the distance between it and
shortened side. With forward bend or in the sitting position, the shorter heel. This measurement eliminates flexion con-
the scoliosis disappears, as there is no longer need for any tractures at the hips, knees, and ankles as a source of error. It
compensation. A rotatory thoracic or lumbar component is also measures the soft tissue component of the lower
absent with forward bend in a compensatory scoliosis extremity as well as the bones. The measurement was found
whereas in a structural curve it persists. There is pelvic to be more reproducible than tape measurements or block
obliquity with the iliac crest lower on the shortened side. measurements in a comparative study of several practitioners
When the child is standing or walking, the discrepancy can in 96 patients with a mean of 5.2 cm length discrepancy. The
be hidden either by flexing the knee on the longer side or by technique is yet another way of estimating lower extremity
6.3 Limb Length Determination 619

length discrepancies in accurate fashion clinically and decreasing accuracy in documenting limb length discrepan-
bypasses concerns with hip, knee, or ankle flexion contrac- cies owing to angular distortion (Fig. 6.2a).
tures. Morscher and Figner have described clinical and
radiographic methods of measurement in detail [42]. 6.3.3.2 Orthoroentgenograms
This technique was developed by Green and associates in the
late 1940s to document accurately lower extremity length
6.3.2 Segments to Be Considered in Assessing discrepancies [44]. Both femurs and tibias are radiographed in
Lower Extremity Length their entirety. A single long X-ray cassette with a single long
Discrepancies X-ray sheet is used. Three X-ray machines are built onto the
ceiling of a specifically defined chamber 72 in. from the X-ray
Lower extremity length discrepancies can involve any or all of film. There is one machine to be centered over the hip, one to
four segments: pelvic height, femoral length, tibial length, and be centered over the knee, and one to be centered over the
foot height. The clinical assessment measuring the length ankle. Three radiographs are taken in rapid succession.
discrepancy with the patient standing on blocks takes each of Studies documented that the magnification factor is under 1 %
these four segments into consideration while most of the other with this technique. As well as providing extremely accurate
length determination modalities do not. The clinical mea- length determinations, the film allows for radiographic visu-
surement from the anterior superior iliac spine to the medial alization of the entire bone for indication of structural or
malleolus eliminates consideration of the foot and only par- angular deformities. This technique is infrequently used today
tially addresses the pelvic height; the characteristic radio- because of concerns about the total amount of radiation
graphic determinations of femoral and tibial length do not exposure with sequential studies (Fig. 6.2b).
consider either the pelvic or the foot regions; and virtually all
the limb length determinations and surgical corrections relate 6.3.3.3 Scanograms
to the femur and tibia alone without considering the other two This is a commonly used technique today for lower
segments. Although the femur and tibia are responsible for the extremity length discrepancy documentation. It can be
vast majority of the limb height, there can be situations where accurate if details of performance are rigidly adhered to
foot and pelvic abnormalities contribute meaningfully to the although in practice many inaccuracies are seen. Three
length discrepancy. In these situations, specific radiographic radiographs are taken similar to the orthoroentgenogram
determination of their height is important. centered over hip, knee, and ankle but spot films only are
taken with the intervening femur and tibia diaphyseal seg-
ments spared any radiation. A ruler is placed beside the limb
6.3.3 Radiographic and Other Imaging and the radiographic projection of the ends of the femurs and
Documentation of Lower tibias over the ruler allows for a measurement at that level.
Extremity Length Discrepancies The ruler should be calibrated in millimeters. A major
problem with this technique is patient movement during the
At least seven imaging techniques have been used to doc- repositioning of the single machine. This can lead to inac-
ument the length of the lower extremity bones and the extent curacies which if slight cannot be detected by those subse-
of lower extremity length discrepancies. Many of the quently reading the radiographs. High levels of quality
screening surveys referred to in Sect. 6.2 above documented control are needed to ensure accurate measurement.
length discrepancies using standardized standing positions
but radiographs of only the lower spine, pelvis, and hips. 6.3.3.4 Computerized Tomography Scans
The relative femoral head positions allowed for length dis- The CT scan can provide accurate length measurements
crepancy measurements but not for absolute owing to the high-resolution calibration of the technique. It
femoral/tibial/foot height measurements. The many technical is difficult to justify the use of this technique for sequential
considerations in making accurate radiologic measurements studies due to high levels of radiation exposure but lower
have been detailed [12, 16, 42, 43]. dosing can be used to lessen this concern since bone detail is
not being assessed.
6.3.3.1 Teleoroentgenograms
Teleoroentgenograms are limited to use during the 1st year 6.3.3.5 Ultrasonography
of life. They refer to a plain X-ray of the entire lower Ultrasound can be used to document bone length [45]. It is
extremity centered over the knee. If taken at a 72 in. height, particularly helpful in the first 2 years of life when cartilage
they are accurate in terms of length owing to the minimal elements compose the bulk of the epiphyses. Although it has
magnification with the small limb. After this age, they are of not been adopted for widespread use, a recent study in young
620 6 Lower Extremity Length Discrepancies

Fig. 6.2 Radiographic measurements used to determine lower extrem- which can be moved along a track are mounted at a standardized 72 in.
ity lengths. a Teleoroentgenogram. A single exposure of the entire distance from the cassette holder. Three radiographs are taken in rapid
lower extremity centered over the knee provides a radiographic image succession with one camera centered over the hip joint, one over the
of both the entire femur and the entire tibia and fibula. The longer each knee joint, and one over the ankle joint. The perpendicular rays
bone is, the greater the magnification error owing to the increased intersect the ends of the bones recording the true length. Each long
divergence of the rays. b Orthoroentgenogram. In performing an bone is imaged completely allowing for structural and angular
orthoroentgenogram a single long X-ray cassette is used. Three cameras deformity assessments as well

adults showed that a laser-based ultrasound method was with the scanograms, although they improve measurement
extremely accurate compared with radiographic length accuracy by decreasing magnification and parallax errors.
measurements with the intraclass correlation coefficients
(ICC) value for agreement between methods 0.97 [46]. 6.3.3.7 Magnetic Resonance Imaging
The measuring accuracy of magnetic resonance imaging can
6.3.3.6 Full-Length Standing Anteroposterior also be applied to extremity length determinations [48]. It is
Radiographs extremely accurate (almost exact) in cadaver studies [49].
Some centers are reintroducing the use of full-length standing While not used clinically to any great extent, it can be
anteroposterior radiographs of both lower extremities to valuable in specific situations, especially if limiting radiation
assess not only the length discrepancy but also the deformity is a consideration.
with one study [47]. Computed radiography is used to assess
the images obtained but the study is not a formal CT scan.
Sabharwal et al. compared same day full-length standing AP 6.4 Causes of Lower Extremity Length
radiographs to scanograms in 111 patients with limb length Discrepancies
discrepancy [47]. The age range was wide (9 months to
73 years) with a mean of 18.8 years and 71 % <18 years of A large number of disorders during the growing years can
age. For the standing AP film, the pelvis was leveled with a either stimulate or retard the growth of epiphyses unilaterally
shoe lift and one film centered at the knees was taken at a or asymmetrically such that a lower extremity length dis-
minimum patient-to-tube distance 203 cm. Scanograms were crepancy occurs. Virtually any childhood disorder which
done with the patient supine using 3 separate images centered affects an epiphysis can lead to stimulation or retardation of
over hip, knee, and ankle at a patient-to-tube distance of growth and possible limb length discrepancy must be con-
101 cm. Compared with the scanogram the mean magnifi- sidered in relation to overall management. Even disorders
cation with the standing AP radiographs was 4.6 % (3.3 cm). which are present throughout the skeleton, such as hereditary
The radiation dosage is actually as much as 2–3 times higher multiple exostosis, can affect sides unequally. The causes,
6.4 Causes of Lower Extremity Length Discrepancies 621

effects, and extent of lower extremity length discrepancies and often semi-annually, from the time of onset or detection
throughout the spectrum of disorders affecting the growing of the disease to maturity. These patients were followed
skeleton are listed in Table 6.1. prospectively because they had an affection in which lower
extremity length discrepancy was known to occur, rather
than being seen only after a clinically apparent discrepancy
6.5 Developmental Patterns in Lower had developed. With the exception of the group of patients
Extremity Length Discrepancies with a fractured femoral diaphysis, the patients included in
this review had to have had a discrepancy of 1.5 cm or more
The discrepancies that develop in children are susceptible to at some time during the period of assessment. The classifi-
considerable change with time, as the involved physes have cation does not refer to any change in discrepancy that fol-
the potential for increasing the discrepancy, maintaining it at lowed surgical physeal arrest, diaphyseal lengthening, or
a stable level, or correcting it spontaneously. Not all length osteotomy.
discrepancies increase continually with time during the The disease entities studied and the number of patients in
growing years. In a review of lower extremity length dis- each group were as follows. There were 18 patients with
crepancies in 803 children who were followed by at least proximal femoral focal deficiency, 102 with congenital coxa
annual orthoroentgenograms for 5 or more years to skeletal vara and a congenitally short femur (some with associated
maturity or to time of corrective surgery, it was demon- anomalies of the leg and foot), 17 with Ollier’s disease
strated that several patterns of developmental discrepancy (enchondromatosis), 21 with physeal destruction, 115 with
can occur [50]. These are dependent on the nature of the poliomyelitis, 33 with septic arthritis of the hip, 116 with a
conditions causing the discrepancies and on the place and fractured femoral diaphysis, 29 with a hemangioma, 17 with
time of their occurrence. They do not refer to changes fol- neurofibromatosis, 46 with hemiparetic cerebral palsy, 113
lowing bone surgery. Table 6.1 gives a broad categorization with hemiatrophy or hemihypertrophy (anisomelia), 36 with
of disorders which can be associated with lower extremity juvenile rheumatoid arthritis, and 140 with Legg–Perthes
length discrepancies, an indication of whether they cause disease. The distribution of pattern types, the average dis-
growth retardation or stimulation, and a range of length crepancy in cm, and the range of discrepancies before sur-
discrepancies with which they are associated. gery were assessed for each group.

6.5.1 Patient Population 6.5.2 Classification of Developmental

Patterns in Lower Extremity
Longitudinal data on lower extremity length discrepancy Length Discrepancies
from patients who were followed in the Growth Study Unit
at the Children’s Hospital, Boston, over a 40-year period Type I, upward slope pattern: The lower extremity length
(1940–1980) were studied. The patterns of developmental discrepancy develops and increases continually with time, at
discrepancy that developed were demonstrated by charting the same proportionate rate.
the extent of a discrepancy directly against time as repre- Type II, upward slope–deceleration pattern: The lower
sented by the patient’s chronologic age [50]. The patterns extremity length discrepancy increases at a constant rate for
were also related to skeletal age to show their independence a variable period of time, and then shows a diminishing rate
from that parameter. Lower extremity length discrepancies of increase independent of skeletal maturation.
were documented by standard techniques using teleoradio- Type III, upward slope–plateau pattern: The discrepancy first
graphs for patients who were younger than 5 years of age. increases with time, but then stabilizes and remains
Orthoroentgenograms, from which femoral and tibial mea- unchanged throughout the remaining period of growth.
surements were made, were used for all the older patients. Type IIIA, downward slope–plateau pattern: The discrep-
Skeletal age was determined from posteroanterior radio- ancy decreases with time, but then stabilizes and remains
graphs of the left wrist and hand and correlated with the unchanged throughout the period of growth.
Todd atlas [51] until 1950 and with the Greulich and Pyle Type IIIB, plateau pattern: The discrepancy, detected ini-
atlas [52] thereafter. tially after it has developed, remains unchanged throughout
For inclusion in the review, an individual had to have the remaining period of growth.
been followed by radiographic means at the Growth Study Type IV, upward slope–plateau–upward slope pattern: The
Unit for a minimum of 5 years (or from the onset of disease), discrepancy first increases, then stabilizes for a variable but
either to the time of skeletal maturity or to the time of bone considerable period of time, and then increases again toward
surgery. Virtually all of the patients were assessed annually, the end of the growth period.
622 6 Lower Extremity Length Discrepancies

Table 6.1 Causes, effects, and extent of lower extremity length discrepancies
Stimulation Retardation

A B C D A B C D

1. Femoral disorders Proximal femoral focal deficiency ✓ ✓

Coxa vara ✓ ✓ ✓

Congenital short femur ✓ ✓ ✓

Developmental dysplasia of the hip

• Subluxated hip ✓

• Dislocated hip ✓ ✓

• Avascular necrosis ✓ ✓ ✓

Legg–Calve–Perthes disease ✓ ✓

Slipped capital femoral epiphysis ✓ ✓

Status post varus osteotomy ✓ ✓

2. Tibial and fibular disorders Congenital short tibia (Fibular hemimelia) ✓ ✓ ✓

Tibial agenesis (Tibial hemimelia) ✓

Pseudoarthrosis of tibia (Anterolateral Bowing) ± neurofibromatosis ✓ ✓ ✓

Posteromedial tibial bowing ✓ ✓

Blount’s disease (Tibia Vara) ✓ ✓

3. Foot disorders Club foot ✓

4. Trauma Epiphyseal growth plate Fracture/Separations ✓ ✓ ✓ ✓

Diaphyseal fractures

• Healing with shortening or overgrowth ✓ ✓ ✓ ✓

• Loss of bone mass ✓ ✓ ✓

5. Infection Syphilis (femur/tibia)

Septic arthritis

• Hip or knee ✓ ✓ ✓ ✓

Meningococcal septicemia ✓ ✓ ✓

Femoral/Tibial osteomyelitis (metaphyseal-diaphyseal) ✓ ✓

Tuberculosis

• Hip or knee ✓ ✓ ✓

• Femoral/Tibial shaft ✓ ✓

6. Prolonged unilateral Tuberculosis ✓ ✓

immobilization/9–12 + months
CDH, etc. ✓ ✓

7. Neuromuscular disorders Poliomyelitis ✓ ✓ ✓

Cerebral Palsy-hemiparesis ✓ ✓

Peripheral nerve injury (Unilateral); Sciatic nerve paralysis ✓ ✓ ✓

Myelomeningocele ✓ ✓

Arthrogryposis ✓ ✓

Diastematomyelia ✓ ✓

8. Vascular disorders Vascular malformations ✓ ✓ ✓

• Klippel–Trenaunay ✓ ✓ ✓

• Parkes Weber ✓ ✓ ✓

• Proteus ✓ ✓ ✓

• Beckwith–Wiedemann ✓ ✓

Hemangioma (Newer terminology) ✓

Cutis Marmorata Telangiectatica Congenita ✓ ✓ ✓ ✓

Following use of neonatal umbilical or femoral catheters ✓ ✓ ✓ ✓ ✓

(continued)
6.5 Developmental Patterns in Lower Extremity Length Discrepancies 623

Table 6.1 (continued)

Stimulation Retardation

A B C D A B C D

Synovial hemangioma (knee)

Arteriovenous aneurysms (traumatic) ✓ ✓

9. Hemihypertrophy, with Cerebrovascular malformations ✓ ✓

connective tissue abnormalities
Lipomatosis ✓ ✓

Lymphedema ✓ ✓

Lymphangioma ✓ ✓

Neurofibromatosis ✓ ✓ ✓ ✓

Silver–Russell syndrome ✓ ✓

Malignant tumors: Wilm’s, adrenal, carcinoma, hepatic carcinoma ✓ ✓

10. Hemiatrophy Otherwise normal appearing limb ✓ ✓

11. Inflammatory Disorders Juvenile rheumatoid arthritis ✓ ✓ ✓ ✓

Scleroderma

12. Hematologic Disorders Hemophilia ✓ ✓ ✓ ✓

Thalassemia ✓ ✓

13. Tumors/Focal bone lesions Benign lesions

lesions
• Fibrous dysplasia

• Unicameral bone cyst ✓ ✓ ✓

• Aneurysmal bone cyst ✓ ✓ ✓

• Osteoid osteoma ✓ ✓

• Caffey’s disease ✓ ✓ ✓ ✓

Malignant tumors

• Resection with growth plate damage ✓ ✓ ✓ ✓

• Resection with loss of bone mass ✓ ✓ ✓ ✓

• Radiation-induced physeal arrest ✓ ✓ ✓

14. Skeletal dysplasia Hereditary multiple exostoses ✓ ✓ ✓ ✓

(Asymmetric Lengths)
Ollier’s enchondromatosis ✓ ✓ ✓ ✓

Stippled epiphyses (Chondrodysplasia Punctata) ✓ ✓

Dysplasia epiphysealis hemimelica ✓ ✓

Melorheostosis ✓ ✓ ✓

Camptomelic dwarfism ✓ ✓

Congenital banding (Streeter Syndrome) ✓ ✓ ✓

15. Bone diseases with high Osteogenesis imperfecta ✓ ✓ ✓

incidence of asymmetric
Rickets ✓ ✓
deformities
Renal osteodystrophy ✓ ✓

Vitamin a intoxication of infancy ✓ ✓

Scurvy (lack of vitamin C) ✓ ✓

16. External causes Burns ✓ ✓ ✓

Frostbite ✓ ✓

Code A = 0–2 cm; B = 2–5 cm; C = 5–15 cm; D = 15 cm+

Type V, upward slope–plateau–downward slope pattern: The classification is illustrated in Fig. 6.3a and an indi-
The discrepancy increases with time, stabilizes, and then cation of the various patterns in various disorders is outlined
decreases in the absence of surgery. in Fig. 6.3b.
624 6 Lower Extremity Length Discrepancies

Fig. 6.3 a The developmental pattern classification showing types I–V. (Reprinted with permission from Shapiro F, Developmental patterns in
lower extremity length discrepancies. J Bone Joint Surg Am 1982;64A:639–651) b The distribution of patterns in several of the more frequent
length discrepancy categories is shown
6.6 Lower Extremity Length Discrepancies … 625

6.6 Lower Extremity Length limb with a hand or foot attached more or less directly to the
Discrepancies in Specific trunk; acheiria, absence of a hand; apodia, absence of a foot;
Disease Entities: adactylia, absence of a digit including the associated meta-
Pathoanatomy, carpal or metatarsal; and aphalangia, absence of one or more
Pathophysiology, phalanges. Hemimelia may be complete or partial. The term
Developmental Patterns, paraxial hemimelia indicates that either the preaxial or the
Ranges of Discrepancies postaxial portion of the distal half of the limb is involved. The
anatomical term preaxial refers to the border of a limb on
In this section, we incorporate information from the study which either the thumb or the big toe is situated and the term
reported in the article Developmental Patterns in Lower postaxial refers to the opposite border. The preaxial paraxial
Extremity Length Discrepancies [50] and information from hemimelias are therefore either radial or tibial and the
the extensive literature on the entire range of disorders that postaxial paraxial hemimelias are ulnar or fibular. The vari-
can lead to length differences. The focus is on the pathoa- ous subtypes of paraxial hemimelia are named after the
natomy and pathophysiology of the disorders themselves absent portion; radial hemimelia refers to a deficiency of the
and particularly on the pattern of discrepancy development radius. The terminology of congenital skeletal limb defi-
and the extent of the discrepancies in the specific diseases. In ciencies is shown Fig. 6.4a.
most instances, the ranges of length discrepancy values are
provided. Some studies refer to percentage shortening, in 6.6.1.2 Dysmelia
relation to the normal side. Reference of the Green-Anderson Henkel and Willert proposed a differing approach to classi-
tables can then indicate the range of values in absolute terms. fication of the congenital malformations which they felt
outlined more accurately the teratological sequence [54].
The term dysmelia is used to refer to limb malformations
6.6.1 Terminology of Congenital Limb varying from mild hypoplasia to partial and total aplasia of
Deficiencies the tubular bones of the extremities and even to complete
nonformation of the extremity. They arrange the abnormal-
Congenital limb deficiencies are among the commonest ities according to their degree of severity to form a terato-
causes of lower extremity length discrepancies. We will refer logical sequence linked by a common morphological pattern
to the commonest and most severe types; but in reality, they and enabling subtle variations to be included and to repre-
represent part of a spectrum of disorders affecting appen- sent the abnormalities throughout an entire limb. The
dicular development in both upper and lower extremities. approach addresses three questions: (1) Which region of the
Extensive efforts have been made over the past few decades limb and which skeletal elements are affected? (2) In which
to develop encompassing classifications for these disorders manner are they affected—by hypoplasia, partial aplasia, or
but they are so variable and the terminology used has been so total aplasia? and (3) Have the affected skeletal elements also
awkward that there has been no universal agreement on ways undergone fusion or synostosis? There are five main types of
to refer to them. As a result, individual terms from differing any teratological sequence of dysmelia: (1) distal form of
classifications have come to be used commonly and on ectromelia (ectromelia refers to involvement of the radius or
occasion different terms are used to refer to the same disorder. tibia with their peripheral rays); (2) axial form of ectromelia;
(3) proximal form of ectromelia; (4) phocomelia (abnor-
malities where no remnants of long bones are seen between
6.6.1.1 Frantz and O’Rahilly
the limb girdle and the hand or foot); and (5) amelia (total
The classification of Frantz and O’Rahilly is an all encom-
loss of an extremity). The classification of malformations
passing approach which divides congenital skeletal limb
was derived from a survey of 693 deformed limbs
deficiencies into terminal, where there are no unaffected parts
(Fig. 6.4bi–iii). This approach would seem to offer the best
distal to and in line with the deficient portion, or intercalary,
correlation with gene and molecular abnormalities as they
where the middle portion of a proximodistal series of limb
are increasingly defined in relation to limb development.
components is deficient but the proximal and distal portions
are present [53]. Each of these two main groups then may be
6.6.1.3 International Terminology
either transverse, where the defect extends transversely
for the Classification of Congenital
across the entire width of the limb, or longitudinal, where
Limb Deficiencies
only the preaxial or postaxial portion is absent (hence the
The International Society for Prosthetics and Orthotics
deficiency is longitudinal). Among the terms used in the
organized a working group to propose a terminology for
classification are amelia, absence of the limb; hemimelia,
limb deficiencies which would be accepted internationally
absence of a large part of a limb; phocomelia, a flipper-like
[55]. They utilized both the system of Frantz and O’Rahilly
626 6 Lower Extremity Length Discrepancies

Fig. 6.4 Classifications of congenital skeletal limb deficiencies are of the lower extremities is shown. bii Sequential changes of the tibia are
illustrated. a The classification of Frantz and O’Rahilly is shown in 4a. shown in a series of cases. The femur is relatively normal throughout.
The terms terminal, intercalary, transverse, and longitudinal are biii Sequential changes of the femur are shown with relative normalcy
illustrated. (Reprinted with permission from Frantz and O’Rahilly, of the tibia, fibula and foot regions (Reprinted with permission from
Congenital skeletal limb deficiencies. J Bone Joint Surg Am Henkel and Willert, Dysmelia: a classification and a pattern of
1961;43A:1202–1224). bi–iii The classification of Henkel and Willert malformation in a group of congenital defects of the limbs. J Bone
is shown in this figure bi–iii. bi The teratological sequence of dysmelia Joint Surg Br 1969;51B:399–414)
6.6 Lower Extremity Length Discrepancies … 627

Fig. 6.4 (continued)

and that of Henkel and Willert along with other terminolo- 6.6.2.1 Proximal Femoral Focal Deficiency
gies in efforts to reach agreement. In each of the 18 patients with proximal femoral focal defi-
There is still no unanimity of opinion concerning ciency, severe progressive shortening of the type I pattern
descriptive terms for this wide array of disorders, although occurred [50]. In types A and B, proximal femoral focal
with appropriate clinical and radiologic descriptions there is deficiency as defined by Aitken, the proximal part of the femur
rarely any doubt as to which entity is being discussed. Even is intrinsically maldeveloped, with no effective capability for
after adoption of any uniform terminology, it would take normal reconstitution even though the acetabulum and
several years before the studies and the literature are all femoral head are present [56]. In types C and D, the proximal
conformed to a standard. It remains essential for those structures are even more markedly abnormal, with no visible
involved with these disorders to have a general under- ossified head and the tapered diaphysis displaced proximal to
standing of the differing classifications used. In the next the shallow, often unrecognizable acetabulum (Fig. 6.5a).
sections, the commonest terms will be referred to. Severe growth sequelae in this class of femoral developmental
abnormalities are well known [57]. Proximal femoral focal
deficiency resulted in an average of 27 cm of shortening, with
some lower limbs having as much as a 45-cm discrepancy. The
6.6.2 Congenital Abnormalities of the Femur range of femoral shortening averaged 60 % (range 40–80 %)
compared with the normal side. In patients classified as having
Congenital abnormalities of the femur encompass a spec- type A, B, or C deficiency, the shortening averaged 57 %, and
trum of disorders from those in which the femur is com- in type D it averaged 80 %. Tibial shortening averaged 7.6 %
pletely absent to those in which it is present, structurally (range 0–37 %) and fibular shortening averaged 28 % (range
normal and only somewhat smaller than that on the opposite 0–100 %) in all types. This condition caused the most severe
side. These can be classified into four broad groups discrepancies seen in the series and presents an extremely
including proximal femoral focal deficiency, coxa vara with difficult management problem. Accurate prediction of the final
congenital short femur, congenital short femur with dia- discrepancy is possible from the early years of life in patients
physeal bowing, and anisomelia in which the femur is nor- with this condition, however, owing to the invariable type I
mally shaped but smaller than that on the opposite side. pattern.
628 6 Lower Extremity Length Discrepancies

Fig. 6.5 (continued)

6.6 Lower Extremity Length Discrepancies … 629

Fig. 6.5 a The classification of proximal femoral focal deficiency of lower extremity malformations: classification and treatment. J Pediatr
Aitken is shown. bi–ii The classification of congenital abnormalities of Orthop 1983;3:45–60. Copyright 1983 Wolters Kluwer Health). c A
the femur into 9 types as defined by Pappas (Reprinted with permission type I developmental discrepancy pattern in congenital short femur is
from Pappas A, Congenital abnormalities of the femur and related shown

6.6.2.2 Congenital Short Femur Including together. A separate detailed study also based primarily on
Congenital Coxa Vara patients followed longitudinally in the Growth Study unit of
This group included patients with congenital coxa vara, a the Children’s Hospital Boston was published by Pappas in
congenitally short femur with coxa vara, and a congenitally which the large number of patients assessed allowed a more
short femur with lateral bowing and sclerosis but without detailed subclassification into nine types of deformity [63].
coxa vara. Many of these patients also had associated mild or Pappas defined the percent of femoral shortening in each of
moderate anomalies of the pelvis, tibia, fibula, and foot. the nine classes; detailed the femoral and pelvic abnormal-
Excluded from this group were the patients with proximal ities; assessed the associated abnormalities of tibia, fibula,
femoral focal deficiency and those with a normally shaped patella, and feet; and defined the treatment objectives
and only mildly shortened femur who were categorized as (Fig. 6.5bi, ii). The large number of patients available for
having hemiatrophy (anisomelia). The average preoperative this study demonstrated a continuum of abnormalities.
limb length discrepancy in this group was 5.92 cm (range Class I refers to the situation where the femur is entirely
2.2–15.6 cm). Thirty-seven of these patients showed a type absent and the acetabular region of the pelvis is markedly
II or III developmental pattern. If a discrepancy reached hypoplastic. In class II, the proximal 75 % of the femur is
6 cm, it generally persisted with a type I pattern. Those absent. In class III, there is no bony connection between the
patients, however, in whom the discrepancy was less great femoral shaft and head although the femoral head which has
often had a type II or III pattern. Ring noted that patients delayed ossification is present in the acetabulum. In class IV,
with a congenital short femur alone—one with lateral the femur is present to approximately one-half its length but
bowing, cortical sclerosis, increased hip external rotation the proximal abnormalities show the femoral head in the
and minimal to absent internal rotation, but without coxa acetabulum with the head and shaft joined by irregular cal-
vara—will continue to have an increase in the discrepancy at cification in a fibrocartilaginous matrix. It is these four dis-
a regular rate with time (type I pattern) [58]. The relatively orders that are generally referred to as proximal femoral
marked length discrepancies in femoral developmental dis- focal deficiency. In class V, the femur diaphysis and distal
orders are well documented [57, 59–62]. end are incompletely ossified and hypoplastic. In class VI,
Two studies on length discrepancies in congenital the proximal two-thirds of the femur are perfectly normal
femoral anomalies have been published in which both and the hypoplasia is in the distal third with an irregular
proximal femoral focal deficiency and congenital short distal femoral region and no evident distal epiphysis.
femur with and without coxa vara have been assessed Classes V and VI are essentially examples of distal femoral
630 6 Lower Extremity Length Discrepancies

focal deficiency. Class VII is congenital coxa vara with a form, group I, the average shortening was 10 % of the
hypoplastic femur which is shortened and somewhat bowed normal side ranging between 88 and 97 % with the mean
and also demonstrates lateral femoral condylar deficiency. amount of shortening at 13 years of age being approximately
Class VIII is infrequently seen but involves a proximal 2.8 cm. In group II, shortening averaged 30 % of the normal
femur coxa valga, a hypoplastic femur, and an abnormality side ranging between 64 and 80 % normal length and the
of the distal femoral condyles with the lateral condyle being mean amount of shortening around 10 years of age already
somewhat flattened. Most would include congenital short 9 cm. In group III, shortening was in the range of 45 % of
femur in this category which perhaps most represents class the normal side indicating 55 % length compared to the
VIII, although it characteristically has anterolateral bowing opposite side and associated with a mean discrepancy at age
which Pappas does not demonstrate. The class IX femur is 12 of 19 cm. In group IV, the overall length was only 24–
essentially normal and might be defined by others as having 44 % of the normal side indicating in many cases a 75 %
only shortness referred to as hemiatrophy or anisomelia. shortness which translated into a mean discrepancy of 11 cm
Pappas also demonstrates the frequently seen underdevel- although patients in this group had only been followed to a
opment of the lateral femoral condyle predisposing to both a little >2 years of age. In the most severe category, proximal
valgus deformity at the knee referable to the femoral femoral focal deficiency, shortness was 90 % of the involved
deformity and a tendency toward lateral patellar subluxation. side translating into length of approximately 10 % normal
The ranges of femoral and tibial discrepancies found in each only and leading to discrepancies at age 5 years which were
of the varying categories were listed. In class I, the femur already 25 cm.
was completely absent. In class II, the femur was shortened A type I developmental discrepancy pattern in congenital
by 70–90 % of that on the opposite normal side. The tibia short femur is illustrated in Fig. 6.5c.
was also shortened. In Class III, femoral shortening was 45–
80 % of the opposite side and tibial shortening ranged from
none to 40 %. In class IV, femoral shortening was 40–67 % 6.6.3 Congenital Developmental
that of the opposite side and tibial shortening ranged from Abnormalities of the Fibula;
none to 20 %. In class V, femoral shortening was 48–85 % Fibular Hemimelia
and tibial shortening was 4–27 %. In class VI, femoral
shortening was 30–60 %; In class VII, femoral shortening Congenital abnormalities of the fibula are the most common
was 10–50 % and tibial shortening was minimal to 24 %. In lower extremity congenital deficiency syndrome. Coventry
class VIII, femoral shortening was 10–41 % and tibial and Johnson noted the fibula to be the most common bone
shortening was none to 36 %. In class IX, femoral shortening congenitally absent with the congenital absence of the tibia,
was 6–20 % and tibial shortening was none to 15 %. ulna, radius, and femur following in that order of frequency
Vlachos and Carlioz studied bone growth in 40 cases of [65]. Farmer and Laurin reviewed the congenital absence or
congenital anomalies of the femur [64]. They categorized severe maldevelopment of the long bones from 1931 to 1957
their patients into five groups with group I being congenital and noted 32 limbs with congenital absence of the fibula
short femur without coxa vara but with shortening and while complete or partial absence was also noted in femur
curvature of the shaft; group II with congenital short femur 16, radius 13, tibia 5, and ulna 2 [66].
and coxa vara; group III severe coxa vara with a dystrophic The fibular abnormalities are referred to as fibular
or pseudoarthrotic junction between the proximal femur hemimelia or lateral (external) hemimelia. They are always
which was in coxa vara and the diaphysis; group IV coxa accompanied by tibial shortening and frequently accompa-
vara, coxa vara with severe angular deformity proximally nied by same side femoral shortening and it is this tibial and
and discontinuity with the shaft of the femur; and group V femoral shortening that length discrepancy treatment relates
where there was almost complete absence of the proximal to. Since the primary bone undergoing treatment is the tibia,
femur and no hip joint articulation. Relatively few patients the disorder is sometimes referred to as congenital short
were followed to skeletal maturity with many being seen tibia. In those with a hypoplastic fibula (fibular hemimelia),
only to the ages of 3–10 years such that definitive pattern the major treatment considerations are the limb length dis-
progression could not be determined. They felt, however, crepancies although on occasion measures are needed to
that all patients regardless of diagnostic category increased at stabilize the ankle usually by varus osteotomy of the distal
a constant rate with time although this is somewhat distinct tibia and fibula and occasionally by orthotic support [67].
from our findings. Assessment of some of their charts would Those with complete absence of the fibula present greater
also indicate a type II pattern in some patients. They clearly management problems because of the more significant
documented both the percentage shortness and the absolute length discrepancy and the equinovalgus foot deformity
amount of shortness in cm in each group. In the mildest along with a subluxed or dislocated ankle [68]. There can
6.6 Lower Extremity Length Discrepancies … 631

also be anterior bowing of the distal third of the tibia, the associated limb deformities, there was genu valgum and
absence of one or more rays of the foot on the lateral side, a instability, absence of the fourth and fifth rays of the foot,
tarsal coalition, and a ball-and-socket ankle joint [69]. anteromedial shortening and curvature of the tibia, tarsal
Coventry and Johnson developed a classification into coalitions involving the talonavicular or talocalcaneal joints,
three types [65]. In type I, the patients have partial unilateral and a dome-shaped talus. Often, the fibula was absent in its
absence of the fibula with little or no bowing of the tibia. proximal one-third.
There is little or no deformity of the foot. The extremity is Achterman and Kalamchi studied 97 limbs with con-
always shortened but the shortening can be quite minimal genital deficiency of the fibula [72]. They outlined a slightly
and is usually managed with an epiphyseal arrest. In type II, modified classification defining type I deformities as those
the fibula is completely or almost completely absent and with hypoplasia of the fibula and type II deformities as those
involvement is unilateral. There is anterior bowing of the with complete absence of the fibula (Fig. 6.6). They noted
tibia, dimpling of the skin, equinovalgus of the foot, and that congenital anomalies of the femur were present in 76 %
absence or deformity of the lateral rays and tarsal bones. of patients with type I deficiency and in 59 % with type II.
There is also marked shortening of the extremity and The femoral abnormalities were invariably underdevelop-
amputation was frequently needed in this group. They also ment of the femur leading to worsening of the limb length
defined a type III in which either the type I or type II discrepancy. Congenital shortening of the femur was present
deformity was associated with other congenital deformities in 46 of the 66 limbs where femoral abnormality was
which were usually either severe deformities of the ipsilat- detected. Approximately, 20 % of the patients had some
eral femur or contralateral deformities of the other leg. bilateral involvement. Measurements were difficult in
A slightly different three-part classification is favored by patients with a proximal femoral focal defect and leg length
some. Type I is characterized by a slight to moderate inequality was assessed where data were available in 51
shortening of the fibula, proportionately lesser shortening of cases. In those where there was complete absence of the
the tibia, and minimal femoral shortening on some occa- fibula (the type III categorization listed above), the amount
sions. Catagni et al. report tibial shortening of 3–5 cm at the of tibial shortening in the affected limb was 25 % of normal
end of growth with little angular deformity [70]. On occa- with femoral shortening 13 % of normal. In those cases
sion, the associated outer fourth or fifth ray of the foot is also where there was hypoplasia of the fibula, the type I defor-
abnormal but rarely is this of clinical significance. Type II mity group showed fibular shortening 7 % of normal, tibial
has major shortening of the fibula with particular underde- shortening 6 % of normal, and femoral shortening 12 % of
velopment or absent development of the distal one-half to normal and in the type II group where there was major
one-third. The lateral malleolus is usually absent and the shortening of the fibula, in particular with underdevelopment
ankle is unstable with the foot moving into a position of at the ankle, the fibular shortening was 38 % of normal, tibial
valgus deformation. The tibia is shorter than in type I dis- shortening 17 % of normal, and femoral shortening
orders and tends to a valgus deformation and slight distal (although on a small number of patients) 23 % of normal.
recurvatum with posterior bowing and anterior concavity. Although detailed growth data were not presented, they felt
Type III, the most severe form, is characterized by an absent that growth of the abnormal limb was proportional to that of
fibula, showing in addition severe deformation and short- the normal limb and that the degree of tibial shortening
ening of the tibia, a deformed foot held in a position of increased as the fibular deficiency became more marked.
equinus and valgus and often associated with dislocation or Treatment of length discrepancy was either by epiphyseal
severe subluxation of the ankle. Owing to the shortness of arrest or tibial lengthening depending on the clinical situa-
the extremity, the angulation of the distal tibia, and the tion. If percentage shortening numbers are converted to
deformed foot much extended orthopaedic treatment is length measurements for a male patient whose height is at
needed, often including Syme or Boyd amputation for
prosthetic fitting.
In a large series with 291 patients with congenital uni- Fig. 6.6 Achterman and
Kalamchi define a classification
lateral shortening of an extremity, presented by Pappas et al. of fibular hemimelia type Ia (left)
129 patients, i.e., 44 % showed greater than 10 % shortening with fibular hypoplasia which is
of the fibula [71]. The extent of fibular shortening in 58 % relatively mild, type Ib (middle)
was between 10 and 30 %, in 9 % between 31 and 50 %, and with fibular hypoplasia which is
more with a length deficiency
in 33 % more than 50 %. Although absolute length dis- distally leading to a tilt of the
crepancy numbers were not presented, the associated tibial distal tibia and its epiphysis, and a
shortening was often in the range of 10 % or more with type II fibular deficiency (right)
fibular shortening >30 %. There was a clear correlation where the fibula is completely
absent
between the fibula shortening and foot deformities. Among
632 6 Lower Extremity Length Discrepancies

the 50th percentile at skeletal maturity, a 6 % tibial short- equalization were warranted while if projected discrepancies
ening would represent 2.2 cm, 17 % shortening 6.3 cm, and were between 8.7 and 15.0 cm, amputation of the modified
a 25 % shortening 9.3 cm. Syme’s type was in order. In those discrepancies projected to
Lefort et al. reviewed 62 cases of fibular hemimelia be >15.0 cm, retention of the foot and its adaptation to a
concentrating in particular on the pathoanatomy of the leg prosthesis was warranted.
and the associated femoral and tibial malformations [73]. The extent of growth discrepancy as well as management
They stressed in particular the anterior curvature of the tibia considerations were well assessed by Choi et al. [75]. They
in those cases where the fibula was either completely absent evaluated 48 extremities in 43 patients with the disorders
or absent to a great extent. Absolute values for long bone skewed to the more severe types in their series. There were 7
shortening were not presented although percentage values fibulas of the type IA categorization, 2 type IB, and 39 type
were. Charts demonstrated a type I pattern of discrepancy IIB (complete absence of the fibula or presence of only a
development for both developmental abnormalities of the distal vestigial fragment according to the classification of
femur and of the tibia. Achterman and Kalamchi). Treatment of groups varied
Hootnick et al. studied 43 patients with partial or com- between those having amputation and those having length-
plete absence of the fibula and a congenital short tibia [74]. ening procedures. They subclassified their patients according
They also determined that the relative difference in growth to the amount of inequality projected for the lower limbs. In
between the two limbs remained remarkably constant and group I, the percentage of shortening was 15 % or less with
thus adhered to the type I pattern of length discrepancy the foot of the shorter extremity at the distal third of the
development. The patients studied had a strictly unilateral contralateral normal limb; group II, between 16 and 25 %
variant and all measurements were determined radiographi- shortening with the foot of the shorter extremity at the level
cally by scanograms or from films showing both tibias on the of the middle third of the contralateral normal limb; and
same X-ray plate in the youngest children. The serial group III, >26 % shortening with the foot of the shorter
radiographic measurements of leg length were available in extremity at the level of the proximal third of the con-
14 patients covering an average observation period of tralateral normal limb. They concluded that lengthening was
9.3 years. Those with sequential radiographs were in the best suited only for patients in group I who had stable hips,
more severe end of the spectrum with the fibula absent in 11 knees, and ankles, and a plantigrade foot while patients in
patients and present but abnormal in 3. The amount of limb groups II and III were best served by ablation of the foot and
shortening was greater as the number of metatarsal bones a prosthetic fitting. Either Syme or Boyd amputation was
diminished. There were 36 patients from whom assessments used with the latter increasingly favored. The data provided
could be made in terms of the number of metatarsal bones indicated the extensiveness of the shortening in these dis-
and the amount of lower extremity shortening. In 12 patients orders. The group projected the limb length discrepancy at
with 5 metatarsal bones, the average shortening was 8.7 cm maturity which owing to the invariably type I pattern in
(range 3.6–12.7); in 11 patients with 4 metatarsals the these deformities is highly accurate. In 15 patients in the
average shortening was greater at 9.5 cm (range 3.8–13.5); group I category, the mean discrepancy projected to skeletal
in 11 patients with 3 metatarsals the average shortening was maturity was 8.85 cm with a range from 5.0 to 12.07 cm. In
11.8 cm (range 4.8–16.5); and in 2 patients with only 2 group II, 20 involved limbs had a mean projected discrep-
metatarsals the average shortening was 14.6 cm (range 11.9– ancy of 16.29 cm with a range between 12.5 and 22.5 cm.
17.3). The average age reached in the first three groups was Deformities were so great, both in terms of extent and bony
11 years and in the final group 9.5 years of age. The femur deformity in group III, that numbers were not provided.
was only minimally affected in these patients. In the 14 Farmer and Laurin recommended early Syme amputation
followed radiographically, there is excellent documentation when the length discrepancy was projected to be more than
that the percent inhibition of growth in the affected limb 7.6 cm (3 in.) at maturity, especially when severe foot
compared to the normal remained unchanged from the ear- deformity was present [66]. A similar recommendation was
liest documentation to skeletal maturity. Femoral involve- made by Westin et al. in their review of 32 patients with 37
ment at skeletal maturity was relatively small ranging from fibular deficiencies [76]. Many of their patients underwent
86 to 96 % length compared to the normal side, while tibial Syme amputation, the two indications of which were a foot
involvement was somewhat greater ranging from 73 to 82 % deformity so severe that any surgery to make the foot
length of the normal side. In patients followed for several plantigrade and functional was likely to fail and the second
years, although not quite to skeletal maturity, the same that a lower extremity length discrepancy of 7.5 cm or more
pattern persisted with femoral shortening in all patients would be present at skeletal maturity in the absence of any
except one being only 92–99 % of normal with associated management. In their group amputation was performed in 29
tibial shortening 61 to 90 % of the normal side. They felt that of 37 cases. They considered the results of the Syme
if the predicted shortening was <8.7 cm efforts at limb amputations to be uniformly good. They noted the growth
6.6 Lower Extremity Length Discrepancies … 633

inhibition to be constant with time. The growth inhibition in

those treated without amputation ranged from 7 to 12 % in
the tibia and from 0 to 14 % in the femur. In the amputee
group, the inhibition was 22–42 % in the tibia and 0–22 % in
the femur. In a listing of 9 patients with unilateral Syme
amputations who were followed to skeletal maturity and
who had growth data to skeletal maturity, the final femoral
and tibial discrepancy ranged between 7.8 and 24.1 cm with
a mean of 14.4 cm with the tibial discrepancies themselves
ranging from 6.7 to 14.8 cm with a mean of 11.0 cm.

6.6.4 Congenital Abnormalities of the Tibia

6.6.4.1 Congenital Developmental

Abnormalities of the Tibia; Tibial
Hemimelia
Partial or complete absence of the tibia is often referred to as
tibial hemimelia. These disorders are rare and markedly less
frequent than the fibular variant. There is marked shortening
and bowing of the involved leg, a flexion contracture of the knee
and a rigid varus foot. Four basic patterns have been defined
[77]. In type 1A, the tibia is completely absent and there is a
markedly hypoplastic lower femoral epiphysis. In type 1B, the
tibia is also completely absent, but there is a normal lower
femoral epiphysis. In both instances, the fibula rides high lat-
erally in relation to its normal position had the proximal tibia
been present. In type II, the upper proximal tibial epiphysis is
present as is a small portion of the metaphysis but the rest of the
tibia distally does not form. In type III, the proximal tibia is
absent but the distal one-third is present. In type IV, there is a Fig. 6.7 Classification of tibial hemimelia (congenital absence of the
marked diastasis between the proximal tibia and the fibula and tibia) by Jones et al. is shown. Several classifications are in use but this
the distal one-third of the tibia is absent. The most distal tibial one shown defines the abnormalities clearly (Reprinted with permission
from Jones et al. Congenital aplasia and dysplasia of the tibia with
segment tends to be curved medially (Fig. 6.7).
intact fibula: classification and management. J Bone Joint Surg Br
In the type II deficiency, the knee is well preserved. In 1978;60B:31–39)
types I, II, and III, the foot is in an equinovarus position. In the
type IV deformity, the knee tends to be well formed, but the
talus has subluxated proximally between the now separated Kalamchi and Dawe simplified the classification defining
tibia and fibula. Treatment is directed toward early disartic- three types [79]. In type I, there was total absence of the
ulation of the knee and prosthetic fitting for type I lesions, tibia; type II, distal absence of the tibia; and type III, distal
tibiofibular fusions, and prostheses for types II–IV, fibulo- deficiency with tibiofibular diastasis. The fibula was always
calcaneal fusions for types III and IV, and frequent need for present but in type I was subluxed proximal and lateral to the
foot ablation for prosthetic fitting. Schoenecker et al. studied distal femur, with a similar position sometimes being shown
57 patients with congenital tibial hemimelia [78]. There were also in the type II variant. In type I, the disorder was also
33 type IA patients, 6 type IB, 15 type II, 7 type III, and 10 invariably associated with a marked flexion contracture of
type IV. In the 54 limbs with the type I or II deficiency, there the knee, variable rotation of the leg, and marked inversion
were 22 who had knee disarticulation, 25 a Syme amputation, and adduction deformities of the foot. The distal femur was
and 1 a Chopart amputation. The foot was retained only in 6 usually hypoplastic with marked retardation of the ossifica-
with these 2 variants. There were 17 extremities with a type III tion center of the distal epiphysis. The functional status of
or IV deficiency and a Syme amputation was done in 9 and a the quadriceps muscle, the severity of the flexion contracture
Chopart in 4. In 4 the foot was retained. They report that 56 of of the knee, and the position and function of the foot all had
57 patients walked independently. to be considered in planning surgical straightening or
634 6 Lower Extremity Length Discrepancies

ablation. Jones et al. stressed that in all three, the fibula is deformities of the calcaneovalgus type. The foot and leg
relatively normal in form and development although it often deformity responds well to conservative treatment with
is situated in types I and II proximal to the normal rela- repeated application of casts or splints. On occasion,
tionship at the knee [77]. In some, the tibial segment is osteotomy is resorted to but only after conservative man-
greater than it appears at birth since it is present in cartilage agement has reached a plateau. Shortening of the affected
manifesting delayed ossification. Careful clinical exam and leg, however, is progressive, increasing with age, and must
other forms of imaging are important to clearly define the be followed until skeletal maturity. Sequential studies have
anatomic structure in the newborn. demonstrated well the spontaneous correction of the bowing
Courvoisier et al. reviewed 9 cases of type I or II (Jones) which in both anteroposterior and lateral projections
congenital longitudinal deficiency of the tibia managed by becomes either perfectly straight or sufficiently straight that
the Ilizarov technique [80]. Patients were assessed at a mean osteotomy is not needed by the time of skeletal maturity. The
follow-up of 18 years (4–32 years). The mean maximum most rapid straightening occurs between 6 and 18 months of
knee flexion was 35° (0–90°) in type I deficiencies and 118° age. Pappas has indicated that the bowing was reduced by
(90–140°) in type II deficiencies. Only 1 patient underwent roughly 50 % in the first 2 years, but after the age of 3 years,
amputation and 1 had knee fusion. The Ilizarov technique the reduction in angulation continued at a much slower rate
provided satisfactory progressive corrections. [81]. Little further correction should be expected after
10 years of age. The posterior bowing almost completely
6.6.4.2 Posteromedial Tibial and Fibular Bowing resolves with the medial bowing somewhat less likely to
Posteromedial bowing of the tibia which is almost always correct fully. The fibular bowing was equal to or slightly
associated with bowing of the fibula must be appreciated as greater than the tibial bowing, corrected more slowly and
an entity different from those described above. There is a some posterior bowing persisted in most even at maturity
considerable tendency for the posteromedial bow to correct when tibial posterior bowing had fully corrected. The pro-
during the first several years of growth, although consider- portionate difference in lengths between the normal and
able shortening often persists (Fig. 6.8a, b). The condition is bowed tibiae remain markedly stable throughout childhood
unilateral. The deformity is exclusively in the distal showing a type I discrepancy pattern.
one-third of the leg and is associated at birth with foot In the study of 33 patients by Pappas, the female/male
incidence was 20/13 (1.5 to 1) and left/right involvement was
19/13 (1.5 to 1) [81]. In those patients whose limb lengths were
determined radiographically within the first 2 months of life,
the average initial discrepancy was 1.25 cm. Subsequent
studies showed a constant increase with time. When all
patients had discrepancies calculated to skeletal maturity, thus
bypassing values obscured by epiphyseal arrest surgery, tibial
shortening averaged 4.1 cm with a range from 3.3 to 6.9 cm.
Femoral lengths were unaffected and foot lengths little affec-
ted. Owing to the extent of the length discrepancy, some form
of limb equalization surgery was done or recommended for
each patient but results were not reported. The abnormality is
focused in the entire distal one-half of the tibia and fibula and
soft tissues of the leg. The growth discrepancy was exclusively
at the distal end of the tibia and fibula based on radiologic
appearances of proximal and distal tibial and fibular epiphy-
ses. In 4 of the 33 patients, osteotomies were performed at an
early age to correct residual bowing; all healed uneventfully.
Hofmann and Wenger also noted a marked tendency to
spontaneous correction of the posteromedial bowing with
continuing progression of the discrepancy in limb length
[82]. In 13 patients studied, there was a direct relationship
between the degree of initial tibial bowing and the severity
of the subsequent discrepancy which, stated slightly differ-
ently, indicates that slightly greater discrepancies in the
Fig. 6.8 Gradual correction of postero-medial bowing is shown in earlier years of life would lead to greater discrepancies
anteroposterior (8a) and lateral views (8b) toward skeletal maturity. The mean posterior bowing at
6.6 Lower Extremity Length Discrepancies … 635

diagnosis was 30° (range 4° to 60°) and the mean medial tibial shortening were equal; in 20, femoral shortening was
bowing was 27° (range 10° to 45°). They noted the relatively greater than tibial; and in 10, tibial shortening was greater
slow improvement of the posteromedial angulation over a than femoral shortening. On occasion, the shortening was
few years compared with the rapid and complete correction limited to either the femur or the tibia. Of 22 patients who
of the calcaneovalgus deformity over a few months. The reached skeletal maturity, 11 (50 %) had limb length dis-
limb length discrepancy was progressive and present in each crepancies in the range for which limb length equalization
of the 13. The mean discrepancy was 3.1 cm (range 1.9– would normally be recommended. Of these, however, only 5
5.4 cm) but none had been followed to skeletal maturity and (23 %) actually had the procedure. The limb length dis-
10 patients were still only between 1 and 7 years of age. In crepancies at the termination of growth in those who did not
the oldest 3 patients (10 years 4 months to 15 years 5 months undergo growth–arrest procedures measured 2.1, 2.4, 2.3,
of age), the mean discrepancy was 4.7 cm—a value similar 2.6, 3.1, and 3.5 cm. Reasons for not performing surgery
to the 4.1 cm projection of Pappas. There were no femoral were: subsequent planned correction of discrepancies with
length discrepancies. associated opening-wedge osteotomy for deformity on the
A similar picture of the effects of a posteromedial angu- short side or closing-wedge osteotomy on the long side;
lation was reported by Carlioz and Langlais [83]. They difficulty of performing epiphyseal arrests in regions where
reported on 18 cases of congenital posteromedial bowing of large exostoses are present; clinical impression of an
the tibia and fibula, all of which were also associated with acceptable situation despite the roentgenographic measure-
shortening. Both the valgus (medial bowing) and posterior ments; and reluctance of patients and their families to
bowing components corrected over the first few years of life. undergo more procedures.
The valgus or medial bowing ranged between 10° and 56° Taking into consideration all 22 patients who had reached
initially and the posterior bowing or recurvatum between 10° skeletal maturity and also the 7 who were close enough that
and 65°. Although not all of the patients were followed to it was possible to say whether they would or would not
skeletal maturity, 5 had shown evidence of complete correc- require an arrest, there were 29 patients, of whom 12
tion of both deformities during growth. The spontaneous (41.2 %) had discrepancies within the recommended range
correction occurred in 3–4 years. The posterior bowing or for operative epiphyseal arrest.
recurvatum tended to correct more completely than the medial All five patients who were operated on had distal femoral
or valgus deformation. Osteotomy was resorted to in three epiphyseal arrests, and 1 had a proximal tibiofibular epi-
patients and in each instance healing was uneventful. Length physeal arrest as well. The limb length discrepancies at the
differences were invariably seen and ranged between 10 and time that the arrest was performed and the eventual limb
20 % of the length of the normal tibia. In most, the discrep- length discrepancy at the termination of growth were as
ancy increased at a steady rate with time but on occasion with follows: 2.9 cm, corrected to a 1.9 cm discrepancy; 2.8 cm,
increased growth the rate of inhibition on the involved side to 1.2; 2.4 cm, to 1.3; 2.3 cm, to 0.5, and 4.0 cm, to 4.0.
lessened. Five tibial lengthening procedures were performed There were no overcorrections, and although 4 of the 5 limbs
with the preoperative average discrepancy of 4.42 cm while 3 were corrected into an acceptable range, all were short of
epiphyseal arrests were performed with a presurgery dis- equalization. The last patient was operated on too late by all
crepancy of 3.7 cm. Other patients were still being followed criteria. In the other 4, however, the question arose as to
such that additional surgery might well have been needed. The whether growth anomalies in hereditary multiple exostoses
authors projected that discrepancies untreated would have might make prediction from the normal charts slightly
reached between 1.5 and 7 cm at skeletal maturity with the unreliable. Femur-tibia length ratios were plotted along the
majority being in the 3–5 cm range. appropriate percentile distribution in all the patients with
hereditary multiple exostoses and compared them with the
standard charts. The mean value for this length ratio in the
6.6.5 Skeletal Dysplasias with Asymmetric patients with hereditary multiple exostoses was 1.27, which
Involvement was exactly the same as the value from the charts for normal
subjects with the same size distribution. Reference to the
In several of the skeletal dysplasias, asymmetric involvement records of each patient who underwent epiphyseal arrest
is strongly associated with length discrepancies. The fol- indicated that there had been considerable difficulty in
lowing variants are particularly likely to show such findings. assessing the skeletal age from the roentgenograms of the
wrists.
6.6.5.1 Hereditary Multiple Exostoses The pattern of limb length discrepancy that can occur in
Femoral–tibial limb length discrepancy measurements in 32 this condition is variable. The discrepancy can remain
patients from the Children’s Hospital, Boston indicated a unchanged for several years; it can increase at slow or
range from 0.1 to 4.0 cm [84]. In 2 patients, femoral and moderate rates, which is the usual pattern; or it can, on
636 6 Lower Extremity Length Discrepancies

occasion, decrease spontaneously. The growth study data did absent at birth but appear within the first 4 years of life with
not support the belief that there is an increase in longitudinal 25 % occurring during the 1st year. Intracranial tumors of
growth in an affected bone following removal of an exos- cartilaginous origin are seen in approximately 15 % of
tosis. In addition, no correlation was seen between the patients. The incidence of chondrosarcomatous change is
degree of shortening in a particular bone and the number or high, similar to the finding in Ollier’s disease, with the
size of the exostoses present. reported incidence being 25–50 %. The matter is difficult to
Limb length discrepancies in hereditary multiple exos- determine since some authors consider virtually all
toses were frequent, and in approximately half of the patients enchondromatosis tissue after skeletal maturity to be pre-
they were great enough to warrant epiphyseal arrest. These sarcomatous at least. The limb length discrepancy findings
discrepancies point to the asymmetrical growth pattern in are similar to those with Ollier’s disease, in particular where
patients with hereditary multiple exostoses. The discrepan- unilateral lesions predominate.
cies were mild to moderate and were readily managed by
appropriately timed epiphyseal arrests. Extremely careful 6.6.5.4 Dysplasia Epiphysealis Hemimelica
observation is required, however, as the discrepancies can This disorder is often accompanied by a lower extremity
remain stable, increase at varying rates, or even, on occasion, length discrepancy in particular if the epiphyseal involve-
spontaneously decrease. Osteotomies can also alter limb ment is at the distal femur or proximal tibia. It is generally
length relationships. Some difficulties were encountered in referred to as dysplasia epiphysealis hemimelica but other
determining skeletal age accurately due to the associated terms for the disorder are tarso-epiphyseal aclasis and epi-
wrist and knee anomalies, but the Green-Anderson charts physeal osteochondroma. Many of the disorders occur at the
were appropriate for predicted corrections in this condition. ankle joint involving either the distal tibia or on occasion the
The limbs were more affected than the spine, both the femur talus. The discrepancies generally tend to be mild to mod-
and the tibia were involved, and limb involvement was not erate and more clinical difficulty is encountered with the
invariably rhizomelic. asymmetric joint surface than with the discrepancy.
Trevor was the first to delineate the disorder formally,
6.6.5.2 Ollier’s Disease (Enchondromatosis) describing eight patients [86]. He noted that the initial
The 17 patients with this intrinsic bone disease demonstrated description of such a disorder was a case described by
a type I pattern of discrepancy development [50, 85]. As Mouchet and Belot [87] in 1926 involving the talus. There
varus or valgus femoral and tibial deformities were often was no true shortening in the eight patients assessed,
associated with the shortening, corrective osteotomy was although specific limb measurements were not taken. Six-
done frequently and length discrepancy data unaltered by teen patients with the disorder were reviewed by Connor
any bone surgery intervention throughout the growth period et al. [88]. Each had only one leg involved but 12 multiple
were rare. Relentless shortening was demonstrated, however. epiphyses affected. The commonest sites were the distal
One patient with severe involvement who was followed to femur, distal tibia, and talus. Treatment of the lesion was by
skeletal maturity, with no surgical intervention, had a type I local excision and was generally effective around the knee
profile, with a 35.7 cm discrepancy and no decline in the rate although some at the ankle required arthrodesis. The disor-
of increase. In all patients, the extent of shortening paralleled der is characterized by asymmetrical overgrowth of one or
the extent of radiographic involvement. The average short- more epiphyses in a limb or of a tarsal or carpal bone during
ening prior to physeal arrest or diaphyseal lengthening was childhood. In the 16 patients, most of whom were followed
9.79 cm. Enchondromatosis was the second most serious to skeletal maturity, inequalities of limb length were
condition causing extensive discrepancies, being exceeded apparent in 5, 1 with lengthening, and 4 with shortening.
only by proximal femoral focal deficiency. Discrepancies were generally of a minor degree and caused
few problems. In those with shortness on the involved side
6.6.5.3 Maffucci Syndrome the amounts were 2, 1, 1, and 6 cm. In the one instance
The Maffucci syndrome is characterized by enchondro- where there was overgrowth on the involved side it was only
matosis, usually but not always unilateral, and vascular 1 cm and there was multifocal involvement of the distal
malformations. The enchondromas are present primarily in femur, distal tibia, and talus. In the one patient with an
the hands and feet but may involve tubular long bones or any extensive 6 cm of shortening, there was major involvement
bone preformed in cartilage. The vascular malformations are of the lateral half of the right distal femoral epiphysis.
either dermal or subcutaneous and adjacent to areas of Approximately, 3/4 of the lesions are concentrated in 5
enchondromatosis in most instances. They are usually regions which, starting with the most common, involve the
venous but can be capillary or lymphatic. Thrombosis of the talus, distal femoral epiphysis, distal tibial epiphysis, prox-
dilated blood vessels with phlebolith formation occurs in imal tibial epiphysis, and the tarsal navicular bone. When
almost half of the cases. The vascular lesions are usually deformities are present, they tend to involve either genu
6.6 Lower Extremity Length Discrepancies … 637

valgum or genu varum, valgus deformation of the ankle and changes occurring over a several year period afterward. They
equinus deformity of the ankle. noted that the affected extremity was usually shorter although
Kettelkamp et al. reported 15 new cases and also occasionally longer but that the affected limb usually appears
reviewed the literature; they noted that inequality of limb larger in circumference and often has angular bone deformi-
length was found occasionally and that the affected ties. In the epiphyses and carpal and tarsal bones, there is often
extremity could be either shorter or longer [89]. The distal a spotty or patchy collection of increased radiodensity. An
fibular epiphysis and the medial cuneiform were also affec- inequality in limb length ranging from ½ to 4½ in. was found
ted in some instances. When the seven most common sites of in 9 patients; the involved or more severely involved
involvement were included, they accounted for 84 % of all extremity was almost always shorter except in one instance in
lesions. Lower extremity length discrepancies were descri- which it was longer.
bed in only three of their cases, although no specific com- Younge et al. did a study of 14 children with the disorder
ments about measurement were made otherwise. In those [93]. The principal presenting clinical features were unilat-
three instances, the involvement was marked with 1 patient eral soft tissue contractures and inequality of limb length.
having 3½ in. shortness and 2 with overgrowth having dis- The initial bony changes involved endosteal thickening or
crepancies of 1 and 2½ in. hyperostosis marked by streakiness of the long bones and
Fairbank reported on 14 additional cases [90]. The length spotting of the small. As in the Campbell et al. series [92],
of the limb was usually unaffected but on occasion dis- there was equal involvement of males and females. Eleven
crepancies did occur. He reported three instances of length of the 14 were followed at least to 16 years of age. Soft
discrepancy in 14 patients; 1/4 of an inch of shortening, 1 in. tissue contractures causing severe and rigid joint deformities
of lengthening, and ½ in. of lengthening. occurred in all and were the presenting complaint in 11. In
If we summarize the length discrepancy descriptions from eight patients, only one limb was involved, in three two
the papers of Connor et al. [88], Kettelkamp et al. [89], and limbs, in two three limbs, and one had all four limbs
Fairbank [90], the number of instances of clinically signifi- affected. Contractures were most commonly seen at hip,
cant length discrepancy is relatively small with 11 out of 45 knee, ankle (club-foot), fingers, and iliotibial band. Each of
involved or about 1/4 (25 %) of the patients. Of those with the 14 patients had a lower extremity length discrepancy. In
involvement, 6 had shortness on the involved side and 5 13 the affected limb was shorter ranging from 1.2 to 10.0 cm
were longer on the involved side. The range of discrepancy with an average shortening of 4.1 cm. In the one patient
in those with shortening was from 0.6 cm to almost 9 cm whose limb was longer, the discrepancy was 2.5 cm. Firm
(0.6, 1, 1, 2, 6, and approximately 9), while in those with thickening of the skin with tethering of the underlying fascia
overgrowth the values ranged from 1 to 5.7 cm (1, 1.2, 2.5, was seen in 5 patients. The problems of joint deformity,
2.5, and 5.7). In summary, therefore, while clinically sig- bone deformity, and contracture were marked and poorly
nificant lower extremity length discrepancy occurs in only responsive to nonoperative and even operative attempts at
approximately 25 % of patients and appears to be equally correction. Indeed amputation was required on 4 occasions,
divided between shortening and overgrowth, on occasion it almost always after failed surgical procedures. Epiphyseal
can be marked such that examination through the growing arrest was effective in treating the length discrepancies.
years is essential in regard to length discrepancy as well as More recently Marshall and Bradish reported successful
angular deformity and range of motion. tibial and fibular lengthening with the callotasis technique
[94]. The discrepancy at maturity was 4 cm equally divided
6.6.5.5 Melorheostosis between femur and tibia. The regenerated bone in the dis-
The rare skeletal dysplasia melorheostosis is characterized by traction gap had the radiologic appearance of the original
linear radiodensity or sclerosis primarily in the metaphyseal bone.
and diaphyseal regions and can be associated with length
discrepancy owing to asymmetric involvement. Daoud et al.
reported one case in which the predicted final discrepancy was 6.6.6 Destroyed Physes
2.5 cm of shortness on the involved side [91]. Campbell et al.
reported 14 patients with the disorder which was character- If destruction and premature fusion of a physis occurs, a type
ized by the radiographic long bone abnormalities with a pri- I pattern of discrepancy development invariably follows
mary clinical finding of contractures or limitation of joint except at the hip (as will be described), with no tendency to
motion [92]. The tendency of the disorder is to be either compensation by the other physes in the involved bone.
exclusively monomelic or at least concentrated in 1 or 2 of the Such destruction occurs most commonly today with certain
major long bones. Any bone of the body can be involved, physeal fracture-separations. Significant lower extremity
however. The clinical findings at birth or early in childhood length discrepancies are seen with distal femoral growth
involve contractures, fibrosis and abnormal skin with the bone plate fracture-separations, especially if there are several
638 6 Lower Extremity Length Discrepancies

years of growth remaining since 70 % of femoral growth

occurs distally. Successful focal transphyseal bone bridge
resection will allow growth to resume in some instances.
Other causative factors are physeal ablation during tumor
resection and severe osteomyelitis, particularly in the pre-
antibiotic era. Wilson and McKeever documented shortening
in 18 (21.1 %) of 85 infected bones whose physes were
damaged from an adjacent focus of osteomyelitis [95].

