Original Article

A Review of 100 Cases of Ectopia Lentis

with Glaucoma: Its Types, Presentation,
Management and Visual Prognosis
Bakht Samar Khan, Zubeda Irshad, Mustafa Iqbal

Pak J Ophthalmol 2010, Vol. 26 No.1

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See end of article for Purpose: To examine the clinical profile of glaucoma associated or caused by
authors affiliations ectopia lentis, its types and management and visual prognosis.
….………………………… Material and Methods: The medical record of 100 consecutive patients
suffering from Ectopia lentis and glaucoma admitted in Eye Unit of Khyber
Correspondence to: Teaching Hospital, Peshawar from June 2002 to December 2007 were reviewed
Bakht Samar Khan retrospectively. The clinical records were analyzed. The main parameters were
Assistant Professor type of glaucoma, its management, visual prognosis and IOP control.
Department of ophthalmology
Khyber Medical College Results: No age and sex was exempted. Main types of glaucoma were pupillary
Peshawar Pakistan block 26.92% (35 eyes), angle anomaly 18.46% (24 eyes), glaucoma due to lens
in the anterior chamber 16.92% (22 eyes), traumatic glaucoma 14.61% (19eyes),
phacolytic glaucoma in 10% (13 eyes) followed by mixed type of glaucoma
16.16% (18 eyes). In 87.69% (114 eyes) surgical intervention done as peripheral
iredectomy in 33.08% (43 eyes), trabeculectomy 13.85% (18 eyes),
phacotrabeculectomy in 9.23% (12eyes), lensectomy in 23.08% (30 eyes) and
lensectomy with vitrectomy in 8.46% (11 eyes).
Glaucoma was completely relieved in 74.62% cases while 25.48% (43 eyes)
needed antiglaucoma medication. Visual acuity was improved in 70.77% (92
eyes). Amblyopia, optic disc cupping and optic atrophy were the main causes of
visual failure.
Received for publication
March’ 2009 Conclusion: Glaucoma in ectopia lentis patients is very common. In more than
….………………………… 80 % cases need surgical intervention like crystalline lens removal plus
glaucoma surgery. Even after surgery chances of refractory glaucoma is there.

E
ctopia lentis or displaced crystalline lens The management of glaucoma associated with
syndrome may be congenital or acquired .In ectopia lentis is often difficult and challenging. The
either case it is associated with a number of current management strategies until lately were aimed
ocular and systemic complication. The ocular compli- at reduction of intra ocular pressure by topical eye
cations are mainly glaucoma, cataract and uveitis. drops containing beta-adrenergic antagonist,
Lund and Sjontoft conducted study in1950 and found cholinergic agonists, adrenergic agonists or carbonic
glaucoma in 25% of cases. Various type of glaucoma’s anhydrase inhibitor and prostaglandins analougues or
associated with ectopia lentis are, pupillary block, in combination with systemic carbonic anhydrase
angle anomaly, phacolytic, traumatic angle recession, inhibitors and hyperosmotics. Failure of medical
primary angle closure, primary open angle and mixed therapy is followed by laser or surgical intervention.
type. Glaucoma is a serious blinding disease as WHO The main surgical interventions are peripheral
data in 1990 has reported is the third leading cause of iridectomy, trabeculectomy with or without antimeta-
blindness in the world.

