We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
A Rare Case With Aniridic Fibrosis Syndrome After Iris Diaphragm Intraocular Lens and Ahmed Valve Implantation in a Patient With WAGR Syndrome.
- Authors
EKŞİOĞLU, Ümit; YAKIN, Mehmet; ACAR, Mehmet; DUMAN, Melih; ÖRNEK, Firdevs
- Abstract
Congenital aniridia is a rare, bilateral condition associated with foveal hypoplasia, nystagmus, cataract, glaucoma, keratopathy, and dry eye. Aniridic fibrosis syndrome is a newly recognized clinical entity characterized by progressive retrocorneal, cyclitic, or retrolenticular fibrosis which can lead to corneal endothelial decompensation, hypotony, and retinal detachment. A 6-year-old male presented with WAGR syndrome. At presentation he had mental retardation, nystagmus, bilateral rudimentary iris, and bilateral posterior polar cataract. Cataract extraction and implantation of an aniridia intraocular lens (IOL) was performed in the right eye. Glaucoma developed in the right eye approximately 2 months after cataract surgery. An Ahmed glaucoma valve was implanted in the right eye and prophylactic goniotomy was performed in the left eye. Nine months after glaucoma surgeries aniridia-associated keratopathy (AAK) was observed in both eyes (predominantly in the right eye). Keratopathy progressed during follow-up. At the 6th year follow-up aniridic fibrosis syndrome was detected in the right eye, with retrocorneal fibrotic membrane formation, inferior dislocation of the IOL, and endothelial decompensation. During the 6-years follow-up, intraocular pressure was controlled with 2 medications in the right and without medication in the left eye. The management of aniridia is often quite difficult because of the complex involvement of ocular structures. Although satisfactory results can be obtained with surgery for cataract and glaucoma, progression of AAK and development of aniridic fibrosis syndrome (especially in eyes with intraocular hardware) are the obstacles for good visual outcome. This is the first case of aniridic fibrosis syndrome reported from Turkey.
- Subjects
FIBROSIS; WAGR syndrome; CONGENITAL disorders; INTRAOCULAR lens complications; ARTIFICIAL implants; THERAPEUTICS
- Publication
Journal of Glaucoma-Cataract / Glokom-Katarakt, 2017, Vol 12, Issue 3, p233
- ISSN
1305-9173
- Publication type
Case Study