Abstract

Congenital ectropion uveae is a rare disorder with unilateral anterior chamber dysgenesis that leads to unilateral secondary glaucoma. Few syndromes have been found associated with CEU like NF1,Prader- Willi and facial hemihypertrophy. We have two unrelated cases of congenital ectropion uveae which had different systemic associations. A 26 year old female with visual acuity of 6/6 in left eye and no perception of light in right eye, right divergent squint, raised IOP, mild ptosis and glaucomatous optic atrophy secondary to right sided CEU. Patient underwent gastric surgery for colon cancer 8 years back. Another case is 15-year-old male with visual acuity of 6/6 in right eye and 6/9 in left eye. He presented with similar ocular features and had history of left sided cleft lip and left sided cleft palate surgery done in childhood. Glaucoma management was done by performing trabeculectomy surgery augmented with mitomycin C with no progression in nine months of follow up.