A challenging case of refractory childhood glaucoma associated with bilateral congenital ectropion uveae

Abstract

Congenital ectropion uveae (CEU) is a rare disorder, resulting from the proliferation of iris pigment epithelium on the anterior surface of the iris. We report the case of a 3-year-old boy, who was recently diagnosed with glaucoma, on treatment with two topical antiglaucoma medications and referred to us for further evaluation and management. His visual acuity, at presentation, was 6/7.5 (Cardiff) in both his eyes. Slit-lamp examination of both his eyes revealed the presence of ectropion uveae, with intraocular pressure (IOP) of 32 mm Hg. Gonioscopy in both eyes showed anterior insertion of iris. On Fundoscopy, both eyes showed glaucomatous disc changes. He had no systemic associations. Owing to inadequate IOP control, despite optimal medical therapy, he underwent trabeculectomy with intra-operative application of Mitomycin-C. However, the IOP reduction post-trabeculectomy was inadequate, and he subsequently required a bleb needling, which also failed. Hence, he underwent Ahmed glaucoma valve implantation in his right eye, with a favorable final outcome. IOP in the right eye was brought under control following multiple surgical interventions and additional medical treatment. CEU in children can be associated with refractory childhood glaucoma. It is important to evaluate for glaucoma in CEU cases to prevent blindness, especially in young patients and improve their overall prognosis.