Severe Eyelid Coloboma and Related Ocular Surface Disorders in Amniotic Band Syndrome

Introduction

Amniotic band syndrome (ABS) is an extremely rare fetal congenital malformation in which fibrous amniotic bands occur and strangulate fetusʼs organs, causing clefts and amputations of the affected organs [1]. Two main pathogenic mechanisms are proposed for its development. The first, the exogenous theory, suggests that early amniotic rupture leads to the formation of fibrous bands that encircle the fetal body. The second, the endogenous theory, attributes the condition to a vascular origin, rejecting the idea that mesoblastic strings are a causal factor. Amniotic band syndrome can cause a variety of orbital and eyelid malformations, including eyelid colobomas and facial clefts. These malformations can be severe, highly variable, and do not follow typical embryological development patterns [2], [3]. The most common ocular problems are congenital or acquired corneal leukomas, which occur secondary to ocular surface exposure caused by eyelid colobomas [4]. The goal of treatment is to restore eyelid anatomy while promoting the healing of the ocular surface. Although a definitive surgical method for treating these eyelid malformations has not been established, numerous reconstructive procedures are available, with the choice depending on the size of the defect [5], [6].

Here, we present a newborn diagnosed with ABS and severe eyelid coloboma, complicated by ocular surface problems, and discuss the management of this challenging case.

Publication History

Received: 04 October 2024

Accepted: 26 September 2025

Article published online:
07 November 2025

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