6.6.7 Abnormal Growth Following Use

of Neonatal Umbilical or Femoral
Catheters

On occasion prolonged use of neonatal umbilical or femoral

catheters for newborn illness has been associated with mild
growth stimulation on the involved side. More serious
sequelae were reported by McCarthy et al. in four patients in
whom significant lower limb length shortening occurred
subsequent to neonatal catheter use [96]. This involved
either umbilical or femoral arterial catheters or both. The
projected length discrepancies were massive varying from Fig. 6.9 Type I pattern of a developmental discrepancy in a patient
10 to 20 cm at maturity. Autopsies on other patients with with poliomyelitis is shown (Reprinted with permission from Shapiro F,
indwelling catheters demonstrated varying degrees of Developmental patterns in lower extremity length discrepancies. J Bone
Joint Surg Am 1982;64A:639–651)
thrombosis of the major associated vessels along with many
embolic phenomena so the complication should be consid-
ered if length discrepancy problems surface after a pro- developing poliomyelitis before the age of 10 with consid-
longed or difficult neonatal hospitalization. erable difference in paralysis of the legs was almost certain
to develop inequality of leg length [100]. Barr had indicated
in an earlier study that the incidence of leg shortening in
6.6.8 Poliomyelitis poliomyelitis was as high as 78 % of cases but more detailed
studies revealed an even greater increase in the likelihood of
In patients with poliomyelitis involving the lower extremity, shortening. To a certain extent, the shortening was related to
the type I pattern was seen commonly. There was, however, a the age of onset of the poliomyelitis and to the extent of the
distinct tendency for the discrepancy to increase most rapidly musculature weakened but these were not absolute correla-
in the first 4 or 5 years following infection, with the rate of tions. Barr found that 35 % of patients developed a dis-
increase diminishing after that (types II and III) (Fig. 6.9) [50]. crepancy of 1½ in. (3.8 cm) or more, and Green noted that
This fact has been pointed out previously by Green and 8 % developed a shortening >2 in. (5 cm) [100].
Anderson [97], Ratliff [98], and White [99]. Assessment of Ratliff analyzed several factors relating to limb shortening
115 patients who were followed for 10 years or more, either to and poliomyelitis in 225 children in whom paralysis was
skeletal maturity or to the time of surgical physeal arrest, confined to one leg [98]. Patients were assessed between 5
indicated that almost one-third of the patients demonstrated a and 17 years after infection. The study assessed length dis-
type II or type III pattern [50]. Improved function due to crepancy prior to any limb equalization procedures. Mea-
tendon transfers and bracing may be responsible for the surements were clinical from anterior superior iliac spine to
lessening rate of discrepancy with time. Detailed studies medial malleolus. Shortening had occurred in 219 of 225
during the poliomyelitis era described a good but variable children (97 %) of whom 190 had some radiographic studies
correlation between the extent of shortening and the severity which led to effective measurement to within 1/16 of an
of involvement. No correlation between the age at onset and inch. The greatest incidence of onset of poliomyelitis was
amount of shortening was found in one major study, although within the first 3 years of life with relatively few developing
this finding was challenged (Fig. 6.9). the disease after the age of 8 years. The involved leg was
During an era when polio was common, it was noted by always shorter. There were 65 patients followed into adult
Ratliff that lower extremity shortening was seen in 219 of life, with no surgery having been performed, in which the
225 affected patients [98]. Barr had stated that any child shortening varied between 1/4 of an inch and 5 in. There was
6.6 Lower Extremity Length Discrepancies … 639

only 1 patient with the 5 in. shortening such that the natural Ring also studied limb shortening in relationship to
history would best be defined as leading to discrepancies paralysis in poliomyelitis [101]. His study did not provide
between 1/4 of an inch and 3½ in. since each quarter inch absolute numbers of the amount of shortening but rather
gradation between those two numbers had patients involved. assumed (somewhat incorrectly) that it would occur at a
The discrepancy was 2 in. or greater in 25 of the 65 patients. constant rate with time. The degree of shortening was
The discrepancy was almost always greater in the tibia than basically the same in limbs with muscle power graded as 2,
in the femur. In 184 patients assessed in this regard, 12 had 1, or 0. The amount of shortening progressively increased;
tibial shortening only, 94 had tibial shortening greater than however, as the grade of strength diminished from 5 to 4 to
femoral shortening, 47 had equal tibial and femoral short- 3. Shortening was least in the strongest patients. The
ening, 30 had tibial shortening less than femoral shortening, important feature was therefore not the degree of weakness
and only one had femoral shortening only. Ratliff felt, after but whether the muscles could function against gravity;
careful analysis, that it was impossible to predict accurately within this group, the stronger the muscle the less the
the distribution of shortening from a study of the distribution shortening. The cause of shortening in poliomyelitis was not
of muscles paralyzed. In another subset of patients followed felt to be weakness per se but rather the diminished vascu-
for at least 9 years after disease onset (130 patients), 3 larity which accompanied the decreased muscle mass.
patterns of progressive shortening were noted. One involved Absolute correlations, however, were never possible in
a rapidly progressive discrepancy pattern in which 2.5 in. or relation to the age of onset of the disorder or the extent or
more discrepancy occurred within 9 years or less of the onset type of muscle weakness.
of the disease, slowly progressive in which the discrepancy Gullickson et al. determined that the average percentage
increased at a constant rate amounting to 2–3 in. by adult life shortening of unilaterally affected limbs with poliomyelitis
and a nonprogressive variant in which a discrepancy of up to did not appear different between the 0–5 year age group or
1.5 in. was present 5 years after onset of the disease and then the 6–10 year age group in terms of age of onset of the
remained constant until growth ceased (the type III pattern). disorder [102]. They also noted no correlation between
There was no evidence that a decrease in the amount of muscle strength of the leg and shortening of the tibia. There
shortening ever occurred (absence therefore of the type V was, however, definite correlation between atrophy of the
pattern). Ratliff noted that almost 62 % of patients fell into thigh or leg and shortening of femur or tibia. They provided
the nonprogressive group III pattern. The classification of percentages rather than measurements of limb shortening. In
paralysis as mild, moderate, or severe could not reflect the those with age of onset between 0 and 5 years of age, the
pattern of length discrepancy progression. Although there percentage shortening of the tibia was 2.44 and of the femur
was a considerable overlap between groups, in general those 2.52 in 47 cases and in those from 6 to 10 years of age, the
with mild paralysis had lesser amounts of shortening than percent shortening of the tibia was 2.37 and that of the femur
those with severe paralysis, with moderate in between. For 1.96 in 29 cases.
example, each of the five patients without any shortening Green in a discussion published in the Journal of Bone and
had mild involvement. In 102 of the patients assessed Joint Surgery after an article published by Stinchfield et al.
9 years after poliomyelitis, those with mild paralysis had reviewed the fact that, in 257 cases of poliomyelitis studied in
shortening from 0 to 2 in., those with moderate paralysis his unit in which one lower extremity was affected and the
from 0.5 to 2 1/4 in., and those with severe paralysis from 1 other was normal, the average maximum growth inhibition
to 3.5 in. There was no significant difference in the range of occurred from the second to the fifth year after the onset of the
shortening which occurred in patients suffering disease onset disease; there was less inhibition prior to the second year and
between 0 and 2 years of age and those between 3 and less inhibition each year after the fifth year following onset
7 years of age. The range of shortening was also similar [103]. It was thus recognized that not all patients with
whether the paralysis involved only one muscle or all of the poliomyelitis increased their discrepancy at a constant rate
muscles below the knee. No child with paralysis only below with time. Green and Anderson showed that the final result of
the knee, however, showed a discrepancy >1 3/4 in. On epiphyseal arrest treatment for poliomyelitis was within
occasion, some transient lengthening of the involved para- 1.2 cm (0.5 in.) of predicted amount in 88.5 % of 61 cases [97].
lyzed leg was noted during the first 2 years after the onset of Barr provided an excellent review of inequality of leg
the paralysis but this was always temporary and no patient length in poliomyelitis [100]. Based on assessment of 371
was found in the series with lengthening of the paralyzed leg cases during an active era of poliomyelitis, with the onset
5 years or more after onset. It is likely that inequality of leg before the age of 16 years, shortening was 0.5 in. (1.3 cm) or
length was present before the onset of the poliomyelitis less in 41 %, over 0.5 in. but <1.5 in. (1.3–3.8 cm) in 24 %
which would of course not have been observed. Premature and 1.5 in. or more (3.8 cm+) in 35 %. Numbers from his
physeal fusion was not a feature of poliomyelitis. clinic indicated that approximately 80 % of patients with
640 6 Lower Extremity Length Discrepancies

unilateral poliomyelitis developed shortening on the dynamic or static hip and knee flexion contractures and an
involved side. Subsequent studies showed the incidence to expected, but rarely documented, shortness in the height of
be higher with virtually all patients with unilateral the foot may further decrease the functioning length of the
poliomyelitis having some degree of shortening. hemiparetic limb. If a tendo Achilles lengthening is done and
Stinchfield et al. attempted to project how much the the lower extremity length discrepancy is not appreciated,
growth discrepancy would be based on the muscle power in there may be a tendency for equinus deformity to recur on a
the affected extremity compared to the muscle power in the mechanical compensatory basis.
normal extremity [103]. A chart was constructed based on
data from 64 cases which had reached adulthood such that
the final discrepancy was known. The hope for clear corre- 6.6.10 Septic Arthritis of the Hip
lation between muscle strength and limb length discrepancy
was shortly disproven however. Damage to the femoral capital epiphysis in septic arthritis can
produce serious growth discrepancies especially if it occurs
in infancy [105, 106]. In one series such discrepancies tended
6.6.9 Hemiparetic Cerebral Palsy to increase with time, but a type I pattern was seen only in
42 % of the patients, and most commonly when the infection
Most of these patients have a lower and upper extremity had occurred relatively late, after the age of 7 or 8 years [50].
length discrepancy with the shortening on the more distal An assessment of pattern development in this group was
parts of the involved side. Type I and type III developmental obscured somewhat more often than in other groups because
patterns in the lower extremity predominated [50]. Lower of the necessity for early and often frequent surgical inter-
extremity shortening in hemiplegic children occurred almost vention, although femoral osteotomy per se was done infre-
exclusively in the tibia, a correlation also noted by Staheli quently in growing children. The patterns in this assessment
et al. [104]. Growth alterations in the hemiplegic child were were based on femoral and tibial lengths. In following such
studied in 50 children with spastic hemiplegia by Staheli patients, however, it is important to be aware that if dislo-
et al. [104]. The hemiparetic side was always somewhat cation occurs, the practical consideration in discrepancy
shorter than the contralateral normal side. Discrepancies in relates to the distance between the iliac crest and the floor.
the affected upper extremities were actually larger than those This can be documented accurately by orthoradiographs, but
in the lower extremities. The mean difference in the radius in a combination of measured blocks under the shortened
terms of percentage growth inhibition in 25 cases was extremity in association with a standing anteroposterior
greatest representing 6 % while the inhibition in the humerus radiograph of the pelvis is also important.
was approximately 4.2 % and in the tibia approximately 3 %. Even with complete destruction of the epiphysis, how-
There was essentially no difference in the femoral lengths. ever, femoral shortening did not invariably become worse
The limb length inequality was far more significant in terms with time, particularly in the younger patients and in those in
of functional disability in the lower than in the upper limb whom femoral head dislocation did not occur. When the
but lower limb discrepancies were not particularly great greater trochanter overtakes the involved femoral head in
since femoral shortening was not a factor. A discrepancy of height, the femur resumes a somewhat more regular growth
2 cm or greater occurred in only 2 of 16 patients in the older pattern as the greater trochanter and distal femoral physes are
age group and epiphyseal arrest was rarely resorted to. Of normal, thus accounting for the type II and III patterns that
the 16 patients, 11 years of age or older the lower limb were seen. The relatively rare type IV pattern (Fig. 6.10a)
discrepancy was 1 cm or more in 10 children and >2 cm in was also seen with septic arthritis (Fig. 6.10bi–vi). There are
only 2. instances where treatment effectively eradicates infection
Lower extremity length discrepancy represents an allowing physeal growth to continue for several years in
important consideration in many hemiparetic patients. Of the seemingly normal fashion. On occasion, however, premature
46 patients who were followed in the Growth Study Unit for femoral head–neck growth plate closure occurs with no
5 years or more, and who had a discrepancy of more than reactivation of infection leading to a worsening of the dis-
1.5 cm, the average discrepancy just prior to physeal arrest crepancy several years after the infectious insult
or at maturity was 2.0 cm (range 1.5–3.2 cm). Physician (Fig. 6.10bv, vi). The growth of the proximal end of the
referral strongly influenced study of this disease entity, femur, in particular the relationship between the capital
unlike other diseases for which the condition itself was femoral and the greater trochanteric epiphyses, has been
reason for referral. The majority of patients with cerebral discussed in relation to normal, diseased, and experimental
palsy were not assessed for discrepancies. Femoral–tibial situations. The complexities of this particular growth area
shortening alone does not give a true measurement of the must be understood in order to plan the proper time for
functional discrepancy that may be present in the limb, as surgical intervention.
6.6 Lower Extremity Length Discrepancies … 641

Fig. 6.10 a Type IV pattern in a patient with septic arthritis of the Radiographs (i–iv) at 1, 2, 4, 8, 13, and 15 years of age are shown. Note
right hip in infancy is shown. The hip was treated early such that there excellent structural recovery by 8 years of age, mild coxa vara at age
was continuing growth of the physis for several years followed by 13 years with early evidence of premature physeal closure and clear
premature cessation of growth which added an additional centimeter to trochanteric overgrowth with shortened femoral neck at age 15 years
the discrepancy at time of skeletal maturation (Reprinted with owing to premature physeal closure years after the initial infectious
permission from Shapiro F, Developmental patterns in lower extremity insult
length discrepancies. J Bone Joint Surg Am 1982;64A:639–651). bi–iv

Both Betz et al. [107] and Hallel and Salvati [108] found dislocated and subluxated cases the mean discrepancy was
subsequent length discrepancies after neonatal septic arthritis 6.0 cm (range 3.0–9.0 cm). The growth of the femoral shaft
of the hip to range between 3.0 and 3.5 cm if the femoral measured from the proximal tip of the greater trochanter to
head remained located and 5.5–6.0 cm if dislocation the lateral femoral condyle was not affected by the septic
occurred. process and the growth rate of the trochanteric epiphysis
Hallel and Salvati reported on the end result of 24 cases remained equal to the opposite side even when the trochanter
of infantile septic arthritis of the hip in 21 patients, 3 of was placed into the acetabulum in the form of a trochanteric
whom were involved bilaterally [108]. The disorders arthroplasty.
occurred in the first 7 months of life. A clear difference in A long-term multicenter follow-up of the late sequelae of
length discrepancy was noted between those hips which had septic arthritis of the hip in infancy and childhood was
dislocated and those which remained located with the far published by Betz et al. [107]. They defined infantile cases
more serious length discrepancies occurring in the former as occurring from birth to 3 months of age and the childhood
group. Fifteen of the 21 patients had reached skeletal form in those whose onset occurred after age 3 months. All
maturity and each of these others ranged between 11 and patients had reached skeletal maturity and the study involved
14 years of age. Shortening of more than 2.0 cm was noticed 28 patients with 32 affected hips. The lower extremity
in 16 cases. In 8 instances, it was due to arrested or delayed lengths were assessed at skeletal maturity in those in whom
growth of the physis of the femoral head and in 8 other cases no epiphyseal arrest had been performed or were determined
the epiphyseal damage was worsened by a proximal as being the predicted length discrepancy had epiphyseal
migration of the head in either subluxated or dislocated hips. arrest or lengthening not been performed. In the infantile
In those cases where the head remained located, the mean group, the projected lower extremity length discrepancy was
discrepancy was 3.4 cm (range 2.0–5.0 cm) and in the a mean of 3.93 cm with a range from 0 to 7.0 cm. In the
642 6 Lower Extremity Length Discrepancies

childhood group, the mean discrepancy was 4.2 cm with a good/excellent results, 3 fair, and 2 poor. At latest assess-
range from 0 to 8.0 cm. The discrepancy was 2.5 cm or ment, 13 were satisfied with the treatment.
greater in 7 of the 10.
Wopperer et al. studied 9 hips in 8 patients at a mean
follow-up of 31.5 years after infantile hip sepsis, occurring 6.6.11 Tuberculosis
between birth and 3 months of age [109]. Six of the hips had
dislocated and three had remained located. The leg lengths Tuberculosis was frequently associated with lower
were equal in the case with bilateral sepsis and dislocation extremity length discrepancy especially prior to the advent
leaving seven patients in whom length discrepancy owing to of antibiotics [113]. The hip was a common site of infec-
the disorder itself could be determined. In these seven, the tion with the knee involved fairly often. The serious nature
discrepancy ranged from 0 (in 2 patients) to a maximum of of the disease overall and the major sequelae of joint
6.0 cm. The mean discrepancy was 2.86 cm. destruction limited the study of length discrepancy alone.
Cottalorda et al. reported on the growth sequelae of 72 There was early awareness, however, that tuberculous
hips in 60 children with septic arthritis of the hip in early infection of the bones in childhood could affect growth.
childhood [110]. Treatment was variable. In 28, there was no Langenbeck described decreases in growth with tubercu-
lower extremity length discrepancy; in 16 the discrepancy lous involvement of the joints in the lower extremities
was 2.0 cm or less; but in 16 with poor results discrepancies [114]. Dollinger described 41 cases of tuberculosis of the
>2.0 cm were present with an average of 3.5 cm and a range knee joint and found that, in the active phase, the diseased
of 2.0–7.5 cm. leg grew either at the same rate or on occasion more
The type IV developmental growth pattern is limited quickly [115]. Since tuberculous infection was relatively
almost exclusively to abnormalities of the proximal end of indolent, there was a chronic synovitis associated with it
the femur that occur with septic arthritis of the hip, and this stimulation of growth was similar to what one sees
osteomyelitis of the femoral neck, Legg–Perthes disease, and today in childhood rheumatoid arthritis. Dollinger also
avascular necrosis of the femoral head complicating treat- noted that retardation of growth occurred only when the
ment of congenital or developmental dislocation of the disease became quiescent by which time in that era there
hip. In septic arthritis patients in whom damage was rela- had been sufficient damage to destroy the physis and also
tively mild, premature fusion of the proximal femoral capital further decrease height by associated articular cartilage
physis was sometimes noted years after the infectious insult. destruction. Increase in the length of the long bones after
The premature fusion can be detected 2 or 3 years prior to tuberculous infection was also noted by Reschke [116] and
skeletal maturation by the progressive change in the rela- Pels-Leusden [117]. In spite of the fact that tuberculosis of
tionship of the level of the greater trochanteric physis to that the hip had been a common disorder, very little has been
of the proximal femoral capital physis. It is, therefore, written of the extent of length discrepancies resulting,
extremely important to continue periodic assessment of these although one would expect values similar to those with
children by monitoring carefully the relationship of the head septic arthritis.
and neck to the greater trochanter until skeletal maturity, Although the joint affected most commonly in tubercu-
even if the discrepancy has been in a plateau phase for losis was the hip and some of the femoral shortening was
several years. Although the average increase in the late due to damage at the proximal end of the femur, it was
phase was only approximately 1 cm, this amount could frequently noted that there was premature distal femoral
convert a clinically insignificant discrepancy to one of growth plate closure which led sometimes to marked length
2.4 cm or more, and thus warrants special consideration. discrepancies. Gill demonstrated that, contrary to the previ-
Gage and Cary have shown the value of trochanteric epi- ous feeling that it was disuse which led to the length dis-
physiodesis in patients with severe growth damage to the crepancy, there was often premature central closure of the
femoral head–neck physis [111]. epiphyseal cartilage plate of the distal femur and occasion-
Manzotti et al. reported generally favorable results in 15 ally of the upper tibia which caused most of the discrepancy
young adults, average 21 years, who had late sequelae of [118]. He described 15 cases of childhood tuberculosis of the
childhood septic arthritis of the hip [112]. The average limb hip in which the complication occurred. In each case, the
length discrepancy was 6.5 cm. Patients underwent simul- limb had been immobilized for an extremely long period of
taneous hip reconstruction and femoral lengthening using a time. In each instance, the age of the patient at the onset of
modified Ilizarov method. The hip procedure involved a the tuberculosis was <7 years. The distal femoral arrests
proximal femoral support osteotomy with either acetabular, were almost invariably central epiphyseal-metaphyseal bone
subacetabular, or pubic ramus support. Ten patients had bridges which, with continuing growth peripherally, caused
6.6 Lower Extremity Length Discrepancies … 643

formation of an inverted V-shape to the distal femoral epi- saucer-shaped conformation of the proximal tibial epiphysis
physis with a deeper bicondylar notch than on the normal and articular surface. Wilson and Thompson also com-
side. In the tibia, central fusion also tended to occur leading mented on the negative effects on growth with tuberculosis
to a continuing peripheral overgrowth and a saucer shape to of the hip [121]. They did note, however, that in relatively
the proximal end. With any degree of eccentricity of the mild tuberculosis of the knee itself a growth stimulation
central fusion, angular deformity also occurred. In each sometimes occurred.
instance, the premature closure of the distal femoral or
proximal tibial growth plate occurred on the side of the
diseased hip. There was no evidence of tuberculosis of the 6.6.12 Premature Epiphyseal Fusion
knee joint to serve as a direct cause of the physeal fusion. at the Knee Complicating
Gill noted marked osteopenia of the entire affected femur Prolonged Lower Extremity
which he felt was due to a long period of immobilization Immobilization
leaving the cartilage plate more susceptible to traumatic
damage even if slight. Although premature epiphyseal arrest at the knee compli-
Parke et al. also noted a relatively high frequency of cating prolonged immobilization of the hip for tuberculosis
premature epiphyseal fusion at both distal femur and prox- became a well-recognized clinical entity, in reality it was as
imal tibia in tuberculous disease of the hip [119]. They also much the prolonged immobilization as the tuberculosis itself
felt it was due to the associated severe osteopenia of pro- which caused the disorder in the knee region. Subsequent
longed immobilization leading to rupture of the physis and studies showed that prolonged immobilization for many
transphyseal bone bridge formation. Much of the osteopenia other hip disorders led to the same negative sequelae
was also due to the generalized suppression of new bone including such conditions as septic arthritis, chronic
formation by the tuberculous disease. These severe sequelae osteomyelitis, and slipped capital femoral epiphysis treated
were described in the preantibiotic era. The physeal fusion with casting. Negative sequelae have been described with
was primarily central initially. The occurrence was with prolonged immobilization for congenital dislocation of the
prolonged disease which had to have been present for at least hip by Kestler [122], and Botting and Scrase [123]. These
2 years and frequently much longer. They assessed 91 dis- negative sequelae occur with treatments in abduction splints
eased hips with a duration of disease varying from 4 months in recumbency or with hip spicas for 12 months or longer.
to 22 years. It was not trauma per se but rather severe With prolonged immobilization, the disorder appears related
osteoporosis which was the precursor to premature fusion. to severe osteopenia such that even minor trauma can lead to
They noted 29 cases of premature fusion at the ipsilateral transphyseal injury followed by linkage of the epiphyseal
knee in the 91 tuberculous hips. The tibial epiphysis was and metaphyseal circulations leading to bone bridge
involved in 26 patients and the femoral in 17. Both bones formation.
were affected in 14 cases, the tibia alone in 12, and the femur Ross also pointed out the occurrence of distal femoral and
alone in 3. A central bulging defect was seen most com- proximal tibial premature growth plate closure in association
monly in the proximal tibia while in the femur a more with prolonged disability of an extremity where the site of
fragmentary type of central physeal lesion was seen. Physeal pathology was well away from the epiphyseal areas that
growth of the normal limb was not unaffected. There was subsequently fused [124]. He studied 13 patients, 9 of whom
virtually no growth plate problem when the disease was had tuberculosis of the hip with the others suffering from
<2 years of duration. The longer the disease had been pre- septic arthritis of the hip, slipped capital femoral epiphysis,
sent the greater the incidence of fusion such that in the 7 polio, and osteomyelitis of the femoral shaft. Both epiphyses
patients with a 10-year history of the disorder or longer each at the knee can be involved or there can be involvement of
one showed premature fusion. either distal femoral or proximal tibial regions alone. When
Sissons confirmed the histopathology of extreme osteo- the distal femur was involved, there was a high tendency for
porosis in the knee joint region in eight cases of joint closure to occur centrally in almost all instances leading to
tuberculosis from amputation or postmortem specimens, the inverted V appearance of the physis and increased
three of which were studied in detail [120]. The osteoporosis angulation of the intercondylar notch area. In the proximal
appeared quite marked owing to removal of the transverse tibia, peripheral fusions occurred more frequently than
trabeculae making the longitudinal ones more prominent central fusions. The proximal fibula was not affected in any
radiographically and involving also the subcortical bone instance. Post-immobilization epiphyseal fusion occurred
adjacent to the articular surfaces and the bone in the after extremely long periods of cast or splint protection of
neighborhood of the epiphyseal plates. He could not directly the limb, especially in comparison to current treatment
address the physeal cartilage since his studies were per- protocols. In general, the immobilization was >1.5 years.
formed in young adults. The central tibial arrest led to a Examples from the case reports indicate the following
644 6 Lower Extremity Length Discrepancies

periods of immobilization: 2 years and 10, 16 months osteomyelitis [127]. In the preantibiotic era, physeal damage
(plaster cast), intermittent immobilization of the lower with growth retardation was common; afterward, the disor-
extremity for 3 years between 3.5 and 7.5 years of age, der was better controlled although often not eradicated
immobilization in plaster between the ages of 6 and 9.5, 4.5 quickly, such that periphyseal hyperemia persisted and
years (plaster cast) immobilization during the period of rapid overgrowth predominated. Hentschel pointed out in as early
growth with weight bearing not allowed until the age of 13.5 as 1908 that osteomyelitis of the proximal tibia in the
years, a long leg brace between the ages of 6–8 years, 1 year, growing child could interfere with growth [128]. In a series
and 11 months (plaster cast), 7.5 months (plaster cast), and of growing bones affected by osteomyelitis, Speed noted
plaster immobilization between the age of 4–7 years. instances of both shortening and lengthening of the affected
In those patients where radiographs of the knee region bone [129]. Another observer also was noted to have a case
were available during the course of the disorder, one of the where osteomyelitis in a growing child led to 1.5 in. over-
early changes of developing growth disturbance was thin- growth. Speed pointed out that acute inflammation either
ness of the physis and a transverse zone of dense bone on its arising directly in the epiphyseal area or spreading by con-
metaphyseal aspect which would indicate a Harris arrest line. tinuity from the diaphysis could damage and destroy physeal
The growth retardation scars were numerous and osteo- cells leading to growth arrest problems. The associated
porosis of the regional bone was pronounced. The contour of thrombosis served to damage further the blood supply to the
the epiphyseal cartilage was irregular. Bony bridges were growth regions. Depending on the extent of the physeal
then noted to cross the physis. At the distal end of the femur, damage there would be either complete stoppage of growth
the point of arrest was commonly posterior to the central or uneven growth resulting from focal physeal destruction. If
portion of the disk leading to a flexion deformity as well as it became clear that the damaged epiphysis had ceased all
shortening. At the proximal tibia, the arrest was often in the growth and was no longer functional, Speed referred to the
posteromedial quadrant leading to a tibia vara deformity. possibility of excising the contralateral epiphysis to stop its
The tibial tubercle was sometimes the site of premature growth and thus limit any further worsening of the
union leading to genu recurvatum. Histologic examples of discrepancy.
transphyseal bone bridge formation were found. Cartilage In a subsequent work, Speed commented on specific
from the physis showed fibrillation, disorganization, and instances of overgrowth of a long bone owing to stimulation
shortening of the cartilage cell columns and diminution of of the epiphyseal cartilage by inflammatory processes [130].
endochondral growth. Histologic studies from the fibula He recognized that there was an actual stimulation of growth
showed normal physeal cartilage. The osteoporosis led to and that the increased length was not due to a delay in
inappropriate support of the physis and with any closure of the involved physis. He presented one case with
weight-bearing transphyseal injury was prone to occur. The chronic osteomyelitis which began at 6 years of age and
interpretation that weight bearing played a major role in persisted throughout the growing period. In adulthood, the
causing further damage is supported by the fact that the involved side was 2 1/4 in. longer with 1 3/4 in. increased
proximal fibular physis almost invariably remained intact length in the infected tibia. One inch of overgrowth was
and continued to grow. This physis would clearly have been noted in a chronic instance of osteomyelitis of the tibia in a
subject to the same immobilization osteopenia but as it is a 5 year old. Speed was one of the earliest to note that over-
nonweight bearing structure no negative sequelae occurred. growth of long bones could follow infections of those bones,
in particular where the infection did not directly involve the
epiphyseal region. He pointed out that patients should be
6.6.13 Osteomyelitis warned that after infectious disorders of a long bone either
shortening or overgrowth could occur.
Both overgrowth and growth retardation can occur in long Wilson and McKeever studied bone growth disturbances
bones that are the site of osteomyelitis. Growth changes in in 85 cases of osteomyelitis in the preantibiotic era [95].
osteomyelitis vary depending on whether the infection is They also noted that both lengthening and shortening could
controlled in the acute phase or whether it persists as a occur after such infections in a growing bone. The onset of
subacute or chronic osteomyelitis [125, 126]. In addition, the infection in all their cases was prior to 12 years of age.
precise site of the infection dictates the growth sequelae. If it Shortening of the bones was noted in 18 of 85 infections
traverses the physis, then premature growth retardation (21.2 %). The focus of osteomyelitis which caused inter-
occurs leading to a shortening; whereas, if it remains in the ruption of growth was always located in the metaphyseal–
diaphysis and metaphysis juxtaposed only to the physis, then diaphyseal region adjacent to the physeal cartilage. Serial
the hyperemia leads to a growth stimulation with mainte- radiographs showed the epiphyseal line to be interrupted,
nance of physeal function. Pandey et al. have pointed out the narrowed, and eventually prematurely closed in many. The
change over several decades in growth phenomena related to amount of shortening ranged from 1.0 to 4.0 cm although
6.6 Lower Extremity Length Discrepancies … 645

there was one case where 6.0 cm of shortening occurred in a extremity length discrepancies are of great magnitude owing
distal femur although the infection there was complicated by to the early stage at damage and the fact that the majority of
a pathologic fracture. The amount of shortening would be lower extremity growth occurs at the knee region. In the 15
dependent on the extensiveness of the growth arrest and the patients followed either to skeletal maturity or into the sec-
age of its occurrence. Fifteen of the 18 episodes of short- ond decade of life, the maximum discrepancy reached had a
ening occurred in the femur, tibia and humerus and the mean mean of 8.1 cm with a range from 1.0 to 14.0 cm.
amount of shortening in these 3 bones was 2.6 cm.
Lengthening of the involved bones, however, occurred to the
same extent with 18 of 85 infected bones (21.2 %) showing 6.6.14 Meningococcemia
an increase in length of the involved bone. Infection in all
patients with bone lengthening was located in the metaph- Meningococcal septicemia associated with necrotic purpura,
ysis and diaphysis but not necessarily immediately adjacent cardioshock, and neurologic signs is a relatively rare infec-
to the physis. Lengthening occurred in the 18 instances in tious disorder which usually requires intensive resuscitation.
osteomyelitis of the femur, tibia, and humerus. The range of The patient can usually be stabilized medically but a serious
lengthening was between 1.0 and 3.0 cm except for one complication of disseminated intravascular coagulopathy
instance of a 5.0 cm lengthening which appeared somewhat (DIC) can lead to ischemic lesions often with necrosis and
usual. When that single case was eliminated, the 17 instan- on occasion full gangrene of the extremities. Once the initial
ces of overgrowth had a mean of 1.74 cm per bone. and intermediate symptoms are stabilized, there is frequently
Trueta and Morgan performed a long-term assessment of evidence that a growth disturbance of the physes of several
the late results in the treatment of 100 cases of acute lower extremity bones has occurred secondary to emboli of
hematogenous osteomyelitis shortly after the introduction of the epiphyseal vessels directly linked to the DIC state.
penicillin [131]. The earliest studies of the antibiotic era Formal recognition of the late sequelae of meningococ-
soon began to show that shortening was becoming much less cemia on physeal growth came with two papers document-
common than previously. They noted seven instances in ing the phenomenon. Fernandez et al. [134] reported on
their series and in each it was due to primary epiphyseal three patients developing epiphyseal–metaphyseal abnor-
damage usually in infants. Increased growth in length of the malities limited to the lower extremities in multiple joints
affected bones, however, was common and 32 cases while Patriquin et al. [135] described growth-related physeal
demonstrated lengthening although in no case was the changes in four children where development of such lesions
increase more than 2.0 cm. They felt that the increase was had been clinically unsuspected at the time of disease
due to the increased vascularization of the periphyseal area occurrence. Several years after the septicemic event, pre-
following the damage of the nutrient vessels by the infec- mature fusion of part of several physes with subsequent
tious process. Overgrowth following osteomyelitis lasted shortening and angular deformity were noted. Frequently,
until medullary recanalization had occurred, a type III pat- central physeal fusions resulted in a cone-shaped epiphysis.
tern in our classification, by which time the sequestra would Robinow et al. also reported partial destruction of the right
have been resorbed and more normal vascular patterns humeral and right femoral head and physeal regions in a
would have been established [131, 132]. In chronic recurrent 30-month-old girl 2 years after recovery from meningococ-
osteomyelitis of childhood, however, overgrowth will persist cal septicemia and DIC [136]. There were also symmetrical
until the inflammatory focus is totally eradicated. With the epiphyseal–metaphyseal lesions of the lower femoral, and
increasingly widespread use of chemotherapy such as sul- upper and lower tibial physes. The disorder was character-
fathiazole after 1941 and penicillin after 1946, the mortality ized radiographically by progressive narrowing of the physis
from osteomyelitis dramatically diminished as did many of which could be either uniform across the entire extent or
the bone deforming complications in those who survived. focal, and many of the focal deformities tended to be cen-
Patients with infantile osteomyelitis, within the first trally situated (Fig. 6.11).
3 months of life, continue to have serious bone sequelae. A report from the University Hospital of Geneva,
These were well illustrated by Roberts who documented the Switzerland assessed 46 patients with meningococcemia with
long-term disturbed epiphyseal growth at the knee after an average age of occurrence at 4.5 years [137]. Twenty-six of
osteomyelitis of the distal femur or proximal tibia in infancy the patients required immediate resuscitation in the intensive
[133]. The distal left femur was involved in 13 and the care unit and 15 of these subsequently died. Most of the
proximal tibia in 2. In many instances in the distal femur, the survivors had serious complications involving cutaneous
physis and epiphysis were not affected equally across the necrosis and four instances of gangrene of the upper and lower
diameter of the bone and severe varus or valgus malforma- extremities. Two suffered serious bone growth disturbance
tion occurred. Multiple osteotomies may become needed due to partial or complete ischemic destruction of the physis
along with attention to the length discrepancy. The lower with DIC. The negative growth sequelae usually did not
646 6 Lower Extremity Length Discrepancies

nine patients. In each instance, there was no evidence of bone

or joint sepsis during the period of hospitalization and the
acute phase of the disorder. The changes became evident
relatively late, anywhere from 4 months to 6 years after the
septic episode. In these four studies, the patients were young
averaging a mean age of 14 months with a range from
2 weeks to 48 months and had meningococcemia, with or
without meningitis, complicated by septic shock and DIC.
The skeletal abnormalities were of the epiphyses and physes
and involved angular deformities and lower extremity length
discrepancies. Involvement of the upper extremities was rare.
The late radiographic changes appeared either as destructive
of the secondary ossification centers reminiscent of avascular
necrosis or involved epiphyseal–metaphyseal defects by
which is meant narrowing of the physis usually in an asym-
metric fashion or with premature central physeal closure that
produced cupping or peaking of the metaphyseal regions and
often transphyseal bone bridge formation. Involvement has
been reported at the proximal capital femoral growth plate
epiphysis leading to a coxa vara deformity, the distal femoral
epiphysis where central involvement often leads to the
inverted V appearance, the proximal tibial physis where
central involvement was relatively common, and the distal
tibial epiphyses where asymmetric involvement frequently
led to varus or valgus deformation. In those instances where
the lesions were bilateral, length discrepancy was either not
present or was minor. Owing to the age of the patients at the
Fig. 6.11 Radiograph of bone bridge formation following meningo- time of infection major discrepancies have been reported in
coccemia of infancy. X-ray at 7 years of age shows the central bone particular where involvement is at the knee and is associated
bridge clearly with a bone bridge which often can extend to involve almost
the entire physis.
manifest for 1–2 years postinfection. They involve shortening Belthur et al. described 24 children (aged 1.3–11 years)
of the affected limb and frequently angulation owing to seen in their facility in an 11-year period with orthopedic
asymmetric involvement. Radiographs will show generalized sequelae of meningococcal septicemia. The median time to
narrowing of the physis in the more severe instances although presentation was 32 months (12–119) post-sepsis with
often a transphyseal bone bridge forms with the rest of the angular deformity and limb length discrepancy due to partial
physis continuing its growth. growth plate arrest the main problems. Multiple growth plate
O’Sullivan and Fogarty reported two distal tibial physeal involvement occurred in 14 children. The lower limbs were
arrests as complications of meningococcal septicemia which much more affected then the upper and angular deformity at
occurred initially in a 2.6-year-old boy and in an the knee was common. Recurrence of deformity after
18-month-old boy [138]. The first patient returned at 8.5 osteotomy pointed out the need to assess and treat the physes
years of age with a short leg and deformed ankle while a (generally completing the arrest by epiphysiodesis) when
diagnosis of growth abnormality was made 1 year postin- correcting the angular deformities [141].
fection in the 18-month-old boy. The fibula was unaffected.
Monsell et al. reviewed 10 patients (14 ankles) with
angular deformity of the ankle due to distal tibial physeal 6.6.15 Physeal Damage Following Irradiation
arrest as a late consequence of meningococcal septicemia. for Childhood Tumors
The distal fibular epiphysis was unaffected (in 13 of 14) and
continued growth led to varus deformity [139]. Limb length inequality remains a fairly frequent occurrence
Barre et al. documented epiphyseal and physeal abnor- following radiation therapy in childhood for malignant
malities following meningococcal sepsis and disseminated tumors of the lower extremities and also for those of the
intravascular coagulation [140]. They also reviewed three kidney, abdomen and pelvis. Robertson et al. documented
papers from the literature with the four works comprising lower extremity length discrepancy in 12 of 67 patients
6.6 Lower Extremity Length Discrepancies … 647

treated in childhood by radiation therapy in the receiving abdominal, 1 of 2 receiving pelvic, and 6 of 7
above-mentioned regions [142]. The use of high dosage and receiving lower extremity irradiation [145]. There is an
early age of treatment correlate with the severity of the extremely long history of awareness of the negative effect of
growth complications. radiation therapy on epiphyseal function dating back to
The more common tumors were Wilm’s tumor, acute works of Perthes in 1903 [146]. The effects of radiation
lymphocytic leukemia, non-Hodgkin’s lymphoma, Ewing’s therapy on bone growth were well reviewed by Probert and
sarcoma, Hodgkin’s lymphoma, and rhabdomyosarcoma. No Parker [147].
lower extremity length discrepancies were noted in any child
who had symmetric radiation to the abdomen or pelvis. If the
primary tumor was in tibia or femur, limb length inequality 6.6.16 Fractured Femoral Diaphysis
developed frequently. It also developed where there were
asymmetric irradiation fields to the abdomen, kidneys, or 6.6.16.1 Femoral Overgrowth in the Era
pelvis. Limbs were equal where the radiation dose was of Conservative, Non-operative
<2400 cGys whereas length discrepancy was quite common in Treatment
those where the mean level was between 4,000 and 5,000. The The stimulation of femoral growth after a diaphyseal fracture
development of lower extremity length inequality was uni- in children who were 2–11 years old and were treated
formly related to long bone physeal irradiation of 4,500 cGy or nonoperatively was well documented. It appeared to be an
greater. Of the 12 children who developed lower extremity obligate phenomenon and occurred regardless of whether a
length discrepancies as assessed at skeletal maturity 5 had fracture had healed with an overlap, end to end, or in a
length discrepancies of 2 cm or less while 7 of the patients lengthened position, or whether it occurred in the proximal,
developed length discrepancies ranging from 2.5 to 9.0 cm. middle, or distal third of the femur. The average femoral
Katzman et al. pointed to the many skeletal abnormalities overgrowth from the time of fracture-healing in the series
in patients undergoing radiation therapy in the childhood from Children’s Hospital, Boston was 0.92 cm (range 0.4–
years [143]. They assessed material from 19 survivors of 51 1.8 cm) [148]. All patients were treated nonoperatively by a
patients with Wilms’ tumor and 13 survivors of 46 patients combination of skeletal traction and casting. Ipsilateral tibial
with neuroblastoma treated partially or completely with overgrowth, averaging 0.3 cm, occurred in 82 % of the
radiation. Epiphyseal damage was common. Although limb patients. Seventy-eight percent of the overgrowth had
length discrepancy and long bone deformity were noted, no occurred by 18 months after injury. In 85 % of the patients,
detailed analysis of these particular parameters was per- the overgrowth had terminated at an average of 3 years
formed. Lewis et al. studied longer term morbidity in 55 6 months after fracture. The overgrowth phenomenon
patients with Ewing’s sarcoma who survived 2 years or manifested itself as the type III slope–plateau pattern in 108
longer [144]. The dose level required to treat the tumor (93 %) of the patients, with the limb length discrepancy
effectively with a minimal likelihood of recurrence is very remaining unchanged throughout the remainder of growth
close to the dose level that severely damages or destroys (Fig. 6.12). If a fracture heals at length or with lengthening,
physeal cartilage. The length discrepancy is also dependent the overgrowth produces an upward slope–plateau pattern. If
on the age of treatment and the region affected. In those a fracture heals with shortening, the overgrowth leads to a
patients who received <5,000 rads, 18 or 64 % had minimum downward slope–plateau representation. A type II pattern
or moderate morbidity. A dose of 5,000 rads was insufficient occurred in eight patients whose fractures had healed with
to ablate reliably a primary tumor with many at that level
showing recurrence. The intermediate dosage level of 5,000–
6,500 rads was most effective in terms of tumor ablation
although at the higher levels the skeletal morbidity was
proportionately increased. All patients had some degree of
morbidity. Those defined as having minimal problems had
<4 cm of shortening along with joint flexion deformities and
muscle atrophy and fibrosis causing only minor limitation of
activities. In the moderate group, shortening was defined as
from 4 to 8 cm. In the severe group, gross shortening was
present which could not be compensated for by epiphyseal
arrest of the opposite extremity. Fig. 6.12 Type III in the developmental pattern classification is shown
in this example of length discrepancy following a fractured femur and
Dawson described the orthopedic problems with skeletal healing associated with overgrowth (Reprinted with permission from
radiation for various lesions in 35 children. Anisomelia or Shapiro F, Developmental patterns in lower extremity length discrep-
lower extremity length shortening was present in 8 of 9 ancies. J Bone Joint Surg Am 1982;64A:639–651)
648 6 Lower Extremity Length Discrepancies

excessive angulation. In these, continuing overgrowth pre- equal and in only 5 % was the tibia on the contralateral,
sumably occurred due to the prolonged remodeling process. nonfractured side longer. The average tibial discrepancy was
The Children’s Hospital, Boston study assessed femoral 0.29 cm longer on the ipsilateral side (range 0.1–0.5).
and tibial growth after femoral diaphyseal fractures treated Epiphyseal arrest: In the group of 116 patients, 28
conservatively using orthoroentgenograms for documenta- underwent epiphyseal arrest. The average preoperative dis-
tion [148]. Methods of treatment for these fractures by crepancy was 2.39 cm (range 1.7–3.4). The discrepancies
traction and hip spice casting had been described by Griffin occurred as a combined result of overgrowth and healing in
et al. [149]. A detailed review of the length discrepancy anatomical or slightly distracted position. The average dis-
findings follows [148]. crepancy postepiphyseal arrest at skeletal maturation was
Level of overgrowth: In the entire group of 116 patients, 0.66 cm (range 0–1.5) with 86 % of those operated showing
63 % of the fractures occurred in the middle third of the a discrepancy of <1.0 cm. Five of the 28 patients requiring
femur, 28 % in the proximal third, and 9 % in the distal third. epiphyseal arrest had continued to increase their discrepancy
In the 74 patients studied in greater detail, a similar distri- with time due to continuing stimulation on the fractured side.
bution was seen with 66 % in the middle third, 27 % in the Martin-Ferrero and Sanchez-Martin studied femoral
proximal third, and 7 % in the distal third. The site of overgrowth in 71 patients under the age of 14 years [150].
fracture was similar regardless of age or sex. All patients had been treated in various forms of traction
Extent of femoral overgrowth: In the 74 patients with except for a hip spica cast which was applied immediately
initial radiographs within 3 months of fracture, overgrowth after fracture in 7 %. Each had an isolated unilateral femoral
of the fractured femur occurred universally. The average shaft fracture. Femoral overgrowth averaged 0.86 cm (0.1–
femoral overgrowth in all cases from the time of healing 2.1 cm). They felt that the greatest amount of overgrowth
onward was 0.92 cm. The extent of overgrowth was not occurred in those between 3 and 9 years of age who had had
dependent on sex, age at the time of fracture, the position of the most severely displaced fractures. Ipsilateral tibial
healing, or the level of fracture. The 74 completely docu- overgrowth occurred in 60 % and averaged 0.1 cm. Most of
mented patients with fractured femurs were studied the overgrowth occurred during the first year after fracture
prospectively solely on the basis of having had a femoral but continued to a lesser extent during the second year and
shaft fracture. for as long as the fifth year postinjury in 27 %. After this
Temporal aspects of the overgrowth phenomenon: Two time, the growth rate of both femurs was equal in all. These
patterns of overgrowth were seen. In the more common values were remarkably similar to those from the Children’s
pattern, overgrowth continued after fracture healing for a Hospital, Boston study.
limited time period, then ceased with no change in dis- Reynolds studied growth rate changes after fractures of
crepancy throughout the remainder of skeletal growth. This the shaft of the femur and tibia in children using serial
is referred to as the plateau pattern or plateau phenomenon. radiographic measurements of length accurate to the nearest
Much less frequently, overgrowth continued until skeletal millimeter [151]. The fractures were all treated by conser-
maturity although at a much slower rate after the first vative nonoperative methods with no attempt made to
18 months following fracture. In the group of 74 patients achieve anatomical reduction. The increased growth rate
with early and continuing documentation of lengths, 92 % postinjury was greatest within 3 months of injury and was
(67/74) showed temporally limited overgrowth (plateau 38 % in excess of normal with both femoral and tibial
pattern) while 9 % (7/74) continued overgrowth with time. fractures. The rate then decreased but remained significantly
In the entire group of 116 patients, 93 % (108/116) raised for 2 years returning to normal in the tibia approxi-
demonstrated the plateau phenomenon while 7 % (8/116) mately 40 months after injury, and in the femur between 50
persisted in overgrowth. In the 74 completely studied and 60 months after injury. In unilateral femoral fractures,
patients, 64 % of the documented femoral overgrowth the uninjured tibia in the same limb also underwent an
occurred within 9 months of healing (1 year postfracture). acceleration of growth but to a much lesser degree. An
By 1.6 years, postfracture overgrowth was complete in only uninjured femur was not particularly affected by fracture of
12 %; by 2 years it was complete in 45 %; by 2.6 years it the ipsilateral tibia. Reynolds concluded that the acceleration
was complete in 45 %; by 3 years in 77 %; by 3.6 years in in growth of the fractured bones reached a maximum
85 % and by 5.9 years in 91 %. Premature epiphyseal clo- between 3 and 6 months after injury with subsequent
sure on the fractured side with a late change in discrepancy acceleration decreasing and the growth rate returning to
did not occur. normal between 3 and 5 years after injury. He felt that sig-
Tibial overgrowth: The tibia on the side of the fractured nificant measurable overgrowth ceased within 2 years after
femur increased in length from the time of femoral fracture fracture of the femur and within 1.5 years after fracture of
healing such that at skeletal maturity 82 % of patients had the tibia. The observations of greatest value were on 55
slightly longer ipsilateral tibias. In 13 %, tibial length was children with tibial fractures and 32 with femoral fractures
6.6 Lower Extremity Length Discrepancies … 649

followed for between 2 and 5 years. The average increase in those <3 years old, 3–9 years old, and more than 9 years old.
femoral length was 0.7–0.8 cm (range 0.1–1.7 cm) and every Overgrowth also occurred regardless of whether the fracture
femur exhibited some increase in the growth rate. The had been allowed to heal in a shortened position, at length,
average increase in the tibia was 0.3–0.4 cm (this for actual or in a lengthened, distracted position. This is an important
tibial fractures) with a maximum of 1.1 cm. Overgrowth finding as regards the cause of overgrowth and is in agree-
occurred in all except three. ment with Staheli [157] and Viljanto et al. [156]. Over-
Stephens et al. studied leg length discrepancy after growth did not appear to be influenced by whether the
femoral shaft fractures in children and assessed 30 skeletally fracture was in the proximal, middle, or distal third. Because
mature patients [152]. All patients were treated conserva- of the large size of this series and the accurate method of
tively in skeletal traction. Only isolated closed femoral shaft assessment using frequent orthoroentgenograms, these data
fractures without other injury to the limbs were assessed. appeared to reflect the actual situation more closely than
When the fracture had occurred between the ages of 7 and studies which rely on clinical measurements or less accurate
13 years, the limb overgrew about 1 cm regardless of sex, radiologic measurements. Truesdell, in one of the earliest
age, fracture site, or configuration. They recommended that documentations of the overgrowth phenomenon, noted that
treatment aim for 1 cm of overlap at union to compensate for overgrowth occurred whether the fracture was in the upper,
the post fracture overgrowth phenomenon. Treatment was middle, or lower third of the femur [158]. Similar over-
either by skeletal or skin traction for an average of 6 weeks growth regardless of level disagrees somewhat with the
followed by a spica plaster or cast brace. The average tibia opinion of Staheli [157] who felt that proximal fractures
overgrowth was 0.18 cm. After fracture at 7–13 years of age, demonstrated more overgrowth but is consistent with the
limb overgrowth averaged 1.1 cm (range 0–2.4 cm). Since work of Viljanto et al. [156].
tibial overgrowth accounted for only 20 % of the total, the
average femoral overgrowth was 0.92 cm. 6.6.16.2 Physiological Consideration Underlying
Hougaard performed a prospective study of the over- the Overgrowth Phenomenon
growth phenomenon after femoral shaft fractures in 67 The overgrowth phenomenon was recognized as early as
children using radiographic measurements of both lower 1867 by Ollier [159] and received ample documentation in
limbs at 6 weeks and 6, 12, and 24 months postinjury [153]. the early 1900s [160–162]. Increased blood supply to the
Mean femoral overgrowth was 10.6 mm with largest 26 mm. healing bone was felt by Ollier [159], Levander [163], and
There was no relationship between shortening at the time of Bisgard [164] to be the primary cause of the overgrowth.
healing and sex, age, type of fracture, level of fracture, and Although there was early disagreement as to the cause of the
the magnitude of overgrowth 2 years later. Angulation itself phenomenon with some attributing it either to “young bone
caused some shortening. yielding to pull” as the shortening corrected itself [161] or to
The overgrowth phenomenon and its extent with con- a law of compensatory overgrowth [162], most investigators
servative, nonoperative treatment of femoral diaphyseal eventually considered the overgrowth to be a physiologic
fractures in childhood has been well documented. In large process [165] associated with the increased vascularity of the
numbers of patients studied by radiologic measurements, involved bone owing to healing and remodeling. The
Hedberg [154] demonstrated overgrowth in 86 % (38/44); increased vascularity extends to the epiphyseal plate regions
Aitken [155] documented overgrowth in all fractured femur where the overgrowth stimulus occurs. This now appears
patients but one (64/65), and Viljanto et al. [156] docu- amply confirmed especially with the demonstration that
mented overgrowth in 50 out of 51 patients over 2 years of overgrowth occurs regardless of the position of fracture
age. The average documented femoral overgrowth in the healing and that it occurs in all patients treated nonopera-
Children’s Hospital, Boston series was 0.92 cm which tively thus indicating that it is an obligatory phenomenon
compares well with Viljanto et al. [156], 1.07 cm; Aitken rather than one called into play only to compensate for
[155] 1 cm from position on discharge; Hedberg [154], shortening. In addition it has been demonstrated to occur
0.9 cm; Martin-Ferrero and Sanchez-Martin [150], 0.86 cm; with humeral [166] and tibial [167] fractures. Kellernova
Reynolds [151] 0.7–0.8 cm; and Stephens et al. [152] et al. demonstrated increased vascularity to the entire limb
0.92 cm. The same average amount of overgrowth occurred following experimental tibial fracture using venous occlu-
regardless of the age at fracture when the patient group was sion plethysmography [168]. The tibial overgrowth docu-
divided into the age brackets 2–4 years, 5–7 years, and 8– mented postfemoral fracture also provides evidence for a
12 years. Hedberg [154] and Staheli [157] noted slightly total limb response. Increased length of the ipsilateral tibia
greater overgrowth in those 4–8 years of age and 2–8 years averaging 3 mm in 82 % of the patients with only 5 %
of age, respectively, but Viljanto et al. [156] found no sta- showing a longer contralateral tibia is taken as presumptive
tistically significant difference in average overgrowth in evidence of overgrowth in association with femoral fracture.
650 6 Lower Extremity Length Discrepancies

Such tibial overgrowth was also documented by Stephens Wessel and Seyfriedt reviewed 221 patients regarding leg
et al. [152] 0.18 cm, and Martin-Ferrero and Sanchez-Martin length discrepancy whose treatments were divided closely
[150] 0.1 cm. between nonoperative (123) and operative (98) methods
The frequent length assessments and the accuracy of the [170]. Those in the nonoperative group were treated by skin
orthoroentgenographic method in the Children’s Hospital, traction (96), skeletal traction (11), and immediate cast
Boston series have allowed for detailed study of the temporal bracing (16). Most of the operative patients were treated by
aspect of overgrowth. The impression that most of the intramedullary nailing (59) or plate fixation (34). A total of
overgrowth occurs within the first year of fracture and that it 127 patients were followed to skeletal maturity allowing for
is virtually complete by 18 months is valid but it is accurate length discrepancy determination. Of the 127, a leg
demonstrated that the overgrowth phenomenon can persist length discrepancy of 10 mm or more was documented in 45
for 3 or 4 years and, more importantly, in 7–9 % of patients (35 %). Shortening was seen in 7 (mean 14.3 mm, range 10–
it continues for the remaining period of skeletal growth [160, 30) and lengthening in 38 (mean 14.1 m, range 10–25).
162, 165]. Prolongation of overgrowth beyond 18 months or Overgrowth was most extensive with fracture in the 4–9 year
2 years has been alluded to by Hedstrom [166] and Viljanto old group. Plate fixation was most commonly associated with
et al. [156] on the basis of remodeling which can continue increased length. Several cases (7) continued to increase the
for that period of time. These two findings are important in length discrepancy more than 2 years posttraumatically.
following children with femoral fractures especially if they Over the past two decades widely adopted treatment
have been allowed to heal at length or with some distraction. methods for femoral diaphyseal fractures include a Pavlik
In the eight patients whose overgrowth continued, harness for infants up to 6 (or 18) months of age, immediate
assessment following fracture demonstrated overgrowth hip spica casts, or operative intervention (especially after
averaging 1.98 cm in contrast to the entire group which 6 years of age) using such methods as external fixation,
averaged 0.92 cm. The overgrowth 18 months following intramedullary nailing with either a rigid interlocking rod or
fracture was only 39 % in comparison to the overall group two flexible intramedullary nails, or plate fixation using
where 78 % of overgrowth had occurred by that time. either a traditional compression plate or a submuscular
Overgrowth was continuing 8 years postfracture in these locking or nonlocking plate. The implications of these
patients. Five of the 8 had a discrepancy sufficiently large to treatment methods for eventual femoral length are reviewed.
require epiphyseal arrest. In four of the eight patients, no
unusual factor could be identified which might have con- 6.6.16.3 Nonoperative Treatment: Newborn
tributed to the continuing overgrowth but in four of the to 6 Years of Age
patients hyperemic stimuli may well have persisted owing to At present, skeletal or skin traction is infrequently used in
excessive angulation prolonged the remodeling phase and patients with femoral diaphyseal fractures from birth to 6 years
one had myositis ossificans which also is associated with an of age. Femoral length and position of healing must still be
increased blood supply. carefully considered regarding long-term length discrepancies.
During the early weeks of fracture healing there is slight
motion at the fracture site and it was neither feasible or (i) Pavlik harness: Femoral shaft fractures for infants up to
necessary to perform accurate orthoroentgenographic length 6 months of age are increasingly treated with a Pavlik
measurements. There is no accurate radiographic documen- harness. Some report such treatment up to 18 months of
tation of the lengths prior to fracture: Barford and Chris- age. No clinically significant problems were reported in
tensen in a clinical study of 431 normal children found 8 % the initial use of this method by Stannard et al. in 16
with unequal length of the lower limbs although only 0.7 % fractures sustained between birth and 18 months [171].
had a 1 cm or more difference [169]. These limitations in all In a subset of 11 fractures followed for 12–30 months
clinical studies have been discussed in detail [166]. Both (mean 20 months), all fractures healed in good align-
Hedstrom [166] and Bisgard [164] attempted to assess ment with leg length discrepancies <1 cm by clinical
overgrowth from the time of fracture in experimental ani- measurements. This approach is now most common
mals. It is unknown, however, whether the vascular response from birth to 6 months of age where shortening at the
begins simultaneously throughout the whole extent of the start of treatment and during the early stages of con-
femur or whether it spreads from the fracture site toward the solidation is <2 cm.
epiphyses. If the former mechanism occurs, then overgrowth (ii) Immediate hip spica cast: This is generally used from 1
would probably be somewhat greater as it would begin to 6 years of age. Some use the Pavlik harness up to
earlier; if the latter, overgrowth may well represent primarily 18 months and some use the cast until older ages but a
a postconsolidation repair and remodeling phenomenon. consensus seems to indicate more problems in those
6.6 Lower Extremity Length Discrepancies … 651

over 80 pounds weight treated by this approach. With Thompson et al. extended this study a few additional
the child under general anesthesia longitudinal traction years until it included 100 patients [174]. They determined
is applied to the fractured limb as a well padded, snug that 81 % had an acceptable outcome and 19 % an unac-
fitting cast is applied. Most seek to achieve restoration ceptable outcome by the definition of more than 25 mm of
of length at time of cast application. Early spica casting fracture fragment overlap after clinical healing. The tele-
is indicated for fractures with <20 mm of shortening scope test had a sensitivity of 80 % and a specificity of 85 %
desired by most. A single leg or 1½ spica can be used. for predicting outcome. The relative risk of failure of spica
Early spica casting is indicated for fractures with cast treatment with a positive telescope test was 20.4.
<20 mm initial shortening or for fractures that are held A large review of early spica casting by Illgen et al.
in cast after manual reduction with <10–20 mm further clarified the shortening phenomenon in 115 fractures
shortening. Martinez et al. recognized that excessive [175]. The telescoping test was not used. Closed reduction
shortening of the fragments could be a problem with under general anesthesia with application of a 1.5 hip spica
early spica cast immobilization [172]. They reported was done. Average age was 3.2 years. A subset of 62
that shortening >20 mm occurred in 43 % of 55 chil- patients was seen at 18 + months (average 21 months). At
dren 3–11 years of age. If detected early enough presentation, shortening averaged 1.36 cm with 32 > 2 cm.
treatment in 10 was changed to skeletal 90–90 traction Postreduction shortening averaged 0.55 cm with
for a few weeks before resuming spica management none >2 cm. At the time of union, the average shortening
and all healed with <20 mm shortening. Eleven patients was 0.92 cm with 2 ≥ 2 cm. The degree of initial shortening
did not have the shortening detected early enough and was the most significant predictor or loss of reduction in
went on to healing with >20 mm shortening (range 24– cast. With the initial shortening of 0.5 cm, there was a 10 %
35 mm, mean 28 mm). Analysis showed that excessive probability of loss of reduction and progressively 1 cm about
shortening in cast was considered to be >10 mm, and 14 %, 1.5 cm about 18 %, 2 cm at 26 %, 2.5 cm at 36 %,
especially in older patients. 3 cm at 48 %, and 3.5 cm at 60 %. They concluded that the
early hip spica casting was warranted in children aged
Buehler et al. also recognized that shortening of the femur 6 years or less regardless of initial deformity, with the knee
was the most frequently reported complication of early or flexed in cast >50°, and preferably use of 90–90 “sitting”
immediate spica casting treatment [173]. They felt that much spica. An initial shortening of >2 cm was closely observed to
of this problem related to the extent of soft tissue (muscle) make certain further length was not lost in the spica.
injury and periosteal stripping at the fracture site. To assess Ferguson and Nicol studied 101 femoral shaft fractures
this clinically they devised the “telescope test”. With the treated by early spicas in patients from 1 to 10 years of age
patient anesthetized, gentle manual compression was applied [176]. The crucial time period for assessing shortening was
to the distal femur along the longitudinal axis to document 7–10 days. They accepted <2 cm of shortening, measured
the amount of fracture overlap using standardized radio- radiographically, to allow for continuation of spica man-
graphs. The level of overlap was determined when a distinct agement. If shortening was greater than this or other vari-
endpoint of movement was reached. Acceptable shortening ables were beyond acceptable criteria, the patient was
on the test was considered to be 5–15 mm. A 1.5 hip spica switched to other treatment methods. Initial shortening
was applied if the overlap criteria were met; if not a period of of <2 cm occurred in 71 fractures, 2–4 cm in 21, and >4 cm
skeletal traction was used to diminish the shortening and in 9 on initial radiographs. Four patients had casts removed
allow early callus to stabilize the position. Weekly stan- at 7–10 days for shortness >2 cm and they were switched to
dardized radiographs were done with overlap <25 mm dur- traction. Excluding these, 90 patients at review had short-
ing early callus formation considered desirable re subsequent ening <2 cm by scanogram. Of 17 patients 8 years and older,
length. Fifty patients underwent detailed study; 41/50 (82 %) 5 had excess shortening while only 3 of the 81 up to 8 years
had successful outcomes at healing (<25 mm shortening) had excess shortening. The authors demonstrated that in 98
and 9/50 (18 %) failed with >25 mm overlap at 4 weeks. The of 99 fractures, the position accepted at 7–10 days after spica
telescope test for those who failed spica casting was application was the position found 3–6 months postinjury.
37.2 mm. The authors determined that the most predictive Since overgrowth was rarely as great as 2 cm, they subse-
level was above or below 30 mm. A negative telescope test quently accepted only 1.5 cm of shortening. Corry and Nicol
led to a 95 % chance of successful early spica casting. reported a mean of 6.9 mm overgrowth (range 0.2–8.6 mm)
A positive telescope test led to approximately 20 times the in 50 femoral fractures as measured by scanograms at a
risk of failing with early spica. Expansion of the series to 100 mean of 3.9 years after fracture in children <10 years of age
patients found the earlier results accurate and reproduceable. (at time of fracture) [177].
652 6 Lower Extremity Length Discrepancies

Infante et al. studied 175 patients (between 10 and a comminuted fracture lost 7 mm of length. The overgrowth
100 pounds) treated with immediate closed reduction and phenomenon was demonstrated, however, even in those
application of a well-molded hip spica cast under conscious having surgical correction. The mean longitudinal over-
sedation or general anesthesia [178]. With a minimum of growth of the fractured femur treated by operation was
2 years postfracture follow-up, none of the children required 9.8 mm with a wide range from 0 to 30 mm. The corre-
external shoe lifts, epiphysiodesis, or limb lengthening for sponding group of 52 patients treated by traction and casting
length discrepancy. They eventually recommended the had an overgrowth of 10.7 mm. Slightly less overgrowth was
immediate spica cast treatment from birth to 10 years of age seen in those treated with intramedullary nailing with the
for children who weigh <80 pounds. The ages of patients in overgrowth value registering 7.2 mm while those treated with
Group 1 (10–49 pounds) was 1–6 years; Group 2 (50– other means of osteosynthesis had a 13.5 mm overgrowth. In
80 pounds) was 7–11 years; and Group 3 (81–100 pounds) the 19 patients assessed intramedullary nailing with the
was 12–13 years. The average shortening at the time of Kuntscher nail was done in 7, intramedullary Rush rods in 4,
fracture and after reduction and casting was 1.7 cm (range 0– cerclage wires in 3, screw fixation in 2, plate fixation in 2, and
3 cm) decreasing to 0.7 cm (0–2 cm); 1.5 cm (0–4 cm) open reduction in 1. Walsh also found that the greatest
decreasing to 1 cm (1–3 cm); and 2.1 cm (1–4 cm) growth stimulus was seen in cases treated by open reduction
decreasing to 0.9 cm (0–2 cm), respectively. Those with and internal fixation with an average stimulus of 9 mm [181].
>2 cm shortening postcasting were re-reduced. Overgrowth Since the early 1990s there has been markedly increased
corrected all slightly excessive (but <2 cm) discrepancies use of operative treatment for childhood and early adolescent
into a clinically acceptable range. Epps et al. noted short- femoral diaphyseal and metaphyseal fractures. Currently,
ening >2.5 cm in 5 of 45 patients during the course of skeletal traction for several weeks followed by hip spica or
casting but were able to correct the problem by altering the long leg cast immobilization is infrequently used for these
treatment in 4, although one proceeded to healing with a injuries. The matter of the overgrowth phenomenon remains
2.6 cm leg length discrepancy by scanogram [179]. important. In the previous era of traction/nonoperative
Shortening is therefore the main length discrepancy treatment, bones were allowed to heal with 1 cm of short-
concern with immediate/early hip spica or Pavlik harness ening or overlap (bayonet apposition) in the expectation that
management. The use of 90–0 casts with the hip flexed 90° remodeling and overgrowth would allow for normal length
has minimized shortening. If early (1–2 week) shortening is in the long-term. However, with operative intervention the
measured radiographically with early cast treatment, most fragments are positioned to heal at anatomic length (external
recommend to switch to skeletal traction to decrease the fixation or open reduction and internal fixation). It was
shortening to <2 cm for a few weeks until stabilizing callus suggested that overgrowth would be markedly diminished in
is seen, after which casting is resumed. As long as short- those with childhood femoral shaft fractures treated with
ening <1.5–2 cm is present at time of healing this method external fixation or internal stabilization. The matter is of
should give no long-term problem since overgrowth will some importance since it will define the desired position of
further diminish the discrepancy. the bone fragments during healing. If the increased stability
leads to more rapid healing and anatomic reduction favors
6.6.16.4 Femoral Overgrowth in Relation less need for remodeling then stabilization at full length with
to Operative Treatment anatomic reduction minimizes the need for long-term
for Diaphyseal Fractures follow-up. If overgrowth occurs because of fracture itself,
Even when virtually all femoral fractures in childhood were then anatomic reduction and either internal or external fix-
treated by nonoperative means, some centers used operative ation would still lead to overgrowth.
stabilization for certain subsets of patients. Efforts were made
to document postfracture overgrowth in these patients. Vil- (i) External fixation: The tendency is to report few prob-
janto et al. reported on growth responses following operative lems with length discrepancy although many other
treatment of femoral shaft fractures in children [180]. During problems related to the use of external fixators have
a 10-year period from 1957 to 1966 they treated 35 femoral limited its use for uncomplicated fractures. Hedin et al.
shaft fractures (18 % of their patient population) by operative studied 98 fractures treated with external fixation at a
means and assessed 19 of these patients at skeletal maturity. mean age of 8.1 years (range 3–15 years) [182]. Only 1
The average age of the group at time of fracture was 9.8 years length discrepancy was >2 cm. A second paper from
compared with 7.2 years in the group treated conservatively. this group specifically assessed overgrowth after
The age range at fracture in the 19 patients undergoing anatomical reduction and external fixation [183]. All 97
assessment was from 2.6 to 16 years. Sixteen of the 19 patients were followed by standardized orthograms for
patients demonstrated femoral overgrowth. One patient with 1 year and a subset of 45 for 2 years postfracture. The
6.6 Lower Extremity Length Discrepancies … 653

mean overgrowth was only 0.3 cm at 1 year and 0.5 cm technique which continues to the present time. The
at 2 years. They recommended fixing the fractures method is sufficiently stable to allow the child to be
without shortening. mobilized early but without casting but still allows
Blasier et al. wrote an excellent review of their expe- for relatively abundant external callus. The
rience (favorable) with use of external fixation for 139 approach requires end to end reduction/apposition at
femur fractures, mean age 9 years [184]. A small subset full femoral length for stabilization. The method is
of patients followed radiographically for more than now commonly used for children 6 years old or
2 years had lengths ranging from −7.7 to +8.7 mm. older with fractures in the middle 2/3rds of the
These indicated no discrepancy problems but did not femoral diaphysis. Some use it for younger children,
comment on the overgrowth phenomenon in detail. incorporating only one rod if the canal is too small
Miner and Carroll noted variable length discrepancy for two. Care must be taken to prevent distraction at
from −2.1 to +1.8 cm but a high rate (21.6 %) of the fracture site (lengthening) or excess shortening
refracture in 37 cases [185]. with badly comminuted fractures.
(ii) Intramedullary Fixation:
(a) Rigid rod: Proximal to distal insertion of a rigid Ligier et al. documented 123 femoral fractures treated at a
interlocking intramedullary rod in the growing child mean age of 10 years 2 months (range 5–16 years) by the
is limited to those older than approximately 11 years ESIN method using rods of 3.0, 3.5 or 4.0 mm diameter and
due to size considerations. Even in the older group, made of steel or titanium [190]. Length assessments using
however, the main problem limiting use of this radiologic documentation were done in 62 cases followed
approach is the risk of avascular necrosis of the for at least one year (mean 1 year 10 months). A mean
femoral head due to damage of the medial circumflex femoral lengthening of 1.2 mm was noted. In 29 simple
artery when placing the rod though the piriformis transverse fractures, the mean lengthening was 2.06 mm, but
fossa [186]. This event has been minimized by an there were four cases of lengthening of more than 10 mm
insertion approach through the tip of the greater (11, 15, 17, and 23) and three of shortening >10 mm (15, 15,
trochanter but this can cause some damage to tro- and 13). The range in spiral fractures was −12 to +17 mm.
chanteric growth. Raney et al. demonstrated five The study did not assess overgrowth as a phenomenon but
cases of growth damage to the greater trochanteric included final length in effect which combined healing in a
epiphyseal growth plate due to passage of a rigid shortened or lengthened position as well as any overgrowth.
single intramedullary rod in children 9–13 years of Regardless, only 2 of the 62 cases (3.2 %) required eventual
age [187]. This often resulted in a valgus femoral epiphysiodesis for discrepancies of 23 and 20 mm. Stabi-
head and neck but without subluxation. Since the lization was provided by the two elastic nails themselves
single rigid rod method tends to be used in older (internal elastic support) but also by the bone (cortical
adolescent patients, often very close to skeletal end-to-end reduction) and surrounding muscles [189–191].
maturity, overgrowth appears not to be a concern. The elasticity, compared to rigid plate or single wide IM rod
Momberger et al. using a greater trochanteric starting fixation, allows for some movement at the fracture site
approach at a mean age of 13.2 years (range 10–16) ensuring optimal development of the external callus by
in 50 cases noted average leg length discrepancy of reducing shear and converting it into compression and
only 1 mm (range −10 to +11 mm) [188]. traction forces. The fracture hematoma and the endosteal
(b) Flexible intramedullary rods: Intramedullary fixa- circulation remain intact.
tion using two flexible rods became widely used for Mann et al. used two Ender flexible nails in 16 femoral
stabilization of childhood and adolescent femoral fractures in children 9–15 years of age [192]. Their
diaphyseal fractures in the mid 1980s. The approach impression of results was highly favorable. Regarding
referred to (in English) as elastic stable intrame- lengthening, “legs were clinically equal in all patients at a
dullary pinning (ESIP) (or ESIN-nailing) was mean follow-up of 3 years 9 months (range 1½–7 years)”.
developed by Metaizeau and colleagues in Nancy, A larger series by Heinrich et al. of 78 fractures treated by
France using two flexible steel or titanium rods Ender nails at a mean age of 9 years (range 2½–18 years)
introduced in closed fashion via the distal meta- also produced favorable results [193]. In a subset of patients
physeal region [189–191]. Treatment in the United followed for 12 months or longer 20 % had 8.5 mm average
States by Mann et al. [192] and Heinrich et al. [187] “overgrowth” (range 6–26 mm) and 14 % an average of
favored the use of the Ender nails. Excellent results 9 mm shortening.
overall, including reports of minimal to no problems Virtually, all studies of operative treatment of femoral
with length discrepancy, led to wide adoption of the fractures by intramedullary fixation mention length
654 6 Lower Extremity Length Discrepancies

discrepancies but there is variability in the findings which, to 10 % of traction (with or without spica) cases, and 25 % of
a great extent, represents extensiveness of efforts to docu- internal fixation cases (due to excess length).
ment this component of childhood femoral fractures. Post- Several series published since 2000 are reviewed below
fracture limb length discrepancy is fairly complex to study. regarding LLD with intramedullary stabilization.
Not all papers distinguish shortening from lengthening. Cramer et al. in a subset of patients treated with Ender
Some use the term overgrowth when they appear to mean rods seen at least 1 year postsurgery (mean 20 months) and
longer length. Increased length of femur postfracture can assessed by scanogram showed no clinically significant
occur because of (i) healing in a lengthened position and length discrepancy >2 cm [199]. The 22 patients had a mean
(ii) overgrowth of the fractured femur mediated by increased of 7 mm lengthening (range 1–19 mm). All patients were
blood supply to the entire bone stimulating proximal and between 5 and 14 years of age at surgery.
distal physeal growth. Increased limb length also occurs with Ozdemir et al. found only an average lengthening of
a few (2–4) mm overgrowth of the ipsilateral nonfractured 1.8 mm (range 1–3) measured by scanogram 24 months after
tibia, a fact documented in studies of other treatment surgery using two Ender-like nails in children 6–14 years of
modalities. Also an unknown factor is slight prefracture age [200].
length discrepancies in many normal individuals. Houshian et al. reviewed 30 fractures treated with ESIN
To accurately assess overgrowth postfemoral fracture (2 rails) at a median age of 6 years (4–11 years) [201]. At 1–
treated by operative stabilization (external fixation, intrame- 3 years follow-up, leg length discrepancy up to 1 cm was
dullary rodding or diaphyseal plating), a study should men- found in six legs.
tion length of follow-up postfracture, the extent of angular Song et al. showed no discrepancy in 27 flexible retro-
deformity, the quality of the reduction (anatomic, shortening, grade nailings [202].
lengthening, translation), and the method of documenting Oh et al. treated 31 femoral shaft fractures with retrograde
length of the lower extremities (radiographic or clinical flexible intramedullary nailing at a mean age of 6.7 years (5–
assessments). The length of follow-up is particularly impor- 10) years [203]. At a mean follow-up of 27 months, there
tant since overgrowth can continue as long as 2–3 years was no limb length discrepancy exceeding 1 cm.
postinjury and even longer if angular deformity needs to be Ho et al. studied 94 flexible titanium intramedullary nails
remodeled. Vierhout et al. specifically reviewed the at a mean 8.6 years of age (range 3.2–15.1) with a mean
long-term outcome of elastic stable intramedullary fixation follow-up of 13.7 months (0–56) [204]. The length dis-
(ESIF) of femoral fractures in children [194]. They assessed crepancy section of the study was limited by the relatively
71 of their own patients operated at 3–16 years of age in a short follow-up and lack of clarity between shortening and
prospective study for the purpose of evaluating leg length lengthening. They did indicate that lengthening of the frac-
discrepancies. Two and half years postinjury, six showed a tured side was more common. Subjects assessed were not
persistent LLD of more than 10 mm (range 10–25 mm) (with clearly defined. Eleven patients (12 %) had total limb dis-
the fractured limb longer in all) and at 10 years the LLD crepancy >1 cm at 12 months.
persisted in five. They also reviewed 10 published papers Salem et al. assessed 68 femoral and 5 tibial fractures
regarding LLD in ESIF approaches. The findings were treated by ESIN at a mean age of 5.7 years (range 1.9–11.5)
variable with six studies noting no cases of LLD > 1 cm but [205]. There were nine patients with length discrepancy
other studies reported excess lengths (>1 cm) in 4 % of 122 >10 mm. Some were shorter and some longer but follow-up
cases (Ligier et al.) [190], 10 % in 100 patients (Flynn et al.) time (not mentioned) appears to have been limited. The
[195], 14 % (Luhmann et al.) [196] and as high as >1 cm in 8 study did show, however, that transverse fractures tended to
of 17 patients —47 % (Maier et al.) [197]. They stressed not heal with increased length on the involved side while spiral
only awareness of the length discrepancy following intra- fractures showed a tendency for shortening.
medullary fixation but also the need for long-term follow-up Bopst et al. studied use of the flexible intramedullary
preferably until overgrowth stops. nail in 72 younger children at a mean age of 4.1 years
Wright systemically and critically reviewed the treatment (range 1.5–5.9) [206]. Leg length discrepancy was care-
of childhood femoral shaft fractures in 2000 including fully checked in every child with follow-up available in
assessing reports (15 studies) that compared the results of 62 at a mean age of 36.7 months (4–124). Femur over-
two or more forms of treatment [198]. While all of the growth occurred in 27. More than 1 cm of femur over-
common clinical parameters were assessed, leg length dis- growth (always lengthening) was seen in 6 children
crepancies >2 cm were seen in 3 % of early hip spica cases, (8.2 % of the entire group; 9.7 % of the detailed 62
6.6 Lower Extremity Length Discrepancies … 655

studied) all but one of whom had transverse fractures. compression plates for 46 femoral fractures between
Follow-up in these 6 ranged from 39 to 124 months with 4 and 10 years of age [212]. Leg length discrepancy
discrepancies of 12, 13, 20, 21, and 25 mm. They stressed averaged 12 mm (range 0.4–18 mm) with length-
the need to follow children postfracture at least 24 months ening on the operated side in 15 patients with an
or until the lengthening stabilized. average of 12 mm.