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bolites, lensectomy with or without vitrectomy and Type of glaucoma
phacotrabeculectomy. Pupil block glaucoma in 35 eyes (26.92%), angle
anomaly in 24 eyes (18.46%), glaucoma due to lens in
MATERIAL AND METHODS the anterior chamber in 22 eyes (16.92%), traumatic
All the patients of ectopia lentis associated with glaucoma in 19 eyes (14.61%), phacolytic glaucoma in
glaucoma (intraocular pressure more than 22mmHg) 13 eyes (10%) mixed type glaucoma in 09 eyes (6.92%),
admitted to eye ward from June 2002 to Dec.2007 were primary open angle and closed angle in 05 eyes
studied. The history charts chosen were analyzed for (3.85%) and 03 eyes (2.31%) respectively (Table 3).
relevant data. This includes age, sex and visual acuity.
History revealed ectopia lentis to be congenital or Table 1: Sex and Glaucoma
acquired. Ocular examination was done with respect
to visual acuity, anterior chamber examination i.e. Sex No of patients n (%)
corneal edema, peripheral anterior syncline, anterior
Male 77 (77)
chamber depth, iris atrophy, lens dislocation
(luxation)/ subluxation and its site. Intraocular Female 23 (23)
pressure measurement with applanation/ goldmann Total 100 (100)
tonometer taken at the time of admission and
discharge. Gonioscopy finding were recorded.
Posterior segment assessment including disc cupping, Table 2: Ages and Glaucoma
neovascularization, atrophy and hemorrhage.
Systemic examination was performed with specific Age (years) No of patients n (%)
anomalies of cardiovascular system, musculoskeletal Up to 10 19 (19)
system and nervous system in view.
11-19 37 (37)
Where needed electrocardiography, echocardio-
graphy, full blood count, ESR, urine examination, 20 to 9 22 (22)
bleeding time, clotting time, sodium nitroprusside test,
homocystine level in urine and plasma were also done. 40 to 59 17 (17)
The treatment modalities were studied. Anti 60 and above 05 (05)
glaucoma therapy was given to control glaucoma. In
majority cases it was not successful. Surgical Total 100 (100)
intervention was done. These were mainly iridectomy,
trabeculectomy, lensectomy, phacotrabeculectomy and
Table 3: Types of glaucoma
vitrectomy.
Types No. of eyes n (%)
RESULTS
Angle anomaly 24 (18.46)
One hundred and thirty eyes of one hundred
consecutive patients having ectopia lentis with Pupil block 35 (26.92)
glaucoma, hospitalized for management in Khyber Photolytic 13 (10)
teaching hospital were studied. Out of these one
hundred patients, seventy-seven were male and Glaucoma with lens in 22 (16.92)
twenty three were female. Bilateral glaucoma was anterior chamber
present in thirty patients and unilateral in seventy
Angle closure glaucoma 03 (14.61)
(Table 1).
Open angle glaucoma 05 (2.31)
Age distribution Trauma and angle 19 (3.85)
No age was exempted. Up to age 10 years there were recession
19 patients (19%), in 11-19 years age group 37 patients Lensectomy and 09 (6.92)
(37%), in 20 to 39 years group 22 patients (22%) while vitrectomy
40 years and above 22 patients (22%) (Table 2).

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Therapeutic intervention lens dislocation/subluxation (ectopia lentis), lens
In majority cases ectopia lentis with glaucoma was intumescent cataract glaucoma, classical pupillary
unable to be controlled with anti glaucoma block glaucoma, lens particle glaucoma, phacoanaphy-
medication. In 114 eyes (87.69%) needed surgical laxis and phacolytic glaucoma1. Various studies
intervention as peripheral iredectomy in 43 eyes showed glaucoma association is an established fact2-3.
(33.08%), lensectomy in 30 eyes (23.08 %), trabeculec- Glaucoma has been reported in patients with
tomy in 18 eyes(13.85%), phacotrabeculec-tomy in 12 ectopia lentis as part of the syndromes like marfan,
eyes (9.23%) and lensectomy with vitrectomy in 11 Weill marchesani, homocytineurea and aniridia4.
eyes (8.46%) eyes (Table 4). Fibrilin -1 (FBN-1) gene mutations screening analysis
Glaucoma was controlled in 97 eyes (74.62%) shows ectopia lentis with secondary glaucoma is
while in 43 eyes (25.48%) it remained uncontrolled and positive as compare to normal control people.
needed additional anti glaucoma medication (Table 5). This type of genetic and molecular understanding
Visual acuity was improved to 6/6-6/12 in 69 eyes provide information for genetic counseling5.
(53.85%), up to 6/36 in 23 eyes (17.69%), up to 3/60 in Glaucoma in ectopia lentis patients has been
12 eyes (9.23%) and perception of light in 26 eyes reported as 41.5%6. Cross and Jerret has reported the
(20%) (Table 6). presence of glaucoma as 25 %7 while in another study
the glaucoma has been reported as 25 % in marfan
Table 4: Management and glaucoma syndrome8. Maumenee reported glaucoma as
129/1000 in patients of ectopia lentis as compared to
[Link] Type of treatment No of eyes n (%)
normal population where it was 40/1000.
A Medical 16 (11.3) The ectopia lentis is mainly associated with
B secondary type of glaucoma although primary angle
Surgical closure and open angle glaucoma has also been
114 (87.69)
• Peripheral/iridectomy reported9.
• 43 (33.08)
• Trabeculectomy
• 18 (13.85) The secondary glaucoma’s are associated or
• Lensectomy
• 30 (23.8) aggravated by angle anomaly in congenital type and
• Phaco Trabeculectomy
• 12 (9.23) traumatic angle recession in traumatic type. No age
• Lensectomy and
• 11 (8.46) and sex is exempted from ectopia lentis and glaucoma.
virectomy
Glaucoma was noticed in 59% in age group of 20-39
years. Ectopia lentis and its induction of gluacoma
Table 5: Glaucoma status appear in second and third decade of life because of
changes in zonules and hperplastic cillary body. This
Glaucoma Status No of eyes n (%) causes forward displacement of iris accompanied by
Controlled 97 (74.62) unequal lens zonuler tension leading to angle closure
or pupillary block glaucoma or it may appear due to
Uncontrolled 33 (25.48) abnormal structure of anterior chamber10.
The crystalline lens is the main culprit causing
Table 6: Visual prognosis and glaucoma raised intraocular pressure particularly when it is
displaced anteriorly, causing pupil block glaucoma.
Vision Eyes n (%) This condition was noticed in 26.92 % of cases.
PL 26 (20) In our study pupilary block glaucoma was highest
followed by angle anomaly and lens in the anterior
Pl + 3/60 12 (9.23) chamber with glaucoma. In study of Harrison et al
Up to 6/60 23 (17.69) angle anomaly and pupillary block were the main
glaucoma variety11.
Up to 6/12 69 (53.08) Ectopia lentis or lens displaced syndrome
associated with glaucoma remains a therapeutic
DISCUSSION challenge for ophthalmologists. If it is of congenital
The crystalline lens is implicated as causative element origin then presence of other ocular condition like
in producing several forms of glaucoma. These include angle anomaly and aniridia or systemic condition like

