DR. ARKAPRAVA PRADHAN

DR. JYOTIRMAY BISWAS

Abstract

A young male with achondroplasia was having exudative RD. Visual acuity in the left eye was 6/60. FAF showed leopard spot pattern. FFA and ICGA showed no leakage of the dye. USG B scan showed axial length of 20.40 mm and 20.90 mm and normal scleral thickness. The diagnosis of VKH was made elsewhere, 3 doses of intravenous methylprednisolone were given but there was no improvement. On UBM, there was shallow anterior chamber with supraciliary effusion in both eyes. PET CT was normal. Diagnosis of type 3 UES was made and sub Tenon injection of steroid was given in both eyes. Patient responded well. Surgical decompression of the vortex veins is the treatment of choice in uveal effusion related to nanophthalmos or scleral wall abnormalities. Type 3 UES typically doesn’t respond to scleral windows but often responds to oral and periocular steroids. The concurrence of UES in achondroplasia could be due to collagen abnormality raising suspicion of the genetic basis of this rare disorder.