(iii) Femoral Plating: (b) Bridge Plates: In order to minimize soft tissue dis-
section to the fractured femur some use bridge
(a) Compression Plates: Treatment of diaphyseal plating or submuscular plating (with or without
femoral fractures prior to growth plate closure was locking plates). These plates were initially used for
reserved initially for patients with head injury, major problematic cases such as severely comminuted
vessel injury, or polytrauma where rigid stabiliza- fractures. The plates were placed under fluoroscopic
tion was essential in the context of the associated imaging in the submuscular plane against the shaft
injuries. Anatomic reduction was achieved prior to following indirect/closed reduction. Each plate was
plate application. The value of the technique was held with a 2–3 screws at top and bottom. The
shown in the 1990s. Ward et al. used an AO femoral average age in three studies was 11.3 years [213],
compression plate in 25 children ranging in age from 9 years [214], and 11 years [215]. Leg length
6 to 16 years of age. Leg length discrepancy was problems have not been a major concern; they are
described as “not a problem” [207]. Hansen reported either absent or clinically insignificant or, if present,
on 13 femoral shaft fractures treated with an AO attributable mainly to the difficult fractures, many
compression plate [208]. The mean age at operation with significant shortening.
was 11 years (6–14 years) and all patients were
followed until closure of the epiphyseal plates. 6.6.16.5 Summary
Patients were reviewed at an average 8 years post- The overgrowth phenomenon following femoral diaphyseal
surgery. Orthoroentgenography was used for post- fractures in childhood and early adolescence occurs regard-
operative documentation. In the surgical cases less of the method of treating the fracture. When the Pavlik
where the operated femur was longer than the con- harness or immediate hip spice casting are used the main
tralateral femur (one femur was shorter), the mean concern is with femoral shortening that the overgrowth
overgrowth was 8 mm (range 3–25). Only one cannot compensate for. If the fracture ends early in the
patient had a severe overgrowth problem of 25 mm. healing process are overlapped by more than 2 or 2.5 cm.,
Kregor et al. reported on 12 patients who had consideration should be given to switching treatment to
compression plating [209]. Scanograms in 7 patients traction to decrease the overlap (shortening). When operative
with uninjured contralateral femur showed 9 mm intervention with external or internal fixation is used, the
femoral overgrowth (range 3–14 mm). tendency is for excess femoral length to occur since the
Compression plating came to be used more com- fragments are usually reduced anatomically for optimal fix-
monly and was used in some centers as the primary ation. Patient should be followed until the overgrowth has
treatment of choice for many otherwise healthy reached a plateau (no further change) even if this is several
patients. Some subsequent papers tended to show no years postinjury.
leg length problems where follow-up was relatively Excellent reviews of the treatment options in pediatric
short and only clinical assessment was done. Fyo- femoral shaft fractures have been written by Flynn and
dorov et al. used compression plating (DCP) in 23 Schwend [216] and Anglen and Choi [217]. Lascombes
fractures between 8 and 12 years of age with et al. have provided an excellent review of flexible intra-
follow-up averaging 16 months [210]. They indi- medullary nailing in children and adolescents [218].
cated that none of the patients developed a leg length
discrepancy. Caird et al. used AO compression
plates in 60 patients with a mean age of 8 years 6.6.17 Fractured Tibial Diaphysis
(range 3–15) and a mean follow-up of 21 months
(range 7–98) [211]. They did not refer to leg length Tibial overgrowth following tibial fracture has been reported
discrepancy other than mentioning that clinical as being most marked in patients who are <9 years old. In an
assessment of leg lengths was recorded and symp- isolated tibial fracture, overgrowth rarely is severe enough to
tomatic limb length discrepancy occurred in two require continuing long-term length assessment, but it can be
children. Eren et al. however, used dynamic troublesome when there is an ipsilateral femoral fracture.
656 6 Lower Extremity Length Discrepancies

Srivastava et al. [219] and Lascombes et al. [218] have used demonstrated a type I pattern, with the remainder equally
elastic stable intramedullary nailing for difficult tibial frac- divided between type II and type III. The average maximum
tures without significant growth sequelae. discrepancy in these 113 patients was 3.16 cm (range 1.5–
6.90 cm). Beals mentions two patients with hemihypertro-
phy who experienced spontaneous correction of limb length
6.6.18 Overgrowth Syndromes and Lower inequality in early childhood with the maximum discrepan-
Extremity Length Discrepancies cies radiographically documented at 1.4 and 1.0 cm prior to
4 years of age with no discrepancy at 6 years of age [220].
Overgrowth syndromes are relatively rare but are frequently We did not note this pattern in our patient group where
accompanied by asymmetric involvement including over- inclusion required at least a 1.5 cm discrepancy at some
growth of digits, localized parts of limbs, or entire limbs and time. Pappas and Nehme also reported on lower extremity
often present for orthopaedic management of length dis- length discrepancies associated with hypertrophy in a sepa-
crepancies. Overgrowth (macrosomia) of the infant is con- rate study of patients from the Children’s Hospital, Boston,
sidered to exist when birth weight is >4500 g, which occurs Growth Study Unit [221]. Many of these patients would
in 0.4–0.9 % of newborns. There are many causes but have been included in the developmental pattern study so it
assessment for syndromal types and lower (and upper) is not surprising that the results were similar. They divided
extremity length discrepancies is needed. As well as length their assessments into patients with idiopathic hypertrophy
and weight abnormalities, many have associated malforma- and those with vascular disease, neurofibroma, and lym-
tions (especially of the head and neck), vascular lesions, and phangioma. Graphs in their work showing the progression of
neoplasia. Vascular lesions can be composed primarily of length discrepancies with age showed a distribution with
capillary, arterial, venous, lymphatic, arteriovenous, or type I, type II, and type III patterns. In 35 patients with
mixed vessels. Neoplastic involvement is common in kid- idiopathic hypertrophy, the lower extremity length discrep-
neys (Wilms tumor), liver (hepatoblastoma), and adrenals ancy immediately prior to epiphyseal arrest averaged 3.6 cm.
(adrenocortical carcinoma) but many other organ tumors are In those with vascular disease, mean discrepancy in 18
seen such as neuroblastoma, glioblastoma, rhabdomyosar- patients preoperatively was 3.3 cm. The mean discrepancies
coma, pheochromocytoma, and pancreatic tumors. Benign in this group were somewhat larger reaching a mean of
tumors are also common. It has been estimated that neo- 5.3 cm preoperatively. Many patients with neurofibromato-
plasms are present in 7.5 % of those with the Beckwith– sis, however, had either no hypertrophy or clinically
Wiedemann syndrome, 5.9 % with hemihypertrophy, and insignificant hypertrophy. In 90 patients followed until the
2.2 % in Sotos syndrome. time of epiphyseal arrest, the average preoperative discrep-
ancy was 3.4 cm. In each of the four groups, mean values
6.6.18.1 Hemihypertrophy and Hemiatrophy indicated that there was almost always both femoral and
(Anisomelia) tibial lengthening but that tibial lengthening was greater than
This diagnostic category has been frequently used in the femoral in each of the subgroups and usually by a 2 to 1
orthopedic literature to refer to two different diagnoses, margin.
hemihypertrophy and hemiatrophy. A more accurate term is Hemihypertrophy is rarely a simple increase in length of
hemihyperplasia since the disorder is due to an increase in the femur and tibia on the involved side. There are almost
cell number rather than an increase in cell size. One study always mesodermal abnormalities associated, many in par-
reviewed patients who had been assessed over a 40-year ticular with vascular anomalies and also a high incidence of
period and it was frequently not clear what criteria were used neuroectodermal abnormalities. Bryan et al. studied 27 cases
to include a patient under each particular designation, but the of congenital hemihypertrophy in which the entire side of
diagnosis of hemihypertrophy did not include patients who the body was affected or there was segmental hypertrophy in
were noted to have hemangiomas, lymphangiomas, lipo- which only one particular extremity was markedly affected
matosis, or neurofibromatosis (who were assessed sepa- compared to the contralateral side [222]. The maximal lower
rately) [50]. At present, the diagnosis of hemiatrophy is extremity length discrepancies were measured. The ranges of
applied to patients in whom both limbs individually appear length discrepancy involvement seemed to be comparable in
to be structurally and neurologically normal, with the short the hemihypertrophy and segmental hypertrophy cases. In 22
limb diagnosed as being hemiatrophic. The developmental patients in whom discrepancy data were listed, the range
patterns in both groups were similar, however, and for the varied from 0.9 to 6.4 cm. Although the large majority of the
purposes of classification the entity was referred to as ani- patients had reached skeletal maturity, a few still had several
somelia. The term anisomelia, which means a condition of years of growth remaining. The mean discrepancy in 13
inequality between two paired limbs, is nonspecific and is patients 12 years of age or older as measured prior to sur-
infrequently used today. Most of these patients (57 %) gical correction was 3.92 cm.
6.6 Lower Extremity Length Discrepancies … 657

MacEwen and Case reviewed 32 cases of hemihypertro- elephantiasis. By review of many case studies, they defined
phy noting a limb length discrepancy in 26 [223]. Of these hemihypertrophy as a true congenital malformation. They
they felt that 65 % had, or would develop, a discrepancy noted that “vascular dilatations were frequent and encoun-
great enough to require epiphyseal arrest. In six patients who tered in varying degrees in the large majority of instances.”
had already been treated with epiphyseal arrest, the mean There were two types involving skin capillaries in some and
discrepancy was 3.3 cm. presenting in others with birthmarks (naevi) involving
There is a very large subset of patients with hemihyper- lesions of the subcutaneous veins which were often true
trophy, primarily associated with a wide range of vascular varices. The cutaneous and vascular abnormalities were
and other connective tissue abnormalities. These are outlined unilateral, always present on the hypertrophic side, and
in the next few sections. exactly limited to the hypertrophic regions. The cutaneous
naevi were present from birth. These lesions rarely, if ever,
6.6.18.2 Initial Delineation crossed the midline and the spots, veritable naevi of reddish
of the Hemihypertrophy discoloration, had a variable series of configurations but
Syndrome—Trelat and Monod were always limited to the particular region of the hyper-
Initial delineation of the hemihypertrophy syndrome occur- trophy. Varicose veins were often present although some-
red in an 1869 monograph by Trelat and Monod “On Uni- what less frequently than the cutaneous vascular changes.
lateral Partial or Total Hypertrophy of the Body” [224]. These too were limited to the regions of hypertrophy and it
They reviewed the clinical findings, considered previous was felt that they were specifically connected with the
case reports including one of their own and used the term general hypertrophy. Many observers also noted superficial
hemihypertrophy to describe the entity. They indicated that arteries to be more dilated on the hypertrophic side. The
Isidore Geoffroy Saint-Hilaire [225] in his book on devel- large number of observations did not note any particular
opmental anomalies in man had commented on asymmetric changes of the deep arterial circulation but rather frequent
development which often involved only a region or small modifications of the veins and capillaries of the involved
part of one side in relation to the other. They reviewed side of a general character of “angiectasies.” They felt that
reports from 1836 onward to present “a general history of the condition was not hereditary although they considered
this defect of conformation.” They defined it specifically as a that the unilateral hypertrophy or hemihypertrophy was
hypertrophy of one side and not an atrophy of the other since congenital. It progressed after birth and during the entire
atrophic conditions appeared almost always to be associated period of development leading to the increasing limb length
with neuromuscular abnormalities and a weakened state. The discrepancies. The disorder produced hypertrophy of several
initial clinical reports were accompanied by extremely tissues but some of the findings such as the varices were
detailed measurements and they were able to indicate that variable.
the average discrepancy in hemihypertrophy was between 3 Studies over a century later continue to point out asso-
and 5 cm at maturation but that the range was great going ciated developmental abnormalities such as the variable skin
from 1.5 to 19.0 cm. The disorder not only involved lesions, central nervous system abnormalities (hemimega-
increased length but also a proportionate increase in soft lencephaly, macrocephaly, seizures), liver cysts and hyper-
tissue size in those areas affected. They pointed out the large plasia, renal unilateral disorders (nephromegaly, polycystic
number of vascular abnormalities present on the hyper- kidney, abnormal collecting system), a high incidence of
trophic side. In virtually all instances, the limb itself was the unilateral dental and oral maldevelopment, and tongue and
site of abnormality which in the standing position caused an facial hemihypertrophy.
elevation of the pelvis most obvious as an elevation of the
iliac crest on the hypertrophic side; it was not the pelvis or 6.6.18.3 Association of Hemihypertrophy
trunk that were abnormal but rather the changes of position with Neoplasia
were due to the increase in lower extremity length. The There is a well-documented incidence of childhood neo-
truncal abnormalities and pelvic obliquity were not fixed plasia in overgrowth syndromes [226–228]. In a prospective
deformations but were due to positional effects based on study of 168 patients with idiopathic hemihyperplasia
limb length discrepancy in the upright and walking position. (hemihypertrophy), a total of 10 tumors in 9 patients were
In 11 of the 12 cases, the skeleton of the trunk was normal. found, an incidence of 5.9 % [226]. The most common
There were almost invariable skin changes on the involved tumor was the Wilms tumor (nephroblastoma) of the kidney
side involving discoloration and changes in thickness. In (6) with 2 adrenal cell carcinomas and 1 each of hepato-
many instances, the skin was thickened and elevated by blastoma and small bowel leiomyosarcoma. Another study
numerous swollen venules. In one of the cases with the in idiopathic hemihypertrophy found that 3 of 250 children
largest discrepancy, there was enormous hypertrophy of the (1.2 %) developed an abdominal neoplasm. There were two
lower extremity (19 cm), congenital lipomas, and cases of Wilms tumor and one with adrenal carcinoma [227].
658 6 Lower Extremity Length Discrepancies

These studies assessed patients with IHH only, excluding hypermethylation of H19 in children with idiopathic hemi-
those with other malformation syndromes such as Beck- hypertrophy and Wilms tumor at 20 % (3/15) was signifi-
with–Wiedemann or Klippel–Trenaunay. Tumors occurred cantly lower than the frequency in children with Beckwith–
contralateral and ipsilateral to the hyperplastic side of the Wiedemann syndrome at 79 % (11/14) indicating differing
body. epigenotypes [233].
Tumor risk in Beckwith–Wiedemann syndrome has been Other tumors include pheochromocytoma, retroperitoneal
determined to be 10.6 % of a total of 520 patients (or 13.7 % sarcoma, testicular carcinoma, and cerebellar heman-
of 402 who had molecular data available [228]. More gioblastoma. Any patient being followed with hemihyper-
accurate diagnosis of tumor risk is becoming possible with trophy must be assessed periodically for visceral neoplasms.
molecular subtyping. In this meta-analysis, the commonest No specific standard of evaluation has evolved but renal and
lesions were Wilms tumors (nephroblastoma) 67 %, hepa- abdominal ultrasound is an easy and relatively effective way
toblastoma 11 %, rhabdomyosarcoma 5 %, neuroblastoma of screening for renal Wilms’ tumor and other abdominal
4 %, other embryonic tumors 3 %, and a series of other and retroperitoneal lesions.
single tumors. The occurrence of embryonic tumors in
patients with the Beckwith–Wiedemann syndrome varies 6.6.18.4 Association of Hemihypertrophy
between 4 and 21 % in many studies from 1976 on [228]. with Silver-Russell Syndrome
Most occur before the age of 4 years and only rarely do the Silver [234, 235] and Russell [236] described a syndrome
tumors develop after the first decade of life. Since most are which subsequently became well defined and is referred to
intraabdominal, the primary screening-diagnostic study is an as the Silver–Russell syndrome. It was described by Silver
abdominal ultrasound. as congenital hemihypertrophy, shortness of stature, and
Merks et al. studied 1073 children with cancer to deter- elevated urinary gonadotropins. Russell commented on the
mine the frequency of malformation syndromes [229]. They syndrome as involving intrauterine dwarfism recognizable at
diagnosed a syndrome in 42 patients (3.9 %) and suspected birth with craniofacial dysostosis, disproportionately short
the presence of a syndrome in another 35 patients (3.3 %) for arms, and other abnormalities. With further study, manifes-
a total incidence of 7.2 %. Many syndromes other than those tations of the syndrome came to involve the significant
with asymmetric overgrowth were identified but of the 42 asymmetry, shortness of stature present at birth even though
with recognizable syndromes, 14 had hemihypertrophy the child was born at term, variations in the patterns of
(hemihyperplasia) with nephroblastoma 10, rhabdomyosar- sexual development which involved elevated urinary gona-
coma 2, neuroblastoma 1, and Hodgkin lymphoma 1. The dotropins or early sexual development or markedly retarded
two patients with Beckwith–Wiederman syndrome had skeletal age in relation to sexual development, unusually
nephroblastoma and hepatoblastoma. short fifth fingers often with increased curvature, a triangular
While neoplasia can occur in both syndromal hemihy- shape to the face, and occasional cafe au lait areas of the
pertrophy and non-syndromal (isolated or idiopathic) hemi- skin. A detailed natural history study of the Silver–Russell
hypertrophy, it appears to be more common in the better syndrome by Tanner et al. showed height at referral
defined syndromal types, and especially with the Beckwith– (4.6 years mean) averaging 3.6 standard deviations
Wiedemann syndrome. Wiedemann reported a 7.6 % inci- (SD) below the mean with that level persisting throughout
dence (5/66) of embryonal tumors [230]. Beckwith detailed growth [237]. The height at a mean age of 13 years was 3.4
the screening approach of renal sonography, concentrated in SD below the mean. The predicted adult height in males was
those under 10 years of age and done as often as every 153.5 cm and in girls 147.0 cm. The limb asymmetry was a
6 months to allow for earlier diagnosis [231]. Ballock et al. hemihypertrophy of the longer side rather than an atrophy of
reviewed the issue of neoplasia with hemihypertrophy from the shorter. The asymmetry was relatively mild being
the pediatric orthopedic viewpoint [232]. The occurrence <1.0 cm, which they felt was a normal variation, in 31 of 36
rate of 5–10 % appears to be at that level where professional patients. Where hemihypertrophy was present the discrep-
opinion varies as to whether close clinical assessment might ancies, in cm, in children with some growth remaining were
be sufficient compared to exposing the large majority of 1.0, 1.17, 1.25, 3.33, and 6.12.
children (and their families) who will not get the disorder to Specht and Hazelrig reviewed the lower extremity length
the expense and stress of multiple assessments. The gene discrepancies in the Silver syndrome including 4 of their own
assessments outlined by Rump et al. should be helpful in cases and 47 from the literature [238]. The 51 cases gave a
categorizing the likelihood of occurrence [228]. Along those good overview of the length discrepancy involvement. The
lines, Niemitz et al. showed that the frequency of asymmetry was noted either at birth or during the first year of
6.6 Lower Extremity Length Discrepancies … 659

life in 29 of 40 instances where data were available. The 6.6.19 Congenital Vascular Malformations
length difference increased in proportion to the child’s Associated with Lower Extremity
skeletal growth. The length discrepancy varied from 0.5 to Length Discrepancies
6 cm although the length data included relatively few who
had reached the age of skeletal maturity. There was no sex Many lower extremity length discrepancies are associated
preference for the disorder. In 4 patients who had reached with vascular anomalies of the affected limb. This correla-
skeletal maturity, each had a significant discrepancy from 4 to tion has been known for some time but there is a vast array
6 cm. There were 7 instances in younger children in whom of involved vascular disorders with many patterns of irreg-
the discrepancy was already in excess of 3 cm. There were 40 ularity noted at histopathologic examination. Most patients
patients in whom the length discrepancy was referred to. with these disorders do not undergo surgical exploration and
There appeared to be a continuing increase in the discrepancy clinical and imaging criteria alone are used for diagnosis.
with time. If the numbers provided are studied by age Many of these disorders are grouped under the term “he-
groupings, there were 13 values of lower extremity length mangiomas” in the older orthopaedic literature even though,
discrepancy listed from birth to 5 years of age, 15 measure- in a histopathologic sense, the lesions associated with major
ments from 5+ to 10 years of age, and 12 measurements from length discrepancies are not true hemangiomas. Orthopedic
10+ years of age to skeletal maturity. In the youngest group surgeons and geneticists have used syndromal name-based
of 13, the mean discrepancy was 1.75 cm with a range from 0 eponyms based on the clinical appearance of the limbs and
to 5.0 cm; in the next age group, the 15 measurements the presence of overgrowth (hemihypertrophy) or less fre-
indicated a mean discrepancy of 2.44 cm with a range from 0 quently diminished growth (hemiatrophy). A more specific
to 4.0 cm; and in the oldest age group the 12 values listed had biologically based terminology has been utilized by pathol-
a mean discrepancy of 3.44 cm with a range from 1.0 to ogists, vascular surgeons, and plastic surgeons in their
6.0 cm. The impression is of a type I pattern with perhaps dealings with these disorders. Previously, some used the
some showing a type II in the later years of growth. Beals general term “angiodysplastic disorders” to refer to this
describes a patient with the Silver–Russell syndrome with a broad array of conditions. A two part article by Malan and
leg length discrepancy of 3.0 cm at 11 years of age [220]. Puglionisis provided correlations of clinical symptoms,
anatomic findings and histopathologic descriptions of the
6.6.18.5 Association of Hemihypertrophy wide array of congenital angiodysplasias [240, 241]. It has
with Abnormalities of the Cerebral been recommended that the term should no longer be used
Vasculature and replaced with the term “congenital vascular malforma-
Fischer et al. described two patients with congenital hemi- tion”. Current terminology, therefore, outlines two distinct
hypertrophy and associated vascular abnormalities of the types of vascular anomaly. These are vascular tumors rep-
brain on the side of the hypertrophy and in the posterior resented by neonatal/infantile hemangioma which resolve
fossa [239]. The abnormalities included giant aneurysm, and are not associated with limb length discrepancies and
capillary hemangioma, and arteriovenous malformation. congenital vascular malformations (CVM) which do not
Literature review indicated only one previous similar patient, resolve spontaneously and are often associated with limb
a girl who died at the age of 6.5 years with a vascular length discrepancies [242].
malformation of the thalamus. The hemihypertrophy in one Mulliken and Glowacki introduced a biological classifi-
patient reached 5.0 cm at 11 years of age at which time an cation of cutaneous vascular anomalies incorporating cellu-
epiphyseal arrest was performed while in the other patient lar features, physical findings, and the natural history of the
the maximum length discrepancy reached was 1.6 cm at 2.5 various disorders [242]. The two major categories of cuta-
years of age after which the discrepancy diminished by a few neous vascular anomalies were defined as hemangioma, a
millimeters over the next 15 years and surgical correction lesion demonstrating endothelial hyperplasia, and malfor-
was not required. The vascular abnormalities were assessed mation, a lesion with normal endothelial turnover. The use
by CT scans, arteriograms, and examination at open neuro- of the term hemangioma should be restricted to a lesion of
surgical intervention in one case. The extent of the hemi- vascular origin that grows by cellular proliferation. It is the
hypertrophy did not correlate with the presence or extent of most common tumor of infancy and demonstrates sponta-
associated of cerebrovascular malformations. Other neuro- neous regression. Malformations result from embryonic
logical abnormalities reported with hemihypertrophy include errors of vascular morphogenesis and are subdivided into
ipsilateral loss of sweating, ipsilateral indifference to pain, slow-flow and fast-flow lesions. The slow-flow lesions
neurofibromatosis, metachromatic leukodystrophy, ipsilat- encompass capillary malformations, lymphatic malforma-
eral ventricular enlargement, and bilateral and ipsilateral tions, and venous malformations while fast-flow lesions
enlargement of the cerebral hemispheres. involve arterial malformations, arteriovenous fistulae, and
660 6 Lower Extremity Length Discrepancies

arteriovenous malformations. Combined vascular malfor- remainder being type II or III. Involution is a well-known
mations are seen frequently involving capillary-lymphatic, occurrence in some types of hemangiomas and may account
capillary-venous, lymphatic-venous, and arteriovenous for slowing of growth stimulation. Although there were
lesions. Once this distinction was made, it became apparent some well-documented instances where partial resection of
that the vast majority of skeletal changes were associated the soft tissue lesions also diminished the growth stimula-
with vascular malformations. The term hemangioma is now tion, most patients demonstrated a type II or type I II pattern
restricted to common childhood tumors distinguished by in the absence of any surgery. The average discrepancy prior
rapid postnatal growth but followed by slow involution. to bone surgery in this group of patients was 3.09 cm (range
Skeletal malformations are rarely seen with hemangiomas 1.8–5.60 cm). The developmental pattern in this group must
but are commonly associated with vascular malformations. be observed carefully in the middle years of the first decade
Hemangiomas passed through a proliferating and an invo- of life, as considerable discrepancy may develop and pro-
luting phase. Histologic study during the phase of rapid jections that are based on the expectation of the same rate of
growth showed endothelial cell proliferation and hypercel- growth stimulation can be misleading.
lularity while during involution the tissue showed diminished In a study of hemangioma of the extremities in 35 cases
cellularity with onset of fibrosis. Malformations showed a in which a broad array of histopathologic diagnoses were
normal endothelial cell cycle and persisted, growing com- included, McNeill and Ray noted 16 limbs with equal
mensurately with the child. Tissue was not hypercellular but lengths, 8 with overgrowth on the involved side, and 11 with
rather composed of vascular channels lined by flat mature shortening or atrophy on the involved side. It appears that
endothelium. Most of the lesions were predominantly venous overgrowth when present was far more extensive than
but the full range of capillary, venous, arterial, and lymphatic shortening since no indication of the amount of shortening,
vascular elements often combined were seen. other than its presence, was made. Overgrowth, where
When skeletal abnormalities were assessed in relation to amounts were listed, ranged from 1 to 8.75 cm [244].
the hemangioma/malformation categorization, it was noted A generalized overview of growth with limb hypertrophy
that of 356 hemangiomas only 3 (1 %) had bone changes, was provided by Maroteaux [245].
while 224 vascular malformations demonstrated bone Hemangiomas, as currently defined: Owing to the fact
changes in 77 (34 %) [243]. Of the 77 patients with vascular that the vast majority of hemangiomas as defined by
malformations, 27 were in the head and neck region and 50 Mulliken and Glowacki undergo spontaneous regression,
in the extremities. In the extremity group, lymphatic mal- axial skeletal overgrowth is almost never seen in con-
formations were frequently associated with hypertrophy and junction with these lesions [242]. Most hemangiomas first
on occasion with distortion of the shape of the bone. The appear in the early neonatal period and 80 % of them grow
extremity venous malformations, however, were frequently as a single lesion with the rest multiple. Infantile heman-
associated with hypoplasia and demineralization. When the giomas are the most common type of vascular anomaly but
vessel malformations were of the combined type, both shape are completely benign. They are typically noticed in the
distortion and hypertrophy and hypoplasia and demineral- first 2 weeks of life. They are far more common in females
ization were found. High-flow malformations tended to with a 3–5 to 1 female–male ratio. Once established there
produce hypertrophy and shape distortion of the bones in the is rapid neonatal growth during the first 6–8 months of life
terminology used by Mulliken and Glowacki. with a plateau in size being reached at 1 year following
which the lesions grow proportionately with the child with
6.6.19.1 Hemangiomas as Used in the Older regression beginning around 5 years and continuing until
“Orthopaedic” Terminology approximately 10 years of age. As the tumor proliferates in
As used for a diagnostic category in the older orthopaedic the superficial dermis, the skin becomes raised and devel-
literature, including our paper on developmental patterns, ops a vivid crimson color. If it is deeper in the dermis and
“hemangioma” encompassed a wide histopathological vari- into the subcutaneous layer, the overlying skin lends a
ety of vascular anomalies, including capillary hemangioma bluish color to it. Contained blood cannot be evacuated
(port-wine stain), cavernous hemangioma, arteriovenous completely by manual pressure. Hemangiomas can be
aneurysms and fistulae, congenital varicosities, and mixed divided into a proliferating phase during which they
lymphangioma-hemangioma lesions. The term “heman- enlarge and the subsequent involuting phase. Histologi-
gioma” had been used by orthopaedic surgeons over a period cally, they originate from endothelial cells and there is
of several decades in a generic sense to refer to any type of endothelial cell proliferation. Active pericytes are often
vascular anomaly. Ipsilateral overgrowth occurred in 29 seen as well. As the tumor regresses endothelial cell
(83 %) of 35 patients, while in the remainder the limb was activity also diminishes. Mast cells make their appearance
shorter on the ipsilateral side [50]. Nine (31 %) of the 29 during the involuting phase. The term capillary or cav-
patients who showed overgrowth had the type I pattern, the ernous hemangioma should not be used.
6.6 Lower Extremity Length Discrepancies … 661

6.6.19.2 Vascular Malformations recurrence but more serious hemodynamic implications

Vascular malformations are all present at birth but may not since they often present as a persistent fetal remnant (truncal)
always be obvious. Unlike hemangiomas, they do not have a vessel that does not involute normally. They are obstructive
growth cycle and then regress but persist and grow slowly (aplasia, stenosis, membranous) or dilated (aneurysm [local]
throughout life. They are benign. Venous malformations, in or ectasia [diffuse]).
particular, may manifest later during childhood. Males and
females are equally affected. Vascular malformations tend to Mulliken-Glowacki Approach, Now Expanded to ISSVA
grow proportionately with the child. The malformations are Classification (International Society for the Study of
classified as arterial, venous, arteriovenous (AV) shunting, Vascular Anomalies)
lymphatic, capillary, or combined vascular malformation. Once congenital vascular malformations were distinguished
Each structural category of vascular malformation has a typ- from hemangiomas, the Mulliken-Glowacki concept stressed
ical cutaneous appearance. The types of vascular malforma- the hemodynamic differences in the various types of lesions
tions are: (i) capillary (CM) (port wine stains), present at birth in implicating clinic profiles [242]. These were broadly
as pink or purple skin lesions); (ii) venous (VM) (soft color categorized as slow-flow or fast-flow lesions. These terms
disappears when compressed, common on jaw, cheek, tongue depend on the amount of blood supplying the malformation.
and lips); iii) lymphatic (LM) (excess fluid accumulates within The size of the shunt is determined by the volume of blood
lymphatic vessels); (iv) arterial; (v) arteriovenous that enters the feeding vessels. High shunt lesions corre-
(AVM) (abnormal connections between arteries and veins spond to macrofistulous AV malformations.
leading to pulsating collections of blood vessels); and In the ISSVA classification, there are the two broad
(vi) mixed (a combination of any of the other 4 types). Each of groupings of vascular tumors (hemangiomas) and vascular
the major categories of vascular malformation has its partic- malformations [249]. There are several variants of heman-
ular histopathological appearance but combined forms (CVM, giomas including: infantile, congenital, rapidly involuting
CLM, LVM) can be difficult to distinguish even histologi- congenital, noninvoluting congenital, hemangioendothe-
cally. The characteristic histopathologic finding is a lining of lioma (± Kasabach–Merritt syndrome). In the vascular
endothelial cells without high activity similar to what is seen malformation group, the slow-flow variants are capillary
in normal vessels. Vessel walls, in particular with venous and malformations (CM): port-wine stain, telangiectasia; venous
lymphatic anomalies, are of variable thickness and mast cell malformation (VM): several variants including Maffucci
distribution in vascular malformations is normal. syndrome); and lymphatic malformation (LM) and the
fast-flow variants are: arterial malformation (AM), arteri-
Hamburg Classification ovenous fistula (AVF), and arteriovenous malformation
The Hamburg classification of congenital vascular malfor- (AVM). Many combined vascular malformations are inter-
mations (with modifications) has five groups: predominantly mediate in hemodynamic effects including: CVM, CLM,
arterial defects, predominantly venous defects, predomi- LVM, CLVM, AVM-LM, and CM-AVM.
nantly arteriovenous shunting defects, predominantly lym- Upton et al. studied upper limb vascular malformations in
phatic defects, and combined vascular defects (mostly 270 patients seen over a 28 year period [250]. The female:
hemolymphatic) [246, 247]. Each group of defects is then male ratio was 1.5 to 1. The malformations were categorized as
subdivided into two anatomical forms, extratruncular and either slow flow (venous = 125, lymphatic = 47, capillary = 32,
truncular forms depending on the stage of embryonic life combined = 33) or fast flow (arterial = 33). MRI with and
when the developmental arrest occurred [247, 248]. without contrast was the best modality to identify site, size,
The extratruncular (ET) form is an embryonic tissue flow characteristics, and involvement of contiguous struc-
remnant following development arrest at the earlier stage of tures. CM, LM, and VM are generally “slow-flow” anomalies
embryonic life, while the vascular system is in the reticular and AVM and arterial malformations are “fast-flow” anoma-
stage. The tissue remnants retain the characteristics of lies. The lesions can be present in skin and subcutaneous
mesodermal cell origin (mesenchymal cells) and can grow regions but can also occur in most organs including muscles
rapidly with stimulation from trauma, surgery, hormone and bones. Combined forms tend to affect the extremities
therapy, or pregnancy. The lesions carry a considerable risk where they are associated with growth irregularity.
of recurrence after therapy. The ET lesions can be either Vascular surgeons seeing the disorders use increasingly
infiltrating or limited causing mechanical pressure as well as sophisticated studies to provide hemodynamic and ana-
their hemodynamic impact. tomic–physiologic information. Noninvasive tests are used
The truncular (T) form develops in the later stages of primarily including duplex sonography (color Doppler
embryonic life and lacks alterability after losing mesenchy- image and spectral waveform analysis), whole-body blood
mal cell characteristics. They have minimal risk of pool scintigraphy (WBBPS) using radioisotope-tagged
662 6 Lower Extremity Length Discrepancies

erythrocytes, T1 and T2 magnetic resonance imaging, minimally depressed platelets from 50,000 to 150,000/mm3.
transarterial lung perfusion scintigraphy (TLPS) using It is associated not with hemangioma of infancy but with two
radioisotope albumin, and lymphoscintigraphy (TLPS) using vascular tumors Kaposiform hemangioendothelioma and
radioisotope albumin, and lymphoscintigraphy. Invasive tufted angioma. Patients with KTS can develop the milder
studies are rarely needed but can include percutaneous intravascular coagulopathy. The vascular malformations are
phlebography and selective arteriography [248]. associated with the various syndromes leading usually to
There are two characteristic hematologic findings asso- hemihypertrophy and appendicular skeletal overgrowth.
ciated with these disorders: (1) platelet trapping, referred to In most studies the predominantly lymphatic defects are
as the Kasabach–Merritt phenomenon, is a rare complication most common, followed by the predominantly venous vari-
and usually occurs in the neonate with thrombocytopenia, ants and much more common than the arteriovenous shunting
<10,000/mm3. (2) In vascular malformations, an intravas- malformations. The reasonably well-defined syndromes
cular chronic consumptive coagulopathy can occur with associated with hemihypertrophy, or infrequently hemiatro-
phy, are described below. Terminology can vary significantly
in different papers and in particular in papers written several
decades apart. Name-based eponyms have been used for
decades to describe these disorders but they lack precision.
The lesions are increasingly described using the Hamburg
Classification or the ISSVA classification for more precise
clinical, anatomic, histopathologic, embryologic, and hemo-
dynamic definition. An excellent overview of vascular mal-
formations has been written by Gloviczki et al. [251].
Syndromes seen frequently with lower extremity length dis-
crepancies include the Klippel–Trenaunay syndrome (KTS),
Parkes Weber syndrome, the Proteus syndrome, Beckwith–
Wiedemann syndrome, congenital arteriovenous fistula, cutis
marmorata telangiectatica congenita, and Maffucci disease.
The Parkes Weber syndrome is an extremity arterial mal-
formation with arteriovenous fistulas along with capillary,
venous, and lymphatic malformations and skeletal hyper-
trophy. The Klippel–Trenaunay syndrome is a combined
lymphatic-venous malformation with cutaneous port wine
stain (capillary malformations) with or without associated
bone hypertrophy or hypoplasia. Hypoplasia, for example,
was characteristic of venous or combined extremity malfor-
mations and demineralization was another common finding
in venous malformations while intraosseous and lytic chan-
ges were characteristically seen with high flow lesions. Many
of the complex vascular abnormalities are associated with a
spectrum of disordered neuroectodermal and mesodermal
elements often with skeletal overgrowth.

6.6.19.3 General Classification of Angiomatous

Lesions Based on Their Size
and Position. Goidanich
and Campanacci
Fig. 6.13 a A patient with hemihypertrophy owing to unilateral
Goidanich and Campanacci derived a classification of the
overgrowth with the Klippel–Trenaunay syndrome (Reprinted with congenital and developmental vascular disorders based on
permission from Shapiro F, Developmental patterns in lower extremity their position and size in the extremities [252]. Their
length discrepancies. J Bone Joint Surg Am 1982;64A:639–651). approach was based on dissatisfaction with confusing and
b Type V discrepancy pattern in juvenile rheumatoid arthritis. The
variable descriptive terms for this group of disorders. They
initial stimulation led to a discrepancy with ipsilateral overgrowth
which then reached a plateau and eventually self-correction with felt that the clinical management could best be guided by
premature fusion owing to the continuation of the synovitis consideration of the size and position of the vascular
6.6 Lower Extremity Length Discrepancies … 663

anomalies. Their six groups included: (1) localized cuta- dilation which could be of two types—capillary involving
neous and subcutaneous vascular hamartomata; (2) localized the nevae and subcutaneous venous involving the varices
deep vascular hamartomata; (3) extensive deep vascular [224]. In the words of Klippel and Trenaunay, however,
hamartomata; (4) multiple deep vascular hamartomata; “they did not observe the remarkably frequent coexistence of
(5) diffuse deep vascular hamartomata; and (6) infantile the nevus, the hypertrophy of the skeleton and the venous
angioectatic osteohyperplasia. They felt that each group had dilations,” feeling that they were secondary occurrences to
distinctive clinical and pathologic characteristics although the primary symptom which was the hemihypertrophy.
some cases showed transitional features. For each group they Klippel and Trenaunay on the other hand insisted “on the
documented whether there was hemihypertrophy or hemia- simultaneous presence of these three principal signs.” They
trophy, radiographic bone changes, varicose veins, skin pointed out several examples from the literature of the soft
temperature, skin angiomata, pain, functional impairment, tissue abnormalities associated with hemihypertrophy and
and swelling. The classification was derived from a study of referred to previous cases to support their contention that the
94 cases with the largest group, deep localized vascular triad of abnormalities was a unique congenital lesion
hamartoma, comprising 45 cases and the smallest, infantile apparent since birth. The nevus and the hypertrophy existed
angioectatic osteohyperplasia, 7 cases. They considered all since birth and the varices became evident around 8–
the angiomata of the soft tissues of the extremities to be 10 years of age. The length discrepancies were extensive;
hamartomatous with the lesion being present from birth and they listed discrepancies in the lower extremities of 4.5, 4, 2,
cessation of growth after skeletal maturity. In terms of 4, and 9 cm. The triad of disorders present in the same
extremity length, examples of both overgrowth and retar- subject were not lesions grouped by coincidence but resulted
dation were noted. In patients with cutaneous and subcuta- from a single disorder. The hemihypertrophy often involved
neous vascular hamartoma, limb length was essentially equal an entire whole side including the face and skull but was
in each of 11 cases. In all 7 cases with infantile angioectatic often segmental and occasionally just involved either the
osteohyperplasia, there was overgrowth on the involved hand or the foot and often individual digits. The bone was
side. In the other four groups, however, there were examples uniformly increased in size in terms of length, width, and
both of overgrowth and retardation in association with the thickness but anatomic shape was normal without angular or
vascular lesions. In the four groups, there were 16 patients other deformations. The limb hypertrophy was present at
with overgrowth and 17 with decreased growth. In terms of birth but progressed with growth, occasionally increasing to
length discrepancy, therefore, even site and extent of the 10 cm. Owing to the limb length discrepancy, secondary
lesion with the two exceptions noted above offer little deformations of the pelvis and lower spine occurred. The
prognosis as to the extent of any discrepancy and whether or disorder was not particularly rare.
not there will be overgrowth or retardation. The affected tissues do not contain hemodynamically
significant arteriovenous communications but there are often
6.6.19.4 Klippel–Trenaunay Syndrome other soft tissue, lymphatic, and bony abnormalities. It is
This syndrome described in 1900 by Klippel and Trenaunay, similar to the Parkes Weber syndrome but it is better to
refers to a congenital abnormality consisting of a cutaneous consider them separately although some studies link the two
nevus (port-wine hemangioma), varicose veins, and bone describing the Klippel–Trenaunay–Weber syndrome. Bas-
and soft tissue hypertrophy (Fig. 6.13a) [253]. It is usually kerville et al. note that the presence of arteriovenous fistulae
unilateral and affecting the lower limb but occasionally more excludes the diagnosis of Klippel–Trenaunay syndrome and
than one limb is involved. Klippel and Trenaunay stressed is characteristic of the Parkes Weber syndrome [254]. In
that the bones on the hypertrophic side while larger main- their detailed study of 49 KTS patients with 56 abnormal
tained a normal anatomic shape and proportion. They called limbs (53 lower extremity and 3 upper extremity), the male–
the syndrome “le noevus variqueaux osteo-hypertrophique.” female ratio was 1.3 to 1. All 49 had visible varicosities,
Klippel and Trenaunay noted that there was considerable 47 had a naevus, and 47 had limb hypertrophy. In 43 of the
awareness of the existence of hemihypertrophy often patients, the abnormality was noted at birth. There was no
including skull and facial asymmetry, asymmetric soft tissue clinical evidence of an arteriovenous fistula in any of the 49
development of the extremities and in particular asymmetric patients including 22 who had arteriography. In 36 patients,
vascular anomalies of the skin and subcutaneous tissues. the affected limb was longer (>2 cm) and in only two was it
They commented on the unique triad of abnormalities shorter. The feet were also usually hypertrophic. Varicose
involving the nevus, varices, and osteophypertrophic chan- veins were visible in all 49 patients. 68 % had a large
ges. They pointed out that Trelat and Monod as early as incompetent vein on the lateral aspect of the limb which
1869 had described a syndrome in which the characteristics arose on the dorsum of the foot or ankle and extended a
were unilateral bony hypertrophy generally involving the variable distance up the leg. Phlebography showed that
lower extremities and frequently accompanied by a vascular approximately 50 % of the abnormal lateral veins drained
664 6 Lower Extremity Length Discrepancies

into the main stem leg veins with 33 % extending to the syndrome at birth. Capillary malformations (port-wine stain)
buttocks and draining via the gluteal veins into the internal were seen in 246 (98 %), varicosities/venous malformations
iliac vein. More than one quarter of the patients had intra- in 182 (72 %) and limb hypertrophy in 170 (67 %). All three
pelvic venous abnormalities as well. Other abnormalities features were present in 63 % and two of 3 in 37 %. Atypical
included 15 % with lymphedema and 22 % with cutaneous veins including lateral foot/leg veins and persistent sciatic
lymphatic vesicles. Five patients demonstrated gigantism of vein occurred in 72 %. Operations were done in 58 %
the toes. including epiphyseal arrest, stripping of varicose
A convincing argument is made to separate the Klippel– veins/venous malformations, excision of vascular malfor-
Trenaunay syndrome in which arteriovenous fistulae are mations, debulking procedures and amputations. Similar to
absent and the Parkes Weber syndrome which is character- Servelle, lower extremity involvement only was 70 %, upper
ized by arteriovenous fistulae [255]. Appreciable overgrowth extremity only 11 %, and upper and lower extremities 18 %,
of the limb may occur in the absence of arteriovenous fis- with additional involvement of thorax, pelvis, abdomen, or
tulae. The difference in lower extremity length discrepancies head and neck. The mean limb length discrepancy in the
rarely increased after the age of 12 years which would imply 42 % who had scanograms was a mean of 1.75 cm (range
a type II or type III discrepancy pattern. Absolute numbers 0.1–9.6 cm). The involved limb was usually longer, being
for the extent of the discrepancy were not provided but shorter only in 7 %. Jacob et al. provided detailed listings of
epiphyseal stapling was performed in only four patients the numerous other congenital anomalies and symptoms in
(4.5 %) although problems with vascularity make surgical these patients.
length discrepancy management potentially dangerous. Baskerville et al. suggested that KTS was caused by a
Servelle provided much useful information on KTS with mesodermal abnormality during fetal development [255].
his review of 768 operated cases [256]. It was his contention There appears to be no true atresia of the deep veins with
that surgical release of compressive fibrous bands of the abnormalities concentrated in the superficial system. Histo-
deep veins in the lower limb improved the clinical symp- logic studies, similar in all patients, showed an increase in
toms. Elongation of the involved limb was invariably found the number and diameter of the venules in a cross section of
although he did not comment on the extent. He indicated that the deeper layers of the dermis and subdermal fat. There was
he had operated (on the venous system) in 48 children also widespread hypertrophy of the smooth muscle in the
between 6 and 12 years of age with “a pronounced difference walls of the subcutaneous veins owing to response to
in the length of the 2 limbs”. Of deep vein malformation, the chronically increased flow. There were normal deep veins
popliteal vein was involved in 51 %, superficial femoral vein and normal calf pump function in 60 and 84 % of patients,
16 %, both popliteal and superficial femoral veins 29 %, iliac respectively. Both Bourde [258] and Baskerville et al. [254,
veins 3 %, and lower vena cava 1 %. He felt that overgrowth 255] suggest that KTS is due to the persistence of part of the
was due to venous hypertension with blockage of the deep embryological vascular system and that a mesodermal defect
venous channels. In 614 of his operated cases, involvement acting primarily on angiogenesis could explain the condi-
of the lower limb occurred in 80 %, upper limb 13 %, upper tion. The findings were felt to be consistent with a later than
and lower limbs 3.2 % with occasional involvement of all normal regression of the embryonic vascular reticular net-
four limbs or both lower limbs. Varicose veins served as work in the developing limb bud. This would lead to
substitute channels for obstructed deep veins. Venous increased capillary and venular blood flow during
obstructive lesions involved fibrous compressions (espe- intrauterine development and to the superficial varicosities.
cially in the popliteal and superficial femoral sites), perive- A mesodermal abnormality would also explain the other
nous sheaths, hypoplasia (especially in the iliac and venous abnormalities with the syndrome involving devel-
superficial femoral veins), and agenesis (iliac veins). In the opmental abnormalities such as absence of valves in the
upper extremity brachial and axillary veins, compression deep veins or reduplication of axial veins and a large often
lesions were most common. Collateral venous drainage often valveless lateral venous channel.
led to symptomatic varicosities at bladder, rectum, and Management. At present, the general management
vagina. The venous stasis and often associated lymphatic approach is observation and conservative medical manage-
malformations led to soft tissue swelling and edema in 84 % ment with elastic stockings and shoe lifts for mild discrep-
of involved limbs. ancies [257]. Operation should not be done to improve
Jacob et al. reviewed 252 patients from the Mayo Clinic cosmesis at the expense of function. The outcomes of sur-
seen in 1956–1995 [257]. They presented a far more con- gery are limited and recurrent varicosities are common. In a
servative management profile with surgery limited to far review of their experience, ligation and stripping of varicose
more specific indications. Sex distribution was relatively the veins was done in 49 patients with better outcome in only
same (136 female, 116 male) and 91 % had evidence of the 40 %, excision of angioma in 44 with better outcome in
6.6 Lower Extremity Length Discrepancies … 665

60 %, epiphyseal arrest in 41 with a better outcome in 90 %, hypertrophy to dilatation of arterial and venous trunks with a
and debulking procedures in 11 with a better outcome in specific arteriovenous communication [263]. He indicated
65 %. that “the communication between the arterial channels and
A more aggressive approach in younger children, (mean the venous channels may be so free that in it a definite kind
age 10.3 years at surgery) has been reported by Baraldini of thrill or pulsation, rhythmical with the heart’s contrac-
et al. in 29 children involving stripping of persistent mar- tions, is transmitted to the veins as in cases of arteriovenous
ginal vein, 16; multiple ligation of bulking varicosities, 10; anastomosis of traumatic origin.” All cases are sporadic. The
sclerotherapy, 14; laser photocoagulation, 13; and excision involved limb is warm, the skin color is pink and diffuse, and
of lymphatic malformations, 5 [259]. 77 % involve the lower limbs. He referred to the condition as
Management considerations involved correction of bony “congenital or developmental phlebarteriectasis” or
overgrowth, excision of soft tissue hypertrophy, and removal hemangiectatic hypertrophy of limbs. Both his papers were
of varicose veins but only in those veins causing pain or accompanied by abundant descriptions of vascular anoma-
discomfort [254, 257, 258, 260]. The widespread removal of lies and limb overgrowth from the late eighteenth and early
varicose veins had not proven to be particularly successful. nineteenth centuries from English, French, and German
Cohen has summarized the Klippel–Trenaunay syndrome reports.
defining its three features as: (i) combined vascular malfor-
mations of the capillary, venous, and lymphatic types; (a) Sturge–Weber Syndrome: In this syndrome, there is
(b) varicosities of unusual distribution in particular the lat- capillary malformation of the leptomeninges overlying
eral venous anomaly observed during infancy and child- the cerebral cortex with or without choroid and facial
hood; and (c) limb enlargement [261]. The lower limb is involvement (port-wine stain on the ipsilateral side).
involved in 95 % of patients, the upper alone in only 5 % Only capillary malformations are found in the Sturge–
with a few having both upper and lower involvement. The Weber syndrome and limb changes similar to those in
capillary malformations of the skin are bluish to purplish in KTS do not occur. Seizures occur in about 83 % of
color, unilateral or bilateral, without facial involvement, and cases. Overgrowth of the maxilla is common.
often intermixed with lymphatic and venous components. Involvement can be unilateral or bilateral. Capillary
The classic varicosity is the lateral venous anomaly found in malformations can appear anywhere on the body.
about 80 % of patients. The malformation begins as a plexus These can be hemiparesis and mild associated hypo-
of veins on the dorsum and lateral side of the foot and then trophic limb development.
extends superiorly for variable distances. Lymphatic abnor-
malities are found in 70 % of KTS patients. 6.6.19.6 Proteus Syndrome
The Proteus syndrome is characterized by patchy or seg-
6.6.19.5 Parkes Weber Syndrome mental hamartomatous overgrowth and hyperplasia of mul-
Parkes Weber pointed out the combination of vascularization tiple tissues and organs along with susceptibility to the
abnormalities with hemihypertrophy of the limbs. A partic- development of tumors. Associated abnormalities include
ular syndrome has come to be associated with his name; it macrodactyly (overgrowth of the hands and/or feet), hemi-
involves the KTS triad of cutaneous naevus (port-wine hypertrophy, asymmetry of the limbs, connective tissue nevi,
hemangioma), varicose veins, and soft tissue and bone epidermal nevi, lipomas or a regional absence of fat, vascular
hypertrophy with arteriovenous malformations. In his initial (capillary, venous) and lymphatic malformations, and cranial
presentation in 1907, he drew attention “to a group of cases hyperostoses. There is concern about neoplastic disorders
in which hypertrophy of one limb, or else hemihypertrophy, and a large number of developmental abnormalities of many
is found to be associated with tumor-like overgrowth in the types with this syndrome [264, 265]. There is generalized
corresponding portion of the vascular system” [262]. He felt thickening and soft connective tissue swellings of the skin
that the disorder was congenital in most instances. In dif- and subcutaneous tissue. The connective tissue nevi are
ferentiating a particular syndrome from previously known particularly common on the soles of the feet but may involve
developmental abnormalities of the vascular and lymphatic hands, abdomen, and nose. Histologically, they are com-
systems, he pointed out that the condition under considera- posed of dense collagenous accumulations. The overgrowth
tion was distinguished by the associated vascular abnor- may involve the whole body or it may be unilateral involving
malities and the actual increase in length of the bones of the one limb or occasionally it is localized even to a digit. The
affected limb. He pointed out that even in the late 1800s, skin and subcutaneous thickening is associated with lipo-
there were many cases reported of hemihypertrophy with mata, lymphangiomata, or hemangiomata. There is a rela-
some form or other of angiomatous formation. In his second tively high proportion of skeletal abnormalities other than the
communication in 1918, he linked the congenital limb hemihypertrophy including contractures, bony prominences
666 6 Lower Extremity Length Discrepancies

over the skull, angular deformities of the knees, scoliosis or processes such as cell proliferation and apoptosis. AKT1 in
kyphosis along with the dysplastic vertebrae, hip dislocation, murine chondrocytes controls cartilage calcification and can
and hallux valgus. There is disproportionate involvement of activate ectodermal defects and impair bone morphologe-
the hands and feet with macrodactyly and often soft tissue netic signaling. Previously, grups had reported Proteus to be
hypertrophy, in particular over the plantar surfaces of the feet. due to PTEN mutations. It now appears that patients with
The craniofacial skeleton is large and misshapen due to some PTEN mutations were clinically distinct from those with
or all of hyperostosis, unilateral condylar hypertrophy, Proteus and that Proteus patents do not have the PTEN
craniosynostosis, and underlying hemimegalencephaly. mutations. PTEN mutations underly a similar but different
Cohen and Hayden differentiated the various symptoms from syndrome referred to as SOLAMEN syndrome characterized
other overgrowth syndromes, in particular neurofibromatosis by segmental overgrowth, lipomatosis, arteriovenous mal-
and the Klippel–Trenaunay syndrome [266]. The name formation, and epidermal nevus.
Proteus was suggested by Wiedemann et al. who described
four patients documenting partial gigantism of the hands or 6.6.19.7 Beckwith–Wiedemann Syndrome
feet, nevi, hemihypertrophy, subcutaneous tumors, macro- The Beckwith–Wiedemann syndrome is associated with
cephaly, or other skull anomalies, accelerated growth and hemihypertrophy leading to lower extremity length dis-
visceral affections [267]. Developmental abnormalities were crepancy in about 25 % of cases [271, 272]. The hemihy-
described later including progressive kyphoscoliosis, sub- pertrophy may not be apparent at birth. This clinical entity is
cutaneous abdominal lipomas, dilated veins and/or heman- characterized by macroglossia, omphalocele or other
giomas, facial anomalies, mental retardation (20 %), and umbilical anomalies such as umbilical hernias, macrosomia
occasional seizures (13 %) [268]. One of the characteristics with large muscle mass and thick subcutaneous tissues,
of this disorder is the changing phenotype with time. The hemihypertrophy, linear creases in the lobule of the external
patients are normal at birth to clinical assessment and develop ear, and abdominal visceromegaly especially including large
the characteristic findings over the first year of life. This is a kidneys. Whole body overgrowth onset can occur from the
primary differentiating feature from other overgrowth syn- prenatal period to as late as 1 year of age, is rapid throughout
dromes (such as Klippel–Trenaunay) which have hyper- the first few years of life but then shows a decreased growth
plastic overgrowth of the limbs at birth. With Proteus, rate from mid-childhood to puberty leaving mature height in
patients are normal at birth or show mild alterations (hyper- the high normal range.
plasias, hamartoses). Progressive asymmetric overgrowth Other developmental anomalies can be seen. Hypo-
evolves postnatally. Once established, the abnormalities tend glycemia is present in early infancy in approximately ½ of
to be progressive throughout childhood with growth of the the cases and there is an increased incidence of embryonal
hamartomata and generalized hypertrophy increasing during tumors such as Wilms tumor and hepatoblastoma. None of
the first few years of life. The bone and soft tissue overgrowth the three cardinal features [macroglossia, macrosomia
is stable after puberty. Morbidity is considerably greater than (height and weight equal to or greater 97th percentile), and
with the KT or PW syndromes. Of 11 patients evaluated by omphalocoele] is a diagnostic requisite but at least 2 of the 3
Clark et al. 2 required amputation of the leg, 6 had fingers or are usually present. Geneticists recommend two major and
toes removed, and 2 women had breast implants and recon- one minor criteria to make the diagnosis but milder pheno-
struction [269]. Spinal stenosis and neurologic sequelae can types should be considered for tumor surveillance.
develop due to vertebral anomalies or tumor infiltration.
A recent multidisciplinary workshop addressed earlier 6.6.19.8 Congenital Arteriovenous Fistula
orthopedic interventions since if surgery is delayed too long Horton drew attention to the overgrowth phenomena with
the condition may become inoperable. They suggested soft congenital arteriovenous fistula involving the extremities
tissue releases to counteract contractures and aggressive and [273]. He detailed findings in 23 upper and lower extremity
early use of epiphysiodesis to limit limb length discrepancies disorders in which documentation of the length discrepancy
[270]. was made. In 23 patients, the limb circumference was from 2
The Proteus syndrome is caused by a somatic activating to 8 cm greater than the corresponding normal side and in 18
mutation in AKT1, proving the hypothesis of somatic of the cases there was an increase from 0.5 to 7.0 cm in the
mosaicism and implicating activation of the PI3 K-AKT length of the bones on the abnormal side. The involved
pathway in the clinical findings of overgrowth and tumor extremity was hypertrophied and showed marked evidence
susceptibility [265]. Of 29 patients with the Proteus syn- of engorgement, swelling of superficial veins and skin ulcers
drome, 26 had a somatic activating mutation in the oncogene in most. There was marked increase in the pulsations of the
AKT1 encoding the AKT1 kinase, an enzyme mediating arteries and a definite increase in the surface temperature of
6.6 Lower Extremity Length Discrepancies … 667

the extremity involved. He used the term arteriovenous fis- 6.6.19.10 Lower Extremity Length Discrepancies
tula to designate any abnormal communication or commu- in Vascular Malformation
nications between arteries and veins by means of which Syndromes
arterial blood passes from an artery to a vein without passing Klippel and Trenaunay in their original article listed dis-
through a capillary bed. There were 15 patients with lower crepancies of 2.4, 4, 4.5, and 9 cm in some of their patients
extremity length discrepancies described, 12 of whom had [253]. Peixinho et al. described eight patients with the KTW
reached skeletal maturity. Two of the patients had no syndrome treated for lower extremity length discrepancy
increase in the involved side length but the others all showed [278]. There were seven patients who had growth remaining
increase in length. The discrepancies varied from 0.5 to at time of treatment, ranging between 10 and 15 years of age,
7.0 cm. In the 13 patients with a lower extremity length and one patient treated at skeletal maturity at 19 years of age.
overgrowth, the mean discrepancy was 3.2 cm with 9 of the This report concentrates on the more severe variants but
13 showing a discrepancy of 2.5 cm or more. McKibbin and shows the extensiveness of the length discrepanices that can
Ray implicated abnormalities of venous return in experi- develop. The range of disorders treated was between 1.5 and
mental arteriovenous fistulae with bone overgrowth [274]. 10.0 cm with those undergoing epiphyseal arrest between
The direction of blood flow in the vein distal to the fistula 2.9 and 10.0 cm. In these six patients, the average discrep-
was reversed for a considerable difference. As the venous ancy was 6.35 cm. In the five patients with epiphyseal arrest
collateral channels, including those in the bone, developed followed to skeletal maturity, the mean discrepancy initially
the periphyseal blood supply was also altered. was 6.82 cm and at skeletal maturity it had decreased to a
mean of 2.1 cm. An additional patient had shortening of the
6.6.19.9 Cutis Marmorata Telangiectatica femur at skeletal maturity diminishing the discrepancy from
Congenita 4.8 to 0.8 cm. A better overview of the expected extent of
Cutis marmorata telangiectatica congenita is characterized length discrepancies in a large group of patients was pro-
by a persistent vascular mottling of the skin usually vided by Jacob et al. who did scanograms on 105 patients
involving the limbs and usually in an asymmetric pattern. finding a mean value of 1.75 cm (range 0.1–9.6 cm) [257].
The disorder was described initially by Van Lohuyzen [275] A review of 40 patients with the KTS syndrome from the
and a detailed review by Gelmetti et al. listed approximately Mayo Clinic documented equal male-female distribution
150 cases described in the literature. Spontaneous regression [279]. Thirty patients had findings noted immediately at
has been observed in the majority of patients in the first few birth with three being diagnosed before the age of 1 year and
years of life but many lesions persist to adulthood. The four between 1 and 6 years of age. A strict distinction was
disorder is also referred to as congenital phlebectasia. Lower made between the Parkes Weber syndrome and KTS with
extremity length discrepancies occur on occasion in the patients with arteriovenous fistulae not included in the KTS
disorder. As with many congenital vascular abnormalities, grouping. The lower extremity was involved in 38 patients
there is a high frequency of multiple associated congenital (95 %) and the upper extremity in 6 patients (15 %) with 4
abnormalities including neuroectodermal and mesodermal patients (10 %) bilateral. The disorder was unilateral in 34
defects. Gelmetti et al. pointed out that abnormalities of the patients (85 %), bilateral in 5 (12.5 %), and crossed bilateral
central nervous system, musculoskeletal system, and vas- in 1 (2.5 %). The affected extremity was longer in every
cular system were involved. Several instances of hemiatro- case. In 29 patients documented by scanogram, the average
phy or hemihypertrophy on the involved side have been difference in documented length was 2.39 cm with the lar-
described [276]. In the review referred to above, there were gest 12 cm. Hemangioma was present in 39 cases (97.5)
11 cases of shortness or hemiatrophy of the involved side while lymphangioma was the histologic diagnosis in 1 case
and 7 cases of hemihypertrophy of the involved side as well (2.5 %). The 2 together were found in 5 additional cases.
as other categorizations involving retardation of growth and The typical port wine flat cutaneous hemangioma was pre-
asymmetric growth where specific limb length determina- sent in 30 cases (75 %). Varicosities were marked and sig-
tions were not clear. Dutkowsky et al. described a nificant with incompetent perforators in 25 cases (62.5 %).
15-year-old male with 2.9 cm of shortness on the involved Seven of the 25 had persistent large embryonic veins on the
side and two other patients 2–3 years old with 1–1.6 cms lateral aspect of the thigh. Arteriography was performed
shortness on the involved side [277]. These studies indicate in nine cases with no arteriovenous fistulae diagnosed.
that some discrepancies in limb length could be present in as All except two of the patients were still under 10 years of
high as 25 % of cases, that both hemiatrophy and hemihy- age in terms of their assessment for length discrepancy.
pertrophy on the involved side could be seen, and that evi- Guidera et al. described 28 patients with limb overgrowth
dences of growth retardation are perhaps somewhat greater and the diagnosis either of KTW (18 patients) or Proteus (10
than those of growth stimulation. patients) syndromes [280]. The results were pooled. Most
668 6 Lower Extremity Length Discrepancies

patients had a large array of mesodermal abnormalities. All phlebectasia). Overgrowth (lengthening) and undergrowth
patients but one had extremity involvement. Twenty-seven (shortening) were assessed. The male: female ratio was 153
of 28 had lower extremity involvement consisting of overall (42 %): 208 (57 %); CVMs were venous 215 (60 %), arte-
limb hemihypertrophy in 15, localized gigantism in 6, and riovenous 43 (12 %), lymphatic 46 (13 %), and combined
macrodactyly in 10. Nine patients had lower extremity venolymphatic 57 (16 %). On the scanograms, only 26
length discrepancy varying from 1 to 12.8 cm. No specific patients (7.2 %) had a length discrepancy >2 cm with 20 due
analysis of the discrepancies was made. The authors warned to overgrowth and 6 due to undergrowth of the affected side.
of the need to be extremely cautious concerning surgical Statistical analysis showed that a whole leg CVM was the
intervention. Soft tissue debulking operations and vascular single most important risk factor for length discrepancy and
operations in particular often appeared of dubious benefit for overgrowth in particular. Overgrowth was significantly
especially with the danger of heavy bleeding. They felt that more common in females. Venolymphatic lesions were also
the timing of epiphyseal arrests was not predictable in significant for developing length discrepancy. Neither the
equalizing limb length discrepancies, although that does not depth of the CVM lesion nor joint involvement were a sig-
appear to be the experience of others. nificant factor for length discrepancy development.
Rogalski et al. utilized the term angiodysplastic lesions of
the extremities to review 41 patients [281]. They used this 6.6.19.11 Hemangiomas of Bone
terminology rather than the more common syndromal terms Hemangiomas of bone occur but are extremely rare. When
referred to above indicating that “identification of a specific present they almost invariably affect either the vertebral
syndrome appeared to depend on the speciality and training bodies or the skull. In those infrequent instances when they
of the treating physician and was not predictive of initial affect the long bones, they tend to be innocuous in terms of
symptoms or outcome.” They felt they could not categorize clinical significance. Cohen and Cashman reported one
the vascular malformations according to either histologic or instance where hemihypertrophy of a lower extremity was
angiographic descriptions and preferred to describe the associated with multifocal intra osseus hemangioma [284]. In
malformations based on the system of Goidanich and the one patient described involvement of both the right femur
Campanacci [252] based on size and location of the mal- and right tibia led to overgrowth on the right side at 12 years
formations. Eleven patients had limb length discrepancies of age but this was due to a combination of the right tibia
with hemihypertrophy and four of these had epiphyseal being 2.9 cm longer while the femur was 1.1 cm shorter. The
arrest. No specific details concerning the extent of the dis- hemangioma was present in both epiphyseal and metaphyseal
crepancies were given. The shape and size of the extremities, bone but the intervening growth plate was normal. The
and the associated vascular problems as well as the large authors noted that in the few previous cases of long bone
number of additional malformations throughout the body hemangioma described no growth changes were reported.
made the length discrepancy itself relatively less important
than in other individuals. Anatomic location and overall size
were predictive of symptomatology and were the more 6.6.20 Neurofibromatosis
important factors. They determined that the majority of the
lesions (59 %) were subcutaneous, while 20 % were Neurofibromatosis is associated with length discrepancies in
specifically identified as intramuscular. No bony lesions two types of clinical situations [285–288].
were identified. All limb length discrepancies were associ-
ated with overgrowth of the involved limb. The authors also 6.6.20.1 Bones Appear Structurally Normal
commented on the limitations of surgical interventions in The bones may be structurally intact but the soft tissues of
particular where cosmetic improvement was being sought. the affected limb are characterized by cafe au lait spots of the
Paley and Evans also felt that it was the depth and extent skin and/or subcutaneous neurofibromas. Growth stimula-
of the lesions that were the most significant prognostic tion was documented on the involved side in 17 patients who
characteristics [282]. Kim et al. provided an excellent review did not have a tibial pseudarthrosis [50]. Prior to physeal
assessing risk factors for leg length discrepancy in patients arrest, the average discrepancy was 4.40 cm (range 2.0–
with congenital vascular malformations [283]. They studied 8.8 cm). Shortening was also associated with neurofibro-
361 patients from 1994 to 2005 as part of assessments for matosis in the six patients in whom a pseudarthrosis
congenital vascular malformations affecting the lower occurred. The type I pattern was commonest, although type
extremities. Scanograms were used in 229 where discrep- II, type III, and type V patterns were seen also.
ancies were detected clinically. Risk factor analysis was
performed for clinically significant discrepancy > 2 cm in 6.6.20.2 Congenital Pseudarthrosis of the Tibia
relation to age, gender, features of CVM (type, extent, There may be a congenital pseudarthrosis of the tibia, which
depth), and deep vein status (agenesis, hypoplasia, is often seen with neurofibromatosis although cutaneous
6.6 Lower Extremity Length Discrepancies … 669

abnormalities or neurofibromas of the involved segment may There were two patients whose limbs where equal, eight
not be present. As surgical intervention in an attempt to where the difference was between 0 and 1 in.; five between 1
establish union was so frequent in the patients with pseu- and 2 in.; three between 2 and 3 in.; 4 between 3 and 4 in.;
darthrosis, the natural length discrepancy patterns in our one between 4 and 5 in.; and 3, 6 in. of discrepancy. The one
series were infrequently available for assessment. Some patient with tibial overgrowth was 2.0 cm longer.
information is available however on the maximum lower Morrissy et al. analyzed 40 cases of congenital pseu-
extremity length discrepancies reached from large series of darthrosis of the tibia. Half the patients in the series had
pseudarthrosis of the tibia described previously. Virtually all neurofibromatosis [287]. As in the study by Masserman et al.
of these patients will have had surgical interventions, often the diagnosis of associated neurofibromatosis does not affect
multiple times, on the affected tibia in efforts to obtain the overall result in comparison with the group without that
straightening and union. The discrepancies were at times diagnosis. The amount of shortening in congenital pseu-
worsened by angular deformity, the need to resect darthrosis of the tibia correlated extremely well with the
scarred-sclerotic diaphyseal tissue and by intramedullary rod eventual result achieved. This serves as a biologic reflection of
passage through the tibial epiphyses in efforts to enhance the extensiveness of the bone abnormality which affects not
stabilization. Van Nes pointed out, that a considerable only the diaphyseal regions but also the entire bone. In those
amount of the shortening in congenital pseudarthrosis of the with good results, the average amount of shortening was
tibia was due to associated developmental abnormalities of 1.4 cm (range 0–4.0 cm); with fair results, the average amount
the distal tibial epiphysis which often led not only to of shortening was 3.4 cm with one patient having a 6.0 cm
diminished growth but also to premature fusion [288]. The shortening and another a deficit at one time of 8.1 cm; and with
distal tibial secondary ossification center was often delayed poor results the average shortening was 5.5 cm (range 2–8 cm)
in appearance. Throughout the growing years, the physis can with all but one patient having at least 4.0 cm of shortness.
often be noted to be misshapen and thin. Van Nes stated that Amputation was done eventually in 14 patients. There was a
the retardation of growth in the distal tibial epiphysis indi- significantly greater amount of shortening in patients with
cated involvement with the same segmental dysplasia as the tenuous union or with nonunion. Much of the growth dis-
distal part of the diaphysis that caused the pseudarthrosis crepancy was secondary to failure of growth in the distal
originally. Both the pseudarthrosis and the retardation of physis while relatively normal growth proximally continued.
growth were to be considered symptoms of the same
developmental defect of the distal tibia. The closer the
pseudoarthrosis was to the epiphyseal region, the greater the 6.6.21 Juvenile Rheumatoid Arthritis
epiphyseal involvement and the less the associated growth.
Van Nes clearly pointed out that spontaneous physeal fusion Lower extremity length discrepancies occur commonly in
could occur as early as 10–12 years of age. He presented patients with juvenile rheumatoid arthritis where joint
case reports on 22 individuals. Length discrepancy mea- inflammation is prolonged and asymmetric. Asymmetric
surements were listed in 18 patients, virtually all after bone growth occurs in many instances of moderate to severe
10 years of age and prior to any epiphyseal arrest for the juvenile rheumatoid arthritis, characterized by overgrowth
length discrepancy. One patient had no discrepancy and the on the involved side early in childhood, and a tendency to
others ranged from 1.25 to 11.0 cm. Mean discrepancy in the premature physeal closure leading to shortening in those
18 patients was 5.4 cm. The discrepancies were due to a affected toward the end of skeletal growth [289–296]. Griffin
combination of factors including angular deformity, multiple et al. noted overgrowth as great as 2.6 cm in one patient with
surgical procedures some involving resection of bone in knee involvement and shortening as great as 3.8 cm and
efforts to obtain union, diminished function of the distal 5.1 cm in others [297].
tibial epiphysis due to its involvement in the dysplastic A retrospective study at the Children’s Hospital, Boston,
process and premature fusion of the distal tibial epiphysis. determined the course of limb length discrepancies occurring
On occasion, the physis was damaged by intramedullary in patients with monoarticular and pauciarticular juvenile
nails passed through it although many instances of continued rheumatoid arthritis [298]. Data were assessed on 36 patients
growth following this procedure were also noted. followed to skeletal maturity, (group I); on 15 patients who
In a long-term study from the Mayo Clinic, Masserman had not reached skeletal maturity but who had been followed
et al. reviewed 52 cases [286]. Of these, 20 (38 %) had a for 4 years or more, (group II); and on 49 patients followed
known diagnosis or the clinical manifestations of neurofi- for 3 years or less (group III). In 72 of the 100 patients, the
bromatosis. Lower extremity length discrepancies were onset of the disease occurred before they were 5 years old,
documented in 32 of the 52 patients. Of these, five had no and 90 patients had involvement of the knee. Of the 51
specific number mentioned but appeared to be insignificant. patients included in Groups I and II, while all had some
In all instances except one, the involved leg was shorter. length discrepancy, in 35 (70 %) a length discrepancy of
670 6 Lower Extremity Length Discrepancies

