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Windswept: Deformity

1. The document describes "windswept deformity", an abnormal valgus deformity in one knee and varus deformity in the other, commonly seen in young children in parts of Africa. 2. Three case histories are presented of otherwise healthy African children who developed the deformity rapidly between ages 2-4. No known causes of bone disease were identified. 3. It is suggested the deformity may be due to an unrecognized hereditary bone dysplasia associated with a phase of epiphyseal instability during rapid growth.

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0% found this document useful (0 votes)
141 views2 pages

Windswept: Deformity

1. The document describes "windswept deformity", an abnormal valgus deformity in one knee and varus deformity in the other, commonly seen in young children in parts of Africa. 2. Three case histories are presented of otherwise healthy African children who developed the deformity rapidly between ages 2-4. No known causes of bone disease were identified. 3. It is suggested the deformity may be due to an unrecognized hereditary bone dysplasia associated with a phase of epiphyseal instability during rapid growth.

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Isaac Samuel
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WINDSWEPT DEFORMITY

E. H. J. SMYTH

From the Cambridge Military Hospital, Aldershot

The occurrence of “windswept deformity” in the legs of otherwise healthy African children in the
second or third year of life is Illustrated by three typical case histories. The usual causes of epiphysial
abnormality were absent in these and other
similar patients seen recently in central Nigeria. It is suggested
that an unrecognlsed hereditary dysplasla of bone, possibly of local geographical distribution and
associated with a phase of epiphysial Instability due to rapid growth, might underlie the sudden onset of
this striking deformity.

The term “ windswept deformity” describes the evidence of generalised bone disorder. Tests for sickle-cell disease
were normal. His serum alkaline phosphatase was not known. He had
appearance of abnormal valgus deformity in one knee in
a brother with mild bilateral genu valgum. He was treated by
association with varus deformity in the other. It is
corrective osteotomies to the femur and tibia on the valgus side.
commonly seen in young children in certain parts of
Africa, but has received only brief attention in the
medical literature.
Clinical features. The deformity usually occurs in a
healthy child with normal developmental milestones.
During the second or third year a valgus deformity
rapidly develops on the “windward” side quickly
followed by the appearance of a varus deformity of
comparable degree in the other, “leeward”, knee. The
transformation from normal alignment to severe defor-
mity may take only a matter of weeks. General skeletal
or metabolic disease, malnutrition and sickle-cell
disease are not found.
Radiographic appearances. The radiological changes in
the affected epiphyses and metaphyses are not unlike
those described by Blount (1 937) and Golding (1 962) in
bilateral tibia vara, and also bear some resemblance to
those seen in multiple epiphysial dysplasia (Wynne-
Davies and Fairbank 1 976). No other parts of the
skeleton are involved.
Fig. 1

ILLUSTRATIVE CASE REPORTS Case 1 . Boy aged seven.

These three children were seen at the University of Ife Hospitals in


Central Nigeria. Case 3. This girl had walked when one year old and showed no
Case 1. This boy showed no clinical abnormality at birth and walked evidence of abnormality until the age of two, when she gradually
when one year old. At the age of three he had an acute febrile illness, developed a valgus deformity of the right knee, and then a mild varus
possibly measles, after which he rapidly developed the windswept deformity on the opposite side. There was no evidence of rickets or
deformity. When seen aged seven (Fig. 1) he had no clinical or sickle-cell disease and no family history of skeletal abnormality. Varus
radiographic evidence of rickets or other generalised bone disorder, osteotomies ofthe tibia and femur were carried out on the valgus side.
and his serum alkaline phosphatase and sickle-cell tests we;e normal.
There was no family history of similar skeletal deformity. He was
treated by varus osteotomies of the tibia and femur on the valgus side DISCUSSION
and valgus osteotomy of the tibia on the varus side. The term “windswept deformity” is commonly used in
Case 2. This boy was normal at birth and walked when one year old.
Africa to describe the appearance of the legs in these
His development was normal until the age of four and a half when he
rapidly developed windswept deformity to such a degree that lateral
children. Fulford and Brown (1976) applied the term in
dislocation of the patella occurred on the “ windward” side (Figs 2 to a different context to a postural deformity acquired by
4). When seen at the age of six he had no clinical or radiological spastic infants during the first few weeks of life.

E. H. J. Smyth, FRCSEd, FACS, Consultant in Orthopaedics, Cambridge Military Hospital, Aldershot, Hampshire GUi 1 2AN, England.

166 THE JOURNAL OF BONE AND JOINT SURGERY


WINDSWEPT DEFORMITY 167

Case 2. Figure 2-Boy aged six. Figure 3-Photograph


showing lateral dislocation of the patella on the ‘ wind-
ward” side. Figure 4-Radiograph showing anteropos-
. tenor views of both knees. I
Fig. 2 Fig. 4

Abnormal degrees of symmetrical knock-knee and, developed a beak-like projection (Fig. 4). These
more commonly, bow-leg often occur among both white appearances might well be consistent with the mechani-
and coloured children, usually without evident under- cal pressure effects of a rapidly occurring deformity at
lying cause. Blount (1937) described a condition of tibia the time of a growth spurt.
vara in children and called it osteochondrosis deformans Idiopathic genu valgum was found by Morley
tibiae. Golding (1962) reported an extensive survey of (1957) to be present, with two inches or more of
this condition in West Indian children. Both authors inter-ma!!eo!ar separation, in 22 per cent of a group of
recognised a more common infantile form arising in the London children aged three to three and a half years,
first, second or third year of life and a rarer, usually less while only one to two per cent of seven-year-o!ds from
severe, adolescent type. The deformity could be the same environment showed an equivalent degree of
unilateral or bilateral and no certain aetiological factor deformity, indicating that there might be a period of
was identified. potential epiphysial instability at about the third year of
The radiological changes described by these life when any additional stress-factor, such as intercur-
authors, and in another patient with severe bow-leg rent disease or familial susceptibility, could tilt the
encountered by Brennan and Guarino (1961), resem- balance of skeletal growth in one direction and produce
bled those in our patients, with retarded growth on the rapid deformity. Go!ding has reviewed the details of
side of maximal pressure on the upper tibia! epiphysis, our patients and suggested the possibility of a genetic
the medial in genu varum, which became angulated and bone dysplasia of local geographical distribution
of denser consistency, while the adjacent rnetaphysis ( Golding, personal communication).

I wish to express my thanks for help in the production of this article to Miss Elizabeth Paget, Librarian at the Cambridge Military Hospital, and to
Staff Sergeant Casey, RAMC, of the Department of Medical Illustration.

REFERENCES

Blount WP. Tibia vara. J Bone Joint Surg 1937;19:1-29.


Brennan ii, Guarino CA. An unusual case of metaphyseal growth disturbances. J Bone Joint Surg [Am] 1961 ;43-A:88-92.
Fulford GE, Brown JK. Position as a cause of deformity in children with cerebral palsy. Dev Med Child Neurol 1976;18:305-14.
Golding JSR. Tibia vara. J Bone Joint Surg [Br] 1962;44-B:216.
Morley AiM. Knock-knee in children. Br Med J 1957;2:976.
Wynne-Davies R, FairbankTJ. Fairbank’satlas ofgeneralaffectionsoftheskeleton. 2nd ed. Edinburgh: Churchill Livingstone, 1 976;2-3, 200-3.

VOL. 62-B, No. 2, MAY 1980

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