Dr. Ayesha Musthafa

Dr. Ravi Bypareddy, Dr.Sanjana Vinod

Abstract

STUDY DESIGN:Case report
PURPOSE:To report a case of weill-Marchesani syndrome
METHODS:A 42 year old female presented with dimunition of vision since 3 years along with history of stiff joints in fingers since 3 years.
Results:on ocular examination patient had visual acuity of counting fingers at 1/2m in RE and counting fingers close to face in LE,the iop was 18mmhg and 25 mmhg measured with GAT,gonioscopy showed angle of grade 2(shaffers grading),the lens in both the eyes showed Inferotemporal subluxation with cataract of ns1 in RE and ns3 in LE,fundus had CDR of 0.7 in both eyes.On general examination:patient was short in stature and brachydactyly was present.Patient underwent anterior vitrectomy with iris claw lens,surgery was uneventful,post operative vision after 2 weeks was 6/24.
Conclusion:weil marchesani syndrome an inherited connective tissue disorder should be considered in patients with short stature,microsperophakia,subluxated lens with features of secondary glaucoma.