1.5 cm or more developed during the study period. length discrepancy occurs as a result of two factors: (1) a
Twenty-one patients had a discrepancy of between 2.0 and stimulation of the epiphyseal growth plates, predominantly
2.9 cm and in three it was 3.0 cm or more. about the knee joint, during the time the disease is active and
All patients in whom the disease developed before the age for some time afterward; and (2) inhibition of the growth
of 9 years had overgrowth of the involved extremity, but that potential of the involved extremity.
overgrowth never exceeded 3.0 cm. The major discrepancy The epiphyseal growth plates at the knee account for
developed within the first 3–4 years and either increased 70 % of the growth potential of the lower extremity. They
very slowly and thereafter, remained level, or decreased. Of are sufficiently close to the synovial capsule to be affected by
the 36 patients who were followed to skeletal maturity, in 29 the hyperemia that occurs during the inflammatory process,
a discrepancy of 1.5 cm or more developed at some time while not being adversely affected by the concomitant
during the period of assessment. Twelve of the 36 patients destructive process. Since the knee is commonly involved in
had diminution of the discrepancy to the extent that epi- the type of arthritis under discussion and so often is involved
physeal arrest was not required. Fifteen eventually had an unilaterally early in the course of the disease, a great
epiphyseal arrest. Rapid premature closure of the epiphyseal potential exists for growth stimulation of the involved lower
growth plate occurred only in those patients in whom the limb.
disease developed after the age of 9 years. This led to However, the severity of the disease often causes a
immediate shortening of the involved side and on occasion decrease in the use of the involved extremity, either because
to marked limb length discrepancies as much as 5.1 and of the patient’s symptoms or because of the treatment. Such
5.9 cm. decreased use can explain the gradual reduction in epiphy-
In the patients with unilateral disease who had the onset seal stimulation and inhibition of growth. When the patient
of their disease before the age of 9 years, the involved side has activity of the disease in early adolescence, consequent
was almost invariably the longer one (39 of 40 cases). Those hyperemia about the knee could cause rapid and premature
who had the onset of disease within the first 3 years of life fusion of one or both of the growth plates, with sudden
tended to have a discrepancy >1.5 cm (24 of 34 patients) decrease in growth of the involved lower limb.
relatively more often than children 3–8 years old, but The most common and well-defined pattern of develop-
overgrowth in the younger children never amounted to more ment of length discrepancy was ipsilateral lengthening in
than 3.0 cm. When the disease occurred initially after the patients who had the onset of the disease before the age of
patient was 9 years old (5 patients), the involved side usually 5 years. The major part of the discrepancy occurred within
became shorter (with one exception). the first few years of the onset of disease. Thirty-nine of the
Regardless of age at onset, the major discrepancy that 40 patients with unilateral involvement whose disease began
developed did so within the first 4 years after onset of the before the age of 9 showed this pattern. Early in the course
disease. Thereafter, in the Group I patients, the discrepancy of the disease, the predominant factor is stimulation of the
either increased very slowly (6 patients), remained unchan- epiphyses about the involved knee joint. The pattern of
ged (14 patients), or decreased spontaneously (12 patients). development of a limb length discrepancy just described
The other 4 patients, whose discrepancies continued to most often is followed by lack of a significant continuing
increase, were patients with late onset of the disease whose increase in the discrepancy. Although occasionally (in two
epiphyses fused prematurely on the involved side. Contin- patients in this series), the discrepancy will increase over the
uing involvement of the knee for several years in one patient ensuing years; in most patients, it will decrease or remain
resulted in a continuing increase with time until a pre-arrest unchanged with time.
discrepancy of 2.4 cm was reached, but such an occurrence It is important to note the rapid premature closure of the
was unusual. epiphyseal growth plates about the involved joint that
In the 12 patients whose discrepancies decreased, there occurred at the end of growth in four children in this series.
was a gradual inhibition of growth in the involved limb over All of them had onset of the disease after the age of 9 years,
several years. In 7 of the 12, the discrepancy became clini- and all showed shortening of the involved side. Their length
cally insignificant. The changes in discrepancy with time discrepancies ranged from 1.9 to 5.9 cm, with the two
were: 2.8–0.3, 2.5–1.2, 2.3–1.3, 2.2–0.4, 2.0–0.2, 1.8–1.4, patients who were 9–10 years old at onset of the disease
and 1.5–0.9 cm. Evidence for rapid premature epiphyseal showing larger discrepancies at skeletal maturity than the
growth plate closure was noted only in four patients who had other 2, who were 11–12 years old at onset. Early epiphyseal
discrepancies because of shortness on the involved side. growth-plate fusion has been recognized as a complication
In patients with the monoarticular or pauciarticular form of juvenile rheumatoid arthritis for many years.
of juvenile rheumatoid arthritis who have predominant In patients with monoarticular or pauciarticular juvenile
involvement of the major joints of one lower extremity, the rheumatoid arthritis, variable developmental patterns
6.6 Lower Extremity Length Discrepancies … 671

occurred. Types I, II, III, and V were all seen (Fig. 6.13b). 6.6.22 Thalassemia
The knee is the commonest area of involvement in juvenile
rheumatoid arthritis and its involvement is most likely to Premature fusion of the epiphyses is a fairly common
result in clinically significant discrepancy. The type I pattern occurrence in thalassemia of the homozygous or major type.
was seen most frequently in patients whose initial attack of In a series of 79 patients with the disorder, 14 % showed
rheumatoid arthritis occurred after the age of 9 years. In premature physeal fusion almost always most marked in one
these patients, a type I pattern with shortening on the growth plate [300]. All instances however were noted in
involved side developed due to the relatively rapid, prema- those >10 years of age. When that age group alone was
ture physeal fusion of the bones comprising the involved assessed, 23 % of 48 patients demonstrated the physeal
joint. The type II and type III patterns were seen most often arrest phenomenon. The commonest growth plate involved
in patients whose initial synovitis occurred in the first few was that of the proximal humerus with frequent occurrence
years of life and resulted in physeal stimulation and over- in the distal femur and also in the proximal and distal tibia
growth. Once the synovitis had resolved, physeal growth and fibula. Currarino and Erlandson noted that the premature
altered toward a more normal rate and the discrepancy either fusion was almost always focal and generally peripheral
persisted unchanged or increased at a much slower rate. The such that any shortening present was usually complicated by
type V pattern resulted from a slowing of physeal stimula- angular deformity. The growth plate arrest in the proximal
tion over a few years prior to plate closure (Fig. 6.14). humerus was almost always seen medially leading to varus
Whether the type V pattern was due to decreased use or to an tilt of the head in relation to the glenoid. The abnormality
alteration in the timing mechanism for closure due to dis- was not demonstrated in any of the 31 patients under
ease, or to both, is uncertain, but the phenomenon itself was 10 years of age. The incidence in males and females was
well documented. It was not possible to predict which approximately equal. There is very little documentation of
patients would have a type II, III, or V pattern. Similar the extent of the discrepancy or the nature of angular
overgrowth can occur following other inflammatory condi- deformity and little also with any indication that surgical
tions about the knee in childhood such as tuberculosis, septic intervention was performed. No definitive cause of the dis-
arthritis, and hemophilia. In what appears to represent a order has been described.
description of a type V pattern, Phemister quoted Bergmann
as observing “equalization of length years after overgrowth
produced by tuberculosis of the knee beginning in early 6.6.23 Hemophilia
childhood.” [299].
Lower extremity length discrepancies can occur in hemo-
philia [301]. The mechanism appears similar to that in
juvenile rheumatoid arthritis in that a recurrent synovitis in a
single joint, commonly the knee, leads to growth stimulation
in the early years up to approximately 8 years of age fol-
lowing which continuing synovitis tends to cause a prema-
ture closure of the distal femoral and to a lesser extent
proximal tibial physes. It is common for hemophilia to occur
in a recurrent fashion in one joint, which is referred to as a
target joint, with the three commonest affected regions being
the ankle, knee, and elbow. Caffey and Schlesinger descri-
bed overgrowth of the epiphyses themselves in all dimen-
sions in joints with hemophilic arthropathy but they did not
study overall bone length [302].
Kingma described overgrowth of an extremity affected
with recurrent hemarthrosis in one joint [303]. Three
patients, all <10 years of age, suffered repeat knee
Fig. 6.14 Type IV pattern in a patient with Legg–Perthes disease who hemarthrosis and after straightening of flexion contractures
maintained proximal femoral growth for several years after the initial were noted to be longer on the involved side. Each was still
insult but then prematurely fused the proximal femoral capital epiphysis
increasing the discrepancy (Reprinted with permission from Shapiro F,
growing and report of the final discrepancy was not made.
Developmental patterns in lower extremity length discrepancies. J Bone The overgrowth in an 11-year-old boy was 2.5 cm, in a
Joint Surg Am 1982;64A:639–651) 5-year-old boy 2 cm, and in a 7-year-old boy 2.5 cm.
672 6 Lower Extremity Length Discrepancies

Overgrowth in hemophilia was also described by Heim et al. and the average maximum combined lower extremity length
who reported a 2.2 cm overgrowth on the side of the discrepancy was 2.14 cm. Once bracing was discontinued,
involved knee at 5 years of age [304]. Length discrepancy is the tibial discrepancy decreased. Twenty-one patients
seen less frequently now because of improved medical demonstrated a type I pattern; 8, a type II; 52, a type III; 10,
control limiting hemarthrosis and synovitis. Overgrowth a type IV; and 49, a type V. When only the femoral lengths
discrepancies were often hidden and minimized by flexion were assessed, 14 patients (10 %) demonstrated a type IV
contractures and articular cartilage degeneration of involved pattern (Fig. 6.14). The occurrence of the type IV pattern
joints. was analogous to that seen in some patients with septic
arthritis of the hip with only mild destruction. Premature
fusion of the capital femoral epiphyseal growth plate
6.6.24 Synovial Hemangioma of the Knee Joint occurred, with a late alteration of the femoral head-greater
trochanter relationship. It is probable that the type IV pattern
Synovial hemangioma, a rare disorder, can occur with the would have been seen more often, but the performance of
most frequent site of involvement being the knee joint. distal femoral epiphyseal arrests in these patients made it
Moon performed a careful review of the literature and doc- difficult to document the type IV change. There was a good
umented 137 patients with synovial hemangioma of the knee correlation between the age of the patient at onset of the
[305]. There was equal occurrence in males and females disease and the final discrepancy pattern. The average age at
with initial occurrence of symptoms concentrated in the onset in the patients who showed the type I pattern was
childhood years from birth to late adolescence. Approxi- 8.7 years, for type II 6.5 years, for type IV 5.6 years, and for
mately, 75 % of patients were symptomatic prior to age 16. type V 5.3 years. These numbers reflect the better healing
In those reports which mentioned limb length, only 14 cases that occurs in younger patients with Legg–Perthes disease,
were described as having increased limb length on the who have a longer time available for the slow repair process.
involved side with many showing only about 1 cm differ-
ence, in 8 cases the limbs were of equal length, and in 4 the
involved limb was somewhat shorter. Limb length discrep- 6.6.26 Slipped Capital Femoral Epiphysis
ancies were thus variable although a slight majority of
patients had slight overgrowth. Clinically significant lower extremity length discrepancy is
relatively infrequent in slipped capital femoral epiphysis.
There are several reasons for this, the two most important
6.6.25 Legg–Calve–Perthes Disease being the relatively late age of occurrence in skeletal
development and the fact that only 30 % of femoral and
The extent of lower extremity length discrepancy in Legg– 15 % of lower extremity length is due to growth at the
Perthes disease was reported a large group of patients treated proximal femur. Even though treatment induces premature
with a unilateral abduction brace [306]. Both the disease and physeal fusion, there is insufficient growth remaining on the
the treatment used impact on length differentials. In 147 contralateral proximal femur to lead to clinically significant
patients with a lower extremity length discrepancy associ- discrepancy. Other limiting factors include cases with
ated with unilateral Legg–Calve–Perthes disease, the bilateral involvement and the slight discrepancy caused by
involved side was always shorter at some time during the cases with only mild-to-moderate slippage. Concern about
period of assessment. The average maximum femoral dis- discrepancy is increased with unilateral disorders in those
crepancy in this group of patients was 1.38 cm, none of 11 years of age or younger in particular with moderate or
whom had femoral or pelvic osteotomy. All five types of severe displacement. This profile is often seen in the sub-
discrepancy pattern were seen. Femoral shortening occurs group of slipped epiphysis associated with other medical
due to cessation of growth during the phase of necrosis of disorders.
the secondary ossification center, due to subchondral col-
lapse with the coxa plana deformity, and due to disuse in
association with therapy, and it has long been recognized as 6.6.27 Infantile Cortical Hyperostosis: Caffey’s
part of the disease entity [307–309]. If proximal femoral Disease
varus osteotomy is performed shortening will be increased
[310]. Innominate osteotomy tends to increase length on the In this disorder there is thickening of the periosteum and
operated side by 1 cm [308]. The femoral shortening was radiodensity of unknown etiology but the bone involvement
frequently associated with shortening of the ipsilateral tibia is selective and can lead to increased length on the involved
due to decreased use of the limb with unilateral brace ther- side [311]. In those instances where the tibia and fibula are
apy. The average maximum tibial discrepancy was 0.93 cm involved overgrowth has been described. Jackson and Lyne
6.6 Lower Extremity Length Discrepancies … 673

reported an unusual complication of Caffey’s disease, how- clearly occur with alterations involving the pericapsular
ever, which involved shortening on the involved side sec- structures and in those instances where the articular cartilage
ondary to direct involvement of the distal tibial and fibular is destroyed adjacent physeal cartilage would also have a
epiphyses [312]. Radiographs showed cortical hyperostosis high likelihood of destruction.
of the entire length of the tibia and fibula on one side, as well
as the left mandible. Synostoses eventually developed 6.6.28.2 Epiphyseal Growth Arrest Secondary
between the tibia and fibula proximally and distally. In to Freezing
addition the distal tibial epiphysis was wedge shaped. The Severe frostbite in childhood has been shown to lead to
fibula was shortened 1.1 cm relative to the opposite side and epiphyseal damage with premature growth arrest following.
the tibia was shortened 0.7 cm. The authors felt that it was The large majority of cases described involve the hands with
not the synostosis which caused the growth retardation but the phalangeal epiphyses most commonly affected. Initial
primarily the periosteal inflammation and scarring in the observation of this occurrence was made by Lohr who
epiphyseal-metaphyseal regions which affected the vessels to demonstrated physeal closure in several phalanges beginning
the epiphyseal plates. In this report, the patient was followed several months post exposure [315]. Other instances of
only to 4 years of age. phalangeal destruction due to freezing were reported by
Bennett and Blount in which there was complete destruction
of the epiphyses of the distal phalanges of the second to fifth
6.6.28 Limb Length Discrepancies Due digits, and the middle phalanges of digits 2, 3 and 4 [316].
to External Causes The physes in the less involved contralateral hand remained
open. Thelander also reported a case of unilateral frostbite in
6.6.28.1 Burns a 6.5-year-old boy who demonstrated premature physeal
Lower extremity length discrepancies can occur following closure at 9 years of age involving each of the epiphyses of
severe third-degree burns in particular about the lower part the distal and middle phalanges from the second to the fifth
of the leg and ankle joint. Four cases were reported by Frantz digits [317]. An extensive study by Bigelow and Ritchie
and Delgado [313]. The growth slowdown was considered to described 13 patients with frostbite of the hands during the
be due primarily to scarring in the periphyseal region which childhood years all of whom had loss of one or more epi-
could serve as a mechanical tether to growth and also limit physes [318]. The thumb was shown to be involved rarely
the vascularization of the physeal area. The possibility of and it was invariably the distal and then middle phalanges of
direct thermal damage to the epiphyseal plate was also the fingers which were affected first. In any instance where
raised. In two instances, there was premature closure of the the proximal phalangeal epiphysis was affected, those distal
distal tibial physis leading to a maximum discrepancy of to it were also affected. In most instances, shortening
4.6 cm in one case and 4.7 cm in another as measured prior occurred without angular deformity but in some instances
to contralateral epiphyseal arrest for correction of discrep- the growth arrest was partial and subsequent growth led to
ancy. In a third patient, the discrepancy prior to epiphyseal varus or valgus angulation. The epiphyses are damaged both
arrest was 2.0 cm although premature closure of the distal by the direct effect of the freezing and then by vascular
tibial plate did not develop and it was felt that the peri- changes secondary to frostbite including thrombosis.
physeal scar formation was limiting the growth. In a final
patient, a discrepancy of 1.0 cm was noted with early central
sclerosis of the distal tibial physis and the suspicion that 6.7 Projection of Limb Length
growth would worsen with time since there were still several Discrepancies by the Time
years of growth remaining. Skeletal Maturity is Reached
Evans and Smith studied several cases of burn patients,
many in the childhood years, and described bone and joint The projection of limb length discrepancies that would be
changes [314]. In spite of the fact that many children were present at skeletal maturity became of practical importance
assessed no specific comment on physeal changes was made. with the demonstration by Phemister that surgical arrest of
They defined skeletal alterations as falling into three groups: an epiphyseal growth plate on the longer limb with growth
(1) alterations limited to bone with osteoporosis and peri- remaining allowed limb length equalization as the shorter
osteal new bone formation; (2) alterations involving peri- limb continued to grow [299]. Accurate timing of an epi-
capsular structures such as pericapsular calcification, physeal arrest procedure became feasible as documentation
osteophytes and heterotopic para-articular ossification; and of the percentage of growth at each long bone epiphysis and
(3) alterations involving the joint with progressive articular the normal range of femoral and tibial bone lengths was
destruction and joint fusion. Tethering of the physis would obtained.
674 6 Lower Extremity Length Discrepancies

6.7.1 Percentages of Growth at Each End presented a detailed article in 1909 studying “about a
of Major Long Bones thousand” radiographs throughout the growth period [326].
He came “to the conclusion that in the process of develop-
Digby determined percentages of growth in human bones by ment from birth to adolescence the normal changes which
measuring from the nutrient canal to either end taking the take place in the bones of the wrist correspond so closely to
nutrient canal position as a standard and unchanging marker those of other joints that in the great majority of individuals
[319]. He documented proximal femoral growth as con- the wrist may be accepted as a fairly correct index of general
tributing 31.2 % of entire length and distal growth 68.9 %. development.” He then chose about 200 children of all ages
Other measurements included: proximal tibia 57.1 %, distal with normal development of the joints and epiphyses who
tibia 42.9 %, proximal humerus 80.8 %, distal humerus underwent radiographic assessment. The radiographic stud-
19.2 %, proximal radius 25 %, distal radius 75 %, proximal ies were accompanied by listings of chronologic age, sex,
ulna 18.6 %, and distal ulna 81.4 %. Bisgard and Bisgard weight, height, and tooth development. The development of
performed similar studies in goats and showed remarkable the ossific centers of the carpal bones and the lower epi-
similarity of growth patterns [320]. Using the nutrient canal physes of the radius and ulna served as the milestones. He
method their findings for the proximal femur were 32.7 %, referred to the findings as the anatomic age and related them
distal femur 67.3 %, proximal tibia 56 %, distal tibia 44 %, to the chronologic age and other criteria. He developed an
proximal humerus 81.7 %, distal humerus 18.3 %, proximal alphabetical classification A to M which corresponded to
radius 25.5 %, distal radius 74.5 %, proximal ulna 19.6 %, increasing appearance of ossification of the particular carti-
and distal ulna 84.4 %. The rounding off of numbers widely laginous bones and regions, their increased size, and their
accepted for clinical use is proximal femur 30 % of growth, development toward mature shapes. While noting the vary-
distal femur 70 %, proximal tibia 55 %, distal tibia 45 %, ing times of appearance of bone of the metacarpal epiphyses
proximal humerus 80 %, distal humerus 20 %, proximal and the epiphyses of all the phalanges he felt that they were
radius 25 %, distal radius 75 %, proximal ulna 20 %, and “somewhat illusive as factors of development” and that “the
distal ulna 80 %. In terms of overall lower extremity growth, most practical and reliable signs for the different periods of
general percentages are upper femoral epiphysis 15 %, lower developmental age… were a combination of the carpal
femoral epiphysis 35 %, upper tibial epiphysis 30 %, and bones and the lower epiphyses of the radius and ulna.” It was
lower tibial epiphysis 20 %. Two major studies in the quickly recognized that girls developed ossific centers earlier
American literature have provided data which remains than boys. He presented wrist and hand radiographs showing
valuable in terms of overall lengths of femurs and tibias progressive stages of development from A to M which
throughout the childhood years. These are the reports by formed his preliminary anatomic index. Even at this period
Maresh in 1955 [321] and 1970 [322], and Anderson, Green of time (1909) he cautioned that “we should, as a rule, use
and Messner in 1964 [323]. Beumer et al. updated the chronological age as little as possible in solving the prob-
femoral–tibial growh data studying 182 Dutch children by lems of early life.” He felt that “anatomic age as gauged by
serial radiographic studies (orthoradiographs) from 1979 to the epiphyses always means the same conditions, (while)
1994 [324]. They performed 596 measurements (mean chronologic age is very variable.” Height and weight were
3.3/child, range 2–14) and established straight line growth also unreliable indicators of the status of bone development.
curves for girls and boys, similar but not identical to those His rough outline of anatomic age with the approximate
outlined by Moseley. Dimeglio and Bonnel have broken correspondence of chronologic age was: A (1st year), B (2nd
down the growth data to indicate the mean amounts of to 3rd), C(2nd to 3rd), D (2nd to 3rd), E (3rd to 4th), F (5th
growth remaining in males and females at the distal femur to 6th), G (6th), H (6th to 7th), I (6th to 7th), J (7th to 8th), K
and proximal tibia at varying ages [325]. (9th to 11th), L (11th to 12th), and M (12th to 14th). He
concluded that the best way of using the wrist as an anatomic
index was to classify the carpal bones and the lower radial
6.7.2 Systems for Assessing Skeletal and ulnar epiphyses alphabetically in groups. Serial radio-
Development and Maturation graphic studies of developing regions were done extensively
in the first half of the twentieth century. Acheson refers to at
With the discovery of radiography in the first decade of the least nine detailed published radiographic studies between
twentieth century there was almost immediate recognition of 1902 and 1952, including those of Todd and Greulich, and
the value of childhood radiographs in demonstrating pro- Pyle from Cleveland, Ohio. The atlases of skeletal devel-
gressive bone ossification at each joint and body region and opment of the wrist and hand by Todd (1937) [51] and
the application of this information to normal and abnormal Greulich and Pyle (1950, 1959) [52], were based on radio-
development. Rotch of Children’s Hospital, Boston logic studies done at Western Reserve in Cleveland, Ohio
6.7 Projection of Limb Length Discrepancies … 675

under the auspices of the Brush Foundation. Atlases of 6.7.3.1 Hatcher

regional skeletal development were also published by this Hatcher developed the initial approach for timing epiphyseal
group on the knee [327] and foot and ankle [328]. arrest procedures at the femur and proximal tibia [331].
Acheson outlined the strengths and weaknesses of Although his figures were based on averages at chronolog-
determining skeletal maturity from radiographs [329]. He ical age periods, they provided the first quantitative infor-
referred to use of the atlases alone as the “inspectional mation on expected growth increments. Hatcher compiled
technique.” To improve analyses he devised the Oxford data from studies which included percentages of growth of
Method for grading skeletal development [330]. This was the bones of the lower extremities from the proximal and
essentially a semi-quantitative system giving a point for each distal epiphyses as follows: femur 23 % proximal and 77 %
successive distinct shape change in a particular bone. The distal, tibia 56 % proximal and 44 % distal. Growth per-
sum total of points (or units) scored by a bone at any stage in centage contributions of each of the 4 major lower extremity
development would be an exact measure of maturity. The epiphyses to overall lower extremity length were then
technique could be applied to any bone or region of the body determined as proximal femur 12 %, distal femur 40 %,
once the maturity pattern was established for those proximal tibia 20 %, and distal tibia 21 %. From available
bones/regions being assessed. Advantages were: every round growth tables, Hatcher was able to calculate and chart the
bone and epiphysis could be graded, small increases of averages of normal growth to be expected from the femur
maturity were recorded, the same standards applied to both and tibia for males and females from age 4 to age 17 years.
sexes, and maturation was quantitated by the units/points Although the length numbers were averages, they provided
referred to as Oxford maturity units. Acheson demonstrated the initial information in terms of final projected length of
the method for the first 5 years of life on the left wrist and the femur and tibia and the average increment per year.
hand using a posteroanterior film and the left knee on an Hatcher’s growth increment curve was published in the first
anteroposterior film [329]. For the wrist and hand, the edition of Campbell’s Operative Orthopaedics in 1939 [331].
scoring system was as follows: distal radius (3 units), distal With the three above-mentioned pieces of information, one
ulna (2), each of eight carpal bones (3), metacarpal [1 for could make a relatively accurate estimation of the appro-
each epiphysis (secondary ossification center)], and pha- priate timing for any epiphyseal arrest.
langes (1 for each epiphysis). The maturity development
score for the knee was also outlined: distal femur (1–5 units), 6.7.3.2 White
proximal tibia (1–5 units), fibula [1 (presence of epiphysis)], White timed all epiphyseal arrests on the basis that the distal
and patella (1). For each bone/region the skeletal maturity in end of the femur grew 9.5 mm (3/8 of an inch) per year and
units was plotted along one axis and the age in years along the proximal ends of the tibia and fibula grew 6.4 mm
the other to provide the developmental curve. (1/4 in.) per year [99]. White and Warner described their
Acheson also applied the Oxford method of assessing technique for epiphyseal arrest by slightly modifying
skeletal maturity to hip and pelvis of over 500 boys and girls Phemister’s approach [332]. They credited him, however,
assessing the proximal femur-head 0 → 8, greater trochanter with the original idea of epiphyseal arrest and confirmed its
0 → 7, and lesser trochanter 0 → 5; and pelvis-ilium 0 → 7, great value in treating lower extremity length discrepancies.
ischium 0 → 8, acetabular lip 0 → 1, junction of ischial and They used a square chisel which, at the distal femur, was
pubic rami 0 → 4, pubis 0 → 2, and triradiate cartilage placed in the midline medially and laterally. The square
0 → 3 [330]. chisel was to straddle the growth plate diagonally so that two
of the diagonal points were forced into the epiphyseal line
and two into bone on either side. The chisel was driven into
6.7.3 Systems for Projecting Limb Length the femoral condyle to a depth of approximately 3/4 of an
Discrepancy at Skeletal inch and the square of bone and interposed physeal cartilage
Maturation tissue was removed in one piece. The metaphysis and physis
were further damaged by a curette and the plug of bone and
Many systems for predicting limb length discrepancy at time cartilage removed by the chisel was rotated 90° and replaced
of skeletal maturity have been outlined. Accurate knowledge in the femur so that the contained portion of the epiphyseal
of the amount of growth remaining in femoral and tibial line was directed in the long axis of the femur and solid bone
physes at any particular age became of practical importance bridged across the physis. A light plaster cast was used to
after Phemister demonstrated the relative ease and value of immobilize the leg for 2 weeks after which physiotherapy
arresting physeal growth in the longer bone [299]. was started with weight bearing allowed in 4 weeks. They
676 6 Lower Extremity Length Discrepancies

reported successful transphyseal bone bridging bilaterally increased slightly. Even in those cases where no limb length
with no deformities produced in 30 cases. equalization occurred there was at least stabilization of any
White and Stubbins reported on additional cases a few progressive discrepancy.
years later, again stressing the relative ease and effectiveness
of the square chisel approach to epiphyseal arrest [333]. As 6.7.3.4 Gill and Abbott
the operation came to be more widely used they stressed Gill and Abbott improved the accuracy of growth projec-
(i) the importance of recording lower extremity length dis- tions greatly by using percentile height tables and determi-
crepancies more accurately than by tape measurements and nations of skeletal age [334]. Their method took the
(ii) use of a simple method of calculation to project the individual’s relative growth and maturation rate into con-
appropriate timing for the intervention. In a short period of sideration rather than using average values, as had been done
time, the epiphyseal arrest dramatically limited use of the previously, although the limb lengths were based on per-
more involved femoral shortening procedure. They felt that centage determinations from data for total body height. Gill
the bone block removed by Phemister did not [299], in many and Abbott pointed out five major criticisms in relation to
instances, include sufficient depth of bone and that the the methods used by Phemister, Hatcher, White and col-
technique they had developed favored solid bony union leagues, and Wilson and Thompson. Their work began the
since there was more extensive bone surface opposed. era of accumulation and use of more accurate growth data.
Phemister had mentioned chiseling out the epiphyseal plate They pointed out that: average figures for the length of legs
to a depth of 1 cm anterior and posterior to the block of bone should not be applied because of the wide variations in the
and cartilage removed but apparently some surgeons were final length of individual children; leg lengths determined as
somewhat lax in doing this. The square mortising chisel was proportions of overall height were far less reliable than
0.5 in. in size. With 90° rotation of the square bone plug, the radiographic measurements that they were beginning to use;
contained epiphyseal cartilage was at right angles to the no allowance had been made previously for variations in
persisting physis and on either side of it bone tissue crossed sexual and skeletal development in children of the same age;
the physis. With healing metaphyseal bone became contin- use of parents’ measurements was not reliable in determin-
uous with epiphyseal bone. White and colleagues began ing future growth of the child; and the numbers used by
using standardized radiographic projections to document Digby presupposed that the femur and tibia grew at the same
length discrepancy using a standard tube distance. A formula relative rate during the entire period of growth. Many
for calculating growth remaining in the femur and tibia was studies, even then, showed this latter point to be untrue with
also established. Regardless of the age and size of the child, some data showing that the tibia obtained its final growth
a growth arrest operation at the distal femoral epiphysis before the femur. Their method was based on three major
would retard growth at the rate of 3/8 of an inch a year, principles: (i) the final stature of a child could be predicted
while at the proximal end of the tibia and fibula it would by use of the percentile method; (ii) the accuracy of growth
retard growth by ¼ in. a year. They accumulated a series of projection would be increased if the bone maturation age of
202 separate growth arrests; of these 57 % were for the child was considered; and (iii) the relative proportions of
poliomyelitis and 11.5 % for osteomyelitis. the length of the femur and the tibia to overall stature were
maintained with only small variations throughout the ado-
6.7.3.3 Wilson and Thompson lescent period. Among the principles of general and limb
Wilson and Thompson also derived a guide for timing epi- development that they utilized were the relationship of
physeal arrest based on the expected amount of growth from overall patient height to the age and percentile, utilization of
each of the epiphyses [121]. Several years of documented separate tables for males and females and assessment of
lower extremity lengths were needed for each individual. If a skeletal maturation using Todd’s Atlas of Skeletal Matura-
discrepancy had not been increasing with growth, the present tion. They began to utilize skeletal maturation ages for
discrepancy would equal the expected discrepancy. If the growth calculations if skeletal development was >6 months
rate of growth in the longer leg had been greater than in the advanced or retarded from the chronologic age. Length data
shorter extremity a discrepancy would continue to increase were then placed in a specific percentile range to allow final
proportionately. They calculated the expected discrepancy, length to be determined relatively early. Femoral and tibial
related it to the expected growth in the longer extremity, and lengths were quantitated radiographically using either tele-
chose the appropriate physis for ablation. Fourteen patients oroentenograms or scanograms. The expected final lengths
averaging 12.5 years of age at time of surgery had been of each bone were calculated. By subtracting the present
observed from 1 to 4 years postsurgery. Seven of these length of the normal femur from its expected final length, the
showed a lessening in discrepancy of 1 to 1.75 in.; in 3 the future expected growth of the bone was obtained. This was
decrease was 0.5 to 1 in.; in 2 the difference between the done separately for femur and tibia. It was then possible to
2 legs remained the same; while in 2 the discrepancy determine growth from the distal part of the femur which
6.7 Projection of Limb Length Discrepancies … 677

would be 70 % of expected femoral growth. Similar School of Public Health who were performing a longitudinal
approaches were used for the proximal tibia: the expected series of child health and development studies from 1930 to
proximal tibial growth was 55 % of the expected tibial 1956 [336]. The patients assessed involved 134 healthy
growth. They calculated expected final lengths of femur and children, 67 boys and 67 girls, who were followed from birth
tibia by relating them to projected adult stature since num- to 18 years of age. Assessments began in 1930 when the first
bers with appropriate percentiles were available for overall mother was enrolled in the study and continued until 1956
stature but not at that time for femoral and tibial lengths. when the last child was discharged from assessment at
Direct femoral and tibial length measurements were even- 18 years of age. Part of the study involved a completely
tually determined by Maresh [321, 322] and by Anderson longitudinal series of radiographs of the lower extremities in
et al.[323]. The relationship of the femur and tibia to overall the 67 boys and 67 girls who had annual lower extremity
stature, however, was felt to be stable with growth. Based on radiographs as part of the observations in the comprehensive
a femoral radiograph and total body height measurement, longitudinal study program. Very early in the program the
they were able to determine the femoral percentage of stature standardized lower extremity radiographs utilized the
which, for example, was frequently 28 %. They then orthoroentographic technique which ensured a high degree
determined the patient’s final overall stature from the charts of accuracy with each assessment. Radiographs were per-
and calculated the final femoral length as being 28 % of that. formed once yearly. In 1964 Anderson, Messner, and Green
The remaining expected growth of the femur and tibia could published their normal femoral and tibial growth charts
also be calculated as could the growth at proximal and distal [323]. Separate assessments were made for the femur and the
ends of each of the major long bones. The accuracy of their tibia in both boys and girls. Values at each year included the
predictions was dependent on the exactness with which mean length of the bone including the epiphyses and pub-
height could be predicted by the percentile method. The lication of growth charts in which the mean value was
method was felt to be valuable since any given child tended indicated as well as those values 1 and 2 standard deviations
to maintain his or her rank in stature from one age to another. above and below the mean. Values were listed from 1 year
Much data was also presented to indicate that the relative of age to 18 years of age. These are printed as Fig. 6.15a, b.
proportions of femoral and tibial length to overall stature The socioeconomic status and national origin of the
were maintained throughout growth with only small age patients in this study were defined by Stuart and Reed [336].
variations. The percentage of growth from each end of the Enrollment was limited to white males and females of pre-
bone could then be calculated based on the proximal femur dominantly North European stock and all but 48 of the 592
30 %, distal femur 70 %, proximal tibia 55 %, and distal parents of the children initially enrolled were born in the
tibia 45 % numbers. The growth remaining could be further United States, with most of the others born in Ireland and
calculated. Gill and Abbott showed the reasonably high brought to the United States early in life. The vast majority
accuracy with which they could estimate expected growth of of children therefore were born in North America and family
the normal limb. The next problem in terms of treatment of origin was from North America, the British Isles, or
lower extremity length discrepancies involved estimates of Northern European countries. The predominant national
the expected growth of the abnormal limb. In experimental origin of the families enrolled was Irish. The patient popu-
efforts to determine how much growth was occurring in lation was drawn from the clinics at the Children’s Hospital
physes which were open but growing at a slower than nor- such that it tended to exclude those of high economic status
mal rate, for example with poliomyelitis, Gill and Abbott and the indigent. Additional growth data generated by the
inserted small metallic markers in the affected bones through study allowed for charts to be constructed indicating
a special hypodermic needle, although this method could not recumbent and erect overall height in boys and in girls.
be used widely in a clinical setting. Recumbent height was measured between 1 and 6 years of
age and standing height between 6 and 18 years of age.
6.7.3.5 Green and Anderson Charts incorporating the data outlined the mean values and
Shortly after Phemister demonstrated the value of epiphyseal also those 1 and 2 standard deviations above and below the
arrest in the longer extremity to allow for relatively mean. Sitting height charts were also produced for boys and
straightforward correction of lower extremity length dis- girls. In a separate publication, Anderson, Green, and
crepancies during the final years of skeletal growth, it Messner derived charts indicating the growth remaining in
became evident that more accurate growth data would the normal distal femur and proximal tibia observed in a
greatly enhance the timing and thus the accuracy of the longitudinal series following given skeletal ages [335]. The
procedure. Green, Anderson and Messner of the Children’s “growth remaining” data were derived from 100 children, 50
Hospital, Boston played a major role in developing femoral girls and 50 boys, measured at least once a year over the
and tibial growth data [323, 335]. They worked in con- 8 years before growth terminated in their lower extremities.
junction with Stuart and Reed and associates at the Harvard Of this number, 51 children were normal (25 girls and 26
678 6 Lower Extremity Length Discrepancies

Fig. 6.15 (continued)

6.7 Projection of Limb Length Discrepancies … 679

Fig. 6.15 (continued)

 680 6 Lower Extremity Length Discrepancies

boys) and 49 children (25 girls and 24 boys) had In a study of 44 patients having had 53 epiphyseal arrests
poliomyelitis which affected only 1 lower extremity with the who were followed to skeletal maturity, Menelaus indicated
opposite normal extremity used for data accumulation. The that 52 % were within 1/4 in. of calculated discrepancy and
maturity of each child was evaluated from the radiographic an additional 41 % were within 3/4 of an inch [338]. Those
appearance of the bones in the hand and wrist with the with more than 3/4 of an inch error were only 7 % of the
skeletal ages being read from Greulich and Pyle Atlas. The operative cases. He felt that this approach compared quite
normal children were part of the longitudinal study from the favorably with the use of skeletal age as proposed by Green
Harvard School of Public Health whose radiographic studies and Anderson. In their 1957 report of patients with
were also used to derive the normal femoral and tibial length poliomyelitis, 89 % were within 0.5 in. of calculated dis-
charts. The normal and poliomyelitis groups were analyzed crepancy while in Menelaus’ series those with poliomyelitis
separately before the data were combined. No statistically showed an 85 % effectiveness within 0.5 in. [338].
significant differences were found between the two at any In a later study of 94 patients described by Westh and
age either in the patterns of maturation or in the average Menelaus, 85.1 % were within 0.5 in. of that calculated and
amounts of growth that occurred after specific skeletal ages. 94.1 % of those had final discrepancy within 3/4 of an inch
The growth remaining charts indicate values for the distal of that calculated [339]. The values are expressed in that
femur and proximal tibia in boys and girls with data in the fashion since equalization was not specifically sought in
girls beginning at 8 years of age and in the boys at 10 years many of the patients who had poliomyelitis since the
of age. Values are listed as the mean amounts plus those in weakened limb was often deliberately left somewhat shorter
the first and second standard deviations above and below the than the stronger.
mean (Fig. 6.15c). Annual increments in lengths of femur
and tibia in males and females are illustrated compared to 6.7.3.7 Moseley
total height above (Fig. 6.15d). The Growth Study unit at the Moseley, using the Anderson et al. data, developed a
Children’s Hospital, Boston was founded by Green in 1940 straight-line graph for leg length discrepancies by converting
at which time the major cause of lower extremity length the normal growth curve using logarithmic methods into a
discrepancies was poliomyelitis. The unit was initiated with straight line (Fig. 6.16a, bi, ii) [340, 341]. Moseley based his
support from the National Foundation for Infantile Paralysis. straightline graph method on 2 concepts not previously used
Details relating to the use of the Green-Anderson method for in growth projections. The first was that the growth of the
projecting timing of epiphyseal arrest will be described legs could be represented on a graph by straight lines and
below and in the following Sects. 6.6, 6.7 and 6.8. that a nomogram relating leg length to skeletal age could
In a study of 125 epiphyseal arrest procedures in their provide a mechanism for taking the child’s growth percentile
unit followed to skeletal maturity correction to within 1.2 cm into account in predicting at what lengths the growth of the
(½ in.) of prediction occurred in 89 % of cases [337]. legs will stop. The first concept simply involved converting
the exponential curve of normal growth to a straight line by
6.7.3.6 Menelaus plotting the data against a logarithmic scale. The Anderson,
Menelaus developed a simplified approach for projecting Messner and Green data were used to construct the chart.
eventual length discrepancies based on chronological rather Moseley pointed out several of the important consequences
than skeletal age [338]. It was assumed that the lower of this way of depicting the growth data: (i) the growth of the
femoral epiphysis provides 3/8 of an inch and the upper short leg was also represented by a straight line which was
tibial epiphysis 1/4 of an inch of growth each year. It was positioned below that of the longer leg and tended to have a
further assumed that growth of these epiphyses stops at the different slope; (ii) the leg length discrepancy is represented
chronological age of 16 years in boys and 14 years in girls. by the vertical distance between the two lines; (iii) the per-
Skeletal age is estimated but primarily to allow for a cal- centage inhibition of growth of the short leg is represented
culation of the predicted adult height. Epiphyseal arrest is by the difference in slope of the two growth lines designating
then resorted to only if this adult height will be acceptable. the slope of the normal leg as 100 %; (iv) the growth of the
In addition, a marked difference between skeletal and leg that has undergone surgical lengthening thereafter fol-
chronological ages indicates that any calculation for the lows a straight line of the same slope which is displaced
timing of the epiphyseal arrest is likely to be inaccurate. upward on the graph by an amount equal to the lengthening

b Fig. 6.15 Femoral and tibial length charts presented by Anderson, remaining in relation to skeletal age for boys and girls is shown.
Messner and Green. a Femoral and tibial length chart for girls is shown. d Annual increments in length of femur and tibia in males and females
b Femoral and tibial length chart for boys is shown. c The growth are shown below compared to total height above
remaining chart documenting distal femoral and proximal tibial growth
6.7 Projection of Limb Length Discrepancies … 681

achieved; and (v) the length of a leg that has undergone in girls from 5 to 14 years of age, and for boys from 6 to
epiphyseal arrest will follow a straight line of decreased 16 years of age (Fig. 6.16c).
slope where the decrease in slope exactly equals the per-
centage contribution that the fused growth plate would 6.7.3.10 Eastwood and Cole
otherwise have made to the total growth of the extremity. Eastwood and Cole described a clinical method for the gra-
Since the contributions of the proximal tibial and distal phic recording, analysis and planning of lower extremity
femoral growth plates are 28 and 37 %, respectively, of the length discrepancies during the growth years [343]. Their
total growth of the leg it is possible to predict the amount of chart lists length discrepancy in cm along one axis and
inhibition to be introduced by an epiphyseal arrest. The chronological age in years along the other (Fig. 6.17). The
Moseley approach was widely adopted. average maturity lines were marked for girls at 14 years and
for boys at 16 years. Superimposed on the graphs are epi-
6.7.3.8 Beumer et al physiodesis reference slopes (slopes 1–3) which converge to
Beumer et al. performed radiographic length documentation the skeletal maturity lines at zero leg length discrepancy. The
of femurs and tibias in 182 Dutch children to address con- slopes of these lines are based on the average annual growth of
cerns that different groups than those assessed in North 1.0 cm from the distal femoral growth plate and 0.6 cm from
America might have different growth results and that chil- the proximal tibial growth plate after the age of 8 years in girls
dren now grow taller than those of 50 years ago due to and 10 years in boys. These approximate values (3/8 and ¼ in.
improved socioeconomic trends [324]. They then used the respectively) were also used by White and Stubbins, Ander-
data to construct a straight line graph, similar but not iden- son et al. and Westh and Menelaus. The graphs depict the
tical to the way Moselely worked with length data. They estimated mature discrepancy and timing of surgery. East-
developed the Rotterdam Straight Line Graph (R-LSG), wood and Cole indicate that the pattern of differential growth
referring to the Moseley graph as the M-SLG method. At of the legs is determined from the graph such that the devel-
most ages, Dutch children had longer femurs and tibias than opmental patterns defined by Shapiro are documented and
in the data of Anderson et al. although the differences appear then used to predict the pattern of further differential growth
so slight that growth projection should not differ in clinically and eventual leg length discrepancy projected for skeletal
significant ways, especially since in any individual patient maturity. The observed discrepancy line (line 2) is projected
right and left legs are compared. to the skeletal maturity line (line 3). The point of intersection
(Y) gives the estimated mature discrepancy. The mature dis-
6.7.3.9 Hechard and Carlioz crepancy line (line 4) is drawn horizontally from point Y and
Hechard and Carlioz also developed a growth chart for the may intersect one or more of the epiphysiodesis references
long bones based on the data of Anderson, Green and slopes. The slopes are for proximal tibial arrest alone, distal
Messner (Fig. 6.16c) [342]. The limb lengths from 15 cm to femoral arrest alone, or a combination of the 2. Vertical lines
54 cm were listed on a single chart as were the bone ages for are dropped from these points of intersection to give the
both females and males. Since, in the vast majority of chronological ages for epiphysiodesis of the appropriate
instances, normal growth persisted along its same percentile growth plates (X and X1) (Fig. 6.17). This method incorpo-
projections were readily made at a glance. In addition by rates the different patterns of discrepancy into the plotting of
plotting the length of the abnormal extremity with time its the appropriate time for surgery without the need for specific
pattern of change in relation to the normal side could be calculation of the growth inhibition rate as is done in the
viewed easily and projections readily made. The method for Green-Anderson method.
projecting the inequality in length provided a document
which could follow the growth evolution at a glance. Before 6.7.3.11 Paley et al.
4 or 5 years of age the rate of growth per year increased from Paley et al. developed the multiplier method for predicting
year to year; between 4 and 13 years for girls and 5 and limb length discrepancy at skeletal maturity [344]. It is
14 years for boys the rate of growth annually was constant; applicable to congenital or developmental limb length dis-
while toward the approach of skeletal maturity the rate of crepancy where the short limb grows at a rate that is always
growth diminished in a regular fashion. It was evident proportional to the normal long limb. These disorders are the
therefore that the majority of children examined for a lower most frequent causes of limb length discrepancies and also
extremity length discrepancy were seen during the period of lead to the most severe or extensive deformities. The method
linear increase in growth. By eliminating the extremes of age is thus applicable to the type I developmental length dis-
at either end, a linear depiction of growth was felt to be crepancy pattern. The multipliers are derived from the
acceptable in terms of accuracy for the clinical situation. The Anderson et al. data for femoral and tibial growth from 1 to
values for bone ages and femoral–tibial lengths were listed 18 years of age and from the Marsh data from birth to
682 6 Lower Extremity Length Discrepancies

Fig. 6.16 (continued)

 6.7 Projection of Limb Length Discrepancies … 683

skeletal maturity. Data in both studies was obtained by 6.7.4 Discussion of Methods Used
standardized radiographs. Using these databases, they divi-
ded the femoral and tibial lengths at skeletal maturity by the The previous Sect. (6.7.3–6.7.3.10) have shown the evolu-
femoral and tibial lengths at each age for each of the five tion of approaches to determining the expected discrepancy
percentile groups in boys and girls. The resultant number is at skeletal maturity and the appropriate time for epiphyseal
the multiplier. The multipliers for the femur and tibia are arrest to allow for diminution of the discrepancy during the
equivalent in all percentile groups, varying only by age and remaining years of growth either to make both limbs equal at
gender. Because congenital limb length discrepancy increa- skeletal maturity or, as may be the case in certain instances
ses at a rate proportional to growth the discrepancy at of weakness of the short limb, to minimize but not com-
maturity can be calculated as the current discrepancy times pletely eliminate the discrepancy at skeletal maturity. The
the multiplier for age and gender. One measurement alone most detailed conceptual advance and practical work was
can allow for an accurate projection of eventual discrepancy. done by Gill and Abbott who developed the concepts needed
The percentile groups are the mean, mean plus one standard for accurate growth determination [334]. The studies of
deviation, mean plus two standard deviations, mean minus Green and Anderson and colleagues provided the data nee-
one standard deviation, and mean minus two standard ded to construct the appropriate growth charts for femoral
deviations. The values thus correspond (from lowest to and tibial lengths, from which the growth remaining charts
highest) to the 5th (−2.5D), 33rd (−15D), 50th (mean), 67th were also derived [323, 335]. Both Anderson et al. and
(+15D), and 95th (+25D) percentile. Menelaus derived formulae to aid in determination of the
Paley et al. also obtained femoral and tibial growth data appropriate time for epiphyseal arrest. Green and Anderson
and calculated age and gender-related multipliers from based their determinations on skeletal age while Menelaus
additional databases. Overall they assessed 20 databases, 11 used chronologic age for most patients. Green and Anderson
based on radiographic or clinical measurements of living were able to derive a method utilizing their growth charts.
children and 9 based on anthropological measurements of They incorporated the concept of a growth inhibition for-
femoral and tibial bones. The multipliers derived from mula to aid in ultimate timing for epiphyseal arrest. Growth
radiographic, clinical, and anthropological measurements of inhibition was calculated as a formula: [(growth of the long
femurs and tibias were all similar. Femoral and tibial mul- leg–growth of the short leg)/growth of the long leg]. This
tipliers at the same age were also nearly identical, allowing formula enabled them to determine a rate of growth inhibi-
them to average the mean multipliers to obtain overall lower tion which then served to indicate how much the discrepancy
limb multipliers for boys and girls. A more recent radio- would increase over the remaining years of growth. For
logical study in Dutch children between 1979 and 1994 example, from any time period reference to the normal
allowed Beumer et al. to demonstrate that their population growth charts would indicate how much growth of the
was taller than the American groups studied in the 1930s– longer or normal leg was expected prior to skeletal maturity.
1950s [324]. In spite of this, the multipliers were “approx- Growth inhibition was then calculated from a time period of
imately the same” in the two databases. The lower limb sufficient length to provide accurate data. The assumption
multipliers for boys and girls are shown in Table 6.2. was then made that the growth inhibition would be constant
Multipliers have also been determined for upper throughout the period of growth. It is evident that this is not
extremity lengths (humerus, radius, and ulna) [345], the foot true for all discrepancies. In practice, however, the Boston
[346], fetal prenatal lengths for prediction of bone length at Children’s Hospital Growth Study unit frequently took a
birth [347], and adult height [344]. The study by Paley et al. slowing of growth inhibition into account although there was
on the upper extremity used a wide range of radiographic, no specific formulaic method for this. If the growth inhibi-
clinical, and anthropological databases, similar to their tion was calculated as 0.4 and the future projected growth of
approach with the earlier lower extremity studies [344]. the normal leg was 10 cm, then the future increase in dis-
Studies separated males from females and also determined crepancy was considered to be 10 × 0.4 or 4 cm. This would
multipliers for various percentiles. The multipliers for dif- be added to the discrepancy at the time that the projection
ferent national and racial groups were the same. was made to yield the final discrepancy at skeletal maturity.

b Fig. 6.16 a The Moseley straight-line graph is shown in both Figures the length of the lower extremity bones from 150 to 540 mm and allows
ai, ii (Reprinted with permission from Moseley CF, A straight line for representation of boys and girls with bone age in girls going from 5
graph for leg length discrepancies. Clin Orthop Rel Res 1978;136:33– to 14 years and in boys from 6 to 16 years (Reprinted with permission
40. Copyright 1978 Wolters Kluwer Health). bi, ii Detailed presenta- from Hechard and Carlioz, Methode pratique de prevision des
tions explaining use of the straight-line graphs are shown. The inegalities de longeur des membres inferieures. Rev Chir Orthop
step-by-step method is illustrated and described in bi and a case 1978;64:81–87. Copyright 1978 Masson Editeur)
example is outlined in bii. c The Hechard-Carlioz growth chart depicts
684 6 Lower Extremity Length Discrepancies

Fig. 6.17 The clinical leg length discrepancy graph for boys derived reference slope and slope 3 the tibial epiphyseal arrest reference slope.
by Eastwood and Cole is shown. Line 1 shows the skeletal maturity line Y is the estimated mature discrepancy with X and X1 representing the
which for boys is 16 years of age and for girls 14 years of age. Line 2 chronological ages for femoral or femoral and tibial epiphyseal arrests
documents the observed discrepancy in chronological years. Line 3 is respectively (Reprinted with permission from Eastwood and Cole, A
the projected discrepancy. Line 4 is the mature discrepancy line. It is graphic method for timing the correction of leg length discrepancy.
drawn horizontally from point Y. Slope 1 is the femoral and tibial J Bone Joint Surg Br 1995;77B:743–747)
epiphyseal arrest reference slope, slope 2 the femoral epiphyseal arrest

The appropriate timing for epiphyseal arrest would then be indeed simpler than calculation of the growth inhibition
determined from the growth remaining charts. Since the itself and have been adopted by many.
correction would be made by the shortened leg the percentile All these studies are made more accurate by plotting 3 or
along which the shortened leg was growing was determined more time points. Paley et al. recognized that for Shapiro
from the normal femoral and tibial length charts. If this was type I discrepancies which do increase at a constant rate with
a standard deviation below the mean, then that particular line time a multiplier can be determined for each sex, age, and
was referred to on the growth remaining chart and the percentile from the established growth charts in which the
appropriate skeletal age to make up the specific discrepancy current length is multiplied by the multiplier to determine the
was decided upon. The conceptual changes introduced by final length. This method, in effect, needs only one mea-
the Moseley, Hechard and Carlioz, and Eastwood and Cole surement for the final determination.
graphs primarily involved depiction of the growth data It has been recognized for some time that the length
graphically such that growth inhibition would not have to be parameter of normal growth can be represented accurately
specifically calculated but was simply taken into account by by logarithmic plotting [348, 349]. It is incorrect, however,
plotting the growth of the normal and affected limbs on the to assume that pathological processes are as readily pre-
chart which allowed the discrepancy to be read directly. dictable by logarithmic plotting or any other formula. The
These methods, each of which have their advocates, are straight-line graph of Moseley [340, 341] or the
6.7 Projection of Limb Length Discrepancies … 685

growth-inhibition method of Green-Anderson [337, 350] or Table 6.2 .

any of the methods described might well lead to inaccurate Age (years + mos.) Multiplier
projections, particularly if the assessments are done too early Boys Girls*
in patients with conditions in which type II, late plateau type
Birth 5.080 4.630
III, type IV, or type V patterns may be evolving. The types
0+3 4.550 4.155
of pattern that occur in each disease category have been
delineated. Knowledge of the developmental pattern classi- 0+6 4.050 3.725
fication, the natural and specific history of the condition 0+9 3.600 3.300
causing a particular discrepancy, and the pattern type or 1 +0 3.240 2.970
types that occur in the condition allow the physician to 1+3 2.975 2.750
project the ultimate extent of the discrepancy with clinically 1+6 2.825 2.600
acceptable accuracy. The frequency with which clinical and 1 +9 2.700 2.490
radiographic evaluations of the discrepancy should be done
2+0 2.590 2.390
must strike a balance. The evaluations should not be termi-
2+3 2.480 2.295
nated too early or done too frequently on the expectation that
straight-line graph or growth-inhibition projections always 2+6 2.385 2.200
will suffice, and on the other hand they should not be done 2+9 2.300 2.125
unnecessarily often, as though the eventual outcome were 3+0 2.230 2.050
totally in doubt. The developmental patterns themselves 3+6 2.110 1.925
cannot be used to make accurate mathematical projections, 4+0 2.000 1.830
since growth, particularly during growth spurt periods and 4+6 1.890 1.740
immediately prior to skeletal maturity, is not linear with
5+0 1.820 1.660
time. The patterns do, however, permit accurate projections
5+6 1.740 1.560
of discrepancy to be made using the femoral and tibial length
charts and the femoral and tibial growth remaining charts 6+0 1.670 1.510
which do take the nonlinearity of growth into consideration. 6+6 1.620 1.460
The method of Eastwood and Cole incorporates both the 7+0 1.570 1.430
discrepancy pattern classification and the Menelaus method. 7+6 1.520 1.370
The length and growth remaining charts were developed 8+0 1.470 1.330
from information obtained by making yearly orthoradio- 8+6 1.420 1.290
graphs of 67 boys and 67 girls between the ages of 1 and
9+0 1.380 1.260
18 years. Their value lies in indicating the lengths of the
9+6 1.340 1.220
femur and tibia and the growth remaining in those bones
plotted along percentiles in relation to the standard deviation 10 + 0 1.310 1.190
position. Smooth curves of growth are shown, with the 10 + 6 1.280 1.160
individual growth spurt that occurs between the ages of 10 11 +0 1.240 1.130
and 14 years blurred by averaged data. When an individual 11 + 6 1.220 1.100
child’s growth is plotted, the growth spurt will often change 12 + 0 1.180 1.070
the standard deviation position of the limb lengths. If mat- 12 + 6 1.160 1.050
uration is relatively early the limb length will be on a higher
13 + 0 1.130 1.030
percentile; if maturation is late the limb length will be on a
13 + 6 1.100 1.010
relatively lower percentile. Awareness of this factor is
important in determining the amount of growth remaining in 14 + 0 1.080 1.000
a bone and in projecting its final normal length. Growth is 14 + 6 1.060 NA
generally linear between the ages of 4 and 10 years, and if a 15 + 0 1.040 NA
child is on the first standard deviation above the mean at the 15 + 6 1.020 NA
age of 7, at skeletal maturity limb length will also rest along 16 + 0 1.010 NA
that percentile. Thus, length data obtained before the ado- 16 + 6 1.010 NA
lescent growth spurt are of great value in indicating what the
17 + 0 1.000 NA
child’s projected mature level will be. If information is
*NA not applicable
available only from the period between 10 and 14 years,
686 6 Lower Extremity Length Discrepancies

however, awareness of the relationship of skeletal age to extremities; a chart of the relationship between discrepancy
chronological age is important. If the skeletal age is retarded and age, to outline the developmental pattern that is evolv-
or advanced by 6 months or more in relation to chronolog- ing; the percentile of the normal limb and the abnormal limb;
ical age, the correct growth percentile can best be determined and the patient’s skeletal age.
by plotting the femoral and tibial lengths in relation to
skeletal age, not chronological age. The Anderson et al.
growth charts are derived from studies of white North 6.8 Use of the Developmental Pattern
American and northern European children during the time Classification in Projecting
frame from 1930 to 1956. They thus reflect the growth Limb Length Discrepancies
characteristics and height variations of that group which
would differ slightly from different racial groups and even Type I. The type I discrepancy increases at a constant rate
from similar racial groups at differing time periods under with time, as the rate of inhibition or stimulation remains
altered socioeconomic climates. Even if the absolute height uniform throughout the growth period [50]. If one is certain
values between groups are slightly different, however, the that a type I pattern will evolve, one radiographic assessment
pattern and percentile distribution would be unlikely to of length, especially after the age of 2 years, will suffice for
change in any meaningful clinical way. Since the values are accurate determination of the final discrepancy, although
read from the appropriate percentile and not simply deter- more determinations are always performed. In the first
mined as means or averages placing of any individual on his 2 years of life there can be considerable shifting of length
or her percentile, even if this were somewhat higher than the between various percentiles, whereas afterward the distinct
percentile placements for other groups of relatively smaller tendency is for normal growth to persist along the same
stature, would still lead to the appropriate projections with percentile. For example, if at the age of 4 years the involved
time. Beumer et al. have documented femoral and tibial femur in a child with proximal femoral focal deficiency is
growth radiologically in Dutch children from 1979 to 1994 63 % as long as the normal femur, one can project the final
[324]. Pritchett and Bortel studied individual bones creating discrepancy by determining the length percentile on which
single bone (femur and tibia) straight line graphs (using the the normal femur lies from the femoral and tibial length
Anderson et al. data) as well as single line growth remaining charts and noting the femoral length at maturity for that
graphs. The mean, 10th, and 90th percentiles were recorded percentile. Sixty-three percent of the value represents the
on the skeletal age nomogram [351]. This approach with projected final length of the involved femur, and the differ-
multiple plottings of the normal and abnormal limb allowed ence between the 2 lengths represents the projected femoral
for timing of interventions for epiphyseal arrest by com- length discrepancy. The length differential using the multi-
paring curves and growth remaining data by superimposing plier tables would also be accurate.
the normal and abnormal graphs. The ability to perform When the type I pattern is due to physeal destruction the
accurate imaging assessments without radiographic means, femoral and tibial growth remaining data can be localized
for example by use of ultrasonography, should enable newer accurately to the involved physis, and the values for the
charts of differing racial groups and in differing socioeco- distal end of the femur and proximal end of the tibia can be
nomic settings to be established. read directly from the chart. If the proximal femoral physis
The assessment of skeletal age is important in using the has closed, the projected growth loss is determined on the
Green-Anderson method. While a wide variation in basis that 30 % of the remaining growth of the normal
skeletal-age reading can be demonstrated among readers femur would occur at the proximal physis and 70 % at the
who do it infrequently, the assessments become much more distal physis. Similarly, if the distal tibial plate has fused,
reproducible when done by readers who do many. Although projected growth loss is determined on the basis that 43 %
the Greulich and Pyle atlas has certain limitations, it still of the remaining growth would occur at the distal tibia and
serves as a clinically reliable guide to the rate of skeletal 57 % proximally. The amount of growth remaining in the
maturation. The use of skeletal age versus chronologic age in entire femur or tibia is determined from the line that cor-
growth studies will be addressed below in Sect. 6.9.2.2. responds to the standard deviation position of the normal
Management of the growing patient with a limb length bone on the femoral and tibial length charts. Thirty percent
discrepancy can be improved by knowledge of the classifi- of the difference between the present normal femoral length
cation of developmental patterns; the type or types of pat- and the projected final length along the patient’s percentile
terns that can occur with the particular disease process; is the growth remaining in the normal proximal femoral
radiographic documentation of the lengths of the lower physis.
6.8 Use of the Developmental Pattern Classification … 687

Type II. This can be a difficult pattern to project because normal femur, and, since growth is not occurring, adding this
the discrepancy shows a decremental rate of increase which value to the pre-existing discrepancy to give the projected
varies from patient to patient and from condition to condition. final discrepancy.
The information available from the period of constant Type V. If a discrepancy is beginning to correct itself, the
increase has no predictive value, as the discrepancy values growth charts are used to see how much growth remains.
themselves cannot “be aware” that a change in discrepancy A determination can then be made as to whether the spon-
pattern is about to occur. This group therefore requires taneous correction will be insufficient, will result in equal
especially careful monitoring. An example follows: at the age limb lengths, or might result in overcorrection. The type V
of 11 years if a child’s femoral discrepancy measures 5.0 cm pattern is seen characteristically with chronic inflammatory
the length on the short side is a cumulative 87 % of normal. disorders not fully responsive to therapy which stimulate
The growth percentile on which the normal femur lies allows growth under 10 years of age but lead to premature growth
one to project its final length. The growth rate in the most cessation toward the end of skeletal growth. The type V
recent six-month period however indicated that the short pattern is well documented in juvenile rheumatoid arthritis
femur has shown 93 % growth in relation to the normal side, and appears to occur in many cases of hemophilia and
thus demonstrating the deceleration in the development of tuberculosis where therapy is less than fully effective.
discrepancy. The growth remaining in the normal femur is The developmental pattern classification provides a visual
8.6 cm, as indicated by the femoral and tibial length chart. representation of the varying directional changes that can
A projection of the change in discrepancy with time indicates occur with time in lower extremity length discrepancies
that growth on the shorter side, based on the recent 6 month (Fig. 6.3a). The dependence of the patterns on the causes of
deceleration, would be at least 93 % of 8.6 cm, such that the the discrepancies and on the time and anatomical locations
discrepancy would increase only by 7 % of 8.6 cm, or 0.6 cm, of their occurrence is stressed. The demonstrated relation-
yielding a final maximum projection of 5.6 cm of discrepancy. ships between the pattern type and the particular disease
If there is more time before surgical intervention, a further entity (Fig. 6.3b) should aid in planning the nature and
6 month growth assessment might allow for an additional frequency of discrepancy assessments. In those conditions in
calculation. By this time, projections that allow for a clinically which several pattern types occur, the classification serves
acceptable result (discrepancy of <1.0 cm) can be made. mainly to point out that variability. Some of the contributing
Type III. Once a plateau has been reached, the lower factors to the various patterns within each disease entity have
extremity length discrepancy will not change throughout the been assessed further. The patterns provide for an accurate
remaining period of growth. The prototypical type III pattern projection of a final discrepancy in type I and in type III but
is seen with overgrowth following fracture of a femoral not in types II, IV, and V. The patterns do, however, permit
diaphysis. The timing for the corrective physeal arrest is accurate projections of discrepancy to be made using the
arrived at by using the femoral and tibial length charts and femoral–tibial length and growth remaining charts of
the femoral and tibial growth remaining charts. The final Anderson and colleagues.
discrepancy is known once the plateau phenomenon has
been documented to have occurred, as neither further stim-
ulation nor inhibition will occur. 6.9 Management of Lower Extremity
Type IV. Type IV discrepancies characteristically are Length Discrepancies
seen after hip diseases in childhood that affect the proximal
femoral capital epiphysis, such as septic arthritis of the hip 6.9.1 General Considerations
with mild-to-moderate damage, Legg–Perthes disease, and
avascular necrosis of the femoral head in association with As a general guideline, any discrepancy projected to be
treatment of congenital or developmental dysplasia of the <2.0 cm at skeletal maturity should not require limb equal-
hip. Premature closure of the proximal femoral capital epi- ization; those discrepancies between 2.0 and 5.0 cm are
physis can occur after the discrepancy has remained in a usually treated with contralateral epiphyseal arrests to
plateau phase for as long as a decade. Radiographic indi- shorter the longer side; those >5.0 cm warrant consideration
cation of premature fusion of the proximal femoral capital for ipsilateral lengthening; those beyond 8 cm often benefit
epiphysis is demonstrated by a change in the relationship of from a combination of ipsilateral lengthening and con-
the femoral head to the greater trochanter due to relative tralateral shortening; and massive discrepancies in the 15 cm
overgrowth of the latter. The growth discrepancy to be range or beyond might require prostheses with or without
expected from premature fusion, once it has occurred, is partial amputation. The aim of management is to ensure a
obtained by determining the growth remaining in the entire discrepancy of <1.2 cm at skeletal maturity. This goal can be
normal femur, determining 30 % of that valueto give the achieved in five basic ways: (i) by epiphyseal growth plate
amount of overgrowth expected from the proximal end of a arrest in the longer limb at the appropriate time before
688 6 Lower Extremity Length Discrepancies

