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Pejman Bakhtiari, Jeffry Welder, Clara Chan, Jose De la Cruz, Edward J. Holland, Ali R. Djalilian; Surgical and Visual Outcomes of the Type 1 Boston Keratoprosthesis for the Management of Aniridic Fibrosis Syndrome in Congenital Aniridia. Invest. Ophthalmol. Vis. Sci. 2011;52(14):332.
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To report the clinical and histopathological features of aniridic fibrosis syndrome along with outcomes of surgical intervention using the Type 1 Boston Keratoprosthesis (Kpro) in this subset of aniridic patients.
Retrospective chart review of 9 eyes in 9 patients with congenital aniridia that developed aniridic fibrosis syndrome and underwent a type 1 Boston Kpro.
All patients had PAX6 related congenital aniridia and had previously undergone cataract surgery with posterior chamber intraocular lens (PCIOL) and seven patients had existing tube shunts. Seven had previous corneal transplantation, seven had previous keratolimbal allograft, one had an existing Boston Kpro and one had an existing Alphacor Kpro. In all cases, fibrosis presented as progressive retrocorneal and retrolenticular membrane formation causing displacement of the IOL and corneal decompensation. Two had tractional folds in the retina with posterior extension of the membrane. The management in all cases included IOL explantation and removal of fibrosis with anterior vitrectomy (one patient) or pars plana vitrectomy (eight patients). In all nine patients, the Type 1 Boston Kpro was implanted. At a mean final follow-up of 25.8 months (range 3 to 48 months), vision remained improved in all patients. No patient had recurrence of the fibrotic membrane after KPro implantation. Histopathology revealed dense hypocellular fibrous connective tissue with mild acute and chronic inflammation.
This study represents the largest study to report utilization of the Type 1 Boston Kpro in such patients. As the membrane can cause IOL dislocation, corneal decompensation, hypotony and retinal detachment, monitoring for aniridic fibrosis syndrome in congenital aniridia with early surgical intervention is recommended. We found the Type 1 Boston Kpro to be safe and effective for the treatment of this condition.
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