Aniridic Fibrosis Syndrome in a Child with Ahmed Glaucoma Valve: Report of a Case and Review of the Literature

Purpose: To report a case of aniridic fibrosis syndrome (AFS) after Ahmed glaucoma valve (AGV) surgery. Methods: Case report. Results: Two years post-AGV surgery in both eyes, a 3-year-old aniridic female presented with grayish discoloration of the right eye for the past 3 months. A slit-lamp examination of the right eye revealed an aniridic, hypotonic eye with a totally edematous cornea. A tube of the AGV was in place with a mature cataract subluxed superiorly. In addition, a thick, whitish vascularized membrane originating from the rudimentary iris to the inferior part of the subluxed lens was observed. Ultrasound biomicroscopy investigation confirmed a thick fibrotic membrane originating from the iris root and extending to the posterior part of the cataractous lens. The intraocular pressure of the right and left eye was 0 and 11 mmHg, respectively. A B-scan of the right eye revealed an axial length of 21 mm, optic disc swelling, and shallow choroidal detachment in the anterior 2/3 of the posterior segment without retinal detachment. With the diagnosis of AFS, a pars plana vitrectomy, lensectomy, and membranectomy under keratoprosthesis and penetrating keratoplasty were planned. Conclusions: Aniridic patients may undergo multiple ocular surgeries during their lifetime. One of the rarest surgical complications in these patients is AFS. Early diagnosis can be possible with serial slit-lamp examination and ultrasonographic studies to detect the disease in the early stages and prompt intervention. Here, in our case, the parents declined any surgical intervention.