Fig. 6.18 Management

approaches to discrepancies of
increasing magnitude is shown.
The management profiles A–D in
each of the defined disorders
leading either to stimulation or
retardation of growth are shown
in Table 6.1

skeletal maturity; (ii) by metaphyseal or diaphyseal short- colleagues used an index of the rate of growth inhibition to
ening in the longer limb, removing a segment of bone at project the final discrepancy [97, 335, 337, 350, 356].
skeletal maturity; (iii) by lengthening the shorter extremity Moseley developed a straight-line graph for projecting
using metaphyseal or diaphyseal osteotomy and gradual length discrepancies by using logarithmic methods to con-
distraction, transphyseal distraction, or transiliac osteotomy; vert the normal growth curve [340, 341].
and (iv) by combinations of shortening and lengthening Paley et al. derived the multiplier method for determining
cases with the more extensive discrepancies; and (v) for extent and timing of treatment for lower extremity length
relatively massive discrepancies which leave the foot on the discrepancies [344]. Aguilar et al. subsequently showed the
shortened side in the region of the midleg or knee of the clinical value and accuracy of the method both for predicting
longer side, prosthetic fitting and some or all of correction of limb length at maturity [357] and predicting limb length
angular deformity, joint stabilization, limb lengthening, discrepancy and outcome of epiphysiodesis [358]. Each
distal limb rotationplasty or amputation fit may be required method can lead to inaccurate projections, in patients in
(Fig. 6.18). Several excellent reviews of the lower extremity whom the rate of change varies over time, if the assessments
length discrepancy entity were published [309, 352–355]. stop too early. The five patterns of discrepancy and their
prevalence in each of the major conditions causing dis-
crepancies in length have been delineated. Not all discrep-
6.9.2 Procedures to Shorten the Longer Limb ancies increase at a constant rate, but the discrepancy at
skeletal maturity can still be projected if the disease and the
6.9.2.1 Therapeutic Arrest of Growth Plate pattern of development are assessed carefully. The growth of
Therapeutic arrest of the growth plate requires knowledge of the distal femoral, proximal tibial, and proximal fibular
the amount of further growth to be expected in each of the growth plates may be arrested when discrepancies are pro-
growth plates at a particular age and an accurate projection jected to be <5 cm at skeletal maturity. The function of the
of the expected discrepancy at skeletal maturity. The lengths growth plate can be arrested surgically by inducing prema-
of the femur and tibia have been documented radiographi- ture fusion between the epiphyseal bone of the secondary
cally and plotted in percentile charts showing standard ossification center and the metaphysis; length discrepancy is
deviations, and charts of the amount of growth remaining in then corrected by continuing growth of the shorter side.
these bones have been developed. The approximate contri- Complete growth plate arrest of a normal functioning
butions of each of the major long-bone epiphyses to growth physis, if the contralateral affected epiphysis is still func-
have been known for some time. In patients with a dis- tioning, allows the shorter side to catch up in terms of
crepancy in the length of the lower extremities, Green and growth. If the affected contralateral epiphysis no longer has
6.9 Management of Lower Extremity Length Discrepancies 689

any growth, normal side epiphyseal arrest prevents any halt growth [359–362]. The principle involved is dif-
discrepancy from worsening but does not lead to any cor- ferent from the two techniques described above; growth
rection of the discrepancy. Complete epiphyseal arrest is cessation is gradual since the physis must continue to
most commonly used in treatment of lower extremity length grow until the prongs of the staple mechanically pre-
discrepancies. The timing of the procedure is crucial to its vent further expansion. The original reason for this
success but can be determined effectively using any of approach was to allow for subsequent removal of the
several prediction systems. The most common sites for staples and the resumption of growth if the timing of
elective growth plate epiphysiodesis are at the distal femur epiphyseal arrest proved to be too early such that the
and proximal tibia and fibula. In limb segments with 2 long discrepancy was not only eliminated but continuing
bones (the leg and forearm) complete arrest of one growth growth from the shorter side was about to reverse the
plate often mandates arrest of the adjacent growth plate to discrepancy. This rationale was not always realized
prevent worsening of deformity and to maintain articular since in some cases the staples had caused complete
alignment. cessation of growth and there was often no continuing
Five technically effective ways of inducing premature growth of the physis once the staples were removed.
epiphyseal arrest have been used.
Blount and Clarke in their initial report on control of bone
(a) Phemister technique: At open operation a periphyseal growth by epiphyseal stapling clearly laid out the principles
rectangle of bone and cartilage is removed, rotated of the approach [359]. They pointed out that Haas proposed
180°, and replaced at both the medial and lateral sides and demonstrated retardation of physeal growth by a cir-
of the involved bone end (Fig. 6.19a) [299]. The rect- cumferential wire loop; a discovery of “the principle of
angle of tissue removed involves metaphyseal bone, temporary arrest of epiphyseal growth.” [363, 364]. This
epiphyseal bone, and the intervening growth plate with approach utilized the principle of mechanical diminution of
2/3 of the length of the resected block on the meta- growth and was attractive to Blount since when Haas either
physeal side and 1/3 on the epiphyseal side. The size of removed the wire or the wire broke growth continued.
the segment removed and then repositioned varies Pressure inhibition of growth had also been demonstrated
depending on the size of the bone. Phemister defined a experimentally by Arkin and Katz [365].
3 cm × 1.5 cm × 1 cm block of tissue with curettage of Haas had utilized a mechanical principle of limiting
the physis anterior and posterior to the block of tissue physeal growth by passing a wire around the epiphyseal
removed to a depth of 1 cm. Once removed the block is plate with one transverse path across the metaphysis and the
rotated 180° so that the larger metaphyseal fragment other across the secondary ossification center with the ends
bone completely bridges the remaining epiphyseal twisted together to provide a continuous loop [363]. Several
growth plate. The medial and lateral transphyseal bone experiments in the dog were done each of which showed a
bridges stop growth as soon as bone repair occurs and definite loss in length growth of the bone. In some instances,
their peripheral tethering effect induces central physeal the wire either broke or came loose at which time growth
fusion. The White and Warner modification was pop- continued indicating that physeal growth while restrained by
ular with many (Fig. 6.19b) [332]. the intact wire did not lose its full potential which could be
(b) Green-Phemister technique: Green, in his modification realized once the restraint was released. A few similar pro-
of the Phemister approach, removed a larger and deeper cedures were performed on patients with definite evidence of
block of bone and cartilage medially and laterally, growth retardation noted. In the human growth also con-
obliterated the remaining growth plate cartilage with tinued after breakage of the wire. Haas performed additional
drills and a curette and packed adjacent metaphyseal investigations in efforts to make the technique of clinical
bone into the physeal defect [337, 350]. The rectangle value [363]. In a second series of studies, staples were used
of bone removed was approximately 1.5 in. long (1 in. instead of wire loops. The staples applied unilaterally across
diaphyseal; 0.5 in. epiphyseal), 1 in. wide and 3/4 in. the physis arrested growth on the side of insertion and also
deep. At the end of the procedure it was reversed 180° restricted it on the opposite side to a lesser degree. In many
as in the Phemister approach, replaced into the defect, instances, there was evidence of complete cessation of
and the periosteum resutured in place. Metaphyseal– physeal growth. Enormous forces generated by the growing
epiphyseal bone fusions leads to immediate growth physis were readily apparent since either a single staple or
cessation. the wire loop often broke allowing growth to continue. Even
(c) Blount stapling technique: Blount used three large when two staples were placed, there was often separation of
metallic staples placed medially and laterally at the the tips or widening of the staples again indicating the
anterior, middle, and posterior aspects of the physes to powerful forces of growth not fully controlled by the staple.
690 6 Lower Extremity Length Discrepancies

Fig. 6.19 Technical approaches to epiphyseal arrest are illustrated. the Blount stapling technique are shown in this figure ci. The medial
a Drawing from Phemister’s original work shows his outline of the approach is shown at left and the lateral approach at right. In this figure
reversed bone block technique. b The White modification of the cii the correct insertion for the distal femoral and proximal tibial medial
Phemister technique is illustrated. At left the medial and lateral distal staples is shown. Each prong is equidistant from the physis and the
femoral and proximal tibial blocks to be removed are outlined. Once alignment of the staple is parallel to that of the epiphyseal growth plate
removed the ½ in. plugs containing epiphyseal bone, the epiphyseal with the cross bar at right angles to the physeal cartilage and parallel to
growth plate cartilage and metaphyseal bone are rotated 90° and the bone surface. Three staples were placed medially and three laterally
reinserted. Bone tissue now completely covers the physis and the bone in each bone requiring arrest (Reprinted with permission from
bridge formed bilaterally tethers growth and leads to its cessation. The Blount WP, Unequal leg length, in Reynolds FC (ed): Instructional
fibular block was soon recognized as being unnecessarily large and for Course Lectures 17. Rosemont IL, American Academy of Orthopaedic
fibular arrest most now simply curette the physis which also minimizes Surgeons 1960; pp. 218–245)
the chance of damage to the peroneal nerve. c Surgical approaches for

Blount and Zeier pointed to the work of Strobino and remedied simply by removing the staples at which time
Colonna that a force >120 pounds was needed to halt growth would resume. In a second report, Blount and Zeier
proximal tibial growth in a calf [360]. The routine use of reviewed 117 staplings noting few complications [360].
three staples on medial and lateral sides of the physis was They concluded that staples could be left in place at least
then adopted for clinical cases and the procedures used for 2 years and still removed with the expectation that growth
distal femoral and proximal tibial growth arrests which would be resumed. After removal of staples, there was
during that era were generally for poliomyelitis. The pro- usually a local growth spurt lasting a few months.
cedure, which appeared technically quite simple, was Frantz reviewed 10 clinical papers summarizing the first 2
adopted widely with relatively less consideration for timing decades of work with this technique [366]. Benefits and
since it was expected that any imperfect timing could be drawbacks became more clearly defined. One of the
6.9 Management of Lower Extremity Length Discrepancies 691

problems, which was basically present in any epiphyseal reached the skeletal age of 8 years. It was inappropriate to
arrest operation, was that of timing. The complications perform the stapling procedure according to a timing
reported which tended to appear early in any series, included schedule set up for epiphyseal arrest since the stapling
buried staples, metal reaction, overcorrection, premature procedure was based on a different principle. Staples allowed
physeal closure, peroneal palsy, knee joint laxity, misplaced for correction with growth but were not designed to cause
staples, fractured staples, extrusion of staples, angular complete immediate cessation of growth. Staplings needed
deformity, infection, genu valgum, and false aneurysm. It to be done somewhat earlier than epiphyseal arrests. It was
was widely agreed that stapling was not warranted under the important not to bury the staples under the periosteum since
age of 8 and preferably 9 years. Green and Anderson they would be difficult to find at time of removal and were
reported on both formal epiphyseal arrest and stapling and more likely to cause periosteal new bone formation and
felt that both procedures were satisfactory with the incidence growth plate bridging. Blount indicated that “stapling of an
of complications relatively insignificant, although they used epiphysis retards growth 80–90 % for the next few years. It
the stapling for definitive growth cessation [366]. In an causes a temporary growth spurt at the other end of the bone.
experimental series of studies, Heikel demonstrated that At the stapled epiphysis, elongation is decelerated only 50 %
epiphysiodesis of the proximal tibial plate had no effect on during the first 6 months. Some growth continues until a
subsequent longitudinal growth distally [367]. Siffert per- year or less before the normal time for epiphyseal closure.”
formed asymmetric stapling of the distal femoral epiphysis Sengupta and Gupta used two staples on each side of the
in rabbits and noted production of the varus deformity with distal femur effectively in the large majority of cases rarely
gradual histologic thinning of the physis and eventual resorting to three per side [371]. The two staples on each side
transphyseal bone arrest [368]. Goff studied 120 biopsies of were equidistant from each other with their tips pointing
children at various stages of growth deceleration and arrest toward the center of the physis. Seventy-one percent of the 503
following stapling [369]. He observed that the direct com- procedures led to a discrepancy of 0.5–1.0 cm shortening at the
pression by staples served to inhibit the proliferation stage of end of growth with only 3 % showing more than 2 cm short-
the physis. The thinness of the disk increased with time. The ening. The staples should be removed at skeletal maturity.
most sensitive sign of diminished growth was increased Gorman et al. have noted that mechanical axis deviation
degeneration and shortening of the hypertrophic region. following staple epiphysiodesis for limb length inequality is
Eventually all the hypertrophic cells disappeared and new significantly greater than was previously recognized [372].
bone formation crossed from metaphysis to epiphysis. Bone They used final long standing anteroposterior radiographs of
bridge formation was present invariably after 4 years or 54 patients at skeletal maturity to assess mechanical axis shifts
48 months although the markedly abnormal structure prior to from the preoperative state in patients undergoing distal
that time would appear to have had little potential for femoral or proximal tibial or both sites medial and lateral
regrowth. Bylander et al. studied growth of the physeal (complete) epiphyseal arrests for limb length discrepancy.
regions following stapling using highly accurate radio- Fifty percent (27/54) of the patients showed a shift in the
graphic stereophotogrammetric analysis [370]. There was a mechanical axis of 1 cm or greater compared with the preop-
uniform pattern of growth retardation following stapling erative measurement. Varus was the direction of deformity in
which lasted over a period of several months to years. This 89 %. The most frequent sites of epiphyseal arrest causing the
pattern was evidence of the applicability of the original problem were isolated proximal tibial or combined distal
theory of stapling since it indicated slowing of physeal femoral/proximal tibial procedures. The problematic region
growth to basal levels rather than complete cessation of for attaining physeal fusion was the lateral proximal tibia;
growth. They calculated that growth at the distal femur and limited effect there compared to effective medial tibial physeal
proximal tibia in human patients continued at a low basal fusion led to the varus deformity. Distal femoral procedures
level of about 5–10 μm/day, in particular when stapling was alone were safest and most effective due to the more uniform
performed at younger skeletal ages. anatomy. Six patients of the 54 (11 %) eventually underwent
Blount pointed out the need for precise placement of the corrective osteotomy to improve alignment. Although occa-
staples feeling that many of the imperfect results reported sional occurrence of angular deformity as a complication had
were due to improper timing or less than ideal technique been mentioned previously, only Brockway et al. in 1954 had
[362]. The upper and lower prongs of the staple were to be specifically commented on the problem [373]. They noted
equidistant from the physis and the cross member was to be 4/42 patients (9.5 %) who developed poststapling varus and 3
perpendicular to the growth plate and parallel to the surface of whom had osteotomy for correction. The possibility of this
of the bone into which it was being driven (Fig. 6.19ci, ii). occurrence leads to the recommendation of transphyseal epi-
The staples were to be angled such that the tips of the prongs physiodesis, limiting stapling to femoral arrests or (if stapling
pointed toward the central axis of the distal femur or prox- was to be done) very careful placement of staples at the
imal tibia. Stapling was best performed after the patient had proximal lateral tibial physis due to its nonuniform structure.
692 6 Lower Extremity Length Discrepancies

(d) 8-Plate: The 8-plate was designed by Stevens primarily uncommon. Canale et al. in their initial report on 13 per-
for unilateral growth plate slowdown to allow for cor- cutaneous epiphysiodesis operations reported that all growth
rection of angular deformity without resort to osteot- plates appeared fused with no major complications and no
omy [374]. It uses the same principle as the Blount clinical evidence of subsequent angular deformity [376].
staple but is considered to be more reliable. It is used There were no fractures, no neural or vascular complications
by some on both sides of the bone for physeal arrest. and no angular deformities. A later report by Canale and
There have not yet been long-term studies of its effect Christian on 22 children with percutaneous epiphyseal arrest
specifically in relation to use for complete epiphys- noted that arrest was achieved in all with no patient devel-
iodesis but many of the concerns noted in long-term oping angular deformity [379]. Ogilvie and King in 7 epi-
studies of stapling will need to be assessed. If done for physeal arrests reported no failures of fusion, postoperative
length equalization it is important to note that it slows infections, restricted joint motion, or angular deformities
down growth only as growth occurs since it is the [379]. Horton and Olney reported 42 percutaneous epiphy-
extrinsic pressure applied to the physis that retards seal arrest procedures in which all patients achieved physeal
physeal elongation. Bone block epiphysiodesis with arrest radiographically and clinically and no patient devel-
physeal curettage halts growth immediately with oped angular deformity from an incomplete arrest [380].
transphyseal bone bridging developing within a few There were no neurovascular complications or fractures.
weeks. Timperlake et al. in a detailed study from the DuPont
(e) Percutaneous epiphysiodesis technique: Institute reported on 53 consecutive percutaneous epiphys-
(i) Transphyseal drilling/curettage: In this procedure, iodeses in which the medial and lateral thirds of the growth
growth plate obliteration is performed through a small plate are ablated, but the central third is preserved for sta-
incision with physeal visualization by fluoroscopic bility [381]. They approach the physis from both sides, use a
imaging [375, 376]. Lateral and medial incisions are 3 mm wide osteotome driven 1 cm into the growth plate and
made over the physis to be ablated, but some surgeons rotated 180° to create a hole in the cortex, and then use a
use an approach only from one side. The soft tissues are 3 mm wide oval curette which is swept across the growth
dissected down to the physeal region at which time a plate to ablate it. Statistical analysis demonstrated no sig-
guide wire and cannulated 4–6 mm wide drill bit is nificant difference in the actual discrepancy at follow-up and
inserted. Under radiographic control drilling is per- the discrepancy at maturity as demonstrated by standard
formed across the physeal region anteriorly, at the growth charts. Gabriel et al. recorded the results of percu-
midline and posteriorly. This serves both to destroy the taneous epiphyseal arrest in 56 physes using a cannulated
cartilage and to allow for communication between 10 mm drill bit over a guidepin followed by curettage [382].
epiphyseal and metaphyseal vessels thus leading to There were no severe complications of angular deformity,
transphyseal bone bridge formation. The postoperative deep infections, or neurovascular problems.
scars are smaller and rehabilitation is quicker than in
the previous physeal ablation techniques. (ii) Transphyseal screws (PETS): Another method for
epiphysiodesis by minimally invasive surgery was
The procedure was reported by Bowen and Johnson [375] described by Metaizeau et al. in 1998 using screws
in 1984 and by Canale, Russell and Holcomb [376] in 1986. across the physis [383]. Percutaneous epiphysiodesis
Ogilvie provided an experimental report in 1986 [377] and a using transphyseal screws (PETS) was introduced both
clinical assessment in 1990 [378]. Small differences in for epiphyseal arrest for limb length discrepancies and
technique are used although the principles of small incision, hemiphyseal arrest for angular correction without
percutaneous surgery, and growth plate obliteration under osteotomy in growing children. Metaizeau et al. used
fluoroscopic control are common to all. The physis has been two crossed screws for full epiphyseal arrest. One
damaged with the use of a drill, drill and curette, drill and screw started at the medial femoral metaphyseal cortex
burr, or osteotome and curette. Ogilvie has demonstrated and angled distally and laterally to cross the physis
well the importance of several passes through the physeal lateral to the midline and enter the epiphysis ossifica-
plate in a fanlike pattern to assure complete obliteration tion center and stop just short of the articular surface of
[377, 378]. Excellent long-term results by the original the lateral femoral condyle. The other screw went from
authors and by others subsequently adopting the technique the lateral metaphyseal cortex distally and medially to
have been reported. the medial subarticular region. Each screw passed
In the original report of Bowen and Johnson, the results through the physis at the junction of its middle and
of 12 percutaneous epiphyseal arrests noted no complica- inner/outer thirds. An alternative placement used two
tions [375]. In their later series, complications were nonintersecting screws from metaphysis through physis
6.9 Management of Lower Extremity Length Discrepancies 693

into the epiphyseal bone not crossing the midline. The hand remains the most widely used indicator of skeletal
placement at the physeal area had one screw at either age. There is still considerable subjectivity needed,
end of its (physis) middle third. The alignment was however, with this system. The radiographs used are
more longitudinal than with the two oblique crossed spaced at either 6 month or one-year intervals which
screws. represents a considerable margin of difference between
age gradings. In particular, there are no radiographic data
Metaizeau et al. used fully threaded cancellous screws corresponding to 12 and 14.5 years of age in boys and
with long threads. In other reports of this technique Nouh 9.5, 10.5, 11.5 and 12.5 years of age in girls which are
and Kuo [384] used cannulated partially threaded cancellous very important time periods for consideration of timing
screws 6.5–8 mm with the threads across the physis with at for epiphyseal arrest. The Greulich and Pyle atlas is
least four threads protruding into epiphyseal bone and based on radiographs covering the following ages in
Khoury et al. [385] used 7.3 mm cannulated fully threaded males: newborn, 3 months, 6 months, 9 months, 1 year,
cancellous screws. 1 year and 3 months, 1 year and 6 months, 2 years,
Good results were reported for both limb length dis- 2 years and 8 months, 3 years, 3 years and 6 months,
crepancy and angular correction in each of the 3 studies. The 4 years, 4 years and 6 months, 5 years, 6 years, 7 years,
authors felt that the screws provided a tether to limit growth 8 years, 9 years, 10 years, 11 years, 11 years and
rather than doing so by a lag effect to inhibit growth. 6 months, 12 years and 6 months, 13 years, 13 years and
Metaizeau et al. felt that growth would resume once the 6 months, 14 years, 15 years, 15 years and 6 months,
screws were removed although this was not attempted in 16 years, 17 years, 18 years, and 19 years.
their study [383]. Khoury et al. noted growth resumption in In females ages are: newborn, 3 months, 6 months,
all 13 cases where screws were removed at completion of 9 months, 1 year, 1 year and 3 months, 1 year and
angular correction [385]. 6 months, 2 years, 2 years and 6 months, 3 years, 3 years
Similar to use of staples or the eight plate, growth cessation and 6 months, 4 years and 2 months, 5 years, 5 years and
only occurs when some additional growth has occurred to 9 months, 6 years and 10 months, 7 years and 10 months,
allow the compressive effect of the hardware to occur. At the 8 years and 10 months, 10 years, 11 years, 12 years,
other end of the process, growth retardation may sufficiently 13 years, 13 years and 6 months, 14 years, 15 years,
damage the physis such that it either does not resume growth 16 years, 17 years, and 18 years.
or does so at a diminished rate when the hardware is removed.
(b) SauvegrainMethod for Determining Skeletal Age dur-
It is advisable to indicate in advance that growth resumption
ing Puberty:
may not occur with hardware removal.
Dimeglio et al. [386] have used a modified version of the
6.9.2.2 Timing for Epiphysiodesis method described by Sauvegrain et al. [387] in 1962 to
The timing of epiphyseal arrest is still an imperfect science determine skeletal age and development using elbow
and studies continue to appear indicating a range of dis- radiographs. Four regions are assessed with each progression
crepancies at skeletal maturity from acceptable to amounts of ossification graded using anteroposterior and lateral
still leaving lower extremity length discrepancy out of the radiographs of the left elbow beginning at the onset of
desired therapeutic range. It is not essential in a clinical puberty and during the first 2 years of puberty. This is
sense for limb lengths to be equalized since a fully accept- effectively between 10 and 13 years of age in girls and 12
able result by current criteria is to diminish the discrepancy and 15 years in boys. This, however, is the main time period
to under 0.5 in. or 1.2 cm. Even those patients with a dis- for performing epiphyseal arrests for limb equalization. The
crepancy moved to within 3/4 of an inch or <2.0 cm, would regions used for grading are the lateral condyle and epi-
appear to have few long-term problems. There are relatively condyle of the humerus, the trochlea of the humerus, the
few problems reported with the various surgical techniques olecranon apophysis of the ulna and the proximal radial
used to bring about the epiphyseal arrest. epiphysis. The original 27 point scoring system was modi-
There is widespread recognition of the relative inaccuracy fied for greater accuracy by Dimeglio et al. by adding 3
of the skeletal maturation determinations used as a key additional reference points. The lateral condyle has 9 points
indicator of timing for epiphyseal arrest in most systems. of inclusion at skeletal maturity, the trochlea 6, olecranon 8,
Several radiographic approaches to determine the stage of and the proximal radial epiphysis 7. Once each area has been
skeletal maturation have been developed. graded, the 4 scores are totaled and the values placed on a
graph for boys or girls. This provides an indication of
(a) Greulich and Pyle Atlas: The Greulich and Pyle Atlas skeletal age. The system was used to assess skeletal age by 3
using postero-anterior radiographs of the left wrist and observers on radiographs from 60 boys and 60 girls all
694 6 Lower Extremity Length Discrepancies

healthy and to compare values with determinations made on and ulna along with the three metacarpals (first, third,
AP wrist-hand radiographs using the Greulich and Pyle atlas fifth) and their respective phalanges (2, 3, and 3). This
from the same subjects at the same time. There were system is sometimes referred to as the TW3RUS sys-
excellent correlation coefficients among the 3 observers at tem (R = radius, U = Ulna, and S = Short bones being
both assessments and at each elbow region. The same was the metacarpals and their phalanges). In the TW sys-
found using the Greulich and Pyle atlas but when the 2 were tem, the grading (for the RUS components) assesses the
compared the Sauvegrain method was considered to be more epiphyses and adjacent metaphyses.
accurate since it allowed for 6 monthly assessments while
the Greulich and Pyle atlas in this age range was made up of Owing to concerns about differing rates of skeletal mat-
annual assessments for girls while the 6 month age incre- uration in differing racial groups, the TW method has been
ments for boys were incomplete. Based on this study, applied in several countries to provide reference points for
Dimeglio et al. added 3 time points moving from 27 to 30 specific groups of children. These include studies from
and produced recalibrated graphs for girls and boys [386]. North America (Houston, Texas) [390], North India [391],
An interesting part of the study was the use of each Tokyo, Japan [392], Korea [393], Beijing, China [394],
method to see what correlations existed between skeletal age China (5 cities) [395], and Italy [396].
and chronologic age. The two were considered to be the
same if differences were not >6 months. The ages were the (d) Digital Skeletal Age (DSA) skeletal maturity scoring
same for only 33 % boys/35 % girls with the Sauvegrain system: Sanders et al. Sanders and colleagues have fur-
method and for 30 % boys/28 % girls with the Greulich and ther simplified the Tanner-Whitehouse approach using a
Pyle method. This finding clearly supports the value of using digital skeletal maturity scoring system based on just the
skeletal age as part of the timing decision process. metacarpals and phalanges [397]. Defined in a previous
The other problem appears referable to the nature of the article [398] with skeletal age studies based on left pos-
predominant disorders being studied in any particular series. teroanterior hand and wrist radiographs, immature girls
Very few studies take into account the differing develop- with idiopathic scoliosis were followed through their
mental patterns of the lower extremity length discrepancies growth spurt. The various bones underwent changes in
that have been described. In those series where type I pat- an orderly sequence. Eight stages were defined using all
terns predominate, there is relatively little problem with five digits. The study essentially assesses the relationship
utilizing the more straightforward projections of the of the epiphyses (secondary ossification centers) of each
Green-Anderson, Moseley, Menelaus, or multiplier methods. bone to its adjacent metaphysis, including, at later ages,
Where, however, there are types II, III, IV, and V discrep- physeal fusion (Table 6.3).
ancies, the failure to recognize these patterns can further (e) Assessmentsof Results of EpiphysealArrests: A study
worsen the accuracy of timing. Studies reporting on timing by Blair et al. reviewed retrospectively 67 distal
in epiphyseal arrest procedures should be read with these femoral and proximal tibial epiphyseal arrests per-
considerations in mind. formed over a 14 year period [399]. Only 22 patients
had a final discrepancy of <1 cm. Setting aside 10 of 45
(c) Tanner-Whitehouse Methods: The Tanner-Whitehouse failures due to inadequate surgical technique, the
method for determining bone age was originally based remaining 35 failures were secondary to errors in tim-
on a cross-sectional study of 3000 healthy British ing. This report utilized the Green-Anderson growth
children assessing radiographs of the left wrist and tables. Porat et al. on the other hand, reported good
hand. results in 90 % of their patients, although the series was
(i) Tanner-Whitehouse 2 method: In what is commonly small involving only 20 children [400]. In five children
referred to as the TW2 method of assessing skeletal with anisomelia whose expected discrepancy was
maturity, 20 bones were assessed at increasing ages and 4.5 cm results at maturity show an average discrepancy
given a grading letter correlated with a specific of 0.7 cm. In 10 girls with lower extremity length
weighted score. The bones included the distal radius discrepancy caused by ischemic necrosis with con-
and ulna, 7 carpal bones (excluding only the pisiform), genital dislocation of the hip, the average discrepancy
first, third, and fifth metacarpals and their associated at maturity was 0.6 cm with the expected nontreated
phalanges (2, 3, and 3 respectively) [388] value 4.0 cm. In five children with discrepancy caused
(ii) Tanner-Whitehouse 3 method: Modifications to the by infection, the average discrepancy was 3.8 cm at
scoring system were made in 2001 with the revision time of epiphyseal arrest, whereas at maturity it had
referred to as TW3 [389]. The carpal bones were no diminished to 0.5 cm. This group utilized the Moseley
longer included with scores including the distal radius straight line graph, CT scanograms for length
6.9 Management of Lower Extremity Length Discrepancies 695

Table 6.3 .
Stage Key features Tanner-whitehouse-III Greulich and Related maturity signs
stage reference
1. Juvenile Digital epiphyses are not covered Some digits are at stage E Female 8 year + 10 Tanner stage 1
slow or less mo, male 12 year + 6
mo (note fifth middle
phalanx)
2. All digital epiphyses are covered All digits are at stage F Female 10 year, male Tanner stage 2, starting
Preadolescent 13 year growth spurt
slow
3. Adolescent The preponderance of digits is All digits are at stage G Female 11 and Peak height velocity,
rapid—early capped. The second through fifth 12 year, male Risser stage 0, open pelvic
metacarpal epiphyses are wider than 13 year + 6 mo and triradiate cartilage
their metaphyses 14 year
4. Adolescent Any of distal phalangeal physes are Any distal phalanges are Female 13 year Girls typically in Tanner
rapid—late clearly beginning to close (see at stage H (digits 2, 3, and 4), stage 3, Risser stage 0,
detailed description in the text) male 15 year (digits 4 open triradiate cartilage
and 5)
5. Adolescent All distal phalangeal physes are All distal phalanges and Female 13 year + 6 Risser stage 0, triradiate
steady—early closed. Others are open thumb metacarpal are at mo, male 15 year + 6 cartilage closed, menarche
stage 1. Others remain at mo only occasionally starts
stage G earlier than this
6. Adolescent Middle or proximal phalangeal Middle or proximal Female 14 year male Risser sign positive (stage
steady—late physes are closing phalanges are at stages H 16 year (late) 1 or more)
and 1
7. Early Only distal radial physis is open. All digits are at stage I. Female 15 year, male Risser stage 4
mature Metacarpal physeal scars may be The distal radial physis is 17 year
present at stage G or H
8. Mature Distal radial physis is completely All digits are at stage I Female 17 year, male Risser stage 5
closed 19 year

determination and the percutaneous epiphyseal arrest determined that 34 % of the patients (24) had dis-
procedure. Lampe et al. performed a prospective study crepancies >1.5 cm at skeletal maturity and 27 % (19
in 30 children who underwent 33 epiphyseal arrest patients) had discrepancies >2.0 cm. This study was
procedures using the Moseley straight line graph quite detailed in that 8 different methods were assessed
method [401]. The mean predicted length discrepancy involving 4 variations of the Anderson and Green
was 5.2 cm and the mean discrepancy at the end of technique, and two each of the Menelaus and Moseley
growth was 1.4 cm with a range from 0 to 4.3 cm. In techniques. They concluded that it was the inherent
nine patients out of the 30 (30 %) the final length variability of the individuals requiring epiphyseal arrest
discrepancy exceeded 1.5 cm. They felt that the altered that prevented the methods from predicting the out-
skeletal maturation was most problematic in those cases come more satisfactorily. It was the inability to project
where the projection was inaccurate. Variation in the date of skeletal maturity that played the major role
radiographic determination of skeletal age was also a in the imperfect results. CT documentation of the
source of error as noted by Cundy et al. [402]. Little length discrepancies provided the most accurate deter-
et al. reviewed 71 epiphyseal arrest procedures in mination. There should be relatively little difference
efforts to compare the Green-Anderson, Moseley, and between the Green-Anderson, Moseley and Paley et al.
Menelaus methods of projection [403]. They felt that methods, and even that of Menelaus, since each of
each of the different methods did not have a meaningful these approaches uses the same source of growth data,
superiority in projecting the end result and that all had this being the Anderson et al. femoral and tibial length
somewhat limited accuracy. They advocated the charts and growth remaining charts. Westh and
Menelaus method owing to its simplicity. The mean Menelaus assessed 94 patients with a mean length
preoperative discrepancy in their series was 3.12 cm discrepancy of 4 cm and a mean final discrepancy of
(range 1.4–7.4 cm) and the mean discrepancy at <2 cm [339]. Dewaele and Fabry compared the accu-
follow-up was 1.05 cm (range −2 to 4.4 cm). They racy of the Green-Anderson and Moseley methods
696 6 Lower Extremity Length Discrepancies

[404]. In 83 patients, the mean length discrepancy was improved in 87 % of the deformities treated. Two
3.37 cm and the mean final discrepancy 1.69 cm. They staples were used in most patients. There were 64
felt that the Moseley method was more accurate. valgus deformities and 18 varus deformities. The two
largest groups of patients were idiopathic-physiologic
6.9.2.3 Partial Therapeutic Growth Plate Arrest or secondary to poliomyelitis with other scattered dis-
There are two treatment situations which can lead to a rec- orders involving hemihypertrophy, rickets, and occa-
ommendation for performance of an asymmetric or partial sional skeletal dysplasias. Two biologic phenomena
growth plate arrest. were associated with removal of the staples with
growth still active. (i) The group generally allowed for
(a) To complete an already existing focal arrest to prevent some overcorrection counting on the rebound growth
further angular deformity: Partial arrest of an affected phenomenon to occur in which the stapled side of the
epiphysis can be performed to terminate all growth in a bone elongated more rapidly than the other for a few
growth plate which has suffered a focal or partial arrest months after staple removal. (ii) Following that the rate
which is considered to be too extensive for bone bridge of growth tended to remain equal and then the physis
resection. By completing the arrest across the entire closed 4–6 months prematurely on the stapled side. It is
width of the growth plate, development or worsening of evident that a considerable amount of personal expe-
angular deformity is prevented. Projection should also rience and judgment went into the timing of such
be made of the amount of growth remaining in the procedures using staples
opposite physis. If the patient is near skeletal matura-
tion, no additional measures are needed. If further Bowen et al. developed a chart in an effort to project the
growth is in the 2–5 cm range, contralateral epiphyseal appropriate time for correction of any angular deformity
arrest is warranted to prevent an invariable discrepancy (Fig. 6.20a) [406]. Rather than relying on stapling with
from developing. If growth remaining is >5 cm, the removal of the staple after a certain degree of overcorrection
need for ipsilateral lengthening should be discussed. they performed a bony epiphysiodesis asymmetrically based
(b) To treat angular deformity without need for osteotomy: on a timing chart. Their operation was performed for idio-
The second reason for performance of an asymmetric or pathic or physiologic genu valgum or varum in the adoles-
partial growth plate arrest relates to treatment of cent patient. With an asymmetric stapling, for example on
angular deformity rather than to treatment of shorten- the medial side of the distal femur, continued growth from
ing. It has been recognized for several decades that the lateral physis would be expected. The growth however
creation of an asymmetric growth plate arrest would would not be linear but would represent an arc of a circle
still allow the remaining physis to continue to function with the radius equal to the width the bone measured at the
and correct angular deformity without need for physis. The arc of continued growth relates to the angle of
osteotomy. deformity as the circumference of the circle relates to the
(i) Staples: The earliest proponents of widespread use of total number of degrees in the circle. With use of a specific
this treatment were Blount and colleagues who utilized formula a chart was constructed to relate the amount of
stapling on one side of an epiphysis to correct angular growth remaining to the angular change for varying physeal
deformity. The technique has its major application at distances. This information was then combined with the
the distal femur or proximal tibia for angular deformi- Green-Anderson growth remaining chart which allowed the
ties centered at the knee. Blount and colleagues angular deformity to be related to the linear growth
reported on 82 knees treated with epiphyseal stapling remaining for the patient’s skeletal age. An early study of 13
over a 20 year period and followed to skeletal maturity extremities treated surgically indicated that as a general rule
[405]. The deformities were allowed to overcorrect following partial tibial epiphyseal arrest 5° of angular cor-
before the staples were removed and an effective rection could be expected for each year of remaining growth
rebound phenomenon occurred in 22 patients with 35 and following partial distal femoral epiphyseal arrest 7° of
deformities. In older children, the staples were removed correction could be expected for each year of remaining
when the legs looked straight. Blount felt that exag- growth. The mean ages at surgery were quite similar to those
gerated physiological deformities may correct sponta- recommended by Blount and his group. In the 7 patients
neously and should not be stapled before the skeletal having procedures on 13 extremities the average chronologic
age of 11 years in girls and 12 years in boys. Defor- age was 12 years 8 months with the girls averaging
mities secondary to specific disease processes could be approximately 12½ years of age and the boys slightly more
corrected earlier although rarely below 8 years of age. than 13 years. The average preoperative deformity was 11.6°
They concluded that results were satisfactory or of femoral–tibial valgus and the average deformity at
6.9 Management of Lower Extremity Length Discrepancies 697

Fig. 6.20 (continued)

698 6 Lower Extremity Length Discrepancies

Fig. 6.20 (continued)

 6.9 Management of Lower Extremity Length Discrepancies 699

follow-up was 6.6°. An example of using hemiepiphys- rebound deformity, which reflects the fact that the
iodesis with staples close to the time of skeletal maturity to underlying disorder causing initial deformity has not
correct angular deformity without osteotomy is illustrated in been cured but rather inhibited by the plate. The value of
Fig. 6.20bi–iv. the procedure involves correction without osteotomy
and its attendant complexities and problems.
(ii) Transphyseal screws: Hemiepiphysiodesis using
transphyseal screws has also proven to be effective. With renewed interest in the procedure and introduction of
Metaizeau et al. used single screw transphyseal place- a new device (the 8-plate), current assessments of the
ment for 9 cases of angular correction, 6 for genu val- approach are appearing. Wiemann et al. report similar results
gum, and 3 genu varum [373] The average femoral– comparing the traditional staples with the non-locking plate
tibial angle was 7.66° preoperative and 0.86° at skeletal hemiepiphysiodesis [407]. The rate of angular correction was
maturity. Noulth and Kuo also treated 9 cases of angular about 10° per year with comparable complications. Shin et al.
deformity (knee) with hemiphyseodesis using a single found that staples and transphyseal screws at the knee had
transphyseal screw [374]. The mean angular deformity similar effectiveness correcting angular deformity and similar
prior to surgery was 18° (range 8–35°) with decrease to rebound phenomena after fixation removal with an overall
5.5°(1–25°). Khoury et al. corrected deformities at the 21 % rate (12/56 cases) [408]. Complications such as
knee [375]. All were knee deformities involving 30 eight-plate screw breakage have been reported especially in
patients with 66 bones operated. Screws were placed obese patients with Blount disease [409]. Dual plates, solid
across physes at distal medial or lateral femur or proxi- screws, and stronger implants are suggested solutions.
mal medial or lateral tibia. Distal femoral correction was Treatment with the eight-plate is more problematic when used
6.91° (SD ± 3.75°) a rate of 0.75°/month. In the tibia, the in specific growth plate pathologic disorders such as Blount
average correction was 3.88°, a rate of 0.37°/month. In disease [410] and multiple epiphyseal dysplasia [411]. Ste-
13 cases where the screw was removed with consider- vens and Klatt, however, used the eight-plate to good effect in
able growth remaining, there was no premature growth four patients with rickets although only as part of a series of
cessation and minimal rebound deformity. operative treatments ultimately needed [412]. Since the non-
(iii) 8-plate for guided growth: Stevens has used the principle restrained part of the physis does not have normal growth it
of hemiepiphysiodesis to correct angular long bone may not respond in hoped for fashion leading to imperfect
deformities in children as young as 18 months of age correction. The restrained part of the physis also not normal,
[374]. He developed a two-hole plate (with two non- may not resume growth with device removal. Good correction
locking extraperiosteal screws) placed across the physis of flexion contracture with anterior distal femoral plate
with one screw in epiphyseal bone and one in meta- placement has been noted in anthrogrypotic patients with
physeal bone. The plate with diverging screws is con- knee flexion contractures [413]. Klatt and Stevens used two
sidered to act across the physis as a tension band rather 8-plates anteriorly at the distal femur to correct knee flexion
than by physeal compression as with staples. Stevens contractures without osteotomy [414]. Most of the patients
reported in 2007 on its use in 34 consecutive patients had knee flexion deformities in cerebral palsy. In 18 patients
with 65 deformities of femur and tibia. Once correction with 29 deformities, the mean deformity was 23.4° (range 10–
has occurred the plate is removed. It is essential to place 50). Residual deformity was a mean of 8.2° (range 0–30°). At
the plate extraperiosteally and to not pass the screws into full correction, the plates were removed. The average age at
the physis if postplate removal growth is to be main- surgery was 10.8 years (range 4–17) but generally the pro-
tained. The rate of correction was described as 30 % cedure was best done after 8 years of age.
more rapid than noted with stapling and there had been Excellent reviews of hemiepiphysiodesis have been
no permanent growth arrests. Plate breakage has been written by Saran and Rathgen [415] and Eastwood and
minimized with development of 12 and 16 mm plates Sanghrajka [416]. Guided growth is used most commonly to
and solid screws of 24 and 32 mm lengths. Depending on treat coronal plane deformity at the knee. Over- and under-
its position of placement, the screws can correct in correction must be carefully looked for. For those with more
sagittal, coronal, or oblique planes. Recurrence of than 2 years of skeletal growth remaining, temporary
deformity once the plate is removed is referred to as hemiepiphyseal arrest is attractive, although fixation removal

b Fig. 6.20 Asymmetric stapling is performed to allow for correction of al. Partial epiphysiodesis at the knee to correct angular deformity. Clin
angular deformity without need for osteotomy. a A chart developed by Orthop Rel Res 1985;198:184–190. Copyright 1985 Wolters Kluwer
Bowen et al. estimates the correct time of asymmetric epiphyseal arrest Health). bi–iv Clinical example of hemiepiphyseodesis technique for
in relation to the patient’s skeletal age and the degree of angular angular correction shows correction of bilateral knee valgus deformities
deformity correction sought (Reprinted with permission from Bowen et with medial distal femoral stapling
700 6 Lower Extremity Length Discrepancies

is usually needed, leading to the possibility of partial intramedullary rod. When done at skeletal maturity, there is
deformity recurrence. no concern about overgrowth following surgery and no need
to project the final discrepancy. This approach is recom-
6.9.2.4 Metaphyseal Shortening Osteotomies mended primarily for discrepancies between 2.5 and 5 cm that
Wagner has pointed out the value of correcting a longer femur were not detected by or persist at maturity.
or tibia which also has angular or rotational deformity by Shortening of the bones of the longer leg to achieve
performing a metaphyseal shortening osteotomy [417]. The length equality is a much older and simpler procedure than
most common site is the proximal femoral metaphysis fol- elongation of the shorter leg. Blount and Zeier have credited
lowed by the distal femoral metaphysis. He stressed the Rizzoli of Italy with the earliest descriptions of this proce-
maintenance of a medial cortical strut in continuity with the dure [360]. White [420] and Wilson and Thompson [121]
lesser trochanter to allow for stabilization and a greater area of reported early attempts from the late nineteenth and early
bone repair at the osteotomy site. An AO large fragment twentieth century which generally involved fracturing of the
blade-plate is used for stabilization. Shortening osteotomies of shaft of the femur or open oblique osteotomy, allowing the
the distal femoral metaphysis are carried out in a similar fragments to overlap the necessary amount, and then treating
fashion. A strut of medial cortical bone in the metaphysis is the limb until healing occurred. The first formal shortening
also left intact. The distal femoral metaphyseal shortening of the tibia and fibula was reported by Brooke in 1927 in
osteotomy is often complicated by a relatively slow rehabili- which segments of bone, 1 and 2 in. long, respectively, were
tation owing to periarticular scarring, knee joint stiffness, and removed and then applied as grafts to enhance healing [421].
disruption of the quadriceps mechanism. That site is chosen The femur could also be shortened by a step-cut method
therefore only if the angular bone deformity necessitates followed by internal fixation. The first large series of cases
correction there. Bianco has reviewed shortening and angular was reported by Camera who resected a portion of the
correction procedures in the femur [418]. An intact strut can femoral shaft and then used the resected bone as an intra-
only be used for uni-planar corrections. For bi- or multi-planar medullary graft [422]. He reported on 32 cases with the
corrections (rotational or flexion/extension components) a average period of external fixation of 50 days. Moore
complete osteotomy is needed. reported on 13 femoral shortenings with bone resection with
Metaphyseal shortening osteotomy of the tibia and fibula the average amount of shortening obtained 2.5 in. and the
is recommended only if angular deformity necessitates such average period for complete union 2.5 months [423]. White
a procedure. The tibia should rarely be shortened by more reported on 45 cases of femoral shortening with a transverse
than 4 cm. The advantage of the metaphyseal site for osteotomy of the shaft, overlapping of the 2 fragments the
shortening is the rapid bony consolidation. Complications necessary amount and fixation by long metal pins which
can occur proximally in relation to the peroneal nerve with a were then incorporated into a hip spica cast [420]. Treatment
relatively marked shift in length and alignment. If possible, then followed with casting and eventual bracing. Many
diaphyseal procedures are recommended where tibial short- patients were under age 14 years; in younger children he
ening appears mandatory. shortened 0.5 in. more than necessary to compensate for the
subsequent overgrowth phenomenon. The quadriceps mus-
Effects of Opening/Closing High Tibial Osteotomies on cle always maintained its ability to fully extend the knee
Limb Length with no loss of strength detected. The average amount of
Magnussen et al. have done an interesting study assessing shortening obtained was 2.5 in. with a range between 2 and 3
changes in limb length that accompany high tibial osteotomy 1/8 in. Wilson and Thompson reported 5 femoral shortening
for angular correction [419]. Although the study was done in operations using the White technique with the average
adults, the size of the wedges and the amount of angular shortening of 2 1/8 in. obtained [121]. They reviewed the
correction are similar to what is accomplished in adolescent major series of lower extremity shortening procedures. In the
patients. The mean medial opening wedge was 9.3 mm. The works reported above plus their own, there were 98 femoral
entire limb length increased by 5.5 ± 4.4 mm with the tibia and 2 tibial shortening procedures for the 100 cases. The
increasing by 4.3 ± 2.3 mm. The mean lateral closing wedge only 2 tibial procedures were those reported by Brooke. The
was 8.0 mm. The entire limb length decreased by average shortening obtained was 2 in. and the complications
2.7 ± 4.0 mm with the tibia decreasing by 4.1 ± 2.9 mm. reported were relatively small involving separation of frag-
ments necessitating reoperation in 2 cases, infections in 7,
6.9.2.5 Diaphyseal Shortening (Resection) delayed union in 2, and no instances of nonunion or angular
The diaphysis can be shortened at skeletal maturity to equalize deformity. In comparison to the results reported during that
limb lengths. The exact amount of bone required is removed era for limb lengthening, the relative simplicity of the pro-
and the bone is stabilized with a metal plate, screws, or cedure was clearly shown.
6.9 Management of Lower Extremity Length Discrepancies 701

Fig. 6.21 Radiographs of 2 cases of diaphyseal shortening are shown, well. c and d Anteroposterior (c) and lateral (d) radiographs show the
one of a femur stabilized by an intramedullary rod (a–e) and one of a fixation device distally. e Anteroposterior radiograph of entire femur at
tibia and fibula with the tibia stabilized with an AO plate (f–m). 5 cm healing is shown. f Anteroposterior radiograph of tibia and fibula
were removed from the longer femur at skeletal maturity at open 1 week postsurgery. Tibia is stabilized with AO plate and patient is in a
operation. 5.5 cm were removed from the longer tibia and fibula in a short leg cast for comfort. g Anteroposterior radiograph at 6 weeks
15-year-old girl at skeletal maturity. a An anteroposterior film of the postsurgery. h Lateral radiograph at 6 weeks postsurgery. i Anteropos-
proximal 2/3rds of the femur approximately 10 weeks postsurgery terior radiograph at 4 months postsurgery. j Lateral radiograph at
shows the healing osteotomy site. There are 2 trans fixation screws 4 months postsurgery. k Anteroposterior radiograph at 9 months
proximally and 2 distally to serve as controls preventing rotation or postsurgery. l Oblique radiograph at 9 months postsurgery. m Lateral
lengthening at the osteotomy site. b A lateral radiograph at the same radiograph at 9 months postsurgery
time shows maintained position of the IM rod and healing progressing

The large majority of lower extremity diaphyseal short- An effective approach is to shorten the femoral diaphysis
ening procedures are still performed in the mid-diaphyseal or by resection following which an intramedullary rod is placed
proximal subtrochanteric region of the femur where negative to allow for rigid stabilization and immediate weight bearing
postoperative sequelae are less marked. There have been (Fig. 6.21a–e). Our preference is to do open shortening of the
reports of the inadvisability of performing shortening of the diaphysis to remove the diaphyseal segment of the bone.
tibia and fibula owing to increased problems with nerve or There are also reports of closed shortening in which the
vessel kinking or muscle control at the foot and ankle region osteotomy site is not opened so as to limit the incidence of
afterward. The muscle compartments are much tighter than infection and perhaps hasten the rate of healing by leaving the
those of the thigh and they have more structures passing bone removed beside the shortened diaphysis. The operative
through them in a smaller area. As a result, the lag effect and success rate is improved with the use of an AO universal
the compressive effect of vascular disruption are greater in intramedullary nail locked statically at both proximal and
the leg. Many reports of leg shortening with good results are distal sites [424]. Excellent results can be achieved if the
described below, however. complications of malrotation and distraction at the osteotomy
702 6 Lower Extremity Length Discrepancies

site are prevented. As with any femoral fracture or osteotomy should minimize or eliminate these problems. The optimal
in the non-childhood years, fat embolism can be encountered. site for femoral shortening was at the subtrochanteric region
Shortening of up to 2 in. or 5 cm can be performed readily in using an open approach and stabilization with an intrame-
the femur in one stage. There is concern about performing dullary rod with proximal locking. Extreme importance was
more shortening than this owing to the lag effect of the attached to preventing separation at the osteotomy site
shortening on the quadriceps muscle. In the large majority of postoperatively and in achieving appropriate rotation. After
studies performed, any quadriceps lag can be overcome usu- tibial shortening one problem, cosmetic in nature, was
ally within a year of surgery with intensive physical therapy. It complaint by the patient of localized increase in the bulk of
is, however, difficult to guarantee this effect if more than 5 cm the leg. For that reason, they recommended that tibial
of bone is removed. Two complications of the shortening shortenings be limited to 4 cm or less.
technique which must be guarded carefully against are stabi- Closed nailing had been shown to be reliable by Winquist
lization of the femoral fragments with inappropriate rotation or [428] and by Blair et al. who also expressed concern about
subsequent loss of close apposition of the fragments which not controlling rotation, possible instrumentation breakage, and
only increases the likelihood of delayed or nonunion but also the mass of bone which persisted and was occasionally
leads to a failure to gain a complete correction of the dis- troublesome [399]. They also felt that healing in the tibial
crepancy. Both the rotational and distraction possibilities with shortening procedure would be most enhanced with excision
intramedullary rodding can be minimized with application of a at the level of the flare in the lower diaphysis. They used
small four-holed AO side plate with a unicortical grip or with either a standard AO intramedullary nail or an AO plate but
use of a universal AO nail with static locking proximal and felt that with intramedullary nailing locking mechanisms
distal screws. Liedberg and Persson reported on 11 midshaft should be incorporated. Sasso et al. also demonstrated results
femoral shortening procedures where stability was provided after closed femoral shortening [429]. Shortening averaged
with a reamed Kuntscher intramedullary rod and a step 4.4 cm with a range from 3 to 5 cm in 18 cases. Compli-
osteotomy for rotational control [425]. D’Aubigne and cations included 1 episode of fat embolism and 3 cases of
Dubousset described excellent results with a step-cut or Z loss of fixation. They limited the shortening to 5 cm because
shortening followed by intramedullary stabilization and fixa- of concern about quadriceps lag and in no instance at this
tion with screws to control rotation [426]. Femoral shortening amount of resection were any negative sequelae noted with a
can also be done with correction of proximal and distal angular full knee range of motion and excellent strength regained.
deformity by removing appropriately shaped trapezoidal One possible but rare complication of closed intrame-
wedges. dullary nailing of the femur in association with shortening is
Wagner has commented on some of the important princi- avascular necrosis (AVN) of the femoral head. Mileski et al.
ples underlying diaphyseal femoral and tibial shortening have reported such a problem in an adolescent female who at
osteotomies [417]. Wherever possible he has recommended age 11 years underwent a closed intramedullary femoral
the use of an intramedullary nail as distinct from a side plate shortening [430]. The rod was subsequently removed after
and cortical screws since the intramedullary nail allows for healing. AVN was not diagnosed after rod placement but
early weight bearing. With femoral shortening he recom- was noted 15 months after rod removal. The authors felt that
mends removing no more than 6 cm of bone to avoid muscular the vascular insult may have been caused by using a rod
insufficiency and he supports the use of open osteotomy to entry point that was slightly medially placed although it was
remove the bony segment from the diaphysis. Attention to also possible that the rod removal had contributed to the
rotation is important. Even with a tibial and fibular shortening vascular insult. This represents the first report of this com-
osteotomy an intramedullary tibial nail is favored. Reaming of plication with femoral shortening although Herzog et al.
the medullary cavity is essential to insure a tight fit and [431] reported 4 cases of femoral head necrosis following
minimize the likelihood of rotational abnormalities. The tibia intramedullary Kuntscher nailing of femoral fractures in 26
is resected in its narrowest portion at the middle third. The children and O’Malley et al. [432] reported AVN in a
stable mechanical fit by an intramedullary rod makes the 13-year-old boy with a closed intramedullary nailing of a
diaphyseal site for shortening preferable to metaphyseal femoral diaphyseal fracture. This problem of AVN with
except for those disorders where axial correction is needed. femoral intramedullary rodding has also occurred with rigid
In a review of 46 limb shortening operations, 37 in the rod nailing for femoral diaphyseal fractures. Use of a tro-
femur and 9 in the tibia, Kenwright and Albinana felt that chanteric rather than piriformis fossa entry point for the rod
shortening as much as 7.5 cm could be done in the femur has greatly minimized this complication.
and 5 cm in the tibia in adults of normal height without any Broughton et al. reported on 12 patients who underwent
loss of function [427]. Major problems with the technique tibial and fibular shortening by diaphyseal resection to cor-
were technical in nature and involved inadequate stabiliza- rect limb length discrepancy over a 25 year period [433].
tion at the osteotomy site. Attention to detail, however, Each of the 12 patients did well with no major
6.9 Management of Lower Extremity Length Discrepancies 703

complications. A step-cut technique or Z-type shortening conditions in growing children characterized by prolonged
was employed for the tibia accompanied by a mid-fibular hyperemia in the neighborhood of the epiphyseal growth
resection of the required amount. The tibial fragments were plates there was overgrowth of the involved extremity. They
then stabilized by two screws. All operations except in two attempted to reproduce the earlier clinical finding by per-
were performed either at or just beyond skeletal maturity. forming lumbar sympathectomy in experimental animals but
The shortening achieved ranged from 2.5 to 5.1 cm. Normal in no instance were they able to reproduce growth stimula-
function and appearance were documented in all following tion on the ipsilateral side. They performed 70 lumbar
uneventful healing. A healing of tibial and fibular shortening sympathectomies in patients with poliomyelitis and assessed
at skeletal maturity using AO plate stabilization of the tibia the clinical response. Forty six were available for review.
is illustrated in Fig. 6.21f–m. They showed many instances where lumbar sympathectomy
enhanced the growth of the extremity. In 21 of 46 patients
(46 %) the shortness had become less in amounts varying
6.9.3 Procedures to Lengthen the Shorter from 1/8 of an inch to 1 in. The average age at time of
Limb surgery in this favorable group was 8½ years. In 8 cases
(17 %) the amount of shortness present at operation
6.9.3.1 Stimulation of Epiphyseal Growth Plate remained unchanged which still represented a positive
of the Shorter Limb response since the progressive shortening had ceased. Ben-
The earliest approaches to the treatment of lower extremity eficial results were demonstrated in 63 % of the cases. In 17
length discrepancy beginning in the late nineteenth century cases (37 %), the shortness progressed in spite of operation.
and continuing into the first half of the twentieth century In many of this latter group, the authors felt that effective
involved efforts to stimulate growth of the shorter side prior sympathectomy had either not been obtained or maintained.
to skeletal maturity. There was early recognition of the fact When they subdivided their assessment to include only those
that periosteal irritation frequently led to long bone over- with very clear sympathectomy 20 of 29 cases showed
growth and the therapeutic method which evolved from this diminution of shortness, 4 showed no increase in shortness
observation was an attempt to stimulate and irritate the and in only 5 did the shortness increase. Beneficial affects on
periosteum to allow for increased growth of the adjacent growth were thus increased to 82 %.
physis. Ollier was the first to develop this technique [159]. Barr et al. assessed the results in 1950 of 23 unilateral
He performed periosteal elevation in the rabbit tibia causing lumbar ganglionectomy procedures in patients with
overgrowth of 2–5 mm within 3 months. Over the subse- poliomyelitis [436]. Ipsilateral lumbar ganglionectomy had
quent decades many methods were used to stimulate the in some instances a stimulating effect upon the growth of the
periosteum including: cutting the periosteum, circumferen- shorter extremity. The most favorable interpretation of their
tial stripping or elevation of the diaphyseal periosteum, and results showed that the patients had an average decrease in
placing objects underneath the periosteum to allow for discrepancy of 1.5 cm, based upon projections of the
chronic irritation (these objects including ivory pegs). expected discrepancy. In the control group of 23 cases, 21
Several differing techniques were used subsequently both increased and 2 decreased with the average increase of
experimentally and clinically to enhance physeal growth. 1.8 cm; in the ganglionectomy group of 23 cases, 13
increased, 9 decreased, and 1 was unchanged with an overall
(a) Sympathectomy: In 1930 Harris reported on lumbar average increase in discrepancy of 0.3 cm. It was calculated
sympathectomy performed on the short side of a patient that the average decrease in discrepancy with ganglionec-
with poliomyelitis to take advantage of the observation tomy was 1.5 cm.
that those having sympathectomy for vascular disease
frequently developed vascular dilatation and increased (b) Surgically induced arteriovenous fistula: Observations
warmth of the affected side [434]. It was postulated that made in the late 1800s and early 1900s reported over-
this increased vascular response would enhance phy- growth of childhood limbs in patients who had sustained
seal growth. In many instances, overgrowth was indeed an arteriovenous (AV) fistula. Horton reported 23 cases
caused by the sympathectomies but its occurrence was of congenital arteriovenous fistula with overgrowth of the
unpredictable and rarely exceeded one cm. These involved extremity almost always seen [273]. In
techniques are not used today. mid-century, efforts were made to incorporate this
observation into clinical practice by surgically inducing
Harris and McDonald in 1936 reported on the response to arteriovenous fistulas in the mid-thigh region on the short
46 lumbar ganglionectomies [435]. Use of the procedure was side to treat developing limb length discrepancies. Janes
based on the observation that in certain pathological and Musgrove showed the growth stimulation effect of an
704 6 Lower Extremity Length Discrepancies

experimentally created arteriovenous fistula [437]. Janes (c) Elevation and stripping of the metaphyseal and diaphy-
et al. created the first AV fistula in a child with a short seal periosteum: Increased growth in length had long
limb due to polio and 10 years later reported results been noted after stripping of the periosteum of the
[438]. Mears et al. induced 55 fistulas and studied their metaphysis and diaphysis in several experimental animal
results in detail [439]. The fistula was placed between the procedures. An experimental study of stimulation of
superficial femoral artery and vein. There were no major longitudinal growth of long bones by periosteal stripping
cardio-pulmonary complications. Thirty-nine patients in dogs and monkeys was reported by Sola et al. [442].
were available for long-term review. All patients but 3 They assessed not only an initial periosteal stripping, but
had a short limb due to polio. In 28 there was either a the effects following a second stripping 1 or 2 months
decrease or at least no increase in the amount of dis- after the first procedure. Once again there was a tendency
crepancy. In 11 the limb length inequality continued to to show increased growth although it was not particularly
increase after the fistula was established. Thus 72 % of marked nor was it invariably seen. In operation on the
the group had a discrepancy which was diminished or dog involving a single stripping of the femur and tibia,
stabilized by the fistula. Lengthening of the shorter leg the mean increase in length was only 0.16 cm with 63 %
after establishment of the AV fistula was 0–0.5 cm in 11, showing increase of growth on the operated side. When
0.5–2.5 cm in 13, and 2.5–5 cm in 4. When continued two stripping procedures were done, the mean increase
shortening occurred after the AV fistula, the amounts was greater at 0.35 cm but still only 69 % of the animals
were still much less than would have been anticipated showed increase on the operated side. When two proce-
with 5 patients having 0–0.5 cm of additional shortening dures were done on the monkey, the increase was only
and 6 from 0.5 to 2.5 cm of additional shortening. The 0.17 cm although 87.5 % showed an increase. The
growth pattern following establishment of the fistula was stripping procedure was extensive going from growth
unpredictable and the response quite variable in terms of plate to growth plate in both femur and tibia.
extent.
A detailed study of the effects of stripping of the
Petty et al. assessed their results following surgical cre- periosteum in rabbits was performed by Wu and Miltner
ation of a femoral arteriovenous fistula to treat limb length [443]. Periosteal stripping was performed in variable parts of
discrepancy in 28 patients [440]. Of the fistulae made 21 of the right tibia and right femur. In all instances, there was
28 were done when the patient was felt to be the optimal 8 or overgrowth on the operated side. Twenty-two rabbits were
9 years of age. The fistula was created between the femoral used. The right tibia alone was operated on in 18 animals
artery and vein in the mid-thigh region. There were con- and both the right femur and tibia were operated on in 4.
siderable complications secondary to fistula creation Definite longitudinal overgrowth of the operated bone was
although none was limb threatening. Closure of the fistula observed in all instances except 3. The amount was small
was eventually performed in all by 16 years of age. Of the 28 and limited primarily to the first 3 months postsurgery with
patients operated, 17 subsequently had an epiphyseal arrest the overgrowth varying from 0.5 to 6 mm.
on the opposite side. The average length discrepancy in this Chan and Hodgson applied this procedure to 45 patients
group was 4.6 cm at time of fistula creation and the average suffering from poliomyelitis with the operations done
increased to 5.9 cm at the time of epiphyseal arrest. Eleven between 1961 and 1968 [444]. The age at surgery ranged
patients did not have an epiphyseal arrest and in this group at from 5 to 13 years and all patients had a short limb at the
fistula creation the average discrepancy was 4.1 cm and at time of surgery averaging 3.4 cm (1.1 to 9.5 cm). The
fistula closure it had decreased to 2.4 cm. Only 9 of 28 periosteum was completely stripped with an elevator in both
patients (32 %) showed a decrease in length discrepancy of the femur and tibia. A definite overgrowth >4 mm was noted
more than 1 cm as a result of an AV fistula alone. in 31 patients (69 %); there was no significant increase or
Twenty-one of 28 patients (75 %) showed no further loss of growth over the normal side in 9 (20 %); and there
increase in discrepancy however. The authors concluded that was a continuing shortness in 5 (11 %). In the favorable
“artificially created arteriovenous fistulae can accelerate group, the average overgrowth was 1.3 cm during the mean
growth in the lower extremity, but the results vary greatly time period of 9 months with a range from 0.6 to 4.4 cm. In
and are unpredictable”. By the early 1970s, both Janes and many patients the overgrowth affect was noted to persist as
Sweeting [441] and Petty et al. [440] no longer performed or long as 1, 2, and even 4 years postsurgery. The average
recommended the procedure for treatment of length period of stimulation, however, could not be determined
inequality. accurately. The authors concluded that it was best to do the
6.9 Management of Lower Extremity Length Discrepancies 705

stimulation operation when the child was 8 years of age. No third of the shaft. This served to disrupt the medullary blood
meaningful or long-term complications were seen. supply. When animals without fracture during the postoper-
Jenkins et al. studied 13 of these patients at a later time ative phase were assessed there were no differences in length
from 3 to 5 years postsurgery [445]. They continued to note of either the tibia or femur. This led to the conclusion that
some degree of stimulation but again a variable response. In trauma sufficient to interrupt the medullary blood supply but
28 femurs assessed 3–5 years following periosteal stripping, not great enough to cause regional hyperaemia did not con-
there was an increase in growth of a mean 0.5 cm in 17, a sistently cause growth stimulation. Fractures were then made
decrease in growth of 0.81 cm in 8, and no change in 3. In in the femur and tibia between metal markers to assess the
the 26 tibias, 18 had an increase of 0.75 cm, 5 a decrease of overgrowth phenomenon. They demonstrated in agreement
0.5 cm, and 3 were the same. with Bisgard that overgrowth of a long bone may occur fol-
lowing fracture even without shortening and that the longi-
(d) Short-wave diathermy: Doyle and Smart reported that tudinal overgrowth occurred entirely from stimulation of the
short wave diathermy enhanced epiphyseal growth in epiphyseal growth cartilage. They then assessed patients in
rats [446]. Preliminary experiments indicated that a relation to growth of tibias from which bone grafts had been
temperature of 40 °C would be effective to induce taken. Growth arrest lines were used for the assessment. Small
increased growth without tissue damage. The right amounts of increased growth ranging from 0.1 to 0.8 cm
lower extremity was maintained at this temperature occurred following tibial bone grafts. The growth stimulus
throughout the treatment. Twenty female rabbits were lasted only as long as healing of the defect site was occurring.
used in which insulated copper plates, 2 by 8 in., per- They concluded that minimal trauma to the shaft or to the
mitted the administration of diathermy to 4–6 animals metaphysis of the long bone with or without interruption of
via a short wave medical diathermy apparatus. Treat- the medullary blood supply did not produce any definite
ment was directed to the epiphyses of the right knee. increase of longitudinal bone growth. Gross trauma such as
The animals were treated for 0.5–1 h each day or on caused by a fracture or a removal of a large segment of bone
alternating days from the 21st to the 70th day of life. for grafting both of which necessitated extensive bone repair
The average total duration of diathermy was 25 h. Of did reproduce epiphyseal stimulation and increased longitu-
those animals appropriate for assessment, the treated dinal overgrowth. The increased rate of growth continued
right hind limb was longer than the untreated left in all during the period of healing but not much beyond. The growth
instances. The increase in the combined length of the stimulation appeared secondary to the hyperaemia which
treated tibias and femurs varied from 0.4 to 2.8 mm included the epiphyseal region.
averaging 1.4 mm. The advantage of the procedure was Wu and Miltner reviewed clinical situations where
that it would increase the temperature in tissues at overgrowth occurred [443]. Metaphyseal and diaphyseal
deeper levels without causing bodily damage. Dia- fractures were known to cause overgrowth in long bones in
thermy acted by producing deep tissue heating and children. Infection could clearly damage growth if it
increased blood flow. Granberry and Janes repeated the involved the physeal cartilage but in instances where the
experiment on the dog, without showing beneficial physeal cartilage persisted the increased hyperaemia lead to
effects [447]. They used microwave diathermy to overgrowth. They performed several experiments on rabbits
increase the temperatures 3–5° centigrade in the tibia. aged 5–8 weeks to assess growth phenomena. Group 1.
In their experiment, one knee each of 7 young dogs was Insertion of foreign material into a drill hole placed imme-
heated by microwave diathermy at 100 watts for a total diately distal to the proximal epiphyseal cartilage of the tibia.
of 100 h but they noted no significant growth alteration. The foreign materials included cotton, gauze, paper, wood,
(e) Efforts to stimulate epiphyseal growth by insertion of brass and iron shot. There was no difference in length of the
multiple implants and creation of bone damage in the bones operated. Group 2. Indirect interference of circulation
metaphyseal regions: Wilson and Thompson noted that of bone. The epiphyseal circulation was left intact but the
the many attempts to stimulate epiphyseal growth had not nutrient arteries and periosteal vessels were damaged
been successful enough to warrant clinical use, a feeling extensively. There was no appreciable change in longitudi-
still prevalent after additional attempts since then [121]. nal growth of the bone after the experiments. Destruction of
the nutrient artery and the extraperiosteal blood supply in
Compere and Adams addressed the effects of trauma to the particular caused no changes in the longitudinal growth of
diaphysis on subsequent longitudinal growth, performing 2 the bone agreeing with the extensive studies of Ollier and
series of experiments in rabbits with limited trauma [448]. Haas. Group 3. Curretage of bone marrow. The tibial bone
Three drill holes were placed through both lateral and medial marrow was curreted through a metaphyseal drill hole. There
cortices near the upper and lower metaphysis and in the mid was no significant change in the length of the operated
706 6 Lower Extremity Length Discrepancies

bones. Group 4. Stripping of the periosteum. Many variable Into each of these tunnels in the femur and tibia, a beef bone
patterns were used and in virtually all instances definite peg was inserted across the bone diameter. In some, ivory
longitudinal overgrowth of the operated bone was seen from pegs were inserted for comparison. In one part of the series
0.5 to 6.0 mm. performed at a hospital, no leg subjected to a single opera-
Chapchal and Zaldenrust assessed the effect of various tion showed any evidence of acceleration of growth. It was
metals, metal alloys, and ivory placed in the metaphysis and felt, however, that documentation might have been some-
also in the epiphyses of the bones comprising the knee joints what inadequate. In a second series at another hospital, there
of several animals [449]. They concluded that some length- were groups in which effective stimulation took place, some
ening of the bones was obtained but that the amount was in which the operation had no effect on progression and
minimal and uncertain. Pease attempted to assess overgrowth some where the operation was fully ineffective. Only limited
using foreign bodies in the metaphyseal regions [450]. His conclusions could be made. The operation appeared more
work also involved clinical investigation of the phenomenon. effective in younger children than in those close to puberty.
He placed transverse screws across the entire metaphyseal The best that could be said was that the growth was stimu-
diameter of distal femur and/or proximal tibia using vitallium, lated somewhat in 12 of 28 patients with best results seen
stainless steel, vanadium, and ivory screws. Two screws per when operation was done between 6 and 12 years of age.
region were used. In all cases, stimulation of growth followed Once again some stimulation was noted but it tended to be of
operation to a variable degree and there were no deformities a small amount and unpredictable.
indicative of asymmetric stimulation. The screws were placed
parallel to each other and to the adjacent growth plate and 6.9.3.2 Lengthening of the Diaphysis
extended to or slightly through the opposite cortex. Seven
patients were operated with two screws placed in tibia and
femur in most with one patient having the procedure only in (a) Clinical Approaches 1900–1960s: Much interest in,
the femur. The operation was repeated occasionally. Seven- and occasional efforts at, lengthening lower extremity
teen segments were stimulated. In two instances, there was no long bones were reported in the nineteenth century. No
overgrowth while in the others the overgrowth stimulation acceptable techniques evolved but valuable biological
varied from 0.1 to 2.2 cm. The mean length increase for 17 and mechanical principles gradually became evident
cases with growth stimulation was 0.7 cm. with continued work over the next several decades.
Carpenter and Dalton repeated the clinical work in 30
cases in which epiphyseal stimulation was attempted by the Codivilla: Well-documented attempts at long bone
use of intramedullary implants in a distal femur and proxi- lengthening date from accounts by Codivilla of Bologna,
mal tibia [451]. Periosteum was elevated and a cortical Italy in 1903 and 1905 [453]. He began one of his articles
window made to the metaphyseal side of the distal femoral with the following still relevant sentence: “The difficulties to
and proximal tibial growth plates. The medullary canal was be encountered in lengthening a shortened limb are found in
curetted and the cavity then tightly packed with small chips operation to be greater as regards the fleshy parts than as
of ivory. Each patient was followed for a minimum of regards the bones.” Following osteotomy or fracture of the
2 years with radiographic and clinical measurements made at bone, skin traction had been applied characteristically with
3 month intervals. Some increase in growth was obtained in limited effect due to pressure necrosis of the skin, pain, and
26 of the 30 patients. The gain was 1/8 of an inch in 10, 1/4 the fact that much of the force applied “did not reach the
of inch in 11, 0.5 of an inch in 3, 3/4 of an inch in 1 and 1 in. skeleton”. Codivilla went on to describe the evolution of his
in 1. In 70 % of the cases, the maximum gain was only 1/8– technique which eventually involved osteotomy of the bone,
1/4 of an inch and it was concluded that the degree of application of a hip spica cast, removal of the foot portion of
stimulation was neither great enough or predictable enough the cast, application of a force directly to the skeleton with
to warrant clinical use. transverse placement of a calcaneal wire of 5–6 mm diam-
Tupman reported a detailed study to stimulate bone eter, incorporation of the transverse pin and two side bars
growth by inserting beef bone pegs into the epiphyseal and into the plaster cast, cutting of the cast at the level of the
metaphyseal region in children [452]. The first clinical osteotomy and application of counter-balanced traction to
attempt to stimulate growth in dogs by introducing ivory gain length immediately followed by completion of the cast
pegs into the femur and tibia was in 1869 by von Langen- at the desired length until healing. He reported on 26 patients
beck who claimed 1 cm overgrowth in 3.5 months. Tupman who gained between 3 and 8 cm. Freiberg supported the
reviewed 28 patients who had a total of 51 operative pro- validity of the approach using it following a femoral fracture
cedures. Insertions of material were in the distal femoral and in a 9-year-old boy to reduce a 2 1/4 in. shortening to 0.5 in.
proximal tibial metaphyses. Three tunnels were made with a 5 weeks after injury [454]. A double skeletal transfixator
drill in the metaphysis close to but not involving the physis. method, after the length had been achieved, then evolved.
6.9 Management of Lower Extremity Length Discrepancies 707

