Massive retinal gliosis in an infant microphthalmic globe: A case report

Abstract

Objective: Background: Case Report: Conclusions: Congenital defects/diseases Massive retinal gliosis (MRG) is a rare benign intraocular tumor that results from the proliferation of well-dif-ferentiated glial cells in response to long-standing pathological processes, including glaucoma, trauma, chron-ic inflammation, vascular disorders, and congenital anomalies. This lesion is considered to be nonneoplastiand occurs ≥10 years after the predisposing insult. It usually affects children and can mimic other condi-tions, including uveal melanomas, vasoproliferative tumors of the retina, astrocytic hamartomas, and retinahemangioblastomas. We present a case of infant MRG with severe left eye microphthalmia. An 11-month-old boy was presented bhis parents in the Oculoplastic Unit of a teaching university hospital with bilateral incomplete cryptophthal-mos and small globes. An enucleation of the left globe was carried out to stimulate orbital bone growth and to improve the cosmetic outcome. The histopathological examination revealed a microphthalmic globe with sclerocornea and disorganized intraocular anterior segment structures. The retina was dysplastic with prolif-erating spindle-shaped glial cells showing fibrillar eosinophilic cytoplasm and filled most of the vitreous cav-ity. The glial origin of the cells was confirmed by the immunohistochemical markers (glial fibrillary acidic protein and synaptophysin), supporting the diagnosis of MRG. The optic nerve was markedly hypoplastic. MRG is a rare intraocular tumor that is clinically difficult to diagnose. A definite diagnosis can be made onlon the basis of a histopathological examination and immunohistochemical markers.