SAN DIEGO — Ocular surface stem cell transplant and implantation of a Boston type 1 keratoprosthesis are beneficial treatment options for aniridia, according to a speaker here.
“Aniridia is due to mutations of the PAX6 gene on chromosome 11. This can be an isolation or a systemic association,” Clara Chan, MD, FRCSC, FACS, said at the World Cornea Congress.
Aniridia can result in an affected cornea, iris deformities, cataracts, glaucoma, optic nerve hypoplasia and retinal detachment. Other complications of aniridia include deficient limbal stem cells, recurring erosions, ulcerations, pain, photophobia, blindness due to corneal scarring and primary grafts failure, Chan said.
“Timing of the surgery may require management of other ocular problems first,” Chan said.
Chan recommended ocular surface stem cell transplant for younger, healthier patients and KPro implantation for older patients.
Advantages of stem cell transplant include less follow-up once the surface has stabilized, resolution of surface complications and easily monitored IOP.
Advantages of the KPro include a simple operation with no concerns about astigmatism, no requirement for immunosuppression and no visual effect through the clear optical zone with a poor tear film. Disadvantages include inability to measure IOP accurately, lifelong close follow-up as well as topical antibiotics, possible permanent vision loss due to complications and possible problems in severe dry eye cases, Chan said.
“A multi-subspecialty approach is required to manage this challenging panocular disorder, and patients should be monitored for aniridic fibrosis syndrome after any intraocular surgery,” Chan said. “It is important to promptly remove all intraocular hardware to prevent the condition from worsening to a point where the patient develops hypotony.” - by Nhu Te
Disclosure: Chan reports no relevant financial disclosures.