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Is it a Coloboma or Not? A new definition for isolated lens coloboma: focal zonular dysgenesis

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First Author: N.Larivoir BRAZIL

Co Author(s):    N. Larivoir   P. Marinho   I. Teixeira   G. Figueiredo   R. Fernandes   L. Freitas     

Abstract Details

Purpose:

The aim of this study is to report the cataract surgical management of 9 eyes of 7 patients presenting isolated lens coloboma, approached by different surgeons in multiple centers. Also, it has the purpose of analyzing the definition and etiopathogenesis of this entity and to review the best surgical management.

Setting:

Ophthalmology Department, Federal University of São Paulo (UNIFESP), São Paulo, Brazil

Methods:

This study was a retrospective analysis of a small case series of seven patients who were diagnosed with “isolated lens coloboma” and underwent phacoemulsification and implant of intraocular lens (IOL) by four different surgeons in the last five years.

Results:

All patients presented progressive decrease in vision acuity (VA), and in only one of them the complaint was unilateral. There was no positive familiar history or any systemic disease. After pupillary dilatation, slit lamp biomicroscopy revealed straight inferior equator of the lens with scanty zonules, without uveal or retinochoroidal coloboma and fundus examination showed normal outcomes. Phacoemulsification and IOL implantation were performed, and the technique was chosen according to the patient's clinical examination and the surgeon’s preference, supported by the available publications. There were no intraoperative complications. Two eyes (22,22%) presented postoperative complications, one of which was the IOL decentralization.

Conclusions:

In conclusion, considering the ocular morphogenesis and the definition of coloboma, we believe that the ideal denomination for the congenital malformation traditionally known as isolated lens coloboma would be “focal zonular dysgenesis”. In order to accomplish stabilization and centration of the IOL, our surgical experience in these rare cases associated with the literature results indicates that the best results are achieved using a standard CTR in defects of less than 120 degrees and Cionni modified-CTR when it is greater than 120 degrees.

Financial Disclosure:

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