Ombredanne: Ombredanne began utilizing principles Magnuson described femoral lengthening of 2.5–4 in. in
subsequently incorporated in more formal apparatuses at 14 patients using double transfixion wires and plaster with
later times [455]. In 1913, he described an oblique osteot- lengthening at one sitting by skeletal traction over 20–
omy and lengthening of the femur slowly and gradually with 30 min [458]. The osteotomy was a Z-type to allow for bone
an apparatus fitted to the side of the thigh and working contact to enhance healing. The apparatus was left on for
against one pin inserted above the osteotomy site and one 30 days following which a cast was applied. In 10 cases of
below. He achieved up to 4.0 cm lengthening but no detailed femoral shortening which were operated the lengthening
follow-up of the procedure was performed. varied from 3 to 4 in.
Putti: The next technical advance in limb lengthening was Abbott: Abbott directed his attention to lengthening the
described by Putti also of Bologna, in 1921 [456]. He tibia and fibula in patients with poliomyelitis and reported a
described a need for lengthening of the femur when the method of lengthening in 1927 that gained wide acceptance
discrepancy was >2 in. and questioned early on whether [459]. He used Putti’s concepts of skeletal traction and
lengthening of such a magnitude was “possible without countertraction but developed a lengthening apparatus which
damaging the muscles, nerves and vessels.” His own work was the true precursor of apparatuses used even now. His
and that of Magnusson then showed the possibility of method employed a preliminary Z-lengthening of the tendo
lengthening safely by 2–3 in. Putti defined the need for Achilles, oblique osteotomy of the distal third of the fibula,
continuous traction. He developed a unilateral distraction insertion of single proximal and distal 3/16 “ wide traction
apparatus for lengthening the femur which used two large pins which passed completely through the limb to enable
transcortical metal pins on either side of the osteotomy held fixation to a biplanar distraction apparatus to control angu-
apart by a telescoping tube which contained a strong spring lation, circumferential division of the periosteum at the
press moved by a screw. The apparatus was designed to be osteotomy site, a Z-type tibial osteotomy, and a delay in
sufficiently strong to overcome resistance, to stabilize the lengthening from 7 to 10 days postsurgery. The two dis-
osteotomy site and the alignment, and to provide traction. traction devices consisted of telescoping brass tubes and a
Gradual traction was applied to separate the bone fragments strong coil spring with lengthening performed by turning of
but the time taken for lengthening was not reported. a thumb screw. The limb was immobilized on a Thomas
Putti elaborated on operative lengthening of the femur in splint to which were also attached two metal stabilizers to
1934, with an altered technique [457]. He stressed that prevent anterior angulation. Lengthening was done once
operative bone lengthening was particularly valuable during daily and ranged from 1/16th to 1/8th of an inch. In his
the period of “childhood when the reparative power of the initial report, the maximum amount of length which Abbott
long bones is most vigorous thereby minimizing the danger felt could be achieved safely was 2 in. (5 cm). The entire
of non-union.” Skeletal traction mandated the use of skeletal time in traction was between 3 and 4 weeks. Overall the
countertraction. The Z-osteotomy was replaced with an apparatus remained in place for 8–10 weeks at which time
oblique osteotomy. The patient was then put in bed with the limb was immobilized in a cast. The external apparatus
traction placed on the upper wire. Increasing traction was and the wires were removed from 4 to 5 months after surgery
applied to the lower Kirschner wire until the desired length when there was sufficient callus to permit the patient to walk
usually from 2.5 to 4 in. was attained. This gradual skeletal with a splint. Abbott indicated that “in every case treated by
traction usually required 18–21 days. A hip spica cast was this method callus filled in this space lying between the
then applied with the transfixion wires included at which fragments in a comparatively short time.” The operation was
time the traction was discontinued. Results were reported in performed in those cases with 1.5 in. or more of shortening.
11 patients. Putti added that “in the eleven cases I have Abbott warned of the extreme care needed in the postoper-
treated by this method no complications whatever arose ative phase and suggested “the surgeon who has not the time
other than a single case of temporary ‘toe drop’ caused by to give for daily adjustment of the apparatus should leave it
overstretching of the external popliteal nerve presumably entirely alone.” Abbott then provided a very detailed account
due to faulty position of the knee. The paralysis promptly of his first six patients of whom one was regarded as a
cleared up with rest.” The concept of gradual traction was failure. In the other five, the gains in length varied from 1 3/4
introduced and Putti indicated that the time required to to 1 7/8 in. with all these increased lengths secured from 21
obtain the desired lengthening was approximately 20 days. to 28 days. Union of the fragments sufficient to allow for
The period of immobilization in the corrected position was weight bearing in a splint was present in all cases from 4 to
4 months in a plaster cast with an additional period of lim- 5 months after surgery and in all the completed cases con-
ited support for several months in which physical therapy solidation of the callus had taken place in 6 months. The
continued. No indication was made of the amount of amount of lengthening was initially kept relatively small
lengthening achieved. because of concerns of negative sequelae with larger
708 6 Lower Extremity Length Discrepancies

lengthenings. The recommendation and determination of the approaches in great detail. Abbott noted that the tibia and
initial paper, however, allowed for increasing lengthening to fibula provided much more dependable results than the
2 in. femoral lengthenings which had more complications [462].
The next year Abbott and Crego reported on a similar There were only two incomplete fractures following the
procedure for operative lengthening of the femur [460]. tibial lengthenings but 7 fractures occurred through femoral
Eight cases were reported with the gain in length ranging callus during the early weightbearing phase. There were
between 1.5 and 3.5 in. The authors felt that with experience three cases of nerve paralysis. One involved paralysis of the
in an average case a gain of 2.5 in. could be secured without sciatic nerve associated with subluxation of the knee.
producing injury to the blood vessels or nerves. The prin- Infection of the pin sites was commonly seen but only one
ciples again involved osteotomy of the bone, direct bone deep osteomyelitis occurred.
traction which was gradual and continuous, maintenance of Other Reviews of Abbott Technique: Following Abbott’s
alignment and contact of the fragments obtained by trans- development and subsequent refinement of his technique
verse wires entirely through the limb above and below the limb lengthening enjoyed a surge of popularity. Three major
osteotomy and attached to the screw extension pieces. The reviews of relatively large series of the Abbott procedure
osteotomy was accompanied by sectioning of deep fascial appeared in the 1930s. These series served to define both the
structures, the iliotibial band, and the biceps tendon to benefits and negative aspects of these interventions although
diminish the resistance of the soft parts. The authors the detailed descriptions of the complications considerably
emphasized that “by far the most important and difficult part diminished enthusiasm for lengthenings. Haboush and
of the entire procedure is the postoperative care of the patient Finkelstein reported on 17 tibial-fibular lengthenings, 16 of
during the lengthening process.” The lengthening began which were done for poliomyelitis [463]. They used
approximately 5–6 days after operation. Lengthening was Abbott’s technique although they did modify his apparatus
performed once daily with the average daily gain of 1/8 of an somewhat. Their major problems involved
inch and the entire time of traction extending over a period (1) anterior-medial angulation during the bone lengthening
4–5 weeks. The apparatus was removed and a cast applied in process; (2) more tibial elongation than fibular; (3) valgus of
10–12 weeks, protected weight bearing was allowed at the foot; (4) equinus of the foot; (5) osteomyelitis both at the
5 months, and full weight bearing in 7–8 months. site of the tibial osteotomy and at the site of pin insertions;
Abbott next presented a review of 48 tibial and fibular (6) delayed union leading in some instances to nonunion.
lengthenings in which he again stressed his three principles: The modifications of their apparatus were designed to
(1) to lengthen a bone traction and countertraction must be address these problems. One of their major conclusions was
taken directly on that bone; (2) to overcome the elastic that the fascia in particular yielded poorly to lengthening
resistance of the soft parts the traction must be slow and which led to both the angular problems and the severe pain
continuous; and (3) after osteotomy and the application of which they described as being “a rather constant feature in
traction complete control of the fragments including their this series of cases”. One of the major changes in their
appropriate alignment must be maintained during the operative technique was a more thorough division of the
lengthening process [461, 462]. The apparatus evolved to the fascia in the limb along with the interosseous membrane and
use of two pins above and two below the osteotomy site. The complete circumferential division of the periosteum of both
lengthening began only when all swelling had disappeared tibia and fibula.
which was usually at 7–10 days. In the 48 patients, the gain A more positive review was published in 1935 by
in length ranged from 1.5 to 3 1/4 in. The average time of Brockway who provided a clinical resume of 46 leg
union to permit weight bearing with a splint was 4–5 months lengthening operations using the Abbott technique and
and consolidation of callus generally occurred in 6–7 months apparatus [464]. He provided average values for the key
with restoration of the medullary canal in 10–12 months. criteria in his long range assessment although ranges of
The complications listed by Abbott were surprisingly numbers and case by case details were not given. The
infrequent involving 2 fractures, one worsening of paralysis average age of the patient was 14 years. His report is most
of the dorsiflexor of the foot, and one infection which instructive to note in comparison with current approaches
involved an osteomyelitis of the bone, and occasional using the distraction osteogenesis principles. The number of
over-lengthening of the tibia allowed for valgus deformation days following surgery before the lengthening process was
at the ankle. started averaged 7.5. The average length obtained was 1 and
In a later paper, Abbott reviewed his results in 73 pro- 9–10 in. The number of days required to obtain this length
cedures of which 48 were in the tibia and fibula and 25 in the was 35 making the average daily increase in length 1.3 mm.
femur [462]. In the femur, the maximum gain was 3.5 in. and The average time before the pins were removed (at which
the minimum 1 in. This paper describes the technique of the time the long leg cast was applied) was 11.4 weeks. The
apparatus and the intraoperative and postoperative average time that plaster was worn following the removal of
6.9 Management of Lower Extremity Length Discrepancies 709

the pins was 13 weeks and the average time before full pain should be experienced.” When sufficient callus had
weight bearing was allowed postsurgery was 9.5 months. formed as indicated on X-rays but prior to firm union the
Brockway did note complications with the procedure but his plaster cast and wires were removed, any malalignment was
overall impression was positive and he indicated that “on the adjusted, and the limb was stabilized in plaster until union
whole, very gratifying results have been obtained by this was complete. Femoral lengthening produced greater diffi-
operation and it is now a routine procedure.” Poor results culty than tibial. In 47 cases of tibial-fibular lengthening, the
involved fracture of the tibia, some cases of skin slough, average time before weight bearing was 6.5 months and the
delayed bone healing, and anterior bowing of the tibia. average lengthening obtained was 2 and 1/3 in. In 40
The most critical of the early reviews was published in femoral lengthenings, the average time for weight bearing
1936 by Compere [465]. The large list of complications which was 5 months and the average lengthening obtained was 1
he referred to led him to the belief that shortening was by far and 5/8 in. The technique was evolving during the course of
the more preferable approach to limb equalization. The paper the series. Allan felt that bone was laid down in parallel lines
describes five patients in detail each of whom had a large between the fragments with the osteogenic material strung
number of complications. There is no indication as to how out across the gap and that most of the repair came from
many patients were operated. The discussion focuses on the periosteum. He noted that all bones returned to normal
negative aspects of the procedure but neglects any positive radiographic appearances within a year or so of consolida-
indications either in his own work or the work of others. tion. Union eventually occurred in every case but there was a
Compere listed 14 complications which, in the absence of any marked delay in 12 cases, union taking from 9 to 16 months.
indication that he had any good results at all, clearly led to a Although Allan recognized that the most resistant structures
major dampening of enthusiasm for this intervention in par- to stretching were the periosteum, the interosseous mem-
ticular in North America. The complications he listed follow: brane, and the deep fascia, he specifically mentioned that
(1) stretch paralysis of the sciatic or the external popliteal these were to be left intact as much as possible. The blood
nerve; (2) increased weakness of lengthened muscles in old vessel response distal to the lengthening site was benign. He
cases of poliomyelitis; (3) fracture of the osteotomy; felt that the external popliteal nerve and the compartment
(4) malunion; (5) delayed union or non-union; (6) os- part of the great sciatic nerve could be stretched to 2 in. in
teomyelitis from wound infection; (7) traumatic arthritis and the thigh without losing function and that it could be stret-
limitation of motion in the knee; (8) late fracture; (9) pressure ched to 3 in. with only temporary impairment. He attributed
or stretch necrosis of the skin in the zone of lengthening; vascular complications experienced by other surgeons to
(10) necrosis of bone due to excessive subperiosteal stripping subperiosteal bone exposure and to dividing the periosteum
which also might increase the likelihood of infection; and fascial structures transversely.
(11) malposition of the foot due to rotation following Anderson: The next technical advance in limb lengthen-
lengthening; (12) circulatory disturbance with prolonged ing came from adoption of the technique described by WV
oedema in the lengthened limb; (13) displacement of the head Anderson of Edinburgh, Scotland in the mid-1960s [467].
or of the distal end of the fibula when this bone is not This technique evolved from an operation in 1954 but he did
lengthened as much as the tibia; and (14) protrusion of the not report the technique in detail until 1967. He described
osteotomy fragment of the tibia through the skin. Complica- the evolution of his technique from that of Abbott which was
tions have characterized lengthening procedures from the practiced with some modifications in his institution for
beginning. Table 6.4 lists the large group of possible disor- approximately 20 years prior to 1954 with “satisfactory
ders. Many of these are seemingly inherent with the proce- results obtained, with increases of length up to 3½ in. in the
dures, but awareness should help minimize them. tibia, with no complications of any severity.” We quote two
The major English language orthopedic journals did not paragraphs from Anderson’s paper which eloquently review
publish any subsequent large limb lengthening review for the subsequent reactions to the Abbott procedure after its
12 years until the report of Allan [466]. He used a tibial seemingly excellent early results. “Unfortunately, this pro-
distraction apparatus which incorporated the principles of cedure appeared to be so simple and satisfactory that its
Abbott and some modifications of Haboush and Finkelstein rapid and deserving popularity almost brought about its
as well as his own. An oblique osteotomy of the bone was eclipse. It was performed widely by many surgeons who
performed to enhance repair after lengthening. To minimize failed to realize the fundamental importance of suiting the
angulation, Allan applied a plaster of Paris long leg cast and operation to the patient; by this failure they were directly
incorporated it into the distraction apparatus with the cast cut responsible for the numerous and terrifying complications
at the osteotomy site. The periosteum was left intact and a which followed this tragic misuse. These ranged from gross
careful technique sought to minimize damage to the soft sepsis to amputation, following vascular failure. The pro-
tissues. Distraction began immediately and proceeded at a cedure was condemned loudly and bitterly because it pro-
rate of 1/16 of an inch per day. Allan reported that “little duced too much pain and was technically difficult and
710 6 Lower Extremity Length Discrepancies

Table 6.4 Possible complications in limb lengthening procedures Table 6.4 (continued)
Bone Premature union • motor: partial paralysis; paralysis
• incomplete osteotomy Vessel Intraoperative phase
• long latency period prior to beginning lengthening • pin skewers vessel
• interruption of lengthening due to other causes • vessel damaged during osteotomy
Malunion Distraction phase
• angular deformity/axial deviation • excess vessel stretching
• unstable apparatus Compartment syndrome
• imperfect pin placement or initial malalignment Hypertension
postosteotomy
• excessive arterial stretching
• altered muscle pull with increased extent of
lengthening Thrombophlebitis
– proximal femur—varus Joint Stiffness: ankle equinus, knee extension, hip
adduction/flexion contractures
– distal femur—valgus
Cartilage degeneration
– proximal tibia—valgus
Subluxation
– distal tibia—varus
• knee
• tibial lengthening greater than fibular → valgus
ankle • hip
• angular deformation post fixator removal Dislocation
(softened bone at distraction site) • hip
Scanty union Septic arthritis (pin placed into joint cavity)
Delayed union Physeal Increased growth of femur following completion of
Nonunion cartilage lengthening*
Osteomyelitis Diminished growth of tibia following completion of
lengthening*
• lengthening site
*in relation to pre-lengthening growth rates
• pin-tract site
Osteoporosis
destructive by those who had, in fact, made it so them-
Late fracture
selves.” For many years, it was practically given up in the
• shortening land where it originated. The alternative methods of equal-
• angular deformity ization were more fully developed in particular of epiphys-
Pin/wire pull-out iodesis and stapling, while shortening the longer side was in
Skin Pin-site irritation/infection much more favor than lengthening.
Skin slough 2° malaligned fragment pressure Eventually, the Edinburgh group where Anderson worked
Fragment protrusion
modified the apparatus and the procedure and began dis-
traction on the operating room table. They noted that
Muscle Fibrosis
“contrary to the findings in America, we had none of the
Contracture
complications which ended in the condemnations of the
Weakness operation. The pain was minimal, even from the day of the
• myopathic operation. There were no vascular or neurologic changes of
• neurogenic any importance and no major sepsis occurred. The operation
Pin skewers muscle to compromise mobility was accepted by operating and nursing staff, and the patient,
Nerve Intraoperative phase as a simple routine procedure. One complication which was
• pin skewers nerve
reported by others and appeared in the Edinburgh series was
the slowness of the lengthening of the fibula which led to
Distraction phase
valgus deformation of the ankle. This was greatly minimized
• excessive stretching; sciatic, peroneal, posterior
by a distal tibial-fibular synostosis obtained prior to the
tibial, radial nerve
lengthening procedure. The Anderson technique as subse-
• sensory: paresthesia, hyperesthesia, anesthesia,
transitory, permanent quently practiced evolved in a patient who suffered a
(continued) transverse fracture of the mid-tibia as he was waiting for a
more formal Abbott type lengthening. This opportunity
6.9 Management of Lower Extremity Length Discrepancies 711

presented itself such that the apparatus was applied for sta- Non-union occurred in 4 patients but satisfactory union was
bility and lengthening was performed. There was surpris- subsequently obtained with bone grafting. The authors
ingly good maintenance of alignment and it was noted that modified their technique to do a bone grafting procedure in
the lengthened site healed readily. The advantage of this all patients who at 4 months after surgery “show lack of
approach was that a very small linear incision only was complete bone bridging”. There were no wound infections
required, there was very little disruption of the periosteum and only pin tract infection. There was no permanent
and the transverse osteotomy was made following posi- detectable injury to the neurovascular structures.
tioning of several transcortical drill holes and manual Manning reported on a large series of femoral and tibial
osteoclasis. There was a 1/8 in. lengthening on the operating lengthenings using the Anderson apparatus and technique
room table with subsequent lengthenings starting on the 3rd [470]. The patient remained in bed for the lengthening
or 4th day of 1 turn daily equaling 1/16 of an inch. Anderson procedures with the distraction apparatus supported in trac-
indicated that “in the average case the length of 2–3 in. may tion. Distraction took place once daily with a gain of 1/16 of
be obtained without difficulty” and “up to 1 and ½ in. can be an inch (1.6 mm). Once length had been achieved and early
expected before any tendency to foot deformity healing was underway the patient was transferred to a cast
(tendo-Achilles tightness) becomes evident.” The patient and ultimately to a brace. Lengthenings were done with 211
remained in bed with the limb suspended in a traction frame. procedures performed, 161 on the tibia, and 50 on the femur.
When new bone was seen radiographically external fixation The average gain was 3.06 cm with 122 of the tibias
was removed and the limb was immobilized in plaster. lengthened 5.0 cm or more, and 21 femurs 5.0 cm or more.
Anderson noted empirically that 1/16 of an inch increase per Major complications related to fracture after lengthening had
day remained the most satisfactory rate of lengthening. been completed and delayed union or nonunion. Fractures
Anything less may predispose to early bone union with occurred in 30 limbs (14 %). These all subsequently united
callus formation overcoming the rate of lengthening, thus relatively quickly but shortening occurred in many lessening
preventing full length desired being obtained, and a faster the advantage of the original lengthening procedure. Bone
rate may cause delay in union, considerable pain and pos- grafting was resorted to for slow union or nonunion present
sible nerve and vascular complications. Anderson specifi- 6 months after the lengthening had been completed. Such
cally remarked that “pain is so exceptional (in their grafts were used in 23 tibia lengthenings (14 %) and 2
lengthening unit) that apart from the immediate postopera- femoral lengthenings (4 %). Each then healed uneventfully.
tive pain (in itself minimal) it is regarded as indicative of Chacha and Chong reported on overall favorable results
something abnormal and subsequently of importance.” Fol- with 35 tibial lengthenings (31 Anderson type) with an
lowing new bone formation deemed sufficient to prevent average gain of 5.2 cm and a range of 2.8–6.5 cm [471].
collapse, the apparatus was removed and the limb was Malhis and Bowen reported on 12 tibial lengthening using
placed in a long leg cast followed by eventual transfer to a the Anderson method [472]. The mean amount of length-
walking brace. Femoral lengthening was performed in a ening achieved was 6 cm (range 3–10 cm) and the mean
similar fashion. percent lengthening was 24.5 % (range 13–42 %). Bosworth
A major review of the Anderson approach was published used the Abbott technique but recommended not beginning
by Coleman and Noonan from the Salt Lake City Shriners lengthenings until 10 days after the osteotomy [473].
Unit in 1967 [468] and by Coleman and Stevens in 1978 One-Stage Lengthening Procedures. Le Coeur: Le Coeur
[469]. Thirty-one tibial lengthenings using the Anderson of Paris described a one-stage lengthening procedure with
technique with limited surgical exposure were reported. immediate stabilization and applied the technique 169 times
They performed the Anderson technique as he had described between 1952 and 1962 [474]. The amount of length gained
it emphasizing “the advantage of osteotomy of the tibia by a varied between 3.0 and 4.7 mm and owing to the immediate
limited surgical exposure, in which the hematoma remains fixation used the amount gained was maintained with cer-
localized, periosteal stripping is avoided, there is relatively tainty. The period of immobilization was similar to that used
little soft tissue damage, and the periosteal tube is preserved. for fracture repair. The tibia was the more favorable site for
A distal tibial-fibular synostosis was performed initially prior lengthening but femoral procedures were also performed.
to lengthening to prevent valgus deformity at the ankle but The operation was accompanied by multiple transverse
eventually stabilization was done using a transfixion screw at muscle and fascial releases which allowed for lengthening of
the same time as the lengthening procedure. A cast was the soft tissues readily in conjunction with the bone elon-
applied at the same time as the distraction apparatus and gation. A lengthy oblique incision of the tibia was made
osteotomy to help minimize the development of equinus at following which a bipolar traction apparatus was applied to
the ankle. Distraction was at the rate of 1/16 in./day. In the upper and lower regions of the tibia. Once the soft tissue
thirty-one patients satisfactory union was obtained in all with releases had been performed, the oblique osteotomy made,
an average gain in length of 5.0 cm (range 2.0–6.0). and the traction apparatus positioned, lengthening began
712 6 Lower Extremity Length Discrepancies

with the knee in partial flexion. The knee remained in flexion performing the procedure twice. The operation was done
during the lengthening procedure. Once the desired length with relatively strict limits in terms of the maximum amount
had been reached and the surrounding muscle and fascial of lengthening that could be achieved owing primarily to
tissues released, osteosynthesis was performed with four or limitations involving stretching of the sciatic nerve. One
more transverse screws. No cast was used at this stage. The hundred and eleven of the 175 cases were concentrated in
patient remained in bed with the knee flexed for approxi- the lengthening range of 3.1–4.0 cm. The usual limit in the
mately 45 days to allow healing to occur. The patient then child was 3.5 cm and maximum lengthening to be aimed for
began walking with crutches. If a long leg cast was needed it in the adult was considered 4.5 cm. The extent of length-
was placed with the knee still flexed. Le Coeur indicated that ening was in the range of 10–15 % of the length of the
femoral lengthening could also be done with the same femoral shaft. Only one case of nonunion was observed in
technique although it was more difficult. In 125 cases the children with 11 cases in adolescents and adults (15 %).
involving 88 tibial and 37 femoral lengthenings, the amount In the latter group the average time for bony union was over
gained ranged between 3.0 and 4.7 mm. In younger children 6 months. During the evolution of the procedure, the sub-
with open growth plates, there was often an added over- periosteal approach to the femur was gradually replaced with
growth providing an additional 1–2 cm of lengthening. the musculoperiosteal decortication approach. Knee motion
There were no vascular complications. There were 11 neu- was either not affected or only minimally affected by the one
rologic complications in the 125 procedures, the large stage lengthenings. Of 180 patients 157 experienced no
majority of which resolved fully. There were two full change in knee motion. There were no vascular complica-
paralyses of the sciatic nerve which lasted for a year, two tions in the series. Nerve complications were evident;
partial sciatic lesions which cleared in 1 or 2 months, three however, there was one case of sciatic nerve palsy with a
paralytic lesions, and four partial neuralgias with the tibial 4 cm lengthening and two cases of quadriceps palsy, both
lengthenings. Bone repair was uneventful occurring in most with femoral nerve involvement with partial improvement.
in 45 days with no pseudarthroses created. Fractures did Additional complications involved infection, early breakage
complicate the procedure and occurred both during the early of internal fixation, nonunion, and late fractures of the
repair phase and also following healing anywhere from lengthened femur which occurred on eight occasions. The
3 months to 4 years post procedure. There were 14 fractures authors stressed that sustained flexion of the knee during the
reported in the 125 lengthenings (11 %) with 7 in the femur procedure prevented vascular and nervous complications.
and 7 in the tibia. The vast majority of lengthenings were They felt that the procedure was warranted for moderate
performed for shortening secondary to poliomyelitis. discrepancies although strict observance of the maximum
Cauchoix, Morel et al..: A one-stage femoral lengthening lengthening guidelines was essential to minimize
was also described by Cauchoix, Morel and colleagues [475] complications.
with results from the 100 initial patients reported in 1972 D’Aubigne and Dubousset: D’Aubigne and Dubousset
[476] and a total of 180 cases reviewed in 1978 [477]. The described a one-stage lengthening of the femur using a
operation involved a lengthy mid-diaphyseal Z-osteotomy of transverse osteotomy with lengthening performed immedi-
the femur in the frontal plane with the length of the longi- ately over an intramedullary rod and the lengthening stabi-
tudinal cut 8 cm in the adult and 6 cm in the growing child. lized by insertion of a cortical bone block [426]. They
Distraction was performed against two transverse 5 mm described 16 patients in whom transient peroneal palsy
Steinmann pins placed in the transverse axis through the occurred three times, delayed or nonunion three times, and
proximal and distal femoral fragments. The knee remained knee flexion contractures requiring surgical release twice.
flexed during the lengthening procedure and care was taken Wagner has stressed that one-stage lengthenings should
to keep the bone fragments in contact using a bone holder be restricted to the femur and that a maximum of 4 cm
which was alternately opened and closed during the increase can be obtained safely [478].
lengthening procedure. Considerable releases of the fascia Kawamura. Gradual Lengthening: Kawamura and asso-
were done along with decortication of the middle third of the ciates began lengthening immediately after osteotomy feel-
femur and formal exposure of the sciatic nerve to check that ing that “since operative damage to soft tissues is minimized
it was not excessively stretched during the lengthening it is not necessary to delay the start of bone lengthening for a
procedure. Once lengthening had been achieved, internal few days” [479]. Further lengthening was accomplished
fixation was secured by a posteriorly placed vitallium plate. intermittently in three to five sessions under anesthesia.
The bone gaps left by the Z lengthening were then filled with The periosteum was elevated circumferentially following
iliac crest autogenous cancellous bone graft. The average which osteotomy was performed. When the periosteum was
gain in 180 cases was 3.7 cm with 169 of 175 one-stage, elevated along the line of osteotomy in experimental dog
one-time lengthenings ranging between 2.6 and 4.5 cm. In models with 10 % lengthening the periosteum tore almost
five instances, greater lengthening was achieved by completely. In the second group, the periosteum was incised
6.9 Management of Lower Extremity Length Discrepancies 713

longitudinally and detached for about 5 cm above and below 1 cm incision was made over the mid tibial region with the
the line of osteotomy. With distraction, the periosteum periosteum incised longitudinally and elevated from the tibial
persisted as a tube localizing the fracture hematoma and surface. The periosteum was freed circumferentially. The
rupture did not occur until 20 % lengthening. Histologic oblique osteotomy was outlined by several drill holes pene-
study of 130 dog procedures was done. After tibial osteot- trating the cortices with the osteotome subsequently cutting
omy lengthening was performed gradually up to 10 % over only the cortex in an effort to spare the nutrient intramedullary
2–4 weeks. At 3 weeks, new bone formation from the inner vessels. The initial lengthening was carried out to a distance
surface of the periosteum was seen. Preservation of the not exceeding 3 % of the tibial length immediately. Kawa-
periosteal tube was helpful in enhancing early bone repair. mura did not find it necessary to divide the deep fascia,
The repair response was mediated both by periosteal and interosseous membrane, or intermuscular septum. Lengthen-
nutrient vessels. Kawamura also felt that injury to the ing of the tendo-Achilles was performed if it was tight or if
nutrient artery should if at all possible be prevented. Oblique tightness was anticipated. Since operative damage to soft
osteotomy was used to lessen slightly the length in the gap tissues was minimal it was not considered necessary to delay
needing repair. The nutrient artery was intact at this time the start of lengthening to allow for soft tissue repair. Further
frame also. In the center of the lengthened area hematoma lengthening was achieved by a small amount each day or in 3–
was seen. At 3 weeks, there was excellent vascular supply to 6 sessions a few days apart with more lengthening done. It was
the repair callus and at 5–7 weeks there was vigorous pro- felt that 3–6 weeks might be required to gain the desired
liferation of arterial supply from nutrient branches although length. When the required gain was achieved and consolida-
a slight avascular zone was still seen centrally. By 8 weeks, tion of callus had occurred, the limb was placed in a plaster
most of the avascular zone had disappeared indicating that cast incorporating the pins. A similar procedure was per-
the lengthened area had reunited. At 12 weeks, there was a formed for the femoral lengthening.
well-reconstituted medullary and cortical blood supply. The Over a 17 year period this group performed 252 tibial and
study showed that after a 10 % gradual lengthening fol- 58 femoral lengthenings in children [480]. The tibial tech-
lowing cortical osteotomy, the bone union progressed sim- nique was standardized and 223 of 252 cases were reported
ilar to that seen after a fracture. as obtaining “highly satisfactory results.” The 58 femoral
Kawamura also noted the effect of diaphyseal lengthening lengthenings were also considered to be satisfactory. The
on the physis in dogs at either end. The effect of tibial vast majority of patients had good or excellent results with
lengthening on the longitudinal growth of the bone was tibial lengthening up to 15 % with similar findings in the
assessed in young dogs between 1 and 3 months of age. femur with lengthenings up to 11 %. Their observations in
Osteotomy was performed and lengthening carried out to 0, young dog experimental lengthenings done before the age of
10, 15, and 20 % in 4–6 stages over 2 weeks. Subsequent closure of the epiphyseal growth plates showed that gains in
longitudinal bone growth was then studied between 2 and length were preserved in lengthenings of 10 % but in
18 weeks postoperation. In lengthenings of 15 and 20 %, the lengthenings of more than 15 % subsequent growth was
growth plate showed marked narrowing both by radio- often markedly decreased. Fifty-seven patients were studied
graphic and histologic study. There was marked growth plate in terms of subsequent longitudinal growth following tibial
deformity by 3 weeks in bones lengthened between 15 and lengthening. Of the total of 57 patients, 35 showed a
20 % with disturbance of endochondral ossification. Early decrease in expected growth. Kawamura stressed that com-
closure of the epiphyseal growth plate was seen in 1/3 of the plications in this regard could be avoided with lengthenings
dogs with 10 % lengthening and in all dogs lengthened 15 kept to within 10 or 15 % of overall length. The lengthening
and 20 %. In those animals lengthened 15 and 20 %, the was to be carried out gradually in 3–6 stages over 3–6 weeks
ultimate gain compared to those lengthened only 10 % was and the initial lengthening done immediately should not be
negated “since the normal bone growth was lost in the larger more than 3 % of the bone length.
mechanical lengthenings.” They concluded that lengthening Complications. Kawamura listed in detail the possible
should be carried out gradually and limited to about 10 % of complications with limb lengthening. 1. Complications due
the original bone length. to overstretching. These included angular deformity, arthri-
Technique. The distal metaphysis of the fibula was resec- tis, stiff joints, loss of muscle power, stretch paralysis, and
ted subperiosteally for a distance of 2–3 cm and when neuralgia. 2. Complications due to interference of blood
lengthenings were projected to be more than 11 % it was felt supply. These included delayed union and non-union, bone
advisable to fix the distal fibular fragment to the tibia with a necrosis, and circulatory disturbances distal to the operative
screw. Four Steinmann pins were then introduced into the site. 3. Complications due to inadequate fixation of frag-
tibia 2 above and 2 below the proposed osteotomy site and the ments. These involved overlying skin slough, fragment
leg was placed in the distraction apparatus cradle. A small protrusion, anterior bone angulation, late fracture, and pin
714 6 Lower Extremity Length Discrepancies

site infection which could lead to osteomyelitis. 4. Direct Excellent reviews of limb lengthening techniques have
operative complications included fracture of the osteoto- been provided by Caton [483] and Paley [484]. Some of the
mized shaft, pin pull out, hypertension, and shock. less well-known earlier European techniques have been
reviewed by Wiedemann [485].
(b) Improved results due to more rigid fixators. Gradual Wagner Technique: Wagner in 1978 reported his tech-
Distraction. Judet Technique: The Judet technique, nique which had major improvements from previous
developed in France and reported in 1969, provided approaches and re-stimulated widespread interest in length-
much more rigid stabilization thus enhancing comfort ening procedures [478]. His unilateral fixator was struc-
in association with lengthening [481]. It involved a turally very stable and allowed patients to be ambulatory
lengthening by gradual distraction in association with with crutches immediately after the surgery and throughout
an oblique osteotomy, decortication to hasten bone the lengthening procedure. This alone was a major advance
repair but use of neither graft nor internal metallic since virtually all previous lengthening procedures required
stabilization. The external fixator used 5 mm diameter prolonged bed rest, at least during the lengthening and early
pins with three or four placed in the proximal fragment consolidation phases. Diaphyseal lengthening began imme-
and an equal number distally. A unilateral distractor diately with approximately one cm of distraction performed
was attached. The procedure was designed for use in in the operating room at time of instrumentation (described
the tibia. The distal fibula was stabilized by a trans- by Wagner as lengthening “until stabilization is achieved by
fixion screw to the distal tibia to allow for lengthening soft tissue tension”). A one-time daily lengthening of
of both bones simultaneously and maintenance of the 1.6 mm was performed. Once the desired length was reached
orientation of the ankle mortice. The oblique osteotomy a second operative procedure was done in all patients
of the tibia was made in the frontal plane and was of the involving application of a long side plate, autogenous iliac
greatest length possible with the average amount being crest bone grafting, and removal of the external fixator
10.5 cm. A plaster splint immobilized the knee in (Fig. 6.22a–h). The advantage of the second operative
extension and the foot at a right angle at the termination approach was that rigid internal stabilization was achieved
of the operative phase. The limb was maintained in the immediately and rehabilitation was enhanced since range of
cast during the period of the lengthening which was motion exercises could be performed more easily once the
performed at a rate of 1.5 mm/day. Each day hip, knee, bone and soft tissue transfixing pins were removed. The
and ankle range of motion exercises were done. When Wagner technique represented a significant advance over the
the lengthening was completed, the lower extremity Anderson and previous methods owing to the fact that the
was placed in a long leg cast which was then replaced patient could remain ambulatory and pain was considerably
at varying times with a lighter plastic brace. At diminished owing to the increased stability of the external
6 months, the distractor and fibular-tibial pins were fixator.
removed with the tibia continuing to be protected in By the time Wagner developed his approach, the etiology
brace for an additional 6 months. of limb length discrepancy had changed with fewer children
suffering from poliomyelitis. Of the 58 patients in his initial
Pouliquen et al. reviewed 108 tibial lengthenings per- paper, only 10 had poliomyelitis with the largest group
formed by the Judet method [482]. Of these, the large involving congenital short femur and the next largest
majority, 79, were due to poliomyelitis. The average sequelae of epiphyseal fracture. Wagner found that preop-
lengthening obtained was 4.37 cm with a range from 2.6 to erative consideration of the entire limb was of increasingly
6.0 cm. The average gain was in the range of 16 %. Bone great importance. Of particular importance prior to length-
union in the most favorable cases was obtained at an average ening procedures was the correction of joint contractures and
of 4 months and a secondary bone graft was needed in only 5 surgical intervention to correct acetabular dysplasia and
instances. There were 17 instances of neurologic impairment angular bone deformities. He reviewed the advantages and
although only 2 showed persistent anesthesia and 1 perma- disadvantages of operative limb lengthening and the various
nent paralysis of the extensor hallucis longus muscle. shortening procedures.
Postlengthening fractures occurred in nine patients, five of Carlioz et al. reported on their first 30 cases of the
which were nondisplaced and treated only with a simple cast Wagner lengthening involving 15 femoral lengthenings, 7
with four requiring additional surgical intervention. Overall, tibial lengthenings, and 8 cases associated with correction of
10 secondary bone grafts were required, 5 for a delay in angular deformities or pseudarthrosis [486]. In the straight-
primary healing, and 5 for pseudarthrosis or fractures. The forward femoral lengthenings, the gain ranged between 4.0
paper also performed detailed comparison with tibial and 7.0 cm. Among the complications were three subsequent
lengthening studies from 7 other series. fractures. The percentage of lengthening was in the range of
6.9 Management of Lower Extremity Length Discrepancies 715

Fig. 6.22 Illustrations of a diaphyseal lengthening technique are there was one major advantage in comparison to previous methods,
shown. End result following lengthening of a femur using the Wagner where the distraction device was left on until full healing, in that
method is shown in clinical photographs. As with any lengthening rehabilitation in terms of range of motion of the adjacent joints and
technique, the aim is to attain a symmetric appearance of both lower muscle strengthening were markedly quickened and improved. a–
extremities with a full range of motion of adjacent joints. Following d Clinical photographs show symmetric appearance of lower extrem-
attainment of the desired length by distraction, additional operation is ities posthealing looked at from the front (a) and side (b) showing the
performed at which time a Wagner plate and bone graft are applied. The operated limb with full knee extension. Side views of the patient with
plate designed by Wagner for this procedure has no holes spanning the the knee maximally flexed show comparable hip and knee flexion on
lengthened gap as these are prone to breakage if a regular AO plate is non-operated (c) and operated (d) sides. e–h Show anteroposterior (e),
used. Although additional surgeries were required with this technique lateral (f), and oblique (g, h) projections at healing

15 % with no cases beyond 20 %. In the straightforward (Blachier et al.) [487]. The mean lengthening obtained was
tibial lengthenings, the range of correction was between 4.0 5.2 cm with the most extensive 11.2 cm. In terms of per-
and 5.8 mm with an average percentage increase of 15 % centage gained lengthening values were 15.6 % of initial
with one case being 26 %. Early experience of this group bone length. On six occasions, the lengthening was >20 %
was quite favorable. A few years later, their group reported with a maximum of 24 %. Many complications were noted
on 48 femoral lengthenings using the Wagner technique varying from minor to much more serious in importance.
716 6 Lower Extremity Length Discrepancies

Superficial infection was frequent but easily managed. In 22 Many groups reported their results with the Wagner
of the 48 cases, varus deformation occurred during length- technique. Wagner reported on 58 femoral lengthenings in
ening but was easily corrected in most at the time of plate patients below the age of 17 years in which the average gain in
application and grafting. In some instances, however, the length was 6.8 cm [478]. Bjerkreim and Hellum [491]
varus was not corrected even at the time of plate application. reported average lengthening of 5.8 cm in the femur; Alde-
Subluxation at hip and knee was seen on occasion as were ghiri et al. [492] 4.9 cm in the femur and 4.0 cm in the tibia;
contractures without subluxation at these two joints. Five and Paterson et al. 5.8 cm in the femur and 5.2 cm in the tibia
patients had neurologic complications; in four there was full [493]. Stephens reported an average femoral lengthening of
and rapid recovery of the anterior foot dorsiflexors but in one 5.7 cm in 18 Wagner procedures and average tibial length-
paralysis of the peroneal and tibialis posterior nerves was ening of 5.6 cm in 7 Wagner procedures [494]. Osterman and
complete and recovering only slowly at time of publication. Merikanto reported on a mean increase with 26 tibial
There were 12 deep infections which on occasion compro- lengthenings of 4.1 cm and with 9 femoral lengthenings of
mised the long-term result. On occasion, fracture or pseu- 4.9 cm [495]. Mahlis and Bowen reported 27 femoral
doarthrosis at the site of infection further complicated lengthening with a mean increase of 6 cm (range 3–9.5 cm)
treatment. (mean percentage increase 17.6 %, range 7–36 %) and 11
Caton et al. also reported early favorable results with the tibial lengthenings with a mean increase of 6.1 cm (range 4.5–
Wagner technique in 33 lengthenings of which 20 were in 9 cm) (mean percentage increase 20 %, range 10–32 %) [472].
the femur and 13 in the tibia [488]. The mean lengthening Ahmadi et al. compared results with 50 Anderson, 40 Reza-
obtained was 5.35 cm and although many complications ian, and 51 Wagner lengthenings all done for poliomyelitis
were seen 70 % of cases had none. The mean percentage [496]. The mean gain was 4.8 cm and complications showed
lengthening was in the range of 16.2 % with a mean six refractures (4 %) as well as others commonly seen, but in
lengthening of 4.8 cm for the tibias and 5.7 cm for the relatively low rates. Results in the three techniques were
femurs. similar; the Wagner was favored for relative ease of use.
The use of more rigid fixators such as those of Wagner or Complications with the Wagner procedure: The study of
Judet became popular in the 1970s. Some series are reported and literature on the complications of limb lengthening
in which the fixators were used although differing techniques surgery is somewhat unique in relation to the rest of the
than initially described by the developers were applied. orthopedic literature, primarily because of the intrinsic nat-
A study by Rigault et al. assessed 36 femoral lengthenings in ure of the difficulties with this procedure. A characteristic
which 21 used the Judet distractor and 15 the Wagner [489]. approach is to divide what would generally be considered
In most instances an oblique femoral osteotomy was used, complications into (i) problems, which are felt to be intrin-
there was an initial 5–10 mm lengthening at the time of sic, cannot be avoided, and include such disorders as delayed
surgery and progressive lengthening was then performed at a union and nonunion, pin track infections, and transient
rate of 1–2 mm/day. The bone was allowed to heal without restriction and motion of adjacent joints, and (ii) complica-
application of the graft and side plate as recommended by tions which are extrinsic and should be avoided including
Wagner. The mean lengthening gained in the 36 cases was infection, nerve damage, fractures, and subluxations. Others
5.2 cm which was an 18.3 % increase in length. The series use the terms minor and major complications.
was complicated by a high rate of fracture (9 of 36 or 25 %). DeBastiani et al. reported a 26 % complication rate with
Those having the oblique osteotomy had a mean gain of the Wagner method [497]. Hood and Riseborough [498]
5.6 cm while those having more transverse osteotomies had noted 37 complications in 40 procedures, while Wagner
a mean gain of 4.7 cm. Bone graft was performed in 6 of the [498] noted a complication rate of 45 % in 58 patients.
36 cases because of delayed healing. Rigault et al. also Luke et al. specifically reviewed fractures after the
described 48 tibial lengthenings using either the Judet or Wagner limb lengthening procedure [499]. In a series of 27
Wagner apparatus along with a long oblique osteotomy, cases, there were 10 fractures following lengthening in 8
decortication, and multiple soft tissue releases to stabilize the patients of which 6 were spontaneous and 4 traumatic. The
knee and minimize equinus deformities at the ankle [490]. In fracture occurred through the lengthened area after plate and
general, the distractor was left in place until radiographic screw removal in 8 patients, through a proximal screw hole
bone consolidation was evident following which a brace or and plate in 1 patient, and through an external fixator pin
walking cast was applied to protect the limb during the hole with hardware intact in another. Seven fatigue fractures
return to full weight bearing. The mean lengthening was occurred after plate removal in Wagner’s series. Hood and
4.2 cm or 17.5 % of preoperative bone length. Fractures Riseborough [498] reported 4 fractures after 40 Wagner
were seen in 10 %. In the 10 more complicated cases, the lengthenings; Mosca and Mosely [500] reported 63 Wagner
mean lengthening was 5.5 cm or 16.5 %. lengthenings with 16 subsequent fractures; and Chandler
6.9 Management of Lower Extremity Length Discrepancies 717

et al. [501] reported 21 lengthenings with 2 subsequent (c) Distraction Osteogenesis: Although many good and
fractures. The above 4 studies thus reported a total of 182 excellent outcomes were obtained with the Anderson
Wagner lengthenings with 29 subsequent fractures (16 %), and Wagner techniques, problems and complications
while the most recent report of Luke noted a 37 % rate (10 of still occurred. The Anderson technique frequently
27). The amounts lengthened in both tibia and femur were required supplementary bone grafting while the Wag-
not remarkable in those suffering either spontaneous or ner approach required at least three surgical interven-
traumatic fractures with the tibial percent lengthening at tions and subsequent fractures after plate removal were
approximately 16 % and the femoral 20 %. Some attempted a problem. Work continued with different techniques to
to minimize the fracture sequelae by a four-stage procedure improve results.
involving the osteotomy, plating and bone grafting of the (i) Clinical Techniques. Distraction Osteogenesis. Ili-
lengthened area, and then instead of removing the hardware zarov; Monticelli and Spinelli; DeBastiani; Alde-
at one stage, doing a two-stage procedure with removal of gheri and associates; Canadell and associates:
alternate screws and partial loosening of the plate followed Techniques developed by Ilizarov [504–508] in
several months later by removal of all remaining hardware. Russia; Monticelli and Spinelli in Italy [509];
Osterman and Merikanto noted that a major complication DeBastiani, Aldegheri, and associates in Italy [497];
was late femoral fracture which occurred in 6 of the 26 and Canadell and associates [510–512] in Spain
instances although there were no tibial fractures [495]. There have allowed for lengthening and bone repair such
was 1 hip dislocation, 1 talar deformation, 1 peroneal nerve that bone grafting and plating were infrequently
entrapment, and 1 infection which delayed bone union. The required. These techniques, described collectively
authors cautioned about early removal of the stabilizing under the term distraction osteogenesis or callotasis,
plates and also indicated the need for bone lengthening to be are dependent on four principles, the first two of
performed by well-trained teams. which are truly integral to the effectiveness: (a) de-
Karger et al. reported primarily on Wagner lengthenings lay prior to the initiation of bone lengthening for 7–
involving 51 femurs and 18 tibias. Complications were more 10 days to allow early repair, or callus formation, to
marked when the lengthening exceeded 25 % of initial bone occur, (b) gradual lengthening of 1 mm/day per-
length [502]. They were also much higher in the femur than in formed at four separate times to minimize damage
the tibia. Some modifications to the Wagner procedure were to newly formed vessels and repair tissue, (c) corti-
incorporated; while 44 patients had the entire sequence cotomy leaving the medullary vasculature intact or
described by Wagner, 18 had only the stage-one procedure at least minimally disturbed, and (d) maintenance of
but did not have grafting and plating since the authors felt an intact periosteum. There has been somewhat of a
initially that healing would be appropriate. In 51 femurs polarization between schools performing the dis-
lengthened by the Wagner technique, the mean lengthening traction osteogenesis technique based on the type of
achieved was 7.57 cm which represented a 25 % increase external fixator used. The Ilizarov technique uses
whereas in 18 tibias, the mean length achieved was 6.07 cm circular and hemispheric external fixators stabilized
accounting for 23 % increase in length. The complications by multiple narrow wires; the Monticelli-Spinelli
were divided into groups referred to as problems, obstacles, method uses a single circular fixator to hold epi-
minor sequelae, and major sequelae. They were commonly physeal wires and two diaphyseal level rings held
seen in the femoral lengthenings but somewhat less frequently together by longitudinal rods between which the
in the tibial. Fractures were common occurring in 18 of 51 metaphyseal corticotomy is performed; and the
femoral procedures and 3 of 18 tibial. Angulation was noted in Orthofix and Monotube methods use a unilateral
25 in the femoral group all of which were varus with a mean fixator with two upper and two lower 5 mm wide
deformity of 25°. Some had flexion deformities as well. There pins (Fig. 6.23a). The early results were promising
were 10 of 18 angular deformities in Group 2, all of which but even with these techniques problems similar to
were valgus with a mean of 16°. Pin tract infections and deep those reported with earlier methods are encoun-
infections were noted as were joint restrictions of motion tered. Healing time still remains prolonged and
including 12 transient knee subluxations. associated with osteopenia, muscle atrophy, and
Salai et al. described three hip subluxations during femoral joint stiffness.
lengthening with the Wagner technique [503]. Each of the
patients had some predisposing hip and acetabular abnor-
mality indicating the importance of assessing the hip prior to Paley summarized the work of Ilizarov and the Kurgan
and during the course of lengthening and fully correcting school who worked extensively on distraction osteogenesis
malposition surgically prior to the lengthening procedure. [484]. More than 1,000 publications from their institute had
718 6 Lower Extremity Length Discrepancies

Fig. 6.23 (continued)

described various approaches to the technique, with most removal of the apparatus, and the average time to full weight
published in Russian. Major studies were reported by Ilizarov bearing. They subdivided results into the amount of length-
of 237 femoral lengthenings with an average lengthening of ening from 4 to 5 cm in 1 group, 5.5–9.5 cm in another, and
7.4 cm, (range 3–15 cm) and complications listed in 12 10 cm or greater in the next. Tetsworth and Paley showed
patients (5.6 %). Ilizarov also published results of 217 tibial excellent lower extremity alignment correction as well as
lengthenings in both children and adults with the mean gain of length correction with the Ilizarov method [519].
7 cm, (range 4–15 cm). Paley [513], Aronson [514], and Birch Monticelli and Spinelli reported 43 cases of metaphyseal
and Samchukov [515] have reviewed the complications with lengthening using the distraction osteogenesis technique with
the technique in detail. The Ilizarov apparatus has three major their own fixator with a mean gain of 7 cm and a range from 4
components: bone fixaton elements (wires, half-pins), exter- to 10 cm [509] DeBastiani and colleagues using open corti-
nal supporting elements (rings, arches), and connecting ele- cotomy and callus distraction with their monolateral fixator
ments (rods, plates, hinges) to connect the rings and arches (Orthofix) performed limb lengthenings on 100 segments
and allow for manipulation of the relationship between them. with a lengthening index of 1.2 months for the femur,
Stanitski et al. reported the results in 36 femoral length- 1.4 months for the tibia, and 0.8 months for the humerus
enings using the Ilizarov technique with the average length- [497]. The average amount of lengthening obtained in the
ening 8.3 cm (range 3.5–12) and a lengthening index, months femur in patients with achondroplasia was 7.8 cm (5.5–12)
of treatment/cm lengthening, of 0.74 [516]. Stanitski et al. representing a mean 26 % increase, while in the tibia the
reported on 62 tibial lengthenings using the Ilizarov technique average amount of lengthening obtained was 7.8 cm (6–
with the average lengthening of 7.5 cm (range 3.5–12) rep- 10.5 cm) representing a 36 % increase. In patients with limb
resenting the equivalent of a 32 % average overall increase length discrepancy from congenital and acquired disorders,
[517]. Franke et al. reported good results with the Ilizarov the average amount of lengthening in the femur was 4.7 cm
technique whether they used distraction epiphysiolysis or (3–9; 11 %), in the tibia 4.7 cm (3–9; 17 %) and in the humerus
metaphyseal corticotomy [518]. In the distraction epiphysi- 7.5 cm (7–8; 34 %). Aldegheri et al. reported on 270 femoral
olysis procedure, they lengthened 22 tibias with an average and tibial lengthenings using the Orthofix callotasis method
lengthening of 8.25 cm (range of 4–18 cm) and 30 tibias using [520]. Ninety-five patients had limb length inequality and 45
the metaphyseal-diaphyseal corticotomy with an average of had achondroplasia/hypochondroplasia. The average length
7.9 cm lengthening (range 4–15 cm). Their report is detailed increase was 6.6 cm or 24.6 % of initial length. The mean
assessing the average time of distraction, the average time to healing index was 39 and the complication rate 13.3 %.
6.9 Management of Lower Extremity Length Discrepancies 719

Fig. 6.23 (continued)

Long-term studies of relatively large numbers of was 5.2 cm (3.5–8.5 cm) which represented a mean
patients with the callotasis technique show findings similar increase in femoral length of 17.7 %. In the 79 cases,
to other lengthening techniques. Glorion et al. reviewed however, there were 87 complications, a rate of 110 %,
79 cases of femoral lengthening by the callotasis technique although several complications were often encountered
with 9 patients having the Judet apparatus and 70 the during one procedure. They noted that 30 % of the
Orthofix [521]. They concluded that the incidence of lengthenings were performed without any complication
complications did not seem to be less than encountered and 62 % with relatively mild complications such that
with previous methods of lengthening. The complications additional surgery was not needed. The healing index was
were the same in terms of nature and extent whether 39.6 days/cm which was comparable to other callotasis
callotasis had been performed using the Ilizarov or studies. Dynamization was considered to be an important
Orthofix techniques. The averaging lengthening achieved adjunct to the healing process.
720 6 Lower Extremity Length Discrepancies

Fig. 6.23 (continued)

 6.9 Management of Lower Extremity Length Discrepancies 721

Canadell summarized the experience of his group from evenly spaced time periods. The ideal age to conduct
Pamplona, Spain. Assessment of 93 lengthenings performed lengthening was between 8 and 12 years at which time the
over a 3 year period using the principles of distraction osteogenic capacity was greatest. In those younger than
osteogenesis and a unilateral fixator was reported along with 8 years of age repair was often so rapid that the desired
conclusions realized over a 25 year period involving more lengthening could not be achieved. Lengthening was con-
than 800 lengthenings [510–512]. The 93 lengthenings traindicated after the age of 30 years. Considering all
involved 27 with unilateral discrepancy owing to pathologic lengthenings done, the index of maturation was
conditions and 34 patients having bilateral lengthenings for 1.16 months/cm. Distraction was begun at different times
symmetrical shortening with skeletal dysplasia disorders. after percutaneous metaphyseal or diaphyseal osteotomy and
The average lengthening obtained was 8.37 cm with com- application of a monolateral fixator dependent on the age of
plication rate of 2.1 per lengthening. The repair index was the patient using a rough guideline of 1 day delay per year of
the same for both femur and tibia but humeral lengthenings age. Thus a child of 8 years of age had lengthening started
healed in a much quicker fashion. There was only a slight 8 days after initial surgery and for someone 15 years of age
overall difference in repair between metaphyseal and dia- they waited 15 days. The quickest healing rate was most
physeal osteotomies and those involving distraction epi- favorable in those with short stature conditions averaging
physeolysis. The diaphyseal and metaphyseal lengthenings 0.8 months/cm and it was much longer in those with uni-
provided slightly greater increases in length. The rate of lateral length discrepancies being in the range of
distraction was somewhat slower in the epiphyseal distrac- 1.5 months/cm. The greatest lengthenings were also
tion procedures although the rate of healing was somewhat obtained in those with short stature with an average of
quicker once length had been obtained. Canadell also noted 11.2 cm per segment lengthened. In 8 patients with a
that repeat lengthenings could be performed readily on the chondrodysplasia he reported extensive lower extremity
same bone generally with an interval of 2 years between lengthenings gaining 23.2 cm, 12.35 in the femur and 10.85
each procedure. The number of complications was greater in the tibia. The ideal site for osteotomy was the metaphysis.
during the course of the first lengthening than with the More sensitive angiographic studies indicated that in 90 %
second. Two femurs had been lengthened 5 times and 2 of diaphyseal osteotomies the medullary vessels were dam-
others had been lengthened 3 times. Lengthening was aged which was one of the reasons the metaphyseal site was
resorted to for discrepancies of 3 cm or more. It was difficult favored since the vascularity was more diffuse and richer in
to prevent traumatic rupture of the medullary cavity and in that region. On both a clinical and experimental basis it was
only 30 % of cases were the contents intact. Protection of the vastly more important to respect the continuity of the
periosteum was considered essential. The ideal rate of dis- periosteum than the medullary circulation. Dynamization
traction was 1 mm/day with 0.25 mm lengthened at four was strongly supported for enhancing repair.

b Fig. 6.23 a Examples of the distraction osteogenesis technique are seen. The patient has continued with normal function and increased
shown. ai1ΓÇô8 Anteroposterior films of tibia and fibula lengthening bone density with a full range of motion at hip and knee. aiii(1–13)
show distraction gap from day 1 until full healing at 1 year. The fibula Results of the tibial lengthening are shown using distraction
has never fully united but has not been a clinical problem. Progressive osteogenesis principle and the Orthofix apparatus. Images showing
new bone formation is seen resulting in full cortical reconstitution of the leg in cast were during the period following heel cord lengthening.
the tibial diaphysis on anteroposterior (ai-7) and lateral projections b Radiographs of rabbit tibia undergoing distraction at 22 days
(ai-8) with reformation of the marrow cavity. Lengthening was begun following surgery (i-anteroposterior; ii-lateral). Healing pattern is
one-week post surgery. Note that early bone regenerate is more dense similar to that in the human with central region healing more slowly.
adjacent to the upper and lower persisting bone with the central part of c Specimen radiographs allow for better demonstration of bone repair at
the gap region showing lesser ossification and radiodensity. A unilateral varying times following surgery and distraction. Distraction began at
fixator was used. aii1–8 Results of a femoral lengthening are shown time of surgery. The specimen c i and ii (AP and lateral) were done 14
using distraction osteogenesis principle and the Orthofix apparatus. days after surgery and initiation of distraction, in (iii) 32 days (lateral);
Anteroposterior films of the femur are shown from time of initial and in (iv) 44 days (lateral) postsurgery. Note the excellent cortical
osteotomy and insertion of apparatus to complete healing at 10 months. reconstitution and marrow reformation in the final lateral radiographs.
Note the close apposition of the cortices at time of initial procedure. d Specimen photographs obtained after decalcification and hemi-
Lengthening was begun at 1 week. At 12 days, there is just the sectioning the bone repair gap and the adjacent cortices prior to
beginning trace of new bone formation just lateral to the cortical histologic sectioning. Photograph of rabbit sacrificed at 46 days shows
regions. At 1 and 2 months, new bone is clearly forming in the gap and the excellent reconstitution of the cortex as well as marrow continuity.
slightly lateral and medial to it along with increasing length noted. The lengthened region which totaled 9 mm or 9 % of the initial bone
A central gap region is seen, at 2 months (aii-3) with more bone formed length is indicated by the darker staining more vascular marrow
adjacent to the ortices above and below. A higher power view shows centrally. e The lengthened tibia is shown at sacrifice after the fixator
this formation pattern more clearly (aii-4). Progressive new bone had been removed and surrounding soft tissues had been dissected. This
formation is seen at progressive months. Note the marrow cavity had been lengthened 11 mm, an amount which corresponded to 10.7 %
reconstitution at 6 months particularly in comparison with the of its initial length. Lengthening proceeded for 14 days and the fixator
appearance at 5 months where bone across the gap was relatively was left on for an additional 30 days with sacrifice 44 days postsurgery
uniform. Final cortical and marrow reconstitution at 10 months of age is
722 6 Lower Extremity Length Discrepancies

Noonan et al. subsequently reviewed femoral and tibial involved percutaneous osteotomy referred to as compacto-
lengthening cases from the clinic of Canadell and colleagues tomy when at the level of the metaphysis or corticotomy
in relation to etiology, age and site of the osteotomy on the when done within diaphyseal bone. The cortex alone was cut
outcome, the prevalence of complications, and need for with a small 5-mm osteotome with care taken not to enter the
additional procedures [522]. They reviewed distraction medullary cavity in an effort to spare the nutrient artery and
osteogenesis of 114 femurs and 147 tibias. The group used medullary circulation. The periosteum remained intact. The
monolateral external fixators: Wagner for 96 femurs and 100 remainder of the cortex was then broken either by rotating
tibas, Orthofix for 2 femurs and 3 tibias, and Monotube for the osteotome to distract the bone ends or by rotating the
16 femurs and 44 tibias. fixator pins. Distraction did not begin until 7 days post
The femurs were lengthened an average of 11 cms (range surgery at a rate of 0.25 mm elongation 4 times daily. The
3.5–17) or 48 % of the original femoral length (range 8–86 %). average time to healing is still extensive and the lengthening
There were 114 complications leading to 87 additional oper- index (months healing/1 cm lengthening) has most studies
ations. The femoral lengthenings done for limb length dis- showing an index around 1 (if 5 cm are lengthened the time
crepancy had significantly higher complication rates for each to healing is 5 months).
amount of length gained than those performed for achon- Suzuki et al. studied 26 femoral lengthenings using the
droplasia or other skeletal dysplasias. The rate of complication Orthofix callotasis technique from the viewpoint of dislo-
for femoral lengthenings in those 14 years old or older was cation and subluxation of the femoral head during the
significantly higher than those under 14 years of age. Femoral lengthening procedure [525]. Lengthening began 1 week
lengthenings through the metaphysis had significantly higher postsurgery with the rate of distraction 0.25 mm every 6 h.
rates of complication than those through the diaphysis. In the The mean amount of lengthening obtained was 5.0 cm with a
femurs, 52 or 114 (46 %) had no additional operative proce- range from 2.0 to 7.5 cm. One group of 14 hips with a CE
dures. Complications/additional operations included: interval angle of >20° at the start of lengthening showed no deteri-
fixation and bone grafting for non-union, femoral osteoclasis oration in position with the lengthening while the other
(to improve position), pin removal, internal fixation for frac- group of 12 hips with an angle of 20° or less showed
ture through callus, osteotomy, closed manipulation under deterioration of femoral head position in 5 of the 12 hips.
anesthesia and cast application, repositioning or exchange of One developed a complete dislocation and the other four
fixator, hip region tenotomy for contracture or subluxation subluxed, showing a decrease in the CE angle. Four of the 5
(rectus femoris, iliotibial band and/or adductors.) had history of congenital dislocation of the hip and the other
The tibias were lengthened an average of 9 cms (range 3– had multiple epiphyseal dysplasia. The authors recom-
15.6) or 41 % (range 9–100) of original length. There were mended that in cases in which the CE angle was 20° or less
196 complications leading to 219 additional operations. The preoperatively bone procedures such as innominate osteot-
Achilles tendon was lengthened during or after 73 (50 %) of omy should precede the femoral lengthening.
the lengthenings. The rate of complications and additional Detailed presentations of distraction osteogenesis are
operations in those 14 years of age or older was significantly illustrated in Fig. 6.23ai 1–8, aii 1–8, aiii 1–13. Limb
greater than in those <14 years of age. The site of tibial lengthenings were performed increasingly with the callotasis
osteotomy did not affect the rate of problems or need for technique for those with symmetrical limb shortening due to
associated operations. In the tibias, 42 of 147 (29 %) had no skeletal dysplasias [526–528]; these are discussed in greater
complications. Complications/additional procedures inclu- detail in Chap. 3.
ded: equinus contracture needing tendoAchilles lengthening
(73), valgus angulation before fixator removed (19), knee (ii) Comparison of Techniques Within The Same Centers:
contracture or subluxation (3), and premature healing of Many studies from centers where large numbers of
fibula (4). Operative procedures included the tendoAchilles lengthening procedures are done have presented data
lengthening, removal of pins, osteotomy for malalignment, comparing differing techniques. Pouliquen et al. com-
removal or manipulation of fixator, interval fixation with pared femoral lengthenings in 82 cases divided between
bone grafting for non-union, knee manipulation, fibular 6 techniques of which 5 were used relatively frequently
osteoclasis, and peroneal nerve repair. [529]. These involved the one stage lengthening, 14
Fractures continue to be seen fairly often even after dis- cases; Judet technique, 20; Wagner, 13; a tranverse
traction osteogenesis lengthenings. Danziger et al. reported 9 osteotomy and graft technique, 11; and callotasis, 20.
femoral fractures after 18 Ilizarov femoral lengthenings but The authors concluded that the callotasis technique was
no tibial fractures with 8 tibial lengthenings [523]. Glorion the best since there were no serious complications in 20
et al. using primarily the Orthofix technique in 61 length- cases. The amounts lengthened, however, were similar
enings had fractures in 6 instances [524]. Their technique with the several techniques with the exception of the one
6.9 Management of Lower Extremity Length Discrepancies 723

stage lengthening which was reserved for relatively fracture, and axial deviation. The list of complications in 17
smaller discrepancies. In that group, the average femoral lengthenings included 3 with delayed union, 8 axial
lengthening was 3.6 cm or 7.8 % of bone length. The deviation, 3 late fracture, 2 loss of length, and 1 premature
other four techniques had lengthenings on average consolidation. As far as the joints were concerned, there was
ranging from 4.6 to 5.5 cm or 12.7 to 15.5 %. In the restriction of motion at the hip in 2, knee in 14, and ankle in
one-stage lengthening, a Judet distractor was applied 2 with 1 hip subluxation, 2 proximal tibial subluxations, and
followed by oblique osteotomy, application of tempo- 1 patellar subluxation. There was 1 deep wound infection
rary cerclage wires, lengthening by the distraction and 10 pin track infections. One patient had loss of muscle
technique intraoperatively with the knee flexed and power, and complications leading to discontinuation of the
osteosynthesis with a side plate once the desired amount lengthening procedure occurred in 1 with serious restriction
of lengthening had been achieved. The Judet technique of hip motion and in another with hip dislocation. Only 6
involved a unilateral distractor similar to the Wagner but tibial procedures were performed with complications
with 4 heavy pins below and 4 above the osteotomy site. involving 1 non-union, 3 axial deviations, 4 late fractures,
The lengthening was at a rate of 1.5 mm/day. Once and 2 premature consolidations. As far as the joints were
lengthening had been achieved the distractor was left in concerned, there was restriction of the motion at the knee in
place while healing was allowed to occur. Walking 1 and at the ankle in 3 and also 1 pin tract infection.
began again with protection of a brace which incorpo- A review of 100 lower limb lengthenings from Brazil
rated both the pelvis and the external fixator and was assessed 25 tibial lengthenings by the Anderson technique,
generally maintained until 12 months post initial sur- 45 femoral lengthenings by the Wagner technique, and 16
gery. The complication rate in the callotasis technique femoral and 14 tibial lengthenings by the Ilizarov technique
was extremely low at 5 % while it ranged from 27 to [531]. The amount of lengthening achieved and the average
35 % in the 4 other major approaches. The study also healing time showed very little difference between the
reviewed the literature from the 1970s and 1980s in techniques. The complications seen widely in limb length-
relation to each of the major approaches then used ening were present in each although certain types of com-
involving the one stage lengthening, the Judet length- plication tended to occur with certain types of lengthenings.
ening, the transverse osteotomy plus graft lengthening, Good results could be achieved with each method and
Wagner lengthening, Ilizarov lengthening, and callotasis considerations such as comfort and relative ease of the
lengthening. The one-stage lengthenings assessed procedure for both patient and surgical team would play a
involved 229 cases with lengthening achieved ranging primary role in choice. In the Anderson group the average
between 3.2 and 3.7 cm and a complication rate between lengthening achieved was 4.2 cm, range 3–6 cm, and the
10.5 and 35 %. Two series of Judet lengthenings with an average healing time was 197 days with a lengthening index
oblique osteotomy were reviewed involving 56 cases of 1.72 months. In the Wagner group, the femoral length-
with a mean lengthening of 5.2 cm and a complication enings had an average of 4.6 cm, range 1–12.5 cm, with an
rate between 25 and 41 %. There were 11 cases of the average healing time of 185 days with a lengthening index of
transverse osteotomy plus bone graft group also with a 1.32 months in a subsection having percutaneous osteotomy
5.5 cm mean lengthening and a 27 % complication rate; and a healing time of 166 days with a lengthening index of
120 cases of the Wagner lengthening with a range of 1.23 months in a subgroup having corticotomy. In the Ili-
5.2–6.8 cm increase and a complication rate of 12.5– zarov group, the average femoral lengthening was 4.7 cm,
31 %; Ilizarov lengthenings in 21 cases with a mean range 1–7.5 cm, with a healing time at a mean of 186 days
lengthening of 5.0–6.1 cm and a complication rate of 6– and a lengthening index of 1.31 months and for the Ilizarov
25 % and; the most favorable group the callotasis tech- tibia lengthenings the average was 4.5 cm, range 1–7.5 cm
nique involving 78 cases with a mean lengthening of with a healing time of 184 days and a lengthening index of
4.7 cm and a 6 % complication rate only. 1.35 months. With the Anderson method, the most common
complication was delayed union; with the Wagner technique
Faber et al. reviewed several limb lengthening procedures the most common complications related to bone healing and
divided between the Wagner approach, the subsequent fracture; and with the Ilizarov method the most
Monticelli-Spinelli metaphyseal corticotomy and distraction, common complication was incomplete corticotomy.
and the Monticelli-Spinelli distraction physiolysis [530].
Complications with each of the three procedures were (iii) Effect of Lengthening on Muscle Strength, Articular
reviewed in detail and were frequent. The Wagner diaphy- Cartilage Structure and Nerve Function: The effects of
seal osteotomy involved more cases of delayed union, late lower extremity lengthening on muscle strength,
724 6 Lower Extremity Length Discrepancies

