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Year : 2023  |  Volume : 5  |  Issue : 1  |  Page : 16

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

1 Department of Ophthalmology, Federal University of Juiz de Fora, MG, Brazil
2 Vision Institute, IPEPO; Department of Ophthalmology, Federal University of Sao Paulo, Brazil
3 Department of Ophthalmology, Santa Casa de Sao Paulo; Sao Paulo Eye Hospital, H.Olhos, SP, Brazil
4 D'Olhos Day Hospital, SP, Brazil
5 Department of Ophthalmology, Federal University of Sao Paulo, SP, Brazil

Correspondence Address:
Nicole B Larivoir
MD, Rua Pedro Scapim 165/202, São Matheus, Juiz de Fora, Minas Gerais
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_15_23

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Purpose: Ocular coloboma is defined as a congenital malformation caused by defective closure of the fetal fissure, resulting in a notch in any structure. However, the entity known as “isolated lens coloboma” does not seem to have a similar etiological basis with other ocular colobomas. This congenital malformation can induce an important decrease of the visual acuity, and facectomy has an increased risk for intraoperative and postoperative complications. Better surgical planning could be achieved with an accurate definition of the diagnosis and pathophysiology of this lens abnormality, as the extent and mechanism of zonular disease will have a significant impact on the selection of the modality of intervention. Methods: Nine eyes of seven patients, who were diagnosed with “isolated lens coloboma” and underwent phacoemulsification and implant of intraocular lens (IOL), were studied (retrospective case series). The surgeries were performed by four different surgeons, according to their preferred technique, in the last 5 years. Results: There were no intraoperative complications. Two (22.22%) eyes presented postoperative complications, one of which was the IOL decentralization. Conclusion: Considering the ocular morphogenesis and the definition of coloboma, the ideal denomination for the congenital malformation traditionally known as isolated lens coloboma would be “focal zonular dysgenesis.” The best surgical approach, contemplating the pathophysiology, in addition to the cases described and review of literature, is discussed.

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