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Bilateral asymmetrical isolated lens coloboma with persistent pupillary membrane

Ramasubramanian, Aparna MD, MBBS; Sukhija, Jaspreet S. MD; Ram, Jagat MD; Das, Pranab MD; Radotra, Bishan D. MD

Journal of Cataract & Refractive Surgery: December 2007 - Volume 33 - Issue 12 - p 2153-2155
doi: 10.1016/j.jcrs.2007.07.042
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A 12-year-old girl presented with a bilateral decrease in vision. The best corrected visual acuity (BCVA) was 6/60 in both eyes with −10.0 diopter (D) correction, and the axial length was 27.59 mm. Ocular examination showed superior notching of the crystalline lens in the right eye and nasal notching in the left eye (Figure 1). In both eyes, the anterior capsule showed pigmentation simulating sago grain (Figure 2).There was bilateral posterior subcapsular cataract but no anisocoria, corectopia, or iris or ciliary coloboma. No associated retinochoroidal coloboma was seen (Figure 3). There was no positive family history or evidence of any systemic disease. Preoperatively, the refraction was −11.0 −3.50 × 125 in the right eye and −10.0 −3.50 × 90 in the left eye.

Figure 1
Figure 1:
A: Slitlamp photograph of dilated right eye on retroillumination showing notching of the lens equator superiorly with absence of zonules in the corresponding area. B: Slitlamp photograph of dilated left eye on retroillumination showing notching of the lens equator nasally with absence of zonules in the corresponding area.
Figure 2
Figure 2:
A: Fine pigment deposit in the form of sago grain on the anterior lens capsule in the left eye. Also seen is the lens coloboma. Inset shows magnified view of the pigment clumps, which appear like stacked cells, one above the other. B: Fine pigment deposit on the anterior lens capsule in the right eye. The appearance is similar to that in the left eye.
Figure 3
Figure 3:
A: Fundus photograph of right eye shows no retinochoroidal coloboma. B: Fundus photograph of left eye shows no gross pathology.

Phacoemulsification and posterior chamber intraocular lens (IOL) implantation were performed in both eyes with the aid of a capsular tension ring (CTR). The surgery was accomplished using 4 iris hooks beneath the anterior capsule margin to stabilize the capsule (Figure 4). A CTR with a 12.0 mm diameter was inserted into the capsular bag, followed by phacoaspiration and IOL implantation in the bag. The iris hooks were removed at the end of surgery. Postoperatively, the refraction was +2.25 −4.0D × 180 in the right eye and +1.75 −3.25 × 160 in the left eye and the BCVA was 20/200 in both eyes. The patient was amblyopic but did not have any tropia. Electron microscopy of the anterior capsule was consistent with the diagnosis of persistent pupillary membrane on the lens capsule (Figure 5).

Figure 4
Figure 4:
The capsular bag after phacoaspiration. Note the silicone iris hooks applied to the continuous curvilinear capsulorhexis in the area of the coloboma to stabilize the capsular bag during surgery.
Figure 5
Figure 5:
A: Electron microscopy photograph of the anterior capsule shows the presence of cuboidal epithelial cells (top). Also present is a cell with melanin pigment derived from an iris melanocyte (bottom), suggestive of persistent pupillary membrane type 1. B: Magnified single cell showing numerous dispersed melanin pigments suggestive of an iris pigment epithelium origin.

Coloboma of the lens is usually unilateral.1 It is typically located inferiorly, and if bilateral, is symmetrical.1,2 Our patient had asymmetrical lens colobomas, highlighting a rare presentation.


1. Aggarwal A, El-Bash A-R, Inker S, Musarella MA. Symmetrical bilateral lens colobomas in two brothers. J Pediatr Ophthalmol Strabismus. 2004;41:302-304.
2. Angra SK, Gupta S, Dada VK, Gupta AK. Coloboma of the lens. Indian J Ophthalmol. 1984;32:21-22.
© 2007 by Lippincott Williams & Wilkins, Inc.