Abstract

Congenital microphthalmia with intraorbital cyst is rare. Colobomatous cyst stems from defect in closure of embryonic fissure at the time of invagination of optic vesicle, leading to protrusion of neuroectodermal lined cystic mass from the defect in the wall of microphthalmic eye. Clinical presentation usually comprises progressively enlarging orbital cyst with microphthalmia. Enlargement of orbital cyst is attributable to continuous secretions from its wall. Imaging assessment evaluates lesion and its internal characteristics, organ of origin, and associated anomalies. Differentials include microphthalmia with cystic teratoma and meningoencephalocele. Management strategy comprises removal of cystic lesion or aspiration, followed by sclerotherapy with bleomycin injection and ocular expander to maintain orbital volume for prosthesis.

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