BACKGROUND: Axenfeld-Rieger Syndrome represents a group of developmental disorders involving ocular and extraocular anomalies. Axenfeld’s anomaly is a clinical variation within this spectrum of disorders in which the defects are limited to the peripheral anterior segment. Glaucoma is often an association.
CASE REPORT(S). A 67-year-old male presented to the Bascom Palmer Eye Institute complaining of decreased vision in the right eye for three months. The patient reported a history of a "stroke" in the right eye in 1991 and was treated with laser. Visual acuity was 20/50 OD and 20/25 OS. Pupils and external examination were unremarkable. Intraocular pressures were 22 mmHg OD and 32 mmHg OS. Prominent anteriorly displaced Schwalbe’s lines were present 360 degrees OU. Gonioscopy revealed multiple peripheral iridocorneal adhesions OU. Dilated fundus examination of the right eye showed diffuse retinal hemorrhages throughout the superior hemisphere, panretinal photocoagulation scars in the inferior retina, and optociliary shunt vessels. Examination of the left eye revealed a larger C/D ratio with vertical elongation of the cup. Visual fields demonstrated a superior altitudinal defect OD, and generalized depression OS. A diagnosis of superior hemi-retinal vein occlusion OD and old inferior hemi-retinal vein occlusion status-post PRP OS was made. In addition, Axenfeld’s anomaly and glaucoma were diagnosed. The patient was treated with Xalatan daily OU, and one month later the IOP’s had decreased to 11 mmHg OD and 15 mmHg OS.
CONCLUSIONS. This case represents an interesting presentation of Axenfeld’s anomaly associated with a unilateral recurrent hemi-retinal vein occlusion. Elevated intraocular pressure is a known risk factor for retinal vein occlusions, therefore Axenfeld’s anomaly may have indirectly contributed to the pathogenesis of the hemi-retinal vein occlusions in this patient. This case illustrates an atypical late presentation of glaucoma at the age of 67 associated with Axenfeld’s anomaly. This poster will illustrate the ocular photographs and visual fields of this patient, and discuss the clinical spectrum of Axenfeld-Rieger Syndrome.