Dr.kavitha cr

Dr. KAMALA SUBRAMANIAN

Abstract

Hallermann-Streiff syndrome(HSS) is a rare genetic disorder characterized by distinct cranio-facial abnormalities with a prevalence of less than 1/1,000,000 people. We reporting a case of HSS with glaucoma, as only a small minority of HSS reported to have glaucoma. 25yrs old male presented with blurred vision both eyes(BE) since few days and it was a preterm normal delivery with delayed milestones. Best corrected vision in BE was 6/12 and refraction shows high myopia. Anterior segment- micro cornea, intra ocular pressure(IOP) was 40 and 42mmhg in right and left eye respectively, gonio-open angles, fundus- tilted discs. Head to toe examination- sparse scalp and facial hairs, beaked nose, hypoplastic mandible, hypodontia, short stature and syndactyly of both hands. started on glaucoma medications and IOP was stable. Raised IOP in HSS may be due to developmental anomalies of the angle and inflammation sequelae. medical treatment can be more advantageous over surgical management.