DR. AMBER DUBEY

Dr. VIKAS KHETAN

Abstract

LNSS is a rare congenital oculo-neuro-cutaneous syndrome.It consists of a triad of midline facial linear nevus sebaceous, central nervous system and ocular abnormalities.A 10-year old boy, known case of seizure disorder, presented with linear facial and scalp nevus,right-sided congenital cryptophthalmos associated with an upper lid boggy lesion.An epibulbar choristoma was seen in the left eye in the superonasal quadrant.Fundus evaluation showed a subtle peri-papillary scleral cartilage causing yellowish-orange discolouration confirmed on ultrasound as hyperreflective echos in the left eye.Neuroimaging revealed sphenoid dysplasia,thickened cranial nerves and lipochoristoma of right CP angle.The child underwent debulking of lesion in the right eyelid which was confirmed to be a neurofibroma on histopathology followed by a prosthetic shell fit in the socket for better cosmesis.This case elicits the ophthalmic features of LNSS,its association with neurofibromatosis and management.