Cataract Management in Congenital Coloboma

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CATARACT SESSION - I 71 The purpose of this study was to evaluate the outcome of cataract surgery in congenital coloboma with phacoemulsification with endocapsular ring and foldable intraocular lens implantation. Materials and Methods This is a prospective non-comparative case series of 18 eyes of 18 patients of lens coloboma associated with retinochoroidal coloboma. The patients reported with further progressive diminution of vision attributed to cataract on clinical examination. All these patients had bilateral ocular malformations and no associated systemic disease. The patients were operated in a tertiary eye care centre between July 2001 and June 2007. Examination included visual acuity assessment, detailed anterior segment examination and posterior segment examination where media permitted. Horizontal corneal diameter measurements, intraocular pressure and presence of nystagmus checked in all patients. Biometry was done in all cases. All the cases were done under peribulbar anaesthesia with 2% lignocaine and 0.5% bupivacaine. Surgical technique included phacoemulsification with clear corneal incision opposite to the zone of coloboma. Continuous curvilinear capsulorrhexis was done in all cases. This was followed by hydrodissection and hydrodelineation where possible. The endocapsular ring was inserted through the side port after hydro procedure. Stop and Chop technique of lens nuclear phacoemulsification was done followed by placement of foldable intraocular lens implantation. Patients were reviewed as scheduled in proforma which included visual acuity measurements, refraction and detailed fundus examination to determine posterior extent of coloboma. Follow up ranged from 1 year to 7 years. Results Mean age of 18 cases was 40.16 ± 9.25 years. There were 11 males and 7 females. 12 patients were below 50 years. These eyes had soft nuclear and cortical cataracts including two with total cataracts. 6 patients who were more than 50 years had brown nuclear cataract. Nystagmus was present in 25% patients. There was some difficulty in assessing correct axial length by A scan due to the presence of retinochoroidal coloboma. So B scan was also done for confirming A scan measurements. Unsatisfactory pupil dilatation was seen in 2 cases. Proper dilatation was obtained by injecting viscoelastic into anterior chamber. Size of clear corneal incision ranged from 2.8 to 3.2 mm. Capsulorrhexis was done under sodium hyaluronate and could be completed in all but 1 case. In that case there was difficulty in continuing rhexis near area of absent zonules which necessitated use of capsulotomy scissors to proceed further. Phacoemulsification was uneventful in 17 cases. Foldable intraocular lens was implanted in the bag with an injector after cortical wash. In 1 case there was posterior capsular rent with some vitreous loss. Limited anterior vitrectomy was done with automated vitrector. Scleral fixated lens was implanted in a second sitting after 2 months. The endocapsular ring was explanted in this case in the first sitting. Postoperatively, there was no serious complication. Striate keratopathy was seen in 2 eyes. There was no posterior segment complication either. Visual acuity improved in all cases after 1 month postoperative. Best corrected visual acuity was 6/9 or better in 11 eyes, 6/12 – 6/18 in 5 eyes and 6/24 – 6/60 in 2 eyes. There was well compartmentalization of the eyeball with no vitreous loss in all cases except 1case of posterior capsular rent. Visual outcome was dependent on presence of nystagmus, extent of uveal coloboma posteriorly and pre existing amblyopia. All eyes that had vision better than 6/12 did not have nystagmus or retinochoroidal coloboma extending upto posterior pole. The final vision in all patients was ≥ 6/18, and all these patients were satisfied with this vision as it was apparently close to what they could see prior to developing cataract. It was pleasantly rewarding that they could regain independent mobility and resume most of their daily activities. Discussion Cataract surgery in patients with lens coloboma associated with retinochoroidal coloboma is challenging because of anatomical peculiarities, compromised structural integrity of globe and much higher risk of complications. Surgery requires extra care as every step is potentially
72 AIOC 2009 PROCEEDINGS hazardous. Combined A and B scan evaluation is a must both for axial length measurement and prognostication. The results of this study affirm that clinically significant cataract develops at a younger age in eyes with congenital coloboma than in eyes with typical nuclear sclerotic cataract. Risk of complication is less in younger patients with soft cataracts. The use of endocapsular ring minimizes much of the complication which is likely to be associated with compromised zonules. Phacoemulsification ensures closed chamber technique of cataract surgery and lens implantation enables compartmentalization of the globe. The improved vision in this study indicates that phacoemulsification with endocapsular ring and foldable intraocular lens implantation offers the best option for patients with typical congenital coloboma and cataract. References 1. Yu Y.S, Lee J.H, Chang B.L. Surgical management of congenital cataract associated with severe microphthalmos. J. Cataract Refract Surg 2000; 26: 1219-24. 2. Gimbel H.V, Sun R. Clinical applications of capsular tension rings in cataract surgery. Ophthalmic Surg. Lasers 2002;33:44-53. 3. Jaffe N.S, Clayman H.M. Cataract extraction in eyes with congenital coloboma. J. Cataract Refract Surg. 1987;13:54-8. 4. Nordlund M.L, Sugar A, Moroi S.E. Phacoemulsification and intraocular lens placement in eyes with cataract and congenital coloboma: visual acuity and complications. J. Cataract Refract Surg. 2000;26:1035-40.
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Cataract Management in Congenital Coloboma

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