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P. Bakhtiari, A. Djalilian; Postsurgical Progressive Aniridia Fibrosis Syndrome in Patients With Congenital Aniridia. Invest. Ophthalmol. Vis. Sci. 2010;51(13):1137.
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To report the clinical features and histopathology of patients with aniridia fibrosis syndrome.
A retrospective chart review of 4 patients with congenital aniridia who developed aniridia fibrosis syndrome at University of Illinois Eye and Ear Infirmary. Histological examination of excised inflammatory membrane was performed in one eye.
All patients had PAX6 related congenital aniridia. One patient actually had WAGR syndrome. The mean age was 58 (11-68). All patients had previously undergone cataract surgery and 3 of the 4 patients had existing tube shunts. Two had undergone corneal transplantation and keratolimbal allograft and one patient had an existing Boston Keratoprosthesis. In all eyes, the fibrosis syndrome presented as progressive retrocorneal and retrolenticular membrane formation that caused displacement of intraocular lenses and endothelial cells damage. Two patients had tractional folds in the retina due to posterior extension of the membrane. The management in all cases included IOL explantation with removal of fibrosis with anterior vitrectomy (2 cases) and pars plana vitrectomy (2 cases). In three of the four cases a type I Boston Kpro was implanted. The visual outcome in one patient was poor after the patient developed a suprachoroidal hemorrhage, otherwise the other three patient had good visual outcome. Histopathology revealed dense fibrous connective tissue with mild acute and chronic inflammation
Aniridic fibrosis syndrome is characterized by the development of progresive fibrosis in anterior chamber typically after multiple intraocular procedures that may extend up to ciliary process and anterior retina. Irritation of immature blood vessels in the rudimentary iris in the setting of inflammation and fibrosis may play a role in its development. Monitoring for aniridic fibrosis syndrome in congenital aniridia patients and early surgical intervention to limit intraocular damage is recommended.
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