DR. NEELIMA KARNE

DR.VIJAY PRAKASH KINIKAR

Abstract

PURPOSE:To study the ocular and systemic manifestations of Gorlin-goltz syndrome.

METHODS:A 30year old female with odontogenic keratocysts(OKCs) was referred to ophthalmologyOPD for complete ocular examination.patients history, BCVA, IOP, Slitlamp biomicroscopy, dilated fundus examination,biopsy and imaging was done.

RESULTS:O/E -multiple basal cell naevi in periocular region and scattered over face, Hypertelorism with wide nasal bridge,plantar and palmar pits were noted.A/S -pciol(RE),cataract( LE), IOP WNL.Fundus-myelinated optic nerve fibre layer in BE’s with macular pucker RE. Imaging revealed bifid rib and calcification of falx cerebri.

CONCLUSION:presence of 2major signs(>10basal cell naevi,OKCs) 1minor sign(hypertelorism) confirmed the diagnosis as GGS.It is important to make early diagnosis as case presents malignant predisposition.Ophthalmologists has a role in multidisciplinary team in management and timely therapy could improve visual prognosis of these patients.