Ophthalmic manifestations of linear nevus sebaceous/organoid nevus syndrome.

Linear Nevus Sebaceous Syndrome (LNSS) is a rare sporadic oculoneurocutaneous disorder, also classified as Organoid Nevus Syndrome. It consists of a triad of midline facial linear nevus sebaceous, central nervous system and ocular abnormalities. To the best of authors' knowledge ophthalmic features of LNSS have never been reported in Pakistani population. We report two cases of LNSS, associated with multiple cutaneous nevus sebaceous lesions, complex ocular choristomas and rare bilateral presentation in one patient. Ocular choristomas included limbal dermoids, dermolipomas at superior fornices and chroidal choristoma. Ocular surface was successfully reconstructed by excision of limbal dermoids, partial keratectomy and amniotic membrane transplant.