Axenfeld-Rieger Syndrome And Cataract Surgery: A Case Report

To report a case of a patient with bilateral Axenfeld-Rieger syndrome and glaucomatous neuropathy and the surgical planning and to cataract surgery

Axenfeld-Rieger syndrome is a rare anterior segment dysgenesis. Commonly subdivided in axefeld anomaly (posterior embriotoxon); Rieger anomaly includes pupillary irregularity and defects. If several systemic features are present the Riger syndrome is diagnosed.

A 47-year-old man as referred for suspected glaucomatous neuropathy and cataract. Main complaint was symmetric progressive visual acuity decline. No relevant ocular or systemic history was known. Family history was forthcoming for axenfeld-rieger syndrome on a patient’s son. On observation he had best corrected visual acuity of 20/50 OD and 20/200 OS. Pupillary light reflexes were normal with no RAPD. Ocular motility was preserved. Autorefraction was +10D in the OD and unable to measure in the OS. Anterior segment examination revealed posterior embryotoxon and inferiorly anterior synechiae and grade 2 corticonuclear cataracts. No iris defects were observed. Goldmann applanation tonometry was 18mmHg in the OD and 20mmHg in the OS. Fundus examination revealed optic disk pallor and disk drusen. No macular abnormalities were appreciated. Optic coherence tomography and autofluorescence confirmed optic disk drusen and retinal nerve fiber layer loss. Biometry revealed a 20 mm axial length and anterior chamber depth of 2.2mm. Visual fied testing was ordered to assess the degree of glaucomatous neuropathy. After a thorough discussion on the risks and benefits, the patient accepted phacoemulsification. A monofocal (due to glaucoma) intraocular lens was choses with +38D. Phacoemulsification was performed under topical anesthesia and previous vitreous dehydration with mannitol. The surgical procedure and postoperative were uneventful.

Axenfeld-rieger syndrome is a rare anterior segment dysgenesis. It is frequently associated with glaucoma and hyperopia. Cataract surgery in these patients needs to be carefully planned as, commonly, the anterior chamber is shallow and axial length is, usually, small which increases the risk if complications. Uveal effusion is the most dreaded complication and vitreous dehydration and/or prophylactic sclerostomy are helpful in these scenarios. Nevertheless, phacoemulsification allows good visual and safety outcomes.