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.