Microphthalmia with pseudogliomatosis proliferation in the retina and orbital cyst

Abstract

A 28-year-old systemically healthy male presented with a history of decreased vision in the right eye and a blind, undersized left eye since birth. On evaluation, the patient had typical inferonasal iris coloboma and retinochoroidal coloboma in the right eye and left disorganized microphthalmic globe with colobomatous cyst and contracted socket. The patient underwent left eye injection of 0.25 ml ethanolamine oleate injected into the cyst after aspiration of the cyst contents. The patient had persistent colobomatous cyst, for which he underwent left eye excision of the microphthalmic globe with orbital cyst. Histopathological examination revealed thickened cyst wall, multiple nodules of dysplastic retinal tissues without proper lamination and extensive surrounding fibrosis, deposition of eosinophilic material around the blood vessels and retina, and multiple foci of calcification without atypia and mitosis. The diagnosis of a unilateral microphthalmic phthisical eye with pseudogliomatosis proliferation in the retina and in the orbital cyst was made.

Keywords

• colobomatous cyst
• microphthalmia
• pseudogliomatosis proliferation