PURPOSE
To characterize the ophthalmological findings, assess surgical outcomes,
and review visual outcomes in aniridia.
METHODS
A retrospective case review was performed and data were collected,
including patient demographics, incidence of aniridia-associated keratopathy,
glaucoma, cataract, retinal breaks or detachments, optic nerve hypoplasia,
macular hypoplasia, poor vision, and nystagmus. All outcomes from surgery,
including penetrating keratoplasty, trabeculectomy, Ahmed valve insertion, and
cataract extraction, were recorded.
RESULTS
Six children (12 eyes) had corneal abnormalities, 4 had optic nerve
hypoplasia, 9 had nystagmus, and 2 had retinal detachments. Four patients (7
eyes) required penetrating keratoplasty. Five patients (9 eyes) developed
glaucoma and only 1 of the 4 trabeculectomies performed succeeded. Of the 6
Ahmed valve procedures performed, all succeeded in maintaining a satisfactory
intraocular pressure but some required needling and 5-fluorouracil. Eight
patients developed cataract and 7 required surgery. Visual outcomes were poor
despite treatment. Nine patients had Snellen acuity of 6/60 or less and
required low visual aids to function.
CONCLUSION
Aniridia is a disorder that requires multiple surgeries. It has a poor
visual prognosis despite early diagnosis and aggressive management. Newer
techniques such as Ahmed valves and Boston keratoprostheses offer hope, but its
proliferative nature makes treatment difficult.