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homocytinuria further complicate the condition. On therapy. Left eye visual acuity was 6/9 and normal
other hand traumatic variety with hypaema and angle intra ocular pressure13.
recession is also difficult to manage. In polish journal klin oczna it has been reported
Medical treatment may be helpful to control that in 116 eyes of ectopia lentis parsplana lensectomy,
intraocular pressure temporarily in cases like angle vitrectomy and scleral fixation intraocular implant-
closure, open angle or traumatic glaucoma. But tation done. Visual acuity improved in 89% of cases
insuffient to control glaucoma in majority of types,12 while glaucomatous optic atrophy and amblyopia
which need surgical intervention like peripheral were the main cause of decreased visual acuity16.
iredectomy, trabeculectomy/ phacotrabeculectomy, In our study visual acuity improved in 92 eyes
anterior vitrectomy and lensectomy. (70.77%) while in 38 eyes (29.23%) the glaucomatous
In our study 11.3% cases of glaucoma were optic cupping was the main cause of decreased visual
controlled with medication while 88.7 % cases needed acuity. The glaucoma was relieved in 74.62% eyes.
surgical intervention. In 74.62% glaucoma was cured. In conclusion ectopia lentis is very challenging
While 25.48% cases still needed medication. clinical field. Glaucoma association makes it further
Peripheral iredectomy is useful in pupullary block complex. In more than 80 % cases need surgical
glaucoma. If peripheral iredectomy fails, lensectomy intervention like crystalline lens removal plus
with or without intraocular implantation is done. The glaucoma surgery. Even after successful surgery
intraocular pressure becomes normal13. chances of refractory glaucoma is there.
Harrison and his colleagues studied forty five
patients with ectopia lentis in homo cystinuria. Sixty Author’s affiliation
two patients had lens into the anterior chamber or Dr. Bakht Samar Khan
pupil block glaucoma. Eighty-four surgical procedures Assistant Professor
were done. Peripheral iredectomy was not successful Department of ophthalmology
alone. Lensectomy and trabeculectomy were the Khyber Medical College
choice of operation11. Peshawar
In another case angle closure glaucoma with Dr. Zubeda Irshad
progressive myopia due to ectopia lentis was treated Lecturer Girls Medical College
with propyhlactic lensectomy. That proven most Peshawar
effective9.
Prof. Mustafa Iqbal
Kluppel et al operated 23 eyes having decreased Department of ophthalmology
visual acuity and uncontrolled secondary glaucoma. Khyber Medical College
Main surgical procedure was lensectomy and Peshawar
transcleral posterior chamber intraocular lenses
implantation suture technique. Visual acuity
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