Dr.Shaifali Chahar

Dr.Santosh G. Honavar

Abstract

Purpose: To describe the ophthalmic and neuro-cutaneous features of nevus sebaceous of Jadassohn (organoid nevus syndrome). Method: Retrospective analysis of 8 patients with oculo-neuro-cutaneous manifestations. Results: Ten eyes were analyzed (8 patients, 2 bilateral, 7 males), with age range of 2-22 years. All patients exhibited typical nevus sebaceous, 1 had hairy nevus. Neurological manifestations were seen in 2 – lipomatosis, arachnoid cyst, dysgenesis of corpus callosum, and seizures. Epibulbar complex choristoma was present in all, confirmed histopathologically in 2. Features included sclerocorneal involvement (1), upper eyelid coloboma (1), ptosis (2), choroidal osteoma (3), optic disc pit (2), sclerochoroidal calcification (1). Conclusion: Nevus sebaceous is a type of phakomatoses with diverse manifestations, requiring thorough systemic examination. Management depends on visual and cosmetic concerns.