articular cartilage structure, and nerve function have Kawamura et al. also noted no significant histochemical
been assessed clinically and by more sensitive inves- or electromyographic changes in those lengthened up to
tigational methods. 10 % of their initial bone length [479]. Carroll et al. using a
relatively rapid distraction model in sheep, noted that tibial
Muscle strength before and after femoral and tibial lengthening >11 % of initial length consistently produced
lengthening: Maffulli and Fixsen studied quadriceps strength irreversible changes in the gastrocnemius and flexor digito-
in those with congenital short femur before and at the termi- rum profundus muscles, including loss of myofibrils, central
nation of femoral lengthening [532]. The Orthofix technique migration of nuclei, and irregular shapes and sizes of
was used. Seven patients had an average lengthening of myofibrils [535]. Other observers have reported that up until
7.1 cm, a 23.5 % lengthening of the congenital short femur. approximately 20 % lengthening the muscle actually
The normal side was stronger initially than the shortened side. lengthens throughout its entire extent after which lengthen-
The differences in strength, however, between the 2 sides did ing appears to be localized at the osteotomy site and is more
not meaningfully change with a difference of 15.7 % at the associated with fibrosis which itself would tend to weaken
beginning of the procedure and 13.1 % at the end of the study. the muscle. Lee et al. showed, therefore, that lengthenings
When the relationship of the knee extensor strength to the up to 10 % have little histopathologic change in muscle and
muscle and bone area of the mid-thigh was calculated there that progressively greater lengthening to 20 % and then 30 %
was no change post operatively in the normal side but a slight led to more conspicuous changes [534]. They also felt that
increase in the extensor strength in the operated side. That once lengthening extended beyond 20 % irreversible chan-
report is similar to the clinical impression that once a ges were more likely to occur in the muscle itself. Carroll
lengthening has been completed effectively and range of joint et al. felt that the changes in the muscle were primary rather
motion regained the muscle strength is maintained. than being secondary to nerve stretch phenomena [535].
Barker et al. prospectively studied the effect of limb They concluded that lengthening of the tibia by more than
lengthening on muscle function and noted a decrease in 11 % consistently produced muscle changes in the leg and
muscle strength in patients undergoing lower limb length- cartilage damage in the ankle joint.
ening 6 months after fixator removal but a full return to Effects of lengthening on articular cartilage of adjacent
preoperative status by 2 years with the procedure having no joints: Stanitski et al. documented the effect of femoral
adverse effect on function [533]. lengthening on the articular cartilage [536]. They felt that
Lee et al. studied changes in the gastrocnemius muscle in 30 % femoral lengthening causes reproducible knee cartilage
relation to the percentage of lengthening in the rabbit tibia injury evident by actual loss of cartilage substance and fib-
using the callotasis technique [534]. Lengthenings were rillation. Application of a modified Ilizarov apparatus to the
done of 10, 20, and 30 % assessing 75 rabbits, 25 in each femur and tibia with coaxial hinges at the knee followed by
group. The study was based on histopathologic and mor- 30 % lengthening resulted in less severe damage than when
phometric assessments of the muscle. Biopsies were the femur was lengthened independently suggesting that
obtained from the medial gastrocnemius of both hind legs there was a protective effect of the femoral–tibial apparatus
immediately prior to sacrifice at termination of the length- on joint compression.
ening procedure. As compared with the control side the Carroll et al. noted histologic changes in the articular
lengthened side had substantial differences with fiber size cartilage of the tibiotarsal joint at both gross and histologic
variation noted in all 3 lengthening groups. Significant dif- levels with tibial lengthenings >11 % including fibrillation,
ferences, however, in internalization of muscle nuclei and empty lacunae, and matrix degeneration. The tibia had been
endomysial fibrosis, which represent more definitive chan- rendered short initially by a proximal tibial epiphyseal arrest
ges, were observed only after 20 and 30 % lengthenings. using the Phemister technique, after which a lengthening
There were no differences in degeneration or regeneration was performed in 16 [535].
among any of the lengthening groups. The fiber size varia- Nakamura et al. studied knee articular cartilage changes
tion was thought to be due to an increased number of in association with limb lengthening by the callotasis tech-
atrophic fibers rather than the presence of hypertrophied nique in 18 rabbits with a distraction rate of 1 mm/day [537].
fibers. As the lengthening percentage increased the Distraction began the day after operation. On the right side
histopathologic scores of each parameter of the lengthened the frequency was 0.5 mm increments every 12 h while on
side showed a linear increasing trend reflecting an increasing the left it was controlled automatically leading to 120
severity of the histopathologic changes. Since the rabbits smaller incremental increases which averaged 0.0083 mm
were sacrificed at the termination of lengthening no infor- every 12 min. Histologic changes were much less in the
mation was available as to whether or not some of these multi step auto-distractor technique side than in the side
changes might have regressed. undergoing twice daily 0.5 mm increments. This study was
6.9 Management of Lower Extremity Length Discrepancies 725

also divided into assessments with length increases of 10, results from this large series were parallel to the overall
20, and 30 %. The incidence of cartilage degeneration on the reports with 5 % intraoperative complications and 17 % total
2-step side was 2/5, 5/6, and 6/7 at the 10, 20, and 30 % complications. Makarov et al. used intraoperative
length increases while on the 120-step side it was much less somatosensory evoked potentials (SSEP) to detect acute
at 0/5, 1/5, and 1/7 at the corresponding length increases. peripheral nerve injury during external fixation application
The numbers 5, 6, and 7 refer to the numbers of animals in [540]. There were 42 Ilizarov surgical procedures of the
each group. lower extremities reported in 40 children. Significant dete-
Nerve changes due to the lengthening procedure: rioration or total loss of SSEP response during surgery
Although nerve stretching in association with limb length- occurred in 4. They proposed the use of monitoring to detect
ening can lead to sensory and motor nerve deficits, in most early abnormalities and possibly minimize or eliminate their
patients even with significant lengthening motor and sensory long-term effects by changes in surgery pattern.
function is maintained. More sensitive neurologic testing is In a report continuing their studies, Makarov et al. con-
beginning to show that the margin between maintained tinued using somatosensory evoked potential (SSEP) moni-
function and diminished function is narrow. In those patients toring to detect nerve compromise for their limb lengthening
who develop weakness in association with significant and deformity correction procedures with external fixators
lengthening, there has also been the question as to whether it is [541]. In 306 procedures in 233 patients (mean age 12 years
myopathic or neuropathic in nature. Young et al. studied six (range 1–21), significant SSEP changes were seen in 58
consecutive patients completing tibial lengthening by the procedures (19 %). In 32 (10.5 %) the changes were tran-
Ilizarov method using nerve conduction studies and elec- sient, resolving with time/steps to reverse surgical cause.
tromyography (EMG) [538]. At the termination of lengthen- The remaining 26 (8.5 %) were assessed based on whether
ing there were no complaints of sensory or motor or not corrective action was taken [16 cases, no action,
abnormalities in the group and all patients were normal to showed 13 (81.2, 4.2 % overall) with postoperative neuro-
clinical examination. All six subjects, however, demonstrated logic defect of which 6 were permanent and 7 resolved
significant sensory and motor nerve response abnormalities. slowly over 5–18 months; 10 cases, corrective action taken,
Electrodiagnostic testing showed abnormalities in 6 of 6 deep showed 4 (40, 1.3 % overall) with a temporary deficit
peroneal nerves and 5 of the 6 demonstrated abnormalities in resolving in 1–8 months but none with permanent deficits.]
superficial peroneal sensory responses. Two of 6 demon- Noguiera et al. retrospectively studied 814 limb length-
strated abnormalities related to the posterior tibial nerve. Five ening procedures to assess associated nerve lesions [542].
of 6 patients demonstrated needle EMG abnormalities. Existence of a nerve problem was based on clinical signs and
Although the study was limited there was clear evidence for symptoms of motor impairment, sensory alterations, referred
an axonal neuropathy based on the nerve conduction and pain, and/or positive results of quantitative sensory testing.
EMG results. A purely muscle etiology would not be expected A nerve lesion was detected in 76 of the limbs (9.3 %) with
to demonstrate sensory nerve abnormalities. The authors did 16 % detected immediately after surgery and 84 % during
additional studies in an effort to implicate slightly increased gradual distraction. Patients with double level tibial lenght-
compartment pressure as part or all of the causation of the enings and those with skeletal dysplasia were at higher risk
neuropathic findings. The mean lengthening was 5.6 cm with for nerve lesions. Nerve decompression was performed in 53
a range from 4.0 to 7.0 cm; the lengthenings, therefore, were cases (73 %) but slowing the rate of lengthening was
well within the normal range in terms of extent. sometimes sufficient. Early recognition and response led to
Galardi assessed peripheral nerve damage during limb better resolution of the problem. Testing with a pressure
lengthening [539]. Electrophysiologic studies on limbs specified sensory device was the most sensitive way to
having 5 bilateral tibial lengthenings showed reduced motor detect developing nerve problems. Once dealt with, contin-
conduction velocity in 2 tibial and 3 common peroneal ued lengthening was done. Complete clinical recovery
nerves after a mean lengthening of 27 %. occurred 74 of 76 cases.
Makarov et al. reviewed the literature concerning neu- Rozbruch et al. report the value of high-resolution ultra-
rologic problems in relation to limb lengthening and pub- sonography in detecting the precise site of nerve involve-
lished the data in chart form in terms of intraoperative and ment [543]. While EMG and nerve conduction studies
total neurologic complications [540]. A study of 8 reports identify problems they do not locate the site of the problem.
encompassing 946 cases reported 51 intraoperative nerve MRI and CT are difficult to use because of the extensive
injuries (5.4 %) while total neurologic complications from metallic instrumentation.
12 reports described 215 complications in 1214 patients
(17.7 %). A study of the Ilizarov technique, originally (iv) Distraction Osteogenesis Research: The distraction
published in Russian, accounted for 703 of the patients and osteogenesis/callostasis technique has a considerable
726 6 Lower Extremity Length Discrepancies

body of animal research data associated with it. The compared with immediate distraction [544]. Histologic
extensive work of Ilizarov and associates has been pre- studies outlined the differences in response to timing.
sented in English by Paley [484] and in translation by The authors pointed out that delay between initial sur-
Ilizarov [504, 505, 507, 508]. Ilizarov showed that the gery and onset of start of distraction characterized
proper biomechanical environment was extremely studies published in the 1930s by Bosworth [473] and
important for bone regeneration which involved not only Abbott and Saunders [545], the latter specifically noting
the interfragment stability but also the timing of the excellent callus formation when they waited 10 days
beginning of lengthening and the rate of lengthening. before distraction. Kojimoto and associates also per-
His early work demonstrated that preservation of the formed callus distraction in the rabbit and demonstrated
intramedullary circulation, in particular the nutrient excellent bone healing even following medullary vessel
artery, was important but subsequent assessments have destruction as long as the periosteum was carefully
shown that even when cut the nutrient artery will repair preserved [546]. Experimental studies in the dog have
quickly over a period of a couple of weeks as long as been reported by Aronson and associates [547] quanti-
stability is present. Ilizarov also showed that the rate of fying mineralization by CT methods and by Delloye and
osteogenesis was closely related to the distraction rate associates [548] documenting regenerate bone formation
and that the optimal rate was 1 mm/day with quicker using microradiography and histology.
rates of 1.5 and 2 mm a day slowing osteogenesis and a
slower rate of 0.5 mm leading to premature consolida- Aronson et al. studied the histology of distraction osteo-
tion. Repair was improved with 4 separate lengthenings genesis using Ilizarov and Wagner external fixators for tibial
of 0.25 mm each 6 h apart distinct from 1 lengthening per lengthenings with 8 dogs in each group [549]. The distrac-
day for the entire distance. The smaller, more frequent tion osteogenesis procedure was performed after a 7 day
lengthenings minimize damage to the repair microvas- latency period and distraction at 0.25 mm every 6 h until an
culature and to the early repair cells and matrices. He osteotomy gap of 2.8 cm (about 15 % of initial tibia length)
showed that bone formed during the course of distraction was achieved. Correlative histologic and radiographic stud-
osteogenesis is well organized and longitudinally ori- ies were then made at varying time periods. Both groups
ented in the direction of the distraction forces. New bone healed equally well. Radiodense columns of bone appeared
forms initially in the medullary canal adjacent to the cut regularly between days 21 and 28 of the distraction osteo-
cortices and then passes progressively toward the center genesis procedure. These took origin from either bone end
of the distraction gap. The central region between either with the most central part of the gap persisting as a radi-
cut cortical zone is referred to as the interzone and is the olucent band. Areas of bone repair were aligned in linear
region where bone forms latest and distraction occurs fashion parallel to the long axis of the gap and of the entire
longest. The region tends to be filled with immature bone. The next phase of healing encompassed continuous
fibroblastic cells which transform relatively late into bone tissue traversing the gap from end to end. The radi-
osteoblasts. In most instances, bone formation is via the olucent band corresponded histologically to parallel bundles
intramembranous route with no cartilage forming in the of collagen intermixed with cells also oriented in the direc-
gap region. The new bone is oriented along the longi- tion of the distraction force. The vascular channels were also
tudinal microvasculature and quickly forms a lamellar noted to be longitudinally oriented and there was no mention
orientation. The interzone region ossifies quickly after of cartilage formation, and at higher power magnification
distraction has stopped. New bone formation is seen as there was direct transformation of fibrous matrix into bone
early as the second or third week after beginning dis- strikingly similar to the intramembranous ossification char-
traction and the interzone region is then usually seen as a acteristic of the embryonic phase. In a subsequent histologic
central transverse radiolucency. During the several analysis of the repair gap in tibial distraction osteogenesis by
months of the remodeling phase the cortex thickens and the Ilizarov method in dogs, it was shown again that
eventually the medullary cavity is reformed. The origi- intramembranous ossification proceeded from each cortical
nal work of DeBastiani and associates is almost totally end toward the central fibrous interzone. There was good
clinical in nature, although use of the apparatus in correlation between histologic repair and mineralization as
experiments distracting the epiphyseal plate has been shown by CT scanning [547]. Aronson has summarized well
reported. Specific reports on distraction osteogenesis in the histologic, biochemical, radiographic, vascular, and
the rabbit have been published by White and Kenwright biomechanical studies on distraction osteogenesis [514].
who noted that delayed distraction in the skeletally Delloye et al. studied bone repair during distraction
mature rabbit tibia led to more vigorous osteogenesis lengthening on the forearms of mature dogs using the
6.9 Management of Lower Extremity Length Discrepancies 727

Ilizarov system [548]. With distraction both periosteal and lengthenings. Lascombes et al. were able to harvest 11
medullary callus on either side of the gap gave rise to new biopsies of repair bone during bone lengthening following
bone trabeculae. These were oriented along the direction of the Ilizarov technique [550]. The mean age of the patients
distraction and progressively approached one another. The was 13.5 years and time after the initial procedure ranged
characteristic central transverse region of gap radiolucency from 23 to 502 days. Bone was noted histologically along a
was also reproduced. Bone in longitudinal alignment tra- long axis of the gap as early as the 3rd week. There was a
versed the entire gap region linking proximal and distal bone distinct linear alignment to the bone trabeculae along the
fragments 4 weeks after the end of the lengthening period. long axis of the bone. New bone formation was of the
Most of the new bone formed by intramembranous ossifi- intramembranous type without evidence of a cartilage stage.
cation with some foci of cartilage seen. Specific delineation Osteoblastic and osteoclastic activities were prominent and
of the cortex began to be noted at 3 months but was still not remodeling was continuing even one year after initial
fully achieved at 5 months. Procedures were performed on intervention. Mature lamellar bone was noted however by
13 adult female dogs, some unilateral and some bilateral, the fourth month postsurgery. Saleh et al. analyzed bone
totaling 20 operative procedures. Slight variations of tech- from 8 patients undergoing distraction osteogenesis using
nique were used to assess their influence on repair. In gen- the Orthofix technique [551]. The specimens were obtained
eral, initiation of periosteal and endosteal callus at the bone from 102 days to 4 years post surgery. The earliest bone
ends became apparent at 3 weeks and with progressive synthesized was woven with high cellular activity. This was
distraction bone repair along the long axis of the gap and soon covered by lamellar bone which with time developed a
adjacent to either cut end was noted. Bone regeneration characteristic Haversian architecture.
occurred equally at proximal and distal ends with the central The results of each of several experimental reports on the
radiolucent region seen. Bone bridging of the gap was histology of repair in distraction osteogenesis have been
usually achieved in a month after the end of distraction but similar. Many have been correlated with clinical studies
full cortical definition was not noted circumferentially even primarily radiologic but on occasion utilizing biopsy mate-
at 20 weeks. Microangiography revealed that the integrity of rial. Among the characteristic features are the orientation of
the medullary artery could be preserved after careful corti- newly synthesized collagen and then bone trabeculae along
cotomy. Callus was vascularized by both periosteal and the long axis of the repair gap parallel to the distraction
medullary systems. The microradiographic study showed the forces. In the vast majority of instances, there is direct
first signs of osteogenesis at both ends of the distracted bone intramembranous bone formation present initially adjacent to
segments from both intramedullary and subperiosteal sour- either cut end with the central region or interzone healing
ces at 2–3 weeks. Longitudinally oriented callus was noted last. There is a contribution also from the inner layer of the
at 4–6 weeks. No evident difference was observed between reconstituting periosteum which also tends to be along the
lengthening after corticotomy or full transverse osteotomy longitudinal axis and to represent new bone formation. On
with respect to the amount of callus. Histologic evidence of occasion, cartilage can be seen within the distraction gap and
bone repair was slightly in advance of radiologic manifes- this subsequently turns to bone by the endochondral mech-
tations. Medullary and periosteal osteogenesis were partic- anism. The presence of cartilage, however, is best interpreted
ularly active at 2 weeks. Woven bone was synthesized as a sign of less than optimal stability and does not represent
initially and there was abundant vascularity associated with true endochondral growth but rather the formation of carti-
this. With progressive distraction and time the longitudinal lage on the basis of increased interfragmentary movement
orientation of the new bone trabeculae was seen. Bone and then conversion of that cartilage to bone once better
formed from a membranous ossification sequence with the stabilization occurs. Callotasis means stretching of the bony
advancing fronts of osteogenesis approaching each other callus.
from either side and fusing approximately 4 weeks after Not all instances of bone lengthening heal with a uniform
distraction was ended. The healing sequence was the same distribution of bone surrounding a central interzone region.
regardless of whether the bone had been broken by corti- Hamanishi et al. classified the radiologic pattern of callus
cotomy or transverse osteotomy. Some areas of highly cel- formation seen with the Orthofix procedure in 35 limbs
lular cartilage and fiber cartilage were noticed during the first (Fig. 6.23b) Table 6.5 [552]. One of the continuing problems
2 months but these were invaded soon by vessels followed with bone lengthening procedures is this variable state and
by endochondral ossification. Bone marrow appeared to be pattern of bone formation even where the surgeons involved
the larger contributor to the amount of interfragmentary appear comfortable that a relatively uniform technique is
callus but periosteal callus also constantly supplied the being used. The categorization defined by Hamanishi et al.
peripheral part of the regenerating bone. involved (i) the external pattern with lateral bulging of the
Similar findings were reported by Lascombes et al. [550] bone, (ii) a straight pattern in which the gap filled uniformly,
and Saleh [551] in studies of bone biopsies in human (iii) an attenuated pattern in which the diameter of bone
728 6 Lower Extremity Length Discrepancies

Table 6.5 Classification of distraction callus based on radiographic lengthening until 5–7 days after osteotomy [554]. They
appearances concluded that autogenous new bone formation in limb
External Callus forms between the fragments but also extends beyond lengthening related primarily to the management protocol
the cortices in symmetric widened fashion and was distinct from the external device used or even the
Straight Callus forms between the fragments across the entire width location of the osteotomy. Their best results were reached
of the gap in full alignment with the cortices when the surgical technique involved circumferential
Attenuated Callus is narrower than the original bone and forms in decortication, partial corticotomy with osteoclasis or crack-
hourglass fashion being narrowest midway between the two distracted
fragments
ing of the posterior element, a postoperative period of fixa-
tion without lengthening of 5–7 days, and an eventual
Asymmetric Callus formation is asymmetric with more forming on
one side of the gap than the other (generally on the concavity when distraction rate of 1 mm/day.
the two fragments become angulated)
Pillar A narrow central linear column of callus is the only bone (v) Cell and matrix deposition patterns in distraction
forming between the two fragments osteogenesis: Rabbit model. Studies have been per-
Agenetic Only scattered discontinuous fragments of callus form in the formed in our laboratory on the histologic responses of
distraction gap tibial bone lengthening procedures using 4–5 month old
Based on the classification of Hamanishi et al. Acta Orthop Scand rabbits [555]. The Synthes Mini Lengthening apparatus
1992;63:430–433 (Synthes, USA, Inc., Paoli, PA) was easily placed and
well tolerated by the animals. Figure 6.23bi, ii shows
formed centrally was less than at either end, and opposite radiographs of the mini-lengthening apparatus attached to
pattern where, usually with angular deformity, more bone the rabbit tibia at 22 days. The initial work with this
was formed on the concave than on the convex side, (iv) the apparatus involved the use of skeletally immature 4–
pillar category where a thin linear bone collection formed 5 month old rabbits with distraction begun on the first
centrally, and (v) the agenetic form where there were only day postsurgery. Tibial lengthenings were then per-
isolated spicules of bone within the gap. The healing index formed on 20 skeletally immature rabbits using the
correlated nicely with the pattern as would be expected. The Synthes apparatus. A complete 360° turn of the spindle
index (correlating the number of months per 1 cm of knob gives 0.7 mm distraction. This represents the
lengthening) was in the respective types 1.1, 1.3, 1.5, 2.1, amount of daily lengthening and was performed in 2
3.7, 4 with an overall mean of 1.7 since most patients were stages, one in the morning and the other in the late
in the external or straight healing pattern category. afternoon. Subsequent studies assessing variable age and
One of the main principles articulated by Ilizarov [507, distraction parameters increased the total number of
508] and deBastiani et al. [497] was the need for delay in procedures to 60. Animals were sacrificed at varying
beginning the lengthening to allow for early revasculariza- intervals from 6 to 46 days postsurgery. Nine animals
tion and early bone formation which would subsequently were allowed to heal for additional periods after length-
enhance the repair process. Aside from the excellent clinical ening was completed. The lengthening achieved in those
evidence, experimental studies also confirmed the value of lengthened to the time of consolidation of the regenerate
delay in distraction. Gil-Albarova et al. used the Orthofix bone, which was generally 21 days, ranged from 11.8 to
fixator to compare results in femoral diaphyseal osteotomy 13.9 % of total preoperative tibial length. Periodic X-rays
on 24 3-month-old lambs beginning distraction in half on the of the leg were obtained at one to 2 weekly intervals. At
first postoperative day and delaying until the tenth post sacrifice the entire tibia was removed and specimen
operative day in the other half [553]. The femur was X-rays were obtained in two planes (Fig. 6.23ci–v). The
lengthened by 2 cm at a rate of 1 mm/day. Both radiographic specimens were then processed for light microscopic
and densitometric studies of the lengthened callus at 1, 2, histologic study.
and 3 months showed that delayed distraction when com-
pared with immediate distraction improved the quality of the Tissue preparation involved removal of the distracted
callus with quicker, denser and more homogeneous bone segment and adjacent bone with a saw, decalcification,
formation. The value of delay prior to distraction was also sectioning in sagittal or coronal planes, and slide preparation
shown clinically by Lokietek et al. comparing clinical results using the JB4 plastic embedding technique. This allowed
with the Wagner technique with immediate intraoperative excellent visualization of the histologic detail which was
lengthening of 1 cm and postoperative distraction of then correlated with the radiographic appearances. Specimen
1 mm/day and the Ilizarov technique which did not begin photographs were also taken after decalcification and
6.9 Management of Lower Extremity Length Discrepancies 729

halving of the specimen (Fig. 6.23d). The specimen X-rays procedures [557]. The intramedullary rod, generally a
of the entire bone were taken using a standard technique long Rush rod, needed to be narrow enough to allow
with a magnification factor <0.01. This allowed accurate distraction to occur and wide enough to allow for sta-
measurements to be made of the length of the entire bone bilization. Each of oblique, step-cut, and transverse
and of the distraction gap. A lengthened tibia at sacrifice osteotomies were used but ultimately they favored the
postfixator removal is shown in Fig. 6.23e. transverse procedure since it was simpler, led to better
Histologic sections were processed from all animals. At control of angulation and showed no difference in
6 days following lengthening the gap was filled with blood healing time than the other two patterns. Traction and
clot and fibrinous tissue with no geometric pattern of orga- countertraction were provided through Steinmann pins
nization noted. Mesenchymal cells were accumulating into the proximal and distal femoral fragments followed
adjacent to each of the bone ends with early intramembra- either by the application of traction upon the limb
nous bone formation seen. The histologic studies from suspended in a Thomas splint or the use of a
intervening time periods demonstrated the pattern of new traction-counter traction apparatus which had an upper
bone formation. Animals which had undergone lengthening femoral ring and a frame on both the inner and outer
16 and 20 days showed the entire spectrum of repair cells aspects of the thigh and leg. Traction was started
and matrices within the gap. The repair was not uniform immediately after the operation and continued at a low
across the lengthening gap. Immediately adjacent to the cut rate until the desired length was obtained or no further
cortical bone ends the repair bone was being transformed to length was obtainable. The patient remained in bed
a lamellar configuration although evidence of initial woven during the lengthening procedure. The amount of
bone persisted. Closer toward the center of the distraction lengthening of the femur varied from 3/8 of an inch to 4
gap the bone repair matrix was aligned longitudinally as 1/4 in. In the entire series of 23 lengthening operations,
were the accompanying blood vessels. Further toward the the average gain was 2.18 in., or slightly <2 1/4 in. or
center of the distraction gap the repair tissue was increas- 5.5 cm. In 20 of the straightforward patients the aver-
ingly more woven in configuration with progressively less age time required for the lengthening was about
lamellar bone deposited. In the center of the distraction gap, 11 weeks. In 13 of the 23 procedures, union of the bone
mesenchymal cells persisted and in some sections areas of occurred in an average of 32 weeks and did not vary
organizing clot persisted. This histologic picture correlated between the various types of osteotomy. After 10 of the
extremely well with the specimen photographs, specimen osteotomies one or more bone grafting procedures were
x-rays and the clinical finding of an inability to further necessary to obtain bony union. All of the bone grafting
distract the bone after 3 weeks. The model reproduces the procedures were performed as secondary interventions
clinical and radiographic findings being described in human because of delayed union and no primary bone grafts at
distraction osteogenesis. Examples of repair sequences the time of lengthening were used. The authors as well
shown by histologic processing are illustrated in as Abbott and Saunders [545] in 1939 specifically
Figs. 6.24a–f. noted that lengthening of the callus was occurring.
They indicated that “during its growth the bone callus
(d) Lengthening along an intramedullary rod: Lengthening may be stretched out in length.” There was one late
along an intramedullary rod has attractive features. The disturbance in circulation during the process of
rod helps maintain alignment during lengthening lengthening but this resolved with reduction of the
eliminating translational and angular deformation, traction weight. A palsy of the peroneal nerve occurred
allows external fixation to be removed earlier lessening in 7 patients but in 5 it was transient. There were 5
the likelihood of infection and improving joint range of patients with a posterior subluxation of the tibia on the
motion, and protects against fracture and deformation femur but after management no major problems resul-
after healing while allowing earlier full weight bearing. ted. Fractures of the lengthened femurs occurred in 4 of
(i) Intramedullary rod with simultaneous external fixator the 23 cases. The primary aim of the procedure how-
distraction: For several decades, some surgeons have ever, the control of alignment during lengthening, was
performed lengthening along an intramedullary rod successfully obtained thus eliminating many of the
which serves to enhance stability and help maintain difficulties associated with lengthening procedures.
alignment at the lengthening site. The earliest formal Transverse osteotomy led to healing as quick and as
paper with this approach was by Bertrand who used sound as the step cut or oblique osteotomies. Once
one or 2 narrow intramedullary rods during distraction reasonable healing had occurred the patients were
of the femur [556]. Bost and Larsen used an intrame- protected with either cast or brace during the transition
dullary rod to assist femoral lengthening in 23 phase to full unprotected weight bearing.
730 6 Lower Extremity Length Discrepancies

Fig. 6.24 Histologic study of the gap region correlates well with the bone persisting. Note the orderly array of osteoblasts on the lamellar
radiographic studies showing advancing fronts of repair adjacent to trabecular surfaces. e A region closer toward the center of the gap at 3
each sectioned end rather than a uniform repair process throughout the weeks. Note the predominantly longitudinal orientation of the new
entire gap simultaneously. a The varying regions from which the bone formation. This is related primarily to the associated longitudinal
histologic studies in this figure were taken. b Early new bone orientation of the vasculature. At the right a primarily lamellar
formation adjacent to cortex is seen at left. At right is undifferentiated trabeculum of bone is seen lined with osteoblasts. At the far left some
mesenchymal tissue at center of gap. c Histologic section near the initially deposited woven bone persists. f Tissue from the center of the
persisting cortex at 10 days following surgery and initiation of gap region at 2 weeks. This is the most radiolucent appearing region
distraction. The initial bone synthesized is woven which is more on the radiographs as it is the newest site of bone formation. The
darkly staining here. Shortly thereafter better-oriented lamellar bone is mesenchymal cells have formed a primarily woven bone matrix. Note
synthesized on the woven scaffold. Osteoblasts line up nicely on the however the longitudinal orientation, the early formation of lamellar
lamellar surfaces. d A higher power view in another rabbit in this bone, the lining up of osteoblasts on these lamellar surfaces and in
same general region 2 weeks following surgery now shows a particular the marked osteoclastic resorption occurring even as
predominance of lamellar bone with only small remnants of woven synthesis proceeds

Wasserstein used an unreamed flexible intramedullary the limb. The technique was used in patients between 5
rod in association with distraction through an external and 25 years old although in selected cases those up to
fixator of the Ilizarov type followed by a cortical allograft 35 years of age were operated. The technique was best for
of the distraction gap using a tubular bone segment once discrepancies >6 cm since lengthenings under 6 cm
the lengthening had been achieved [558]. Wasserstein appeared to heal adequately with distraction osteogenesis
transplanted cylindrical allografts into the distraction gap techniques alone. The technique was used in 300 patients
both to decrease the treatment time as well as to increase over a 15 year period for both femoral and tibial
the stability of the fixation and insure proper alignment of lengthenings (Fig. 6.24).
6.9 Management of Lower Extremity Length Discrepancies 731

Fig. 6.25 a Tibial lengthening

around an intramedullary rod is
shown (Reprinted with
permission from Herzenberg and
Paley, Tibial lengthening over
nails (LON). Tech Orthop
1997;12:250–259. Copyright
1997 Wolters Kluwer Health).
b Adaption of the innominate
osteotomy by using a rectangular
rather than a triangular insert was
proposed by Hall and Mills to add
length as well as acetabular
re-orientation at the pelvic level

Paley et al. have performed femoral lengthening with the patients who required rod removal for osteotomy, all 19
Ilizarov or Orthofix distractors over an intramedullary nail others had no malunion or delayed healing. They cautioned
and compared it with a matched group of patients having that use of simultaneous external/internal fixation could lead
lengthening with the standard Ilizarov technique (Fig. 6.25a) to infection and knee stiffness problems but each of the three
[559]. The Ilizarov external fixator was used in 11 length- who developed deep osteomyelitis had a previous history of
enings and the Orthofix fixator in 21 with a 10 mm intra- osteomyelitis before the index procedure and knee sublux-
medullary femoral rod. The mean amount of lengthening in ation problems occurred in those with >20 % lengthening.
32 procedures was 5.8 cm (range 2–13 cm) and the mean age Popkov et al. reported favorable results in lengthening for
of the patients was 26 years (10–53 years). Results in several Ollier’s disease using circular external fixators combined
categories were compared with the standard Ilizarov femoral with intramedullary nailing using 2 elastic stabilizing nails
lengthening that had been performed in 32 matched patients. [302, 561]. They compared 7 patients with the intramedul-
The authors noted that lengthening over an intramedullary lary nails to 37 having limb lengthening using only the
nail reduced the average time of external fixation by almost external fixators and found markedly improved removal
one half. The range of motion of the knee returned to normal times for external fixation. In the ESIN (intramedullary
a mean of 2.2 times faster in the group that had lengthening nailing) group the mean healing index (to time of external
over the intramedullary nail. There were six refractures of fixation removal) was 23.2 days/cm for the femur,
the distracted bone in the standard Ilizarov group but none in 22.4 days/cm for the tibia, and 11.6 days/cm for polyseg-
those protected with the intramedullary nail. They concluded mental (femoral–tibial) cases compared to 31.6 days/cm
that the advantages of lengthening over an intramedullary (femur), 35.7 days/cm (tibia), and 19.9 days/cm (polyseg-
nail included a decrease in the duration of external fixation, mental) for the external fixators alone. They noted a sub-
protection against refracture, and earlier rehabilitation. stantially reduced period of external fixation with limited
Song et al. reported lengthening in 22 femurs over an postoperative complications with the use of intramedullary
unreamed femoral intramedullary nail [560]. The mean age nails with external fixator limb lengthening.
was 22 years (13–35 years) and the mean lengthening 5 cm Guo et al. also noted that tibial lengthening with the Ili-
(2.7–8.1 cm). The external fixator was removed after 20 (8– zarov method over an intramedullary rod (51 limbs) pro-
30) weeks. The mean external fixation index was 24 days/cm vided advantages over the Ilizarov method alone (23 limbs)
(11–35) and the mean consolidation index was 43 days/cm including a shorter time for external fixation (129 vs.
(26–55) of lengthening. The authors felt that the approach 282 days) and lower complication rates (0.47/tibia vs. 1.0)
reduced the duration of external fixation. Other than three [562]. Kim et al. have reviewed their experience lengthening
732 6 Lower Extremity Length Discrepancies

tibial segments over an intramedullary nail. In 80 segments bone distraction is performed by use of a rotation
(40 patients undergoing bilateral lengthening to increase maneuver of the limb which serves to distract the two
height) the average lengthening was 7.73 cm (23.5 % of segments of the rod at the internal telescoping mecha-
initial length) [563]. In spite of the rod, there was still valgus nism using a ratchet effect. The approach has come to
angulation in 20 limbs (25 %). The same group also reported be described as intramedullary skeletal kinetic distrac-
on 118 tibial segments lengthened over a rod, specifically tion (ISKD). It is primarily used in young to
assessing deep intramedullary infection [564]. Both super- middle-aged adults after skeletal maturity but some
ficial (13) and deep (6) infections occurred (16 % overall, centers are using it in late adolescence either at or just
11 % superficial, 5 % deep). The deep infections required before full skeletal maturity.
either early (4) or delayed (2) rod removed and all had
delayed healing. An early precursor of this technique had been described
by Gotz and Schellmann [567] in which a hydraulic dis-
(ii) Intramedullary rod placement at completion of external tractor was placed in a modified interlocking intramedullary
fixator distraction: Rozbruch et al. report a favorable nail to provide for continuous distraction. Guichet and col-
experience completing the lengthening with the exter- leagues in France developed a model for this form of
nal fixator alone and then placing an intramedullary rod lengthening [568]. Baumgart et al. developed the first
to allow for much earlier external fixator removal motorized lengthening device with a subcutaneous receiver
[565]. They call the procedure “lengthening and then [569]. The system developed by Guichet and collaborators
nailing” (LATN). The nail supports the bone during the has the two telescoping fragments relating to each other
consolidation phase, protecting it from deformation and internally such that with external rotation of the limb a
re-fracture. The procedures were done primarily in clicking and locking mechanism allows for elongation with
young adults after skeletal maturity. The classic versus no movement possible in the reverse direction. Fifteen
LATN groups both has 27 patients at mean ages of 30 external rotation maneuvers of the lower extremity, which
(11–57) and 35 (22–55) years. The time of fixator serve to rotate the distal femoral fragment to which the
removal was reduced from a mean of 29 weeks to a intramedullary rod is attached, allows for a lengthening of
mean of 12 weeks without evidence of negative prob- 1 mm/day. The device was developed for femoral length-
lems with the nailing intervention. ening [570]. An osteotomy is performed such as would be
done for a closed femoral shortening procedure. The prin-
Insertion of locked plate at completion of external fixator ciples of distraction osteogenesis are used with lengthening
distraction: In an additional effort to decrease the time that beginning on the eighth postoperative day. The 1 mm/day
external fixators were on, studies compared healing in 27 lengthening is achieved in four separate time periods 6 h
extremities lengthened with the Taylor Spatial Frame and apart much as is used in the Orthofix and Ilizarov approa-
then insertion of a locked plate (LAP) and the classic tech- ches. Once the appropriate length has been achieved rotation
nique in 27 extremities where the external fixator remained is no longer performed and the rod serves as a regular
in place until full healing [566]. Fixation was on less in the intramedullary rod until healing occurs. Guichet et al. pro-
plating (LAP) group (4.5 vs. 6.2 months), pin-tract infection jected that the mechanical intramedullary system, commonly
was less in the plating group (2 vs. 12), malalighnment was referred to as the Albizzia nail, would be used as an internal
similar (6 vs. 7), but significant varus occurred in the plating dynamic fixator for the progressive lengthening of segments
group in 2 cases of plate breakage. Possible suggested of the lower extremity. The apparatus also has a
solution was “stronger plates.” In the Wagner technique, dynamization capability built in once the desired lengthening
which this approach returns to, the plate used was specifi- has been reached. The rod is fixed to the femur both prox-
cally constructed with no screw holes in the segment over- imally and distally allowing for stabilization at the same time
lying the lengthened healing gap. that lengthening is occurring. The external rotation move-
ment allowing for the lengthening to occur is one of 20°
(iii) Intramedullary telescoping rod for lengthening without following which the limb then returns to its normal position.
need for external fixators: A more recent innovation in Each rotation corresponds to a lengthening of 1/15 of a mm
limb lengthening has been the development of an such that 15 movements correspond to 1 mm of lengthening.
intramedullary rod which contains an internal tele- Each movement provides an audible clicking sensation.
scoping mechanism such that the lengthening can be Some patients experience considerable pain with the rela-
performed in the complete absence of any external tively large degree of rotation necessary to activate the dis-
fixators. Following osteotomy and fixation of the upper traction. Other devices are the fully implanted motorized
and lower portions of the intramedullary rod to the intramedullary nail system (FITBONE) based on the
6.9 Management of Lower Extremity Length Discrepancies 733

principles introduced by Baumgart et al. [569] and the ISKD internal device was compared with lengthening using
(Intramedullary Skeletal Kinetic Distractor) a mechanically external fixation and mean lengthening up to 90 days was
activated implant which distracts by milder rotations of 3°– 3.9 cm which was actually somewhat more than with the
9° [571]. The rotation oscillations are part of the physio- external fixator. Bone regeneration in the intramedullary
logical gait pattern. A distraction of 1 mm is achieved by group was completely satisfactory. Advantages are attractive
160 rotations of 3°. The actual amount of distraction is in the sense that there is no external apparatus and the skin is
controlled by an external handheld monitor which measures virtually intact. There are no structures to impede the mus-
the orientation of a magnet on the distal part of the internal cles such that joint motion should be more readily obtained.
rod. Patients can measure the daily distraction; if length is Stability is maintained and angulation either prevented or
insufficient the leg is rotated under the control of the monitor markedly minimized.
until the desired daily distraction is reached. Krieg et al.
reviewed 8 adolescent patients average 15.7 years (range (e) Predictability of Complications with Bone Lengthening
13–18 years) who had femoral or tibal lengthenings with the in Children: An overview of the previous sections
FITBONE motorized intramedullary lengthening device relating to lower extremity lengthening shows that
[572]. The average lengthening was 3.8 cm (range 2.9– relatively few problems occur with lengthening a long
4.7 cm). They felt that the difficulties commonly associated bone by 10 % but that increasing problems occur as
with external fixators were reduced with this method. lengthening progresses beyond 20 % of pre-procedure
Lenze et al. also reported excellent functional results in bone length. Antoci et al. studied all aspects of 116
late adolescent/young adult patients with complications procedures (62 tibial, 54 femoral) from one institution
reduced from those expected with external fixators [573]. [579]. The focus was on the relationship between
Nine of 11 patients were under 20 years of age (14–34 years complications and amount lengthened. Almost all
of age) with a mean age of 18.5 years. segments were lengthened by the Ilizarov (108/116)
The ISKD method is well illustrated in an article by method. The mean patient age at time of surgery was
Leidinger et al. [574]. They treated 25 patients at an average 13.5 years (range 4–20) and the lengthening percentage
age of 25 years (range 16–45 years) with better results in the was 21 ± 16.5 (range 2–94). There was a positive linear
femur. Hankmeier et al. describe the ISKD technique and relationship for all complications with increasing extent
principle well [575]. Burghardt et al. presented 242 lower of lengthening. Neurological complications started at a
limb lengthenings with a device mechanical failure rate of 5 % increase in bone length; with a 15 % increase in
6.2 % [576]. Schiedel et al. studied 69 of their unilateral length the complication rate was 20 %; with a 25 %
lengthenings with the Intramedullary Skeletal Kinetic Dis- increase −35 % rate; and with a 40 % increase −60 %
tractor and found eventual better results in the femur (52 of rate. Residual axial deformities began to occur with a
58, 90 %) than in the tibia (5 of 11, 45 %) [577]. However, 5 % increase; a 15 % increase −20 % rate; a 25 %
there were many problems with only 24 of 69 having increase −30 % rate; and a 40 % increase −50 % rate.
problem-free courses while there were 116 difficulties in the External fixation pins began to break or become
other 45 patients. Premature removal of the device was problematic with 10 % length increase with further
needed in 7 cases, 4 due to breakage. worsening at a 40 % increase in length leading to a
Betz et al. recently reviewed and compared each of three −50 % problem rate. Joint contractures developed with
commonly used techniques: Albizzia, Fitbone, and ISKD approximately a 15 % increase with further worsening
[578]. These devices have been used much more often in at a 40 % increase in length leading to a −60 %
Europe then in North America so far. The primary patient problem rate. They showed that lengthening a bone by
group is the young to middle-aged adult, postskeletal 25 % or greater led to appreciably greater complica-
maturity, with length sequelae of uncorrected childhood tions. Dahl et al. [580] noted increasing frequency of
disorders or severe limb trauma. complications at an increase of 15 % of original bone
Caton et al. have presented a brief report with the elon- length, Maffulli et al. [581] at 18 % or greater, and
gating intramedullary nail based on a series of experiments Karger et al. [502] at 25 % or greater.
in sheep [483]. They inserted the nail in the femurs of four (f) Longitudinal growth after diaphyseal lengthening done
sheep bilaterally. Each rotation applied to the limb allowed prior to skeletal maturity. Clinical studies: While an
0.1 mm lengthening with elongation of 1.25 mm/day. In the increased rate of femoral growth has been reported after
first group of animals, the mean lengthening obtained was one-stage procedures for lengthening of the femur and
3.2 cm over 24 days with a percentage elongation of 14.2 %. a variable rate of growth has been noted after
Regenerated bone was noted radiographically at 15 days and Judet-type procedures for lengthening of the femur and
consolidation took place at 5 months. In a second series, the tibia, there have been few detailed radiographically
734 6 Lower Extremity Length Discrepancies

documented studies of growth of bone after lengthen- invariable tendency for increased growth in the femur after a
ing of the diaphysis. Authors of earlier papers that have one-stage lengthening [583]. They noted stimulation of
included the results of lengthening of the diaphysis growth in 33 of 36 patients who had poliomyelitis, in 8 of 13
have commented on somewhat variable but generally who had a congenital short femur (including some who had
good growth after the procedure. However, these proximal femoral focal deficiency), and in 4 of 5 in whom the
studies were not directed toward the specific assess- shortening had another etiology. Overgrowth after fracture of
ment of growth after lengthening, nor did they report the femoral diaphysis treated by skeletal traction followed by
data from radiographic measurements; thus, the exact casting has been recognized for several decades. Assessment
rate of growth after lengthening cannot be determined of 74 patients using serial orthoroentgenograms documented
from them. overgrowth as a universal phenomenon in patients who are
<13 years old, regardless of whether the fracture healed with
We noted from the data on growth in patients at the anatomical reduction, with shortening, or with distraction.
Children’s Hospital Boston, that when lengthening was Increased vascularity to the entire bone brought about by the
performed on a bone that had several years of growth repair process has been thought to stimulate growth at the
remaining the lengthened bone continued to grow at a proximal and distal growth plates. As the repair response that
slightly increased rate in some patients, while in others is engendered by lengthening of the diaphysis is much more
growth became more inhibited than it was before the oper- extensive and prolonged than after fracture of the diaphysis,
ation [582]. Data on growth were assessed from 18 patients overgrowth is expected. The primary pathological condition
who underwent lengthening of the femur or tibia by that causes the discrepancy and resistance of soft tissue to
mid-diaphyseal osteotomy and the gradual distraction tech- distraction, which can exert compressive forces on the
niques of Anderson and of Wagner. The goal was to define growth plates to restrain their growth, can both minimize the
the growth responses to these lengthening procedures. effects of stimulation. However, in the series reported above,
Femoral Lengthening. In each of 7 patients, the rate of the patients who had lengthening of the femur all had an
postoperative growth of the lengthened femur, in relation to increased rate of growth [582].
that of the normal bone, was increased compared with the The decreased rate of growth that was seen in all patients
preoperative rate. The average rate of preoperative growth of after tibial lengthening in the series does not appear to occur
the short femur was 82 % that of the normal side, whereas universally. Variable growth responses to Judet-type
the average postoperative rate of growth was 90 % of nor- lengthening have been documented radiographically by
mal. In one patient, the rate of postoperative growth was Pouliquen and Etienne [584] and by Pouliquen et al. [585].
21 % greater than the preoperative rate, but in all of the In the more detailed of the 2 articles, the authors reviewed
others the increase was from 5 to 8 %. The amount of sur- the results after 39 lengthening procedures; 6 were per-
gical lengthening of the femur averaged 18 % of the pre- formed on the femur and 33 on the tibia although the report
operative length of the bone, with a range of 6–35 %. did not deal with the femoral and tibial procedures sepa-
Tibial Lengthening. In all 11 patients, the rate of growth rately. Their report and the Children’s Hospital Boston series
diminished from the preoperative levels, ranging from a are not strictly comparable since the criteria for inclusion of
46 % diminution to a 3 % diminution. The average preop- patients and documentation were much stricter in the latter
erative rate of growth of the shortened tibiae was 88 % that series. Still, comparisons can be made. In the series of
of the normal side, whereas the average postoperative rate Pouliquen et al. of the patients who had poliomyelitis the
diminished to 64 % of the normal side. The amount of growth was normal after lengthening in 18, slowed in 7, and
lengthening of the tibiae averaged 20 % of the preoperative arrested in 2. Of their patients who had congenital agenesis
length of the bone, with a range of 14–30 %. growth was normal in one and slowed in 5. The findings in
Long bones have differing growth responses after the later group were similar to those in the patients who had
lengthening of their diaphyses. However, when growth is lengthening of a congenital short tibia in the Children’s
assessed according to the specific bone that was lengthened Hospital Boston series. Pouliquen et al. noted almost no
(that is, according to whether the femur or tibia was operated retardation of growth after lengthening of 5.0 cm or less and
on), more uniform patterns of response are seen. A slight progressively greater slowdown of growth when the
increase in the rate of growth was noted in each of 7 patients lengthening, expressed as a percentage of the preoperative
who had lengthening for congenital short femur, and the length of the bone, increased beyond 15 %. The crucial
increased rate was maintained for several years after the determinant appears to be the percentage of lengthening
procedure. There are insufficient data to determine whether rather than the absolute amount of the lengthening. Of their
this stimulation of growth is maintained until growth ceases patients who had lengthening of 10–15 %, only one (11 %)
at skeletal maturity. Suva et al. documented an almost of 9 had a slowing of growth, whereas 9 (45 %) of 20 who
6.9 Management of Lower Extremity Length Discrepancies 735

had lengthening of 15–20 % and 8 (80 %) of 10 who had growth with lengthening of the tibia. The interosseous
lengthening of 20–25 % had a slowdown or cessation of membrane and the Achilles tendon appear to be more
growth. resistant to stretching than the tissues surrounding the femur.
Sharma et al. also showed frequent growth slowdown fol- The negative effects of increased pressure on epiphyseal
lowing tibial lengthenings for total fibular hemimelia with growth have been well outlined. Shortening was noted in
lengthening prior to skeletal maturation [586]. The average this series even after extensive releases of soft tissue,
tibial lengthening in 8 cases was 6.7 cm (range 5.3–10) with an including lengthening of the heel cord. Attempts have been
average percentage lengthening of 26 % (range 19–40 %). The made to document the impression that retardation of the
average postoperative growth rate compared to pre-operative growth plate can be due to increased pressure in the limb
rate showed 2 with no growth at all, and only 1 growing at the generated by the distractive forces during lengthening of the
pre-operative rate. In 5 femurs, one increased the rate of tibia. A pressure gauge was developed by Pennecot et al. to
growth but 4 also showed slowing. Hope et al. noted no change measure the forces generated during distraction, and the
in growth following 10 femoral and 10 tibial lengthenings measurements were then correlated with the rate of subse-
using the Wagner technique for congenital shortening of the quent growth [589]. It was concluded that for the tibia there
lower limb [587]. It is unclear why their data differ from the was good correlation between retardation of growth,
Children’s Hospital, Boston and the Pouliquen group data or lengthening of >15 % of the preoperative length of the bone,
the experimental data of Lee et al. reported below. The abso- and the increased forces that were registered.
lute amounts of lengthening were not given by Hope et al. only In the Children’s Hospital Boston series the growth
growth ratios and it is known for the tibia that the greater the response after lengthening of the tibia was different than that
amount of lengthening the greater the growth slowdown. No after lengthening of the femur [582]. In each of 11 patients
patient in the series who had lengthening of the femur had a who had lengthening of the tibia, growth was retarded in
slowdown of growth, even though the increases in length were comparison with the preoperative rate. The patients who had
often >15 % of the preoperative length. a congenital short tibia and the patients who had Ollier’s
Reports made before 1978 on lengthening of the tibia disease had marked retardation of growth of the tibia, while
raised the matter of postoperative growth, but none included the one patient who had poliomyelitis had only a 3 %
rigorous radiographic documentation of this specific phe- decrease. Greiff and Bergmann demonstrated overgrowth in
nomenon. The absence of detailed data allows for only the tibia after tibial fracture [167]. As the process of repair
general, qualitative impressions concerning growth after after lengthening of the tibia is more extensive than that after
lengthening. Moore noted that of 19 skeletally immature fracture, it is likely that the factors that cause stimulation
patients with poliomyelitis who had lengthening of the tibia after fracture are present after lengthening. In the patients in
using the Abbott method, the correction was maintained in the present series, however, it appears that the factors lim-
15, growth actually increased beyond the normal side in 3, iting growth were more influential than those stimulating it.
and only one showed increased retardation [423]. Growth responses do not appear to be dependent on the
In another report on Abbot-type lengthening, many techniques that are employed to lengthen the bones. Femoral
patients who were operated on before the age of 12 years growth was stimulated with the use of the Wagner apparatus
showed an increased discrepancy between the lengths of the and in another study it was stimulated with the use of a
extremities at skeletal maturity compared with the amount of one-stage lengthening. Growth was maintained in some
lengthening achieved. In these reports, it is unclear how patients who had poliomyelitis after the use of the Abbott,
much of the final discrepancy was due to the condition itself, the Anderson, and the Judet method, while retardation of
to postoperative complications, or to postoperative retarda- tibial growth increased in patients who had a congenital
tion of growth. A review of the results after 31 Anderson short tibia, Ollier’s disease, or another non-paralytic condi-
procedures for lengthening of the tibia revealed a variable tion using the Anderson, the Wagner, and the Judet method.
degree of postoperative recurrence of limb length discrep- In the human, variable growth responses are demon-
ancy in patients who had undergone the procedure between strated after lengthening of the diaphysis in bones that have
the ages of 8 and 19 years, especially in those who had a several years of skeletal growth remaining. In Children’s
congenital short tibia [468]. Gross indicated that in some Hospital Boston series, the 7 lengthening procedures that
patients growth was stimulated after the Anderson length- were performed for congenital short femur all led to an
ening of the tibia whereas in others the opposite occurred increased rate of growth. It is anticipated that lengthening
[588]. The majority of patients referred to in these 4 reports can be performed on femora that have several years of
had poliomyelitis. The Children’s Hospital Boston series growth remaining with the expectation of a continuation of
included only one patient who had poliomyelitis [582]. growth at a slightly increased rate. The tibial lengthening
It has been proposed that extensive resistance by soft procedures that were done for patients who had a congenital
tissues in the leg is responsible for increased inhibition of short tibia or Ollier’s disease all led to retardation of growth
736 6 Lower Extremity Length Discrepancies

that was more extensive than the preoperative retardation. dislocation of associated joints and with axial
Hadlow and Nicol have incorporated this growth informa- malalignment. Wagner clearly pointed out the need to
tion into a formula used to aid in timing for femoral and stabilize the joints and correct any axial malalignment
tibial lengthenings incorporating altered growth expectations prior to initiation of any lengthening procedure and also
as well as projections of the pre-operative growth rate [590]. the need to carefully observe for joint and alignment
Lengthening of the tibia should include slight overcorrection changes during the course of any lengthening proce-
to compensate for an expected retardation of growth if it is dure [478]. Saleh and Goonatillake strongly reiterated
done on a bone that has several years of growth remaining. the need for adherence to these principles [592]. The
Preferably, the tibia should be lengthened at or near skeletal disorders they discussed included femoral, tibial and
maturity to avoid loss of correction secondary to retardation fibular congenital abnormalities in 92 patients with the
of growth. The timing of surgical intervention for discrep- three commonest groups encompassing 77 patients
ancies in the lengths of the lower extremities before skeletal involving proximal femoral focal deficiency, proximal
maturity need to consider both the developmental patterns femoral focal deficiency and fibular hemimelia, and
and the patterns of growth after lengthening. hemihypertrophy. They indicated that joint stabilization
Experimental studies: A detailed experiment assessing was mandatory for good function and was an absolute
longitudinal growth of the rabbit tibia after distraction prerequisite prior to beginning limb lengthening. To
osteogenesis was reported by Lee et al. [591]. They divided prevent or minimize the likelihood of hip subluxation or
99 5-week-old immature rabbits into 5 groups according to dislocation, femoral head-acetabular congruity would
the percentage of lengthening done with group I 10 %; group have to be established and pelvic or shelf osteotomy
II 20 %; group III 30 %; group IV 40 %; and group V a sham along with proximal femoral osteotomy would often be
operation with osteotomy without lengthening. They clearly needed. The greatest challenge is in cases of proximal
demonstrated that tibial lengthening did not cause retarda- femoral focal deficiency where there is often need for
tion of growth when the bone was lengthened by 10–20 % the above-mentioned procedures and on occasion in the
but in those instances where it was lengthened by 30–40 % more severe variants femoral–pelvic fusion or at least
growth retardation was evident. These data correlate well definitive placement of the proximal femoral shaft into
with the clinical studies from Children’s Hospital Boston the acetabulum. Knee instability due to anterior cruciate
and those of Pouliquen reported above. In groups I, II, and ligament and/or posterior cruciate ligament deficiency is
V, there were no statistically significant growth differences common in dysplastic limbs. Although no specific
between the operated and control tibias. There were signif- treatment is needed for this, development of subluxation
icant differences in the growth ratio in groups III and IV with of the knee during the lengthening procedure must be
relative growth ratios of left to right decreased significantly carefully observed for and managed. Areas of concern at
in group III (average 4.2 %); and in group IV (average the ankle involve tendo Achilles tightness leading to an
7.0 %). Histomorphometric studies were also performed on equinus deformity and varus-valgus instability with
the physes in each of the groups. These studies correlated abnormal relationships of the lateral malleolus to the
well with the gross measurements of length. In groups I, II, medial malleolus, or with changes in the rate of distal
and V, there were no statistically significant differences, but tibial versus distal fibular lengthening. In some instan-
in groups III and IV there were significant decreases in the ces, the tendo Achilles lengthening, posterior ankle
total thickness of the operated tibial growth plates, both capsulotomy, and distal tibial-fibular stabilization are
proximally and distally compared with controls. There was performed prior to the lengthening procedure. In other
thinning of both the proliferative and hypertrophic zones. instances, orthotic devices are used along with physical
The overall heights of the growth plate were measured and therapy to maintain flexibility and anatomic integrity at
average decrease in the proximal growth plate was 10.4 % in the ankle region with surgical intervention occurring
group III and 23.9 % in group IV. Distally it was 11.9 % in only with changes that develop. Abnormalities in
group III and 12.4 % in group IV. Similar ratios were found femoral or tibial alignment of more than a few degrees
with diminution of the thickness of both the proliferative and should be corrected pre-lengthening with appropriate
hypertrophic zones studied separately. osteotomies. Soft tissue contractures at hip, knee, and
ankle must also be released. The final area of stabi-
(g) Increased awareness of need for joint stabilization and lization possibly needed prior to lengthening relates to a
axial correction as well as limb length equalization in pseudoarthrosis either of the proximal region of the
complex abnormalities: Many lower extremity length femur such as can be seen in a proximal femoral focal
discrepancies are complex such that shortness of the deficiency or a formal congenital pseudarthrosis of the
limb is often combined with subluxation and tibia. Lengthening of a congenital pseudarthrotic tibia is
6.9 Management of Lower Extremity Length Discrepancies 737

rarely done although on occasion it has been attempted overall there were 34 rabbits in which gross disruption at the
in that part of the tibia well away from the pseu- plate did not occur. The response of the physis to
darthrosis where the bone structure appears normal. transphyseal force therefore was clearly dependent on the
amount of force applied. The combined force in excess of
6.9.3.3 Transphyseal Lengthening. Distraction 2 kg almost always caused fracturing. There was, however, a
Epiphyseolysis or Chondrodiatasis consistent increase in length on the involved side whether or
not fractures in the plate were produced. In the absence of
fracturing, they felt that hyperplasia and hypertrophy of the
(a) Early Experimental Findings: Transphyseal lengthen- plate were representative of an increased physeal stimulation
ing procedures were conceived initially and performed which in turn led to the length increase.
experimentally by Ring [593]. He achieved lengthening Jani also noted fracture in the hypertrophic zone and
between 11 and 32 mm in 20 puppies between 4 and subsequent healing by endochondral ossification in 42
6 months of age using an external distraction apparatus puppies in whom distraction epiphyseolysis had been per-
which elongated both distal radius and ulna. He rec- formed [596, 597]. Further insight into the effect of chon-
ognized that when sufficient traction was applied there drodiatasis on the physis itself was provided by Elmer et al.
was a physical transphyseal separation after which [598]. They performed a study in the rabbit to assess the cell
continued distraction opened up a space allowing activity in the physeal region and noted that the procedure
lengthening to occur with subsequent repair with a did not produce any significant change in the percentage of
cylinder of new bone from the periosteum. The physis cells labeled with tritiated thymidine, the intensity of
continued to function and both it and the metaphysis radioactive sulfate labeling the matrix, or the blood supply of
filled the distraction gap with repair bone centrally. the physis. They felt that lengthening was not the result of
Some animals suffered premature growth plate fusion stimulating cell division or increased synthetic function of
to limit somewhat the length gain achieved but some the plate but rather by mechanical passive stretching of the
continued with growth postlengthening. matrix.
Transphyseal lengthening is referred to as distraction
Continuous transphyseal traction was applied by Fishbane epiphysiolysis where the distraction force is sufficiently great
and Riley across the proximal tibial growth plate in 10 pup- that a physeal fracture-separation occurs or chondrodiatasis
pies [594]. Histologic examination revealed fracture to have where lesser distraction forces leave the physis intact with
occurred in all cases through the metaphyseal zone of primary hyperplasia occurring in the proliferating and hypertrophic
trabeculae just distal to the hypertrophic zone of the carti- zones. In the former repair occurs by an intramembranous
laginous growth plate. The physis itself appeared undamaged. bone mechanism while in the latter the endochondral
Growth was felt to continue but complete follow-up to mechanism continues. The distraction device spans the
skeletal maturity revealed early fusion preceding the normal growth plate with pins anchored in the secondary ossifica-
limb by several weeks in 5 puppies. Rapid bony healing was tion center of the epiphysis and in the metaphysis and the
noted in the distraction gap. The authors felt that the technique distraction forces lengthen the limb by causing a separation
could be applied to the human as an effective and reasonably at the plate. DeBastiani et al. slowed the rate of lengthening
safe way of obtaining increase in limb length. to 0.25 mm every 12 h and advanced the concept that, in so
Sledge and Noble performed transphyseal lengthening doing, a transphyseal fracture did not occur but that a
experiments in the distal femur of the rabbit [595]. They also stretching of the hypertrophic zone only was the lengthening
varied the forces across the physis and compared histologic phenomenon [599]. The term chondrodiatasis was used to
findings in efforts to determine the precise site of lengthen- refer to this occurrence.
ing. A Salter Harris type I transverse fracture occurred in 13
of the 16 animals to which 5 kg or more force had been (b) Clinical Use: The usual sites of limb lengthening in the
applied. The fractures occurred at the lower part of the human have been within the diaphyseal or metaphyseal
hypertrophic zone. At lesser levels of force, however, there regions of bone but work by Zavyalov and Plaksin
were markedly fewer or no fractures and lengthening [600], Ilizarov and Soybelman [601], and Monticelli
appeared to have occurred in relation to increased thickness and Spinelli [602–604] showed that transphyseal
of both the proliferative and hypertrophic zones of the lengthening was both clinically feasible and advanta-
physis. Thirteen of 21 animals to which 2 kg were applied geous in some regards. The advantages involved the
and 18 of 19 animals to whom 1 kg was applied showed fact that healing was much quicker than in diaphyseal
only a partial microscopic fracture or no fracture. In 17 of 56 lengthenings since dense cortical bone did not have to
animals distracted, there was no fracture of any type and be repaired but only metaphyseal bone much as would
738 6 Lower Extremity Length Discrepancies

occur in a physeal fracture. Early experiments showed patients and most of these were in the tibias primarily
that actual physical separation occurred in the hyper- because of the abnormal shape of the distal tibial epiphyses.
trophic zone of the physis leaving the major growth The procedure is generally performed at the distal femur or
part of the physis intact, a phenomenon referred to as distal tibia just before skeletal maturity. It does not require
distraction epiphyseolysis. The transphyseal lengthen- the use of plates or grafts, because metaphyseal bone heals
ing then occurs mechanically and subsequent growth readily in the gap produced.
continues once the physeal defect within the hyper- Franke et al. performed distraction epiphyseolysis using
trophic zone of the metaphysis has been repaired. the Ilizarov apparatus in 22 lower limb segments with an
average lengthening of 8.25 cm (range 4–18 cm) [518].
Although this experimental technique can lead to superb Some of the patients had achondroplasia and it was these
results both clinically and experimentally, it is not currently patients in whom some of the larger lengthenings occurred.
widely used if there are a few years of growth remaining. The healing was considerably quicker in the distraction
There have been reports of premature growth plate cessation epiphyseolysis group compared with the partial metaphyseal
following this procedure. In patients, however, who have corticotomy group. In a group of 9 patients lengthened
virtually no growth remaining, the procedure is attractive. It between 6 and 9.5 cm the average time to full weight bearing
can be performed using either the circular distraction devices was 9.5 months with the repair index 43.6 days/cm.
of Monticelli and Spinelli and Ilizarov as well as unilateral
lengtheners such as the Orthofix device. (c) Growth Consequences Related to Force of Distraction
Eydelshtyn et al. reported extensively on the distraction and Mechanism of Lengthening: Although initial reports
epiphyseolysis in a clinical setting in association with mean- indicated that growth can continue following healing
ingful limb lengthening [605]. They noted epiphyseal sepa- there have been reports of premature growth cessation
ration radiologically within 7–10 days and the ability to obtain and the procedure is best performed within 1 year of
length increments of 4–7 cm. They suggested that in most expected growth plate closure or in situations where
instances there was no negative influence on further growth. metaphyseal or diaphyseal lengthening is not possible.
The cleavage fractures appeared radiologically to have Clinical papers reporting early physeal closure after
occurred in the metaphysis in 26 of 33 patients with the chondrodiatasis are increasingly common. Hamanishi
remaining 7 occurring at the lowest levels of the growth car- et al. reported femoral chondrodiatasis in 5 patients but in
tilage but preserving the growth mechanism more proximally. 4 of the 5 the physis closed shortly after lengthening and
DeBastiani and associates performed chondrodiatasis loss of gained length or further shortening occurred in
using the unilateral Orthofix apparatus in 40 segments of each [608]. They used the Orthofix apparatus and the
patients with limb length discrepancies gaining a mean of lengthening rate was 0.25 mm every 12 h. The 5 femora
3.3 cm in length (range 1.5–7.0 cm) and in 60 segments in were lengthened a mean of 32 mm (25–43) after 70 days
25 achondroplastic patients gaining a mean of 7.1 cm (3– of distraction. Subsequent growth however was markedly
10.5 cm) [599]. In 16 distraction epiphyseolysis procedures diminished in 4 and moderately diminished in the other.
reported by Monticelli and Spinelli, the tibia was lengthened They felt that the 0.5 mm/day distraction caused physeal
by an average of 6 cm (range 3–10 cm) [604]. The patients separation rather than hyperplasia of the growth plate
were all between 13 and 16 years of age with little to no alone. Bjerkreim evaluated 10 consecutive proximal
growth potential persisting. Aldegheri et al. reported on tibial physeal distraction cases with a mean lengthening
chondrodiatasis used for elongation of 170 bone segments in of 6.7 cm [609]. In 6 cases lengthened at 1 mm/day under
75 children, 41 with limb length discrepancies and 34 with 13 years of age, subsequent growth was only 6 mm
achondroplasia [606, 607]. All were operated on with the compared with the normal side of 32 mm.
growth plate open. The Orthofix apparatus was used to
lengthen either the distal femoral or distal tibial epiphyseal Growth retardation has been seen in several experimental
plates. Distraction began the day after operation at a maxi- animals after epiphyseal distraction. Letts and Meadows
mum rate of 0.5 mm/day. There were 92 femoral and 78 performed distraction epiphysiolysis of the proximal tibia in
tibial lengthenings. The average age was 12 years. In those 18 rabbits [610]. The average distraction gained was 0.54 cm
treated for limb length inequalities the mean lengthening which was 6 % the length of the tibia at time of intervention.
obtained was 3.4 cm (10.9 % of average initial length). The Union invariably occurred. In each of 12 younger rabbits
mean lengthening of the femur was 3.0 cm and of the tibia operated once, the distracted area united it was shortly fol-
3.7 cm. In achondroplastic patients, the mean lengthening lowed by premature fusion of the growth plate. In 6 older
obtained was 7.4 cm with virtually equal amounts in femur rabbits operated close to the time of skeletal maturity no
and tibia. Most of the complications were in achondroplastic negative growth sequelae occurred. Subsequent histology
6.9 Management of Lower Extremity Length Discrepancies 739

showed no normal epiphyseal growth plates after repair of forces of 16–18 newtons produced and continued to the end of
the gap. In the study by Fjeld and Stein, subsequent growth the distraction period were associated only with physeal
retardation was consistently experienced in all animals with hyperplasia without fracture. These results were consistent
an average reduction in growth ranging from 40 to 70 % with those of Sledge and Noble [595], and DeBasianti et al.
[611]. The experimental groups involved 10 distal femoral [599] that a slowed rate of distraction could allow for
epiphyseal distractions in the goat, 14 in the proximal tibia lengthening without fracture. It was in the two lower dis-
of the goat and 18 in the distal femur of the dog. They felt traction rates that histologic evidence of fracture was not seen.
that the reduction in gained length with time after the end of
distraction must have been due to a retardation of endo- (d) Histologic findings in physeal distraction: The appli-
chondral growth after the elongation procedure. Jani also cation of physeal distraction leads to 2 modes of
reported negative growth sequelae after distraction epiphy- lengthening. If distraction pressures are comparatively
seolysis experiments in the rabbit [596, 597]. Alberty per- low there is apparent continuity of the physis with
formed a vascular study of the growth region following hyperplasia of cells in the lower proliferating and
physeal distraction in the rabbit [612]. The microangiogra- hypertrophic zones. In most instances, however, there
phy assessments were done in relation to 45 distal femoral is a transphyseal fracture with the break occurring in
transphyseal lengthenings. Most of these were performed at linear fashion within the hypertrophic zone.
a rate of 1.0 or 1.5 mm once daily increases. Both marked
enlargement of the epiphyseal arteries and defective meta- Peltonen: Repair phenomena were studied by Peltonen
physeal capillary filling were noted after 3 days of distrac- et al. in the distal radius of 40 growing sheep [616, 617]. The
tion, changes which persisted in specimens distracted as long distraction was at a rate of 0.5–1 mm a day. The first signs of
as 21 days. New capillaries were observed in the hyper- ossification in the distraction gap were seen radiographically
plastic physes and in separation gaps at 21 days. Of note, about 3–4 weeks after distraction began. Collagen fiber
however, was the fact that vascular anastomoses were noted bundles were oriented parallel to the long axis of the bone in
across the physes at 6 weeks of follow-up. The latter phe- the gap region and served as the focal point for new bone
nomenon was noted in those distracted from 9 to 21 days formation. Abundant osteoid was demonstrated in the cal-
who then were assessed after an interval of 6 weeks between cified sections. Thin trabeculae from both the epiphyseal and
discontinuation of the distraction and the microangiography. metaphyseal sides grew toward the center of the distraction
A premature closure or impaired function of the physis was gap. At the periphery there was active bone formation from
common in association with the transphyseal vascularity. the inner layer of the periosteum. Clinical and radiologic
In spite of the comments of DeBastiani concerning the consolidation of the distraction area occurred within
absence of transphyseal fracture with slower rates of dis- 10 weeks of the surgery. The consolidated bone area was
traction, DePablos et al. found that in each of three models composed of partly woven bone and lamellar trabecular
used production of a fracture between the metaphysis and bone with most of the lamelli organized in the direction of
the epiphysis always occurred but that the lower the dis- distraction. In those instances where the physis remained
traction rate employed the greater was the viability of the intact, there was an apparent stretching of the endochondral
growth cartilage [613, 614]. The optimal rate for distraction growth mechanism owing to the distractive force. In many
was 0.5 mm/day. The Orthofix distractor was used in 45 instances, however, there was actual transphyseal rupture
lambs divided into 3 groups each with the rate of distraction through the hypertrophic zone. At 12 weeks postoperatively,
being 2, 1, and 0.5 mm/day. Histologic studies showed that the repair bone resembled normal metaphyseal bone. Once
at the slowest rate the growth cartilage remained essentially the hematoma of the stretch injury had been removed, the
normal whereas in femurs lengthened at a rate of 1 or gap was filled with a collagenous matrix and collagen bun-
2 mm/day obvious lesions of physeal cartilage were dles could be seen organized in the direction of the dis-
observed in particular in those studied 45 days following the traction. Shortly thereafter, trabecular bone had been formed
conclusion of lengthening and at 6 months of age. from the inner layer of the periosteum and from epiphyseal
Spriggins et al. assessed the response of the growth plate to and metaphyseal sides.
increasing force of distraction [615]. They studied the upper Peltonen et al. also studied gradual physeal distraction of
tibia in 24 rabbits close to skeletal maturity with the distrac- the distal radius in 2–5 month old sheep [618, 619]. The bone
tion rate of 0.13, 0.26, and 0.53 mm every 24 h. They noted 2 formation occurred from the inner layer of the periosteum,
distinct patterns of response. In the group where forces from the metaphysis and from the epiphyseal side toward the
increased to maximum values of 20–22 newtons and then center of the distraction area. Prior to bone formation colla-
suddenly decreased on subsequent distraction, fracture of the gen bundles were organized in the direction of distraction.
growth plate had occurred whereas in the other group lower Bone formation occurred from the epiphyseal and
740 6 Lower Extremity Length Discrepancies

