Ocular diversity of damage in patients with linear nevus sebaceous syndrome

Abstract

Objective To report a variety of ocular manifestations in patients with linear nevus sebaceous syndrome.Methods The clinical,pathological and imaging findings were retrospectively reviewed in 6 patients with linear nevus sebaceous syndrome who were seen by the corresponding author in Zhongshan Ophthalmic Center,Sun Yat-sen University,between Feb.2006 and Dec.2009.Results Among the 6 cases(6 eyes),3 were male and 3 were female.The patients'age ranged from 4 months and 12 years old,with a mean of 4.4 years.Two were the right eye and 4 the left eye.Only one of the six cases suffered from seizures and mental retardation.All patients had the sebaceous nevus located at the scalp,the face and neck on the same side of the involved eye.The computed tomography(CT)scan of the brain showed unilateral or bilateral brain dysplasia and atrophy in all patients.All 6 patients had upper eyelid tumor,corneal/conjunctival dermoid,and a different degree of calcification of the eyewall on CT scan.Other ocular abnormalities included complete ectopia of large lacrimal gland,upper eyelid ectropion,lagophthalmos,ptosis,blepharophimosis,microphthalmia,the absence of inferior lacrimal punctum,strabismus,conjunctival epithelial hyperplasia,corneal opacity,pale optic disc,choroidal hemangioma,optic nerve hypoplasia and so on.Conclusions Patients with linear nevus sebaceous syndrome have a variety of ocular abnormalities.Upper eyelid tumor and corneal/conjunctival dermoid are the most common,ocular computed tomography scan show a different degree of calcification of the eyewall. Key words: Linear nevus sebaceous syndrome; Seizures; Mental retardation; Corneal and conjunctival dermoid; Sebaceous nevus