Aniridic Glaucoma: Diagnosis and Treatment

Aniridia is a bilateral iris aplasia/hypoplasia, associated with other ocular anomalies arising during the childhood: nystagmus, photophobia, amblyopia, keratopathies, cataract and lens luxation, glaucoma, fovea and optic nerve hypoplasia. In 6–75 % of cases aniridia is accompanied by a dysgenetic secondary glaucoma caused by an iridogoniodysgenesis for abnormal migration of neural crest neuroectodermal cells, and a higher vulnerability of the optic nerve head for possible microstructural alterations in lamina cribrosa. Congenital glaucoma associated with aniridia is uncommon. The poor young patient collaboration for several clinical and instrumental analyses entails in many cases the need of examinations under general anesthesia. Medical therapy represents the first step, whereas low-responsive patients may undergo laser treatments (transscleral diode laser cyclophotocoagulation or cyclocryotherapy) and/or surgery (trabeculectomy with or without antimetabolites). Refractory cases, frequently with an early onset, require glaucoma drainage devices (Molteno implant, Ahmed valve, or Baerveldt tube shunt). A prophylactic goniotomy can be performed with a long-term effectiveness in reducing risks of aniridic glaucoma onset or progression.