metaphyseal sides. Under polarized light microscopy, interzone was 10–20 mm wide, faint irregular shadows
lamellar trabecular bone was seen forming along the collagen became visible suggesting early osteogenesis. New bone
bundles. Woven bone was frequently seen. In the distraction formation was seen next to the epiphysis and the metaphysis
area at 11 weeks in the study by Peltonen, both lamellar and with the central region showing initially little bone forma-
woven bone were present within individual trabeculae but it tion. After 1 month of distraction, the interzone was 25–
was clear that the lamellar bone predominated. 30 mm wide and the new bone formation sites began to link
Alberty and Peltonen showed that all physes in 12 longitudinally. Longitudinal bundles were continuous now
growing rabbits undergoing distal femoral distraction between the epiphyseal and the metaphyseal ends. The bone
showed widening of the proliferative and hypertrophic zones formation from the metaphysis was generally more advanced
as well as a fracture separation at the hypertrophic zone or at than that from the epiphysis. After 2 months of distraction,
the junction of the hypertrophic zone and the metaphysis in the interzone had increased to 42–60 mm and the radio-
11 of 12 [620]. Proliferation of the hypertrophic cells with graphic shadows were more clearly striated in a longitudinal
5-bromo-2-deoxyuridine (Brd Urd) labeling was noted even direction. Ossification proceeded upward and downward
though normally not seen. The labeling of the physeal toward the center of the interzone where bone repair was
regions above, however, was normal in both control and ultimately slowest. Only when distraction ceased did the
distracted physes being positive in germinal and proliferat- central region begin to show uniform radiodensity. After
ing cell zones with no labeling in the hypertrophic zone. lengthening of 30–40 mm it took about 2 months for the
DePablos et al.: In the histologic study of dePablos et al. bone cortex to reform. In a few animals, premature epi-
local fracture was noted in each instance [613, 614]. The physeal fusion occurred which led to the recommendation
physis tended to be poorly organized in some instances and that transphyseal lengthening be performed only when the
normal in some. The lengthened zone was first wholly subject’s bone growth was nearly complete.
occupied by a hematoma which quickly underwent fibrous The repair bone from the sheep was subjected to a more
organization. This was replaced by a rich granulation tissue detailed morphologic study at 1–4 weeks and 2, 4, 6, and
composed of numerous fibroblasts and collagen fibers which 12 months [603]. One week: An epiphyseal fracture (epi-
aligned themselves parallel to the long axis and the traction physeolysis) occurred in all specimens. The fracture gap was
axis of the bone. Ossification of the lengthened zone was first filled with hematoma. There was no calcification or bone at
observed on the twentieth day of lengthening. This was this stage. The fracture line occurred within the growth plate
referred to as desmal ossification although there was some around the level where matrix calcification was beginning to
endochondral ossification taking place in the growth cartilage occur in the hypertrophic zone. Two weeks: The hematoma
at its lowest regions. The ossification progressively replaced occupied a wider space but persisted. Most of the chon-
the fibrous granulation tissue. After 4 months, all of the tissue drocytes in the physis were unchanged although cell col-
in the lengthened zone showed complete ossification. umns of the upper cartilage segment were sometimes
Monticelli and Spinelli: Monticelli and Spinelli showed distorted. The cartilage on the metaphyseal side was pene-
that the gap invariably healed with bone provided that the trated by many capillary vessels and looked more irregular
surrounding perichondrium and periosteum was not excised than in the normal control. Three weeks: The distance
[602, 603]. The resumption of endochondral ossification was between the upper and lower cartilage cell segments had
infrequently seen and incomplete when present. The new widened owing to the continuing distraction. Hematoma was
bone consistently formed along the fibrils as well as along becoming organized with fibrous material. No bone was yet
the under surface of the peripheral periosteum. The bone was formed. Four weeks: The gap was now filled with a compact,
noted to be well oriented in most instances. Polarization translucent, grayish tissue and the lower cartilage segment
microscopy showed the fibrils to be parallel to the long axis was less evident than previously. There was now a thin shell
of the bone and to the axis of the distraction. They carried of bone under the periosteum surrounding the distraction
out proximal tibial lengthening experiments on 41 sheep space. This was also seen radiographically. The hematoma
between 3 and 5 months of age. The duration of the dis- had almost been completely resorbed and replaced with
traction varied from 1 to 16 weeks. The lengthening rate connective tissue consisting mainly of elongated cells sur-
varied between 0.5 and 1.5 mm/day with most either 0.5 or rounded by thin fibrils. Many of these were irregularly ori-
1.0. Separation of the epiphysis from the metaphysis was felt ented but the beginning tendency to longitudinal orientation
to occur after 3–5 days and the force needed to induce was seen with fibrils aligned in the same direction of the
fracture was approximately 18 ± 4 kg. Separation was distraction force. A thin layer of circumferential periosteal
always noted within the hypertrophic zone. During the first bone composed of thin trabeculae of primary bone was seen.
and second weeks, the region between the epiphysis and the Two months. A compact gray tissue zone was evident
metaphysis referred to as the interzone was transparent on grossly below the epiphyseal cartilage. Subperiosteal bone
radiographs. At the beginning of the third week when the formation was seen along with metaphyseal ossification. No
6.9 Management of Lower Extremity Length Discrepancies 741

traces of hematoma were recognizable, having been replaced differing type of distraction apparatuses used and the dif-
completely by connective tissue consisting of elongated ferent degree of stabilization provided.
fibroblasts and thick bundles of collagen fibrils both of
which were preferentially oriented parallel to the tensile (e) Treatment of premature physeal closure by means of
force of the distraction. This was particularly evident in the physeal distraction: Transphyseal distraction has been
middle portion of the elongated segment. Some of the col- used in both experimental and clinical situations to dis-
lagenous material was calcified and there were small spi- rupt focal transphyseal bridges. De Pablos et al. used the
cules of osteoblasts seen. Four and six months: A thin procedure in 30 lambs 1.5 months old after partial epi-
surrounding shell of cortical bone at the distraction area was physeal arrest in the distal femur had been induced [613,
seen. There was continuing ossification in the subperiosteal 614, 621]. Physeal distraction was then done followed by
area. New bone was also seen near both the epiphyseal and no subsequent intervention in some animals and inter-
metaphyseal zones. Long thin bundles of collagen fibrils position of fat in others. They demonstrated the ability to
were present in the elongated area. Most of these bundles disrupt the focal physeal bridge by the distraction tech-
were now calcified forming thin parallel oriented trabeculae nique but recommended that fat be interposed since the
whose longitudinal orientation was the same as that of the bridge reformed in all instances where distraction alone
distraction force. Together with these calcified collagen had been used. They recommended physeal distraction to
bundles true bone trabeculae were found, part of which pull apart the bone bridge and if clinically meaningful
developed from the epiphyseal cartilage and part from the amounts of growth were still left there was value in per-
dislocated metaphyseal trabeculae and the periosteum. The forming fat interposition to prevent bridge reformation.
tips of these trabeculae were surrounded by osteoblasts. The The need to resect the bridge however was bypassed.
persisting physeal cartilage in some instances retained its
normal structure but in others it was irregular. No bone Connolly et al. applied transphyseal traction to correct
trabeculae were present near the epiphyseal cartilage indi- acquired growth deformities in the immature dog [622].
cating disruption of the normal endochondral sequence. Bone bridges were created across the medial distal femoral
Twelve months: Ossification had progressed in the elongated physis to produce a varus deformity. They were able to bring
segment and the fibrous tissue had almost completely been about correction by transphyseal lengthening both after
replaced by bone trabeculae. The subperiosteal bone had a removal of the bone bridge directly and also by not inter-
compact appearance. The elongated tibias were almost vening on the bone bridge but stretching and breaking it with
indistinguishable from the contralateral side. the traction procedure. Many of the animals, suffered pre-
The process of distraction epiphyseolysis was divided mature growth plate closure. They showed, however, that
into three stages: 1. Epiphyseolysis with formation of the mechanical epiphyseolysis after a bone bridge had formed
hematoma; 2. Resorption of the hematoma and the formation offered the possibility of treating large areas of epiphyseal
of fibrous tissue which increases in length as long as the arrest in order to restore length and correct angulation.
distraction force is applied; and 3. Ossification of the fibrous Correction of alignment was also performed in two patients
tissue and reconstitution of the periosteal bone. As the with shortening and angular deformity due to enchondromas
fibrous tissue develops in the second phase the cartilage on but without bone bridges. Correction and lengthening were
the metaphyseal side is resorbed and replaced by obtained but premature fusion limited effectiveness.
fibro-osseous and osseous tissue which is well vascularized. Kershaw and Kenwright were able to pull apart bone
In the third stage, there is first calcification and then true bridges by transphyseal distraction but rabbits killed 3–
ossification of fibrous tissue with the formation of longitu- 6 weeks after the distraction showed complete physeal clo-
dinal trabeculae lying almost parallel to each other. Ossifi- sure suggesting that distraction epiphysiolysis along with
cation takes place directly from the periosteum and from the bone bridge disruption would have a high potential for
cellular elements of the fibrous tissue undergoing producing premature physeal fusion [623].
osteoblastic differentiation in connection with the displaced Bollini et al. used the Ilizarov device to treat a centrally
metaphyseal segment. Ossification also takes place from the located bone bridge in the lower tibia of a 10 year old girl
epiphyseal cartilage but is less regular and less impressive caused by an epiphyseal fracture-separation [624]. The epi-
than from the other two sites. On occasion, the endochondral physeolysis occurred on the fourth postoperative day fol-
ossification sequence of the physis persisted but in others it lowing lengthening or distraction of 0.25 mm/day. The bone
was damaged. Cessation of endochondral ossification was bridge which remained attached to the metaphysis was sur-
not of clinical significance if the distraction epiphyseolysis gically removed following distraction and was prevented
was carried out at an age approaching skeletal maturity. from recurring following interposition of methyl methacry-
Varying reports in the literature could easily be due to the late. Follow-up at 2 years showed no recurrence and normal
742 6 Lower Extremity Length Discrepancies

growth. Canadell and De Pablos presented four clinical 6.10 Direct Operation on Epiphyses
examples of breaking bone bridges by physeal distraction thus to Enhance Growth Potential
eliminating the need for the complex open resection of such by Removing Focal
transphyseal tissue [625]. They used an angulated monolat- Transphyseal Tethers
eral fixator which with distraction served both to pull apart the
bone bridge and then allow for angular correction with time. 6.10.1 Bone Bridge Resection
The procedures were performed 3 times in the distal femur
and once in the distal tibia. The physeal bridge broke in each Partial destruction of the function of the growth plate is
instance a few days after distraction began. In those patients associated with the formation of localized transphyseal bone
close to growth maturation there was no effort made to bridges [627, 628]. These develop most commonly after
interpose tissue. If patients were treated at a younger age then certain growth plate fracture-separations [624, 629–636], in
the possibility of a secondary procedure to insert an interpo- severe cases of Blount’s disease (infantile tibia vara), and
sitional tissue would be strongly considered. They made one after infection. The bone bridges retard growth in a localized
important technical point. It was essential to fracture the bone part of the physis and predispose to angular deformity as
bridge first with lengthening forces across and parallel to the well as shortening since the remaining physeal tissue con-
angulated physis. The distraction would have to be symmet- tinues to function. The possibility of removing focal bone
rical and only after the bridge had broken would angular bridges was raised over 100 years ago by Ollier [159]. He
correction be performed. If angular corrective forces were was able to demonstrate the formation of transphyseal bone
applied from the beginning, they would subject the healthy bridges in experimental animals. Experimental work in
part of the physis to compression pressures which could lead rabbits by others at this time also demonstrated partial bone
to its permanent damage. Physeal distraction was also applied bridging of the physis [634]. There was good clinical
by Aldegheri et al. to pull apart bone bridges and then allow awareness in the latter stages of the nineteenth century of
for angular correction utilizing the principle of epiphyseal bone bridge formation following epiphyseal trauma with its
distraction referred to as hemichondrodiatasis [607]. The subsequent affect on growth. Ollier himself made efforts in
Orthofix articulated dynamic axial fixator was used to provide removing bone bridges surgically but recurrence was com-
for the asymmetric pressures. Two cancellous screws were mon owing to the failure to use an appropriate interpositional
placed in the epiphysis and two cortical screws in the diaph- tissue to prevent recurrent bridge formation.
ysis. Earlier work by De Bastiani was quoted to show that it The position of the bone bridge defines not only the type
was possible to elongate just the lateral portion of the distal of deformity, but also the surgical approach to its removal
growth plate of the femur with histologic assessment showing and the type of material interposed to prevent reformation
an increase in thickness only in the lateral part of the physis (Fig. 6.26a). Central bone bridges lead to shortening without
owing to cellular hyperplasia and hypertrophy. The deviation angular deformation whereas peripheral bone bridges lead to
was achieved without fracture of the growth plate. Aldegheri angular deformity as well as shortening. Bright classified
et al. felt that the best results were achieved in post traumatic partial growth arrest lesions into three types: I: peripheral
deformities when the bone bridge occupied <20–30 % of the lesion; II: central lesion; and III: combined central-peripheral
epiphyseal plate. They recommended performance of the lesion [637]. The exact position and extent of bone bridges
procedure with little growth remaining since the possibility of can be shown by CT scanning and magnetic resonance
post procedure growth arrest still existed. imaging (Fig. 6.26b). Examples of bone bridges are shown
in Fig. 6.26a.
6.9.3.4 Transiliac Lengthening Langenskiold refocused attention on focal bone bridge
Millis and Hall showed that in patients with a discrepancy in formation and developed bridge resection and the implan-
lower extremity length associated with acetabular dysplasia, tation of fat for use as a clinical tool [628, 638, 639]. In
primary intrapelvic asymmetry, or a decompensated scolio- laboratory experiments many types of interpositional mate-
sis, 2.5 cm of length can be gained through transiliac rials were used but fat was both the easiest and the most
lengthening by means of a modified innominate osteotomy effective in preventing reformation of bone bridges and
(Fig. 6.25b) [626]. This approach both increases lower maintaining physeal function. Fat is a minimally vascular-
extremity length and corrects structural pelvic abnormalities ized tissue and generally persists as fat when interposed in
associated with the discrepancy. Three possible complica- growth plate defects thus keeping the epiphyseal and meta-
tions involve delayed union, sciatic nerve palsy, and physeal circulations separate and allowing the remainder of
sacroiliac joint disruption. The procedure is most strongly the physis to continue growth. Another interpositional
indicated for primary correction of pelvic deformity in which material used was cartilage which in experimental studies
slight limb lengthening is also desired. had just as effective a result as the fat [640]. In commentary
6.10 Direct Operation on Epiphyses to Enhance Growth Potential … 743

Fig. 6.26 a Mature transphyseal bone bridges can be seen on plain MRI is from coronal (lateral) plane. c A series of radiographs shows
radiographs. A peripheral proximal medial tibial bone bridge following operative approach for removal of a central bone bridge (i) and (ii),
Blount’s disease is seen. Varus formation has developed. b Magnetic filling of the defect by fat followed by reinsertion of bone window [(iii)
resonance imaging defines the extent of any bone bridge. At distal tibia and (iv)] and resulting following growth resumption several months
central bone bridge is black (no signal) and persisting physis white. later (v)

on his first 43 clinical procedures excising local bone bridges growth plates in pigs and filling them with autologous fat.
and interposing autologous fat grafts, the results in general Studies indicated that the volume of fat tissue implanted in
were good to very good; only 7 showed questionable benefit the cavities continuously increased parallel with the growth
“mainly because the procedure was carried out too close to in length of the bone. It appeared that the fat was augmented
the end of the growth period” [628]. The large majority of by fat cells in the metaphysis. Langenskiold et al. recalled
procedures involved the distal femur, proximal tibia and three patients several years after surgery for CT scan
distal tibia. The etiology of partial closure in 38 growth assessment of the epiphyseal/metaphyseal region [641].
plates was fracture in 28, osteomyelitis in 8 and tuberculosis They concluded that the former resection cavities were filled
and Blount’s disease in 1 each. Following interposition of primarily by fatty tissue and that the portion of implanted fat
the fat graft, the radiolucent area of the fat transplant usually had grown in size corresponding with the growth in length
has a rounded or oval shape whereas following subsequent of the bones in the affected ends. Some strands of fibrous
growth the radiolucent area becomes elongated. tissue were intermingled with the fat. A layer of dense bone
The fate of the fat implants was studied experimentally by remained interposed at the periphery of the fat graft. Lan-
making round cavities in the proximal end of the tibial genskiold concluded that the free fat grafts implanted at time
744 6 Lower Extremity Length Discrepancies

of resection continued to grow and thus had filled out the MRI was very helpful not only in pre-operative planning but
elongated cavities. The fat persisted well beyond the period also in following the result postsurgery.
of growth termination and the cavities were not filled with Resection has proven to be clinically feasible in many
fluid or bone. Examples of central bone bridge resection are instances if one quarter or less of the growth plate is
shown in Fig. 6.26ci–v. An excellent overview of physeal involved and there is sufficient growth remaining to warrant
bridge resection has been written by Khoshal and Kiefer removing the focal tether. Interposition of fat, cartilage,
[642]. silastic, or methyl methacrylate can keep the epiphyseal and
Many clinical studies have assessed the treatment of metaphyseal circulations separate, thus preventing the for-
partial physeal growth arrest by bridge resection and fat mation of further bone bridges and allowing the unaffected
interposition. Vickers reported on 15 patients with good growth-plate cartilage to continue to grow normally. Each of
early results [643]. Williamson and Staheli assessed 29 these methods has advocates; the interposition of fat is the
physeal resections, 22 of which were followed for more than easiest and most commonly used approach clinically.
2 years [644]. They interpreted their results in the longer Examples of a bone bridge resection procedure are shown in
term group as 11 excellent, 5 good, 2 fair and 4 poor. Fig. 6.26c.
Twenty of the 29 bridges were caused by fracture, 3 by The extent of the bone bridge must be determined prior to
tumors, 3 by tibial traction pins and 1 by infection. Twenty making a decision to resect the bridge. Plain biplanar radio-
of the bridges were peripheral, 6 were central and 3 were graphs are inadequate. Tomography was shown to provide a
combined. The results correlated inversely with bridge size. good percentage estimate of physeal area replaced by the bone
They were uniformly excellent for bridges <25 % of the bridge but CT or MR imaging with three-dimensional
physeal volume; bridges between 25 and 50 % yielded good reconstruction are used currently [642, 646–649].
to excellent results in 9 of 12 cases; and results were gen-
erally poor in bridges >50 % with only 1 of 4 yielding a 6.10.1.1 Varieties of Interpositional Materials
good result. Bright reported briefly on 100 patients followed Several interpositional substances have been used following
>2 years with silastic interposition material with 81 % of the resection of a transphyseal bone bridge to keep the epiphy-
patients demonstrating some growth after bridge resection seal and metaphyseal circulations and bone apart so as to
and 70 % good to excellent results [637]. Aufaure et al. allow physeal growth to continue. Cady et al. performed fat
studied 18 cases of bone bridge resection in childhood and implants into 14 New Zealand white rabbit physes in an
concluded that there were 9 good results and 9 failures. The effort to determine whether or not physeal regeneration
best results were obtained in cases where the bridge was occurred [650]. They found no instance in which the physis
peripheral, since it was more easily approached, and fol- regenerated transversely across the gap in either the fat
lowing a traumatic injury in young children. The larger the implanted or the control femurs. The fat remained viable and
bone bridge the greater the likelihood of failure. Extensive was gradually replaced with fibrous tissue. Three inert
bridges in particular those located centrally therefore had a materials have also been used as interpositional substances
poor prognosis and all bone bridges due to osteomyelitis with good effects described. These have included silastic
were failures [645]. Most of the bone bridges were resected [637, 651], methyl methacrylate [627], and bone wax [642].
at the distal femoral and distal tibial growth plates. Among biologic substances cartilage itself was shown to be
Hasler and Foster specifically addressed the reasons for excellent in preventing transphyseal revascularization. Len-
poor and fair results after physeal bar resection [646]. They nox et al. performed a comparative experiment in the 5–
had removed bars <40 % of the physis and a remaining 6 week old white New Zealand rabbit in which two adjacent
growth period of more than 2 years. They reviewed 24 4 mm diameter defects were drilled in the distal lateral
physeal bar resections followed by fat interpositioning. Of femoral epiphysis [652]. In the control group, valgus angu-
the patients available for follow-up there were 9 good (40 %) lation had a mean of 43° with 2.4 cm shortening compared to
4 fair (17 %) and 10 poor (43 %) results. They did not find a the opposite side. The group with fat interposition showed a
correlation between bar size and outcome. MRI and CT diminution of the valgus angle although it was still 28° with
scans were very helpful in assessing reasons for imperfect shortening 1.9 cm. Better results were achieved with inter-
outcomes. The commonest cause of recurrence was what position of femoral head cartilage using punch biopsy plugs
they referred to as a secondary tether due either to incom- of fresh bovine cartilage in one group and frozen bovine
plete bar resection or bar recurrence. Associated or other cartilage in another. In both the fresh and frozen groups
reasons were dislocations of the fat graft or premature distal femoral growth continued with difference being only
physeal closure. They concluded that patients with congen- 0.6 cm from the opposite side. The fresh and frozen cartilage
ital physeal abnormalities or postosteomyelitis abnormalities also led to the least extent of valgus angulation showing 11°.
had a less favorable outcome with growth plate trauma Eulert also working on the distal femur of the developing
patients much more likely to have successful resections. rabbit, eliminated or greatly minimized partial premature
6.10 Direct Operation on Epiphyses to Enhance Growth Potential … 745

distal physeal closure by transplantation of iliac crest growth since the transplanted tissue is nourished by the intact blood
cartilage [653]. The best results were obtained when the supply of the host bone.
growth cartilage was transplanted alone or with a thin layer Focal lesions of the physis continue to cause considerable
of bone. The results were poor when the layer of bone morbidity in terms of angular deformity and shortening.
transplanted with the cartilage was too thick. They con- Osteotomy can correct deformity, although it must be
cluded that iliac crest cartilage could be used as a graft repeated frequently if the growth problem has occurred
following bone bridge resection. When a region had been several years prior to maturity. A variety of procedures has
resected and no interpositional tissue placed the mean valgus evolved to treat the length discrepancies. In an effort to
at 24 weeks post surgery was 60°. When the cartilage graft restore normal growth, well-localized focal bone bridges
was inserted with a thick lamella of bone attached angular have been resected and a biological or prosthetic material
deformity was less but still approximately 45°. The best has been inserted to prevent reformation. Free physeal and
results were achieved when the cartilage graft had only an epiphyseal transplantation is attractive, but previous exper-
extremely thin rim of bone attached which led to angular imental and clinical attempts have not been successful
deformity of only 10°. Bright showed the value of silicone– consistently. Vascularized epiphyseal transplantation has
rubber implants in dogs where they were effective in pre- been used with some promising results in experimental
venting bone bridge reformation in a distal femoral epi- animals.
physeal growth plate model [651]. Wirth et al. tried The cartilaginous physis has a dual epiphyseal and
implanting periosteum to see if it would modulate into a metaphyseal blood supply. From the epiphyseal vessels,
cartilage phase; this did not occur and direct bone formation nutrients are carried by diffusion through the cartilaginous
resulted [654]. Studies have also been performed attempting extracellular matrix to chondrocytes; the metaphyseal vas-
to define use of fat grafts in relatively large central defects. culature serves as a source of osteoprogenitor cells that lay
Osterman removed approximately 65 % of the central part of down bone on the calcified cartilage matrix to complete the
the plate of the distal femur in a 3-week-old rabbit and endochondral sequence. A basic requirement for the survival
interposed autologous fat [640]. This work attempted to and normal function of free physeal transplants is that this
show that even large defects involving more than half of the dual blood supply be preserved at the graft site, and that the
epiphyseal plate can be successfully treated. free physeal graft not be covered by tissues that impede the
diffusion of nutrients from blood vessels into the extracel-
lular matrix of the physis. Studies have been done on the
6.11 Treatment of Premature Partial transplantation of free autogenous iliac-crest physeal grafts
Physeal Closure by Means into defects in the lateral aspect of the distal femoral physis
of Growth Plate in rabbits [655]. The graft site in the lateral aspect of the
Transplantation distal end of the femur was carefully fashioned to expose the
epiphyseal and metaphyseal bone and its vessels. Fibrocar-
6.11.1 Free Autogenous Iliac Crest Physeal tilaginous and perichondrial tissue was removed from the
Grafts—Focal Defects surfaces of the free physeal graft to facilitate the diffusion of
nutrients into the physeal cartilage. The physeal grafts were
Efforts have been made experimentally to reestablish growth readily incorporated into the graft site, the morphology of
by the transplantation of a free partial growth plate after the physis was retained and the physeal transplant prevented
resection [655] (Fig. 6.27a–pi, ii). This procedure is bone bridge formation, growth arrest, and valgus deformity.
designed to keep the epiphyseal and metaphyseal circula- Focal physeal bone bridges develop most commonly
tions apart; the partial growth plate also contributes actively following certain fracture-separations or as medial bridging
to growth, rather than serving as a passive spacer such as do of the proximal tibial physis in association with severe
fat, Silastic, or methyl methacrylate. This approach takes Blount’s disease. In such situations, the epiphyseal and
advantage of the fact that the physeal cartilage itself has no metaphyseal blood supply to the bone adjacent to the physis
direct blood supply but, rather, a dual supply from epiphy- is generally normal. The results of the study indicate that
seal vessels on one side and metaphyseal vessels on the free physeal transplants might be used to prevent or decrease
other. If the defect in the growth plate is at the periphery, its the bone bridge formation, growth arrest, or angular defor-
removal leaves the epiphyseal cartilage and bone, the mity that can occur in these conditions.
metaphyseal bone, and therefore the dual blood supply A procedure was first developed for creating a standard
intact. In rabbits, oriented cartilage from the iliac crest, focal defect in the lateral aspect of the distal femoral physis
transferred after the outer fibrocartilaginous portion has been of 3–4 month old rabbits, which consistently led to the
carefully removed, has been shown to survive and function, formation of a bone bridge between the distal femoral
746 6 Lower Extremity Length Discrepancies

Fig. 6.27 (continued)

6.11 Treatment of Premature Partial Physeal Closure … 747

Fig. 6.27 (continued)

 748 6 Lower Extremity Length Discrepancies

epiphysis and metaphysis. The defect was created by bone bridge formation, growth arrest, and marked valgus
removing the outer one-half of the lateral half of the physis. deformity. In the one rabbit, operated on early, in which
The gross appearance of the defect in a mid-coronal section normal growth occurred, a dissecting microscope had not
of the distal end of the femur is shown in Fig. 6.27a. In been used, and it was assumed that the physis had not been
creating the defect, care was taken to remove all of the removed completely.
cartilage from the surfaces of the epiphyseal and metaphy- A procedure was then developed to remove and trans-
seal bone to leave the bone intact, and thus to preserve the plant a free autogenous iliac-crest physeal graft into the focal
host’s dual epiphyseal-metaphyseal blood supply to the defect created in the lateral part of the femoral physis. The
physis. graft was taken from the posterior part of the iliac-crest
In the early stage following creation of the defect, apophysis. The overlying muscle was freed from the crest
undifferentiated mesenchymal cells migrated into and filled and two parallel incisions approximately 2 cm apart were
the defect, but did not differentiate into cartilage. Woven made through the fibrocartilage and physis to the underlying
bone formed, which then transformed to the lamellar con- bone. These incisions were carried through the periosteum
formation. By 4 weeks after surgery, a bone bridge had onto the inner and outer iliac-bone surfaces and then were
formed between the epiphysis and the metaphysis joined by transverse incisions. By elevating the periosteal
(Fig. 6.27b). There was no tendency for the remaining sleeve, the iliac-crest apophysis was gently separated from
physeal cartilage to grow laterally into the defect. At the the metaphysis at the junction of the hypertrophic chon-
periphery of the defect, a fibrocartilaginous mass was drocytes and the metaphysis and was pulled free of the
sometimes identified, but this never developed into a growth underlying bone. On occasion, a few spicules of metaphy-
plate. Seven of the 8 defects that were created resulted in seal bone came free with the cartilage fragment, but specific

b Fig. 6.27 A series of illustrations shows the use of focal iliac crest the nonoperated distal femoral physis, demonstrating the normal
physeal transplants in a rabbit model. (Reprinted with permission from architecture. i Photomicrograph from the same specimen as in this
Olin et al. Free physeal transplantation in the rabbit. J Bone Joint Surg figure h, showing physeal and metaphyseal tissue from the lateral
Am 1984;66A:7–20). a Photograph demonstrating the defect in the physeal transplant. Cartilage and physeal tissue are at the top and
lateral aspect of the distal femoral physis. b Photomicrograph showing metaphyseal tissue, at the bottom. Note the excellent orientation of the
bone bridge formation following creation of a physeal defect. The proliferating and hypertrophic cell layers of the transplanted physis and
lateral femoral physis had been removed, but no graft inserted, 4 weeks the smooth junction with the metaphyseal tissues. j Low-power
before the animal was killed. The persisting physis (P) is on the left. photomicrograph made 3 months after physeal transplantation. The
There is no evidence of an attempt by the physeal cartilage to grow transplanted physis (TP) is at the left and the persisting physis (PP) is at
laterally to re-form cartilage tissue at site of the defect. Bone fills the the right. There is firm coaptation between the two physes. Persisting
defect site (right) and an extensive bone bridge unites epiphyseal bone epiphyseal bone is at the top and metaphyseal bone, at the bottom.
(EB) with metaphyseal bone (MB).c Photomicrograph illustrating the k Higher power photomicrograph from the same specimen illustrated in
iliac apophysis and the metaphyseal bone. Fibrocartilaginous layer this figure j demonstrating the junction of the epiphyseal bone above
(FC); cartilage of the apophysis (C), the cytologically specialized and the transplanted cartilage below. Vascular invasion of the
region of the cartilage referred to as the physis (P); and metaphyseal transplanted cartilage has not occurred. l Photomicrograph illustrating
bone (M). The arrow represents the line of demarcation between the the physeal graft (right) and the persisting physis (left). Cartilage
green-staining fibrocartilaginous tissue and the red-staining cartilagi- continuity between the two has been established. At the upper right,
nous tissue, as demonstrated on a safranin O-fast green preparation. note the bone that has invaded the cartilage part of the transplant as
d Photomicrograph of the iliac apophysis after its separation from the distinct from the physeal part. The metaphyseal bone adjacent to the
metaphysis. Separation has occurred through the zone of hypertrophic persisting physis merges imperceptibly with the metaphyseal bone
cells, with a few spicules of metaphyseal bone persisting on the adjacent to the graft. m Higher power photomicrograph showing, from
transplant specimen. e Photomicrograph showing an iliac physeal graft top to bottom, epiphyseal bone, bone in the cartilaginous part of the
in position in the defect immediately after transplantation. The cartilage graft, cartilage and physeal cartilage from the graft, and metaphyseal
graft is below and the femoral metaphysis is above. A narrow space is bone produced by the graft. n Higher power photomicrograph showing
present between the graft and the femoral metaphysis, which ensures a the junction between the persisting physeal cartilage (PP) and the
firm fit. f Photograph showing an iliac physeal graft in position in the physeal transplant (TP). There is persistence of the proliferating and
lateral femoral physeal defect. g–n This series of photomicrographs hypertrophic cell zones in the graft tissue and continuity between
demonstrates the histological appearance of the physeal graft in relation metaphyseal bone from both segments. At the upper left, epiphyseal
to the persisting physis and associated epiphyseal and metaphyseal bone remains separate from metaphyseal bone. o Photograph demon-
bone following transplantation. Except figure g, the photomicrographs strating a poor result 4 months after transplantation is seen in this figure
are positioned with the epiphyseal areas at the top and the metaphyses o(i). When the animal was killed, the graft was found to have displaced
below. g Low-power photomicrograph illustrating the distal femoral from the defect site, allowing a bone bridge to form with significant
physeal graft at the right 3 months after insertion. Normal growth has shortening and valgus deformity. The non-operated control femur is on
occurred and no distal femoral valgus deformity is present. The graft is the left. Figure o(ii) shows radiographs of the two bones. p Photograph
approximately two times as thick as the persisting non-operated physeal illustrating an excellent result 4 months after growth-plate transplan-
tissue. Cartilage union has occurred at the graft-physis junction and tation in this figure p(i). The control femur is on the left. Figure p(ii)
there is no continuity between the epiphyseal and metaphyseal bone shows radiographs of the two bones illustrated in (i) demonstrating an
and no angular deformity. h Photomicrograph of the medial aspect of excellent result following transplantation
6.11 Treatment of Premature Partial Physeal Closure … 749

attempts to include bone were not made. Histological studies The type of growth-plate defect created led to bone bridge
confirmed the separation between the hypertrophic chon- formation and valgus deformity in experiments where no
drocytes and the metaphysis. The graft was trimmed under cartilage was interposed. Similar defects created by other
the dissecting microscope to remove the investigators have also led repeatedly to bone bridge for-
periosteal-perichondrial sleeve and much of the overlying mation and angular deformity. There have been reports of
fibrocartilage. The graft was then placed in the femoral defects that involved only a narrow, peripheral portion of the
defect with the correct orientation and the femoral periosteal plate in which a bone bridge formed but the bone subse-
sleeve was sutured to the intact femoral periosteum with 4–0 quently yielded to growth, with angular deformity not
Dermalon to hold the graft in place. The animals were not occurring as the intact physis overcame the small bridge.
immobilized postoperatively. The iliac apophysis is com- There also have been reports of small central bone bridges
posed of a physis, epiphyseal cartilage, and a fibrocarti- forming but not restricting growth. There thus appears to be
laginous layer (Fig. 6.27c). The apophysis was separated a relationship between the size of a physeal defect and bone
gently from the metaphysis of the iliac crest, at the junction bridge formation and the effect of the bridge on subsequent
between the lowermost hypertrophic chondrocytes and the growth.
metaphysis (Fig. 6.27d). Most of the fibrocartilaginous layer Since normal growth did not continue in the sham
and all of the perichondrium and periosteum were removed experiments after creation of the growth-plate defect alone,
using the dissecting microscope. The physeal graft was then the large number of good and excellent results following
placed in the femoral defect and the overlying femoral physeal transplantation is attributable to insertion of the
periosteal sleeve was sutured into place (Fig. 6.27e, f). graft. The graft may have functioned simply as a passive
In the transplant specimens assessed as early as 1 week barrier to vascularization, keeping the epiphyseal compart-
postoperatively, the physeal graft appeared to be intact and ment separate from the metaphyseal compartment without
viable. The morphology and organization of the physis were actually contributing actively to growth. If so, the absence of
retained. Union of the graft by cartilage at the graft-host deformity might have been due to persistence of growth of
junction was seen at as early as 2 weeks. Viability of the the remaining growth plate alone. Separation of the two
growth plate was evident histologically by production of circulations is the prime function of any interpositional
metaphyseal bone, maintenance of physeal height and material, be it biological or prosthetic. Intimate communi-
cytological organization, and the absence of vascular inva- cation between the epiphyseal circulation with its associated
sion or bone formation in the germinal, proliferating, and osteoprogenitor cells and the metaphyseal circulation with
columnar cell layers. The physis maintained its bright-red its osteoprogenitor cells allows a bone bridge to form.
stain with safranin O. The residual overlying fibrocartilagi- Examples of passive barriers include heterogenous
nous tissue of the graft underwent vascular invasion and deep-frozen hyaline cartilage, fat, Silastic,
ossification with time, but the growth plate itself appeared methyl-methacrylate, and bone wax. Physeal cartilage is
immune. A detailed description of the histological charac- avascular and also possesses an anti-angiogenesis factor
teristics of the transplanted physis is presented in Fig. 6.27g– which inhibits vessel ingrowth.
pi, ii. The histological studies of the transplants indicate that the
In the definitive study of the capacity of the physeal morphology, viability, and normal function of the trans-
transplant to prevent bone bridge formation, shortening, and planted physis were retained. The proliferating chondrocytes
valgus deformity, 33 rabbits received transplants. remained organized in orderly rows and contributed to lon-
Twenty-seven of the rabbits were killed 21 days or more gitudinal growth. The persisting epiphyseal blood supply
after surgery. The capacity of the graft to prevent growth adjacent to the defect provides nourishment for the resting,
arrest or valgus deformity was then assessed by its gross and germinal, and proliferating cell layers of the transplanted
radiographic appearance and by measurements of the dis- iliac physis; as long as the graft is sufficiently thin, is han-
tances between the femoral head and the medial and lateral dled gently, and is well positioned, it appears that it will
condyles of the distal end of the femur. The results were survive. Cartilage union at the persisting physis-graft junc-
classed as excellent, good, fair, or poor, as already defined. tion was demonstrated histologically in the several speci-
Photographs of the gross appearance and radiographs of mens from animals that were killed at early time-periods.
the femora demonstrate an obvious, striking difference Endochondral bone formation occurred beneath the physeal
between an excellent and a poor result. In the preliminary transplant in a fashion almost indistinguishable from that of
studies, when the experimental technique and the criteria for the persisting physis.
assessment were evolving, there were 7 excellent, 6 good, 4 The importance of a narrow graft must be stressed. As the
fair, and 2 poor results. In the definitive group, when grading cells of the growth plate are supplied by diffusion, removing
criteria were more stringent, 16 were excellent, 3 good, 4 fair most of the overlying fibrous and fibrocartilaginous tissue
and 4 poor (a good or excellent result in 70%). from the iliac crest cartilage graft allowed vascular diffusion
750 6 Lower Extremity Length Discrepancies

from the epiphyseal side to supply the transplant almost turn was superior to fat. The tibias that received free fat as
immediately. With time, the fibrocartilage remaining on the interposition material developed severe varus angulation and
graft underwent vascular invasion, followed shortly by failed to grow in length. The bony bridge redeveloped in all
endochondral bone formation such as occurs in iliac cases in the transferred adipose tissue, contrary to the find-
apophyseal ossification prior to skeletal maturation. This ings of Langenskiold and his group. In the experimental
bone soon merged with that of the secondary ossification group with iliac physeal grafts, varus angulation was much
center of the epiphysis. The physeal portion of the graft, less and the amount of longitudinal growth much greater
however, remained intact without suffering vascular invasion than that of the other groups with fat and silastic. In many of
until the time that the entire distal femoral physis reached the animals “the transferred physis remained viable and
maturity. A bed of well-vascularized epiphyseal and meta- therefore could have contributed to growth and also could
physeal bone is essential, as the nutrition of the free graft is have prevented reformation of the bony bridge through its
derived exclusively from its surrounding tissue. spacer effect.”
Lalanandham et al. transplanted scapular cartilage in the This experimental approach is for well localized, focal
rabbit into the distal ulna following curettage of the ulnar lesions, in this instance involving approximately one-quarter
physis in animals 5–6 weeks old [656]. They then assessed of the physis in the coronal plane and one-half of that seg-
the viability and biochemical function of the transplanted ment in the sagittal plane. The shape of the iliac physis must
chondrocytes and their histologic appearance at varying time also be considered in relation to the shape of the defective
periods. They concluded that the cartilage transplanted in an physis, as few long bone physes are as level as diagrammatic
avascular fashion could remain viable, synthesize proteo- and radiographic projections often imply.
glycan and also be associated with active growth (although It appears that full physeal and certainly epiphyseal grafts
less than normal). Histologic sections at 2 weeks showed the can be successful only if they are transplanted with an
transplanted block of cartilage to be intact and by 8 weeks associated vascular supply. The possibility of using vascu-
the transplanted cartilage appeared to be participating in larized iliac-crest apophyseal transplants has been investi-
endochondral ossification on the metaphyseal side. Histo- gated. In focal physeal lesions, however, when the host
chemical studies from the graft cartilage were consistent at 2, epiphyseal and metaphyseal bone is present and well vas-
7, and 14 days and showed lactic dehydrogenase to be cularized, free autogenous iliac crest might be effective
strongly present in both transplanted and unoperated control clinically. Although not all of the transplants were success-
cartilage. Radiosulfate administered intravenously was ful, the dramatic differences demonstrated between the
clearly present throughout the transplanted tissue also at 2, 7, excellent, good, and even fair results in comparison with the
and 14 days. This incorporation was indicative of chondro- poor and sham results clearly indicate that free physeal
cyte synthesis of proteoglycan. Thymidine incorporation transplants in the appropriate environment can function well.
was seen but was diminished relative to controls. The graft must be freed from much of the overlying fibro-
Several groups have used animal models to compare the cartilage and surrounding periosteum and perichondrium,
effects of interpositional tissues. Martiana et al. used many and must be fitted gently but firmly into narrow defects so
interpositional tissues in physeal regions of the distal left that coaptation is intimate. In addition, the host periosteum at
femur of a 3-month-old rabbit including muscle, fat, physeal the region of the groove of Ranvier should be sutured over
allograft, and iliac apophyseal autograft [657]. A standard the graft to provide both mechanical and physiological
defect was created in the lateral distal physis of the left support. As the majority of lesions due to trauma and
femur in all rabbits. A control group had no interpositional Blount’s disease leave the epiphyseal and metaphyseal bone
material. At 12 weeks following surgery assessments intact and well vascularized, free iliac-crest physis trans-
involved limb length discrepancy and angular deformity. plants may be useful in the treatment of focal physeal arrest
Muscle, fat, and iliac apophyseal autograft had less severe in patients with such conditions.
limb length discrepancy and angular deformity than did the
control group and the physeal allograft group. In terms of
limb length discrepancy and angular deformity, the best 6.11.2 Vascularized Autogenous Epiphyseal
results were seen with the iliac crest autograft. The second and Iliac Crest Grafts
best tissue in each regard was the interposed muscle with fat
third best. Since studies of nonvascularized transplants over several
Lee et al. excised the medial half of the proximal tibial decades stressed poor or imperfect results owing to failure of
epiphyseal growth plate in the rabbit to create a partial rapid revascularization, the use of vascularized transplants
growth arrest, then excising the bone bridge and inserted held great attractiveness and once vascularized bone trans-
either iliac crest physis, fat, or silastic into the gap [658]. The plants became feasible investigation was extended to this
iliac crest physeal transfer to be superior to silastic which in area. Nettelblad et al. transplanted successfully the proximal
6.11 Treatment of Premature Partial Physeal Closure … 751

third of the fibula including the entire epiphysis, adjacent plate, preventing the formation of bony bridges between the
metaphysis, and diaphysis in 22 puppies showing the fea- epiphysis and metaphysis.” Vascularized iliac-crest trans-
sibility of the technique [659]. In the experimental groups, plantation has been used experimentally to augment acetab-
the fibula switch was performed selecting one fibula as a ular growth in dogs with severely deformed hips and damaged
vascularized graft and the other as a nonvascularized graft. femoral growth as a result of epiphyseal lesions. Allieu also
Continuous growth was observed in the vascularized epi- provided a brief report on the feasibility of iliac crest cartilage
physeal transplants and in the controls with no statistical and bone pedicle transplants into the acetabular and proximal
difference noted whereas the nonvascularized transplants femur region [663].
exhibited considerably less or no growth. Varying tech-
niques subsequently confirmed the continued viability of the
vascularized epiphyseal transplants in contrast to the non- 6.11.3 Physeal Reconstruction Using Tissue
vascularized procedures. from Fetal and Early Postnatal
Teot and associates have reported on their investigations Epiphyses
concerning vascularized partial iliac crest growth cartilage
transplants [660–662]. An initial study performed in 50 child- Zaleske and colleagues attempted physeal reconstruction with
hood cadavers and 25 immature dogs assessed the vascular- young fetal-neonatal tissue to take advantage of its greater
ization of the lower end of the femur and the iliac crest [660]. growth potential [664–667]. Experiments were done involving
They were able to identify regions of cartilage from the epi- full and partial physeal reconstruction in mice with most studies
physes that could be transplanted with anastomosis of appro- involving 4-day-old postnatal distal femoral tissue. While
priate pedicles. In a second brief presentation, the value of iliac growth in length over brief periods of time has tended to be
crest cartilage as a graft source was again reviewed [661]. They limited to the 25 % range, autoradiographic studies using triti-
utilized iliac crest vascularized transfers in an experiment in 38 ated thymidine show persistence of cell proliferation after
puppies replacing approximately 80 % of the distal femoral avascular transplantation. Isolated physeal regions appear to
growth plate region. Initial indications were of growth in the maintain their kinetic activity at least in short-term implanta-
range of 85 % of that on the opposite normal side. Early report tions. In the 4-day-old distal mouse femur, which serves both as
was also made of 1 case in a 2-year-old patient with a total the source of tissue and the area into which tissue is implanted,
epiphyseal arrest of the distal femur who had a vascularized iliac the epiphysis is completely cartilaginous and avascular. The
crest transplantation after bone bridge resection. The patient work, summarized by Barr and Zaleske, showed that as a group
was doing well 16 months later but no further follow-up was the transplanted physeal blocks resulted in femurs of signifi-
reported. The second proposed utilization was in reestablishing cantly shorter overall length [664]. Metabolic and kinetic
acetabular growth for the dysplastic hip. analysis, however, showed both tritiated thymidine and
The vascular pedicle had its origin from the deep cir- radioactive sulfate incorporation indicating that cell viability
cumflex iliac artery. A more formal presentation of iliac crest continued even though normal function was not reconstituted.
pedicle graft transplantation was reported from 48 immature They concluded that blocks of cartilage containing important
dogs in relation to repairing distal femoral growth plate cell populations can be transplanted in a nonvascularized
abnormalities [662]. In 1 group the graft was pedicled on the fashion with at least partial maintenance of viability. The bulk of
superficial circumflex iliac vessels and reimplanted in situ. In this transplantation investigation was done with highly inbred
group 2 the pedicle graft was transferred to the groin area as an strains allowing for syngeneic transplantation. The work was
island graft. These two control groups demonstrated conser- expanded using complete epiphyseal replacement via knee
vation of growth activity when the graft was pedicled on its transplantation in the murine model but utilizing tissue of dif-
epiphyseal vessels. In group 3, the graft was transferred to the ferent developmental times in neonatal mice in an effort to
distal epiphyseal area of the femur after resection of the por- determine whether or not a specific stage of epiphyseal chon-
tion of the growth plate (approximately 2/3) located inside the drogenesis led to improved results. Studies were done in
perichondrial ring of Ranvier with conservation of 80 % of the 4-day-old postnatal mice but the distal femoral and proximal
outer cylinder. Microsurgical revascularization was achieved tibial chondroepiphyses in their entirety were transplanted from
by using the saphenous vessels. In group 4 the latter technique a 4-day-old postnatal mouse, a 1-day-old postnatal mouse, and a
was used without revascularization. Results were far more 17-day-old fetal mouse. Similar histologic, metabolic, and
favorable in terms of growth restoration in the pedicled than in kinetic analyses were done, as had been used previously. The
the nonvascularized transplant. In the vascularized distal animals were followed for 2 months post surgery. There was
transplant, the possibility remained that some regeneration of variability in morphology and growth from the transplanted
cartilage was from the surrounding epiphyseal plate which syngeneic knee in all experimental groups but the important
had been left intact. They concluded that the pedicle graft observation was felt to be the presence of an unequivocal joint
“appears to act as a catalyst in the formation of a new growth with distal femoral and proximal tibial secondary centers of
752 6 Lower Extremity Length Discrepancies

ossification in adjacent physeal regions implying some con- (PCP) signaling pathway has been shown to enhance the
tinuing function of the chondroepiphyseal transplant. No dif- formation of columnar growth plate architecture and can be
ferences could be noted between the three groups of slightly seen to function in patterning growth plate chondrocytes
differing ages. Since the chondroepiphyses were transplanted in vitro [670]. The ability to grow chondrocytes which
prior to their vascularization, the fact that they survived and synthesize a cartilage matrix in vitro has led to the hope that
subsequently were vascularized to form secondary ossification chondrocyte suspensions grown in tissue culture might be
centers demonstrated the ability to utilize developmentally placed within focal physeal defects to reconstitute a func-
immature tissue which could progress to increased develop- tioning physis. Experimental reports of this possible
ment in its new position. approach have appeared. Hansen et al. reported on the
growth of chondrocytes into cartilage discs after culturing
isolated epiphyseal chondrocytes from sheep [671]. The best
6.11.4 Cell-Based Therapy for Focal Growth results are achieved in experimental animals using fetal
Plate Cartilage Repair cartilage as the source of chondrocytes. While there is some
evidence that a cartilage tissue subsequently forms in the
Increased understanding of mesenchymal stem cells (MSCs) physeal defect and proliferates, there is no evidence that the
and chondrocrytes has led to multiple attempts to direct them cytologically specialized physis has been reconstituted.
into a growth plate cartilage synthesis and repair mode Tobita et al. transplanted autogenous chondrocytes cultured
[668]. The most common use of cell-based growth plate in collagen gel into growth plate defects in 8–10 week old
therapy is for focal lesions, usually transphyseal bone rabbits [672]. Results in terms of angular deformity and
bridges following growth plate fracture separations. Exper- length in the growth plate defect model were better than in
imental approaches started with efforts at implantation of the groups with the defect left empty or filled with autoge-
chondrocytes. This tended to poor repair since already dif- nous fat tissue. Autogenous chondrocytes were harvested
ferentiated chondrocytes were ineffective in expanding to fill from cartilage of the knee joints, embedded in collagen gel
spaces and, more importantly, did not further differentiate and cultured for a week prior to transplantation.
into the highly structured physeal cartilage. Autogenous
chondrocytes can be harvested, cultured, and expanded
ex vivo prior to being implanted back into the patient. This 6.11.5 Transplantation of Entire Physes
has shown good clinical applicability for osteochondritis and Epiphyses
dissecans lesions in the knee but has not been well adapted
for physeal focal defects. Both nonvascularized and vascularized transplantation of
Attention is now turning to working with mesenchymal epiphyseal plate autografts have been attempted, experi-
stem cells and directing them to a cartilage-line differentia- mentally and occasionally clinically, for over 100 years.
tion. Exogenous growth factors and scaffolds are also being Throughout most of this time, nonvascularized transplants
employed. Two lines of MSC-based treatments are being were studied. By the late 1960s, advances in vascularized
investigated: (i) cell harvesting, MSC isolation and ex vivo tissue transfers allowed investigation into use of vascular-
expansion; (ii) mobilizing endogenous MSCs to the growth ized epiphyseal transfers [673].
plate injury site and enhancing in situ regeneration. The
mesenchymal stem cell approach has also been directed 6.11.5.1 Early Transplantation Experiments
toward physeal cartilage regeneration, as well defined by 1899–1914
Chung, Foster, and Xian [668]. Planka et al. have treated a The results of the initial experimental transplantations of
distal physeal defect by transplantation of autologous and entire growth plates in dogs were almost all poor in allo-
allogeneic mesenchymal stem cells. Newly formed hyaline grafts but there were some favorable reports of reimplanta-
cartilage was demonstrated histologically in both groups tion and autograft viability and growth. Haas reviewed in
[669]. Differentiation along the chondrocyte line has been great detail the studies on growth plate transplantation that
enhanced by use of growth factors such as FGF-2 and var- had been reported in the German literature between 1899
ious TGF-βs. Use of scaffolds has also been applied espe- and 1914 [674, 675]. Although an optimistic report had been
cially natural materials which are biocompatible, presented concerning the effectiveness of allograft (homo-
biodegradable, appropriate in pore size, and suitable for plastic) transplantation of the upper end of the radius
MSC growth and differentiation. Materials used include including the epiphyseal cartilage allowing for growth in
chitosan, collagens, fibrin gels, hyaluronan, and alginate. rabbits he felt that the interpretation of that work by the
Injectable hydrogels allow MSCs to be embedded into them authors was inaccurate and remained unconvinced that
along with growth factors and can be molded easily into actual growth had occurred since very little growth occurred
irregular spaces. Activation of the Wnt Planar Cell Polarity normally in the proximal radius. In assessing all the reports,
6.11 Treatment of Premature Partial Physeal Closure … 753

there was virtually no growth noted in any of the allograft he too concluded that autografts were much superior to
transplantations although there was on occasion some allografts but that even with autografts there was not regular
growth in the autotransplantations and often considerable growth of the epiphyseal cartilage even though some of the
growth in reimplantations. The term reimplantation refers tissue survived and in no case was there lengthening of the
simply to removing a segment of bone or cartilage and then transplanted joint. Axhausen had also transplanted the lower
replacing it in its same site. In none of these approaches were quarter of the femur from a growing rat to the subcutaneous
any vascular repairs performed. tissue of another rat [684]. The physeal cartilage was noted
Among the earliest detailed epiphyseal transplantation to degenerate such that at 20 days only the peripheral parts
studies were those of Helferich [676] and Enderlen [677] remained alive. With further time the entire physis was
who provided separate reports in 1899. The investigators, replaced by fibrous tissue. Similar findings were noted when
who worked together, reimplanted the epiphysis in rabbits the same experiment was repeated in the rabbit. Von Tap-
using the lower epiphyseal cartilage of the ulna with an peiner performed 3 reimplantations and 8 allografts in dogs
adjoining piece of epiphysis and diaphysis. Helferich [678, 679]. In the reimplantations, there were practically no
reported on the macroscopic findings that the epiphyseal changes in the epiphyseal cartilage line even after 6 months
cartilage, under favorable conditions, need not lose its as assessed microscopically. Even in the allograft group
property of producing length growth. A lessening of this some histologic evidence of continuing function was noted.
ability was noted but generally there was not a complete loss The findings of Von Tappeiner were not confirmed by Haas.
of growth. Enderlen reported on the microscopic findings Obata described a progressive degeneration of the epiphy-
and indicated that some of the reimplanted epiphyseal car- seal cartilage line in the reimplantation group until the 50th
tilage remained viable to a large extent in particular those day at which time there was some repair of function [680].
parts adjacent to the perichondrium [677]. Von Tappeiner He also ascribed almost normal functional properties to this
performed reimplantations and allograft transplantations on regenerated epiphyseal cartilage. By 70 days however
dogs 6 and 12 weeks of age using the distal half of the degeneration had again occurred leading Haas to interpret
metatarsal [678, 679]. In the reimplantations, he found no the fact that under some favorable circumstances some of the
disturbance in length growth even after 6 months while in physes continued to function. Otherwise, the changes
the allograft transplantations growth was markedly dimin- described by Obata were similar to those of Haas. In Obata’s
ished. Obata performed reimplantations, autografts and allograft work, the epiphyseal cartilage underwent progres-
allografts of the entire metatarso-phalangeal joint with either sive degeneration with practically no regeneration. Heller
a part or an entire metatarsal and phalanx [680]. He felt that noted some favorable results in reimplantations. There was
shortening occurred in all cases but that it was most marked some regeneration of the epiphyseal cartilage line and some
in the allograft transplantations. new growth but overall the bone growth was retarded. In
An extensive study was performed by Heller who carried allograft transplants, the results were invariably poor. Haas
out reimplantations of the distal epiphysis of the radius and felt that use of the distal end of the radius and ulna made
ulna as well as homotransplantations (allografts) [681, 682]. interpretation of one bone transplantation more difficult than
The epiphysis did not keep its normal length growth in any in the simpler and more straightforward metacarpal or
of the 45 experiments in which the epiphyseal cartilage was metatarsal model. The findings of Minoura agreed closely
transplanted in the form of half joints. The best results with those of Haas. In none of the experiments was there any
occurred in the reimplantation group while the worst, by increase in length of the bone after transplantation.
which is meant the greatest degree of shortening, occurred in
the allograft between non-related animals. In the allograft 6.11.5.2 Haas
transplantation group, there was complete cessation of epi- Haas reported on his 75 experimental procedures on dogs
physeal function. He concluded that in autografts there was regarding the effectiveness of epiphyseal transplantation in
active regeneration of the epiphyseal cartilage from the maintaining growth [674]. The majority of the animals were
perichondrium but that bone growth did not remain at a from 1½ to 4 months of age at time of surgery. The meta-
normal amount. The most favorable conditions for epiphy- carpals and metatarsals were selected for transplantation
seal cartilage transplantation would be in the form of a thin since they had only one epiphysis making it easy to deter-
sheet of physeal cartilage without adhering bone particles so mine whether or not growth from the transplanted region had
that the cartilage could come directly into contact with occurred. The bones were also quite stable after reposition-
nourishment from the host. He reported almost normal ing because of the nonoperated adjacent bones. Review of
growth in instances where autograft transplantation had been the literature indicated failure of allograft transplantation to
performed transferring physeal cartilage only. Minoura be effective in virtually all studies and thus Haas concen-
transplanted metatarso-phalangeal joints of 2 month old trated on reimplantation and autograft transplantation. Fol-
rabbits into soft tissue [683]. Varying models were used but lowing reimplantation of an entire metacarpal or metatarsal
754 6 Lower Extremity Length Discrepancies

which had been removed from its position with the articular of the secondary ossification center, the marrow and bone of
surface and periosteum intact and then immediately replaced the metaphysis and cortical bone. With epiphyseal cartilage
there was complete cessation of growth of the epiphysis. reimplantation, the first evidence of degeneration of the
Autograft transplantation of the entire metacarpal or meta- epiphyseal cartilage was seen at 23 days and appeared as a
tarsal, in which an entire bone was transplanted from one cleavage line extending through the cartilage columns
foot to another foot of the same animal, also showed no dividing the physis into a proximal 2/3 and distal 1/3. With
evidence of growth. No effective growth was seen with split time, progressive degeneration of the epiphyseal cartilage
metacarpal reimplantation or autograft transplantation. In the line occurred and by 85 days there was almost complete
group most likely to succeed, namely those having reim- disappearance of the physeal cartilage. With autotransplan-
plantation of the epiphyseal cartilage, the cartilage was tation the epiphyseal cartilage also showed early degenera-
transplanted with a piece of adjoining epiphysis and diaph- tion and at 23 days a line of cleavage was noted through
ysis and even here there was no effective growth. A similar what appeared to be the hypertrophic zone. By 135 days,
approach with autograft transplantation from one foot to the only a slight remnant of physeal cartilage persisted. Fol-
other showed no definite evidence of growth with the con- lowing reimplantation with varying lengths of the epiphyseal
clusion that after autograft transplantation of the epiphyseal and of the metacarpal and metatarsal bones degeneration of
cartilage its function for linear growth was entirely lost or the cells at the junction of the proximal 2/3 and distal 1/3 of
present to only a minimal degree. There was also uniform the physis was noted followed by fissuring and eventual
failure of growth with reimplantation of varying lengths of degeneration of the entire cartilage. With autotransplantation
the epiphyseal end of the metacarpal and metatarsal bones the epiphyseal cartilage line underwent a progressive and
and uniform failure of growth after autotransplantation of complete degeneration. The epiphyseal cartilage line also
varying lengths. Haas’ conclusions were quite straightfor- underwent progressive and complete degeneration with
ward; the epiphyseal cartilage lost its power to function after either reimplantation or autograft transplantation of the
transplantation either of the reimplantation or autograft entire metacarpal or metatarsal bone. Haas reconfirmed his
transplantation type and with the physis transplanted either observations that the epiphyseal cartilage ceased to function
alone or with an accompanying piece of epiphysis and dia- after either reimplantation or autograft transplantation in
physis. When Haas uses the term epiphyseal cartilage each of several approaches. The longitudinal growth ceased
“alone” he still obtained a piece with small adjacent regions in each case. The cartilage degenerated, there were fre-
of epiphyseal and metaphyseal bone. He concluded that the quently transverse and vertical fissures followed by a dis-
epiphyseal cartilage was very vulnerable and that its viability appearance of the cells, fibrous substitution and eventually
was directly dependent upon its blood supply. In no instance bone transformation. The only evidence for regeneration was
was any vascular repair done with his transplants and the near the periphery beneath the perichondrium although the
physeal cartilage rarely had direct access to the persisting new cartilage possessed none of the length performing
vascularity of the host bone since it was always transplanted functions of the normal physeal pattern. He concluded that
with a thin rim of bone on epiphyseal and metaphyseal sides. the epiphyseal cartilage was the least transplantable of any of
Haas himself addressed this question in an abstract of the the components of bone due to damage to the vascular
discussion printed immediately following the article. He supply to the epiphyseal region.
noted that “in all the excisions of the epiphyseal cartilage a Haas later performed another series of transplantation
piece of adjoining epiphyseal and diaphyseal bone was procedures since others continued to describe some effective
removed so as not to injure that particular region.” Heller results with epiphyseal transplantation [685]. He concluded
had commented on this matter and felt that in his work he again however following 20 additional procedures that “the
had succeeded in transplanting the epiphyseal cartilage in the epiphyseal cartilage plate loses its power of causing length
form of very thin sheets of cartilage which subsequently growth after transplantation.” Haas defined the potential
resulted in almost normal growth. Haas felt that Heller’s clinical value of epiphyseal transplantation but concluded
conclusions were incorrect either in the sense that he could after extensive and repeated experimentation that longitudi-
not truly effectively transplant just cartilage alone, leaving nal growth ceased after reimplantation, autograft or allograft
open the possibility that it was the persisting cartilage not transplantation of the epiphyseal cartilage whether by itself
removed for transplantation that enabled growth to occur. or with a neighboring piece of epiphyseal or diaphyseal
In a subsequent article, Haas reported on the macro- bone. Entire widths of physis in dogs were transplanted and
scopic, microscopic, and in some cases radiologic details most of the many variations used were unsuccessful, as Haas
from 58 of the 75 procedures [675]. The microscopic recognized, owing to failure to provide nutrition to the
description involved not only the epiphyseal growth plate resting, germinal, and proliferating chondrocytes of the
cartilage but also the articular cartilage, the marrow and bone physis. Even transplants of what Haas referred to as the
6.11 Treatment of Premature Partial Physeal Closure … 755

epiphyseal cartilage line, however, included a thin rim of followed for 5 weeks of more, 6 showed little or no short-
adjoining epiphyseal and metaphyseal bone. ening and 3 were failures. The reimplantation procedure
represents the most favorable in terms of prognosis. He then
6.11.5.3 Physiologic Concerns in Physeal performed comparative experiments involving autograft
Transplantation transplantation within the same animal from right to left and
Subsequent studies on physeal transplants continued to use vice versa and allograft transplantations from separate rab-
entire ulnar, radial, metacarpal and metatarsal physes, most bits. Detailed and accurate length determinations of normal
of which had some epiphyseal bone attached both to protect rabbit ulnar growth and of the transplanted animals were then
the physis from mechanical damage during removal and to made over a several week period. Histological and radio-
ensure its complete transplantation. The results were vari- graphic studies were made. He concluded that only five of the
able. It is probable that even thin layers of bone attached to autograft transposition group could be assessed as completely
physeal transplants on the epiphyseal side inhibit rapid dif- successful out of 18 procedures. The ulnar growth had to
fusion of nutrients to the physeal cartilage, result in chon- exceed 75 % of that predicted and stringent radiographic
drocyte death, and lead to growth-plate resorption and criteria had to prevail to give the successful grading. In the
replacement by bone. In support of this contention is the allograft group 26 procedures were done. Using the criteria
report by Heller of good results in physeal transplants when for successful transplantation developed for the other 2
a thin layer of cartilage was transplanted without a sliver of groups, 21 of the 24 were clear failures and while 3 looked to
epiphyseal bone attached to the graft although Haas pointed have acceptable radiographic appearances in each of these the
out that growth might have been due in such situations to epiphyseal cartilage was narrow and in none of the 3 was the
parts of physeal cartilage left behind which continued to growth in the experimental limb within 75 % of the control.
function [681]. Similar good results have been reported with He thus concluded that allograft transplantation was unsuc-
focal cartilage transplants performed using iliac-crest carti- cessful in all the animals studied, a conclusion invariably
lage without overlying epiphyseal bone.Some grafts appear reached by investigators throughout the century. In the
to have led to poor results after transplantation with the autograft transposition group normal growth occurred in only
surrounding perichondrium intact. This tissue layer would 5 of 18 procedures. Some of the failures were due to technical
inhibit nutrition by diffusion from the host tissues, which is difficulties in the sense that it was difficult to obtain a snug fit
essential in free transplants. In addition, most of the studies of the transplanted physis into its new host bone. Even when
on free epiphyseal transplantation have involved total phy- this was present however growth failure occurred because of
ses, which undoubtedly represent too extensive an amount of inability to restore full revascularization in an appropriate
tissue for consistently good results, especially if epiphyseal period of time.
bone, perichondrium, and periosteum are left attached and a
firm fit in the defect site is not achieved. 6.11.5.5 Harris et al.
Harris et al. achieved a 50 % success rate in autogenous
6.11.5.4 Ring transplants of immature rabbit whole distal ulnar physes by
Ring performed reimplantation, autograft and allograft leaving the host perichondrium intact and transplanting only
transplantations of the entire distal ulnar epiphysis in 3– physeal cartilage without a sliver of epiphyseal bone, thus
6 week old rabbits [686]. He had calculated that approxi- substantiating Heller’s results at least partially, as well as the
mately 85 % of the growth of the ulna occurred distally. He importance of allowing nutrition from the host to support the
also recognized that owing to the tight bond between the graft [687]. Extremely careful technique was utilized to
distal radius and the distal ulna there could be some length- obtain the graft. Following subperiosteal dissection of the
ening of the distal ulna region after transplantation which distal ulnar metaphysis and adjacent epiphysis for about 1/3
need not represent physeal growth but was due to a of its circumference the epiphysis was broken from the shaft
mechanical pulling apart of the distal ulna by the continuing with ease by manual pressure. Since the physis was quite
growth of the radius. After a period of time however failure of straight there was a constant line of separation through the
ulnar growth would be reflected by tethering of the adjacent hypertrophied cells of the zone of provisional calcification
radius and its growth in a curved pattern. The ulnar physeal leaving the bulk of the epiphyseal plate attached to the
cartilage was removed following knife cuts made trans- epiphysis. They then inserted a small scalpel blade as close
versely through the bony epiphysis and metaphysis such that to the bone plate of the epiphysis as possible excising only
the isolated cartilage was transplanted “with its thin attached the cartilaginous physis. Since the germinal layer was rela-
slivers of bone” following which it was gently freed from the tively thick during early development it was easier to obtain
radius and removed together with its surrounding perichon- an adequate transplant in the very young animals. They
drium. In a series of reimplantation experiments of 9 animals performed reimplantation in 65 rabbits, autogenous
756 6 Lower Extremity Length Discrepancies

transplantation (right to left) in 65 rabbits and allograft microangiographic. A major part of the cartilage transplant
transplantation using animals paired as closely as possible by survived in each of the rabbits and on one occasion there was
weight in 65 animals. A stainless steel wire was then placed total survival. Any imperfect result was due to the produc-
through the ulnar shaft approximately 0.5 cm proximal to the tion of a hematoma which limited passage of host vessels to
plate to serve as a radiographic guide for length measure- the graft and thus limited revascularization. Measurements in
ments. Sacrifice ranged from 1 to 84 days with 9 time terms of physeal function were not made however.
periods in the first 2 weeks and histologic studies at 21, 28,
56, and 84 days. In the allograft transplantations, 7 of 13 6.11.5.7 Transplantation of Vascularized
long-term (56 and 84 days) animals were successful. Normal Epiphyseal Plates
growth was virtually never noted in comparison to controls The biology of vascularized epiphyseal transplants was well
regardless of the type of procedure. In the reimplantation reviewed by Boyer et al. [673]. Much work was done out-
group 11 of 25 available long-term specimens showed a lining the details of blood supply to the epiphyseal regions
normal histological appearance of the plate and 80 % or (which we have reviewed in previous chapters). Three types
more of anticipated growth. In the allograft transplantations of vascularized epiphyseal plate studies were reviewed.
there was a remarkably uniform success rate up to 28 days. (i) The first involved studies after vascularized epiphyseal
All physes subsequently degenerated however and were plate orthotopic replantation. This refers to microvascular
destroyed between the 56th and 84th day. They concluded replantation into the same host in the same anatomic location.
that satisfactory survival with up to 80 % of normal growth This experimental approach assesses the ability of growth
for the 12 weeks of the experiment was achieved in ½ of the plates to maintain function after (immediate) revasculariza-
autografts with homografts fairing quite poorly in particular tion. (ii) the second approach involved studies of vascularized
after the first 4 weeks. Technical features for a good result epiphyseal plate heterotopic transplantation, meaning transfer
involved the necessity of including the germinal cells of the to different anatomic sites in the same individual. These are
physis within the transplant, an adequate supply of tissue autogenous free tissue transfers which not only define a bio-
fluid (i.e. vascularization) reaching the transplant from the logic principle but also represent some clinical applicability.
epiphyseal side and a snug fit of the transplant in the (iii) the third approach studied epiphyseal plate behavior after
recipient bed. Their interpretation, which appears accurate, allograft transplantation. The clinical problem limiting this
suggested that the transplants had to survive an avascular work is the need for immunosuppression to maintain cell
stage obtaining nutrition from the surrounding tissues before viability. Studies by Bowen et al. with microvascular growth
healing and stabilization occurred so that longitudinal plate transfers of the distal ulna of the immature dog illustrated
growth could continue. Care had to be taken to ensure that the feasibility of these transfers for subsequent growth when
the recipient bed in particular on the epiphyseal side had both epiphyseal and metaphyseal circulations were main-
been curetted to bleeding bone since it is the vascularity of tained [690, 691]. Donski and O’Brien transferred distal ulnar
the host that provides nutrition to the transplanted or reim- growth plates contained within epiphyseal and metaphyseal
planted physeal graft tissue. bone in the distal ulna of the dog [692]. Heterotopic and
orthotopic transplants were done, some vascularized and
6.11.5.6 Silfverskiold; Farine some not vascularized, although relatively few dogs were
Silfverskiold performed ulnar epiphyseal cartilage transfers done (13) with the bilateral upper extremity surgery.
in the rabbit and found that allograft transplantation pro- Heterotopic transplants (vascularized) averaged 69 % growth,
duced a complete cessation of growth while autograft with orthotopic even higher, while grafts without microvas-
exchange produced cessation of growth in 6 of 11 animals cular anastomoses showed no growth. Nettleblad et al. per-
and considerable retardation in the rest [688]. Farine also formed classic free microvascular epiphyseal plate
performed distal ulnar transplantation procedures in the transplantation in puppies in proximal fibulas showing
young rabbit [689]. Sacrifice in most was from the first to the retention of good values [659].
19th day after surgery with occasional animals followed to Experimental work in immature rabbits by Zaleske et al.
180 days. Operations were performed in 16 rabbits 5 weeks has shown that reimplantation of vascularized whole knee
of age. The transplant included the entire distal ulnar physis joints, including the epiphyses, can survive and continue
with two thin epiphyseal and metaphyseal lamellae of bone. growth [693]. At present, however, such procedures have
Autograft transposition from right to left was done in 16, and little clinical applicability, primarily because the epiphyses
in a second group an additional 16 had reimplantation pro- that can be sacrificed for transplantation do not fit the
cedures performed. The results in the two groups were anatomical and mechanical needs of the area to which they
similar. The subsequent study was histologic and would be transferred.
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