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Abstract #15444 Published in IGR 1-3
Anomalies associated with Axenfeld-Rieger syndrome
Ozeki H; Shirai S; Ikeda K; Ogura YGraefe's Archive for Clinical and Experimental Ophthalmology 1999; 237: 730-734
BACKGROUND: To detect the associated anomalies in patients with Axenfeld-Rieger syndrome is clinically important, because early treatment of such anomalies is crucial to both visual and systemic development. This study was conducted to clarify the associated anomalies in the syndrome. METHODS: The authors evaluated 21 patients with Axenfeld-Rieger syndrome encountered at Nagoya City University Hospital over a 16-year period. Patients who presented with a prominent Schwalbe's line accompanying the iris strands were diagnosed as having Axenfeld-Rieger syndrome. RESULTS: The series consisted of nine males and 12 females, ranging in age from one month to 41 years, mean 15.4±12.7 (SD) years. The syndrome was bilateral in 17 cases and unilateral in four. Hypoplasia of the iris was observed in ten eyes of six patients. The associated ocular anomalies included sclerocornea in six eyes of three patients, developmental glaucoma in five eyes of three patients, persistent pupillary membrane in four eyes of two patients, microphthalmos in three eyes of two patients, and typical iris coloboma in one eye. Of ten eyes with hypoplasia of the iris, five exhibited glaucoma. The accompanying systemic anomalies included nine cases of dental anomalies, five of facial anomalies, and three of Alagille syndrome. CONCLUSIONS: All the associated ocular and systemic anomalies appeared to arise from the maldevelopment of the neural crest cells. Patients with Axenfeld-Rieger syndrome should therefore be examined for the presence of anomalies in the tissues of neural crest origin. Patients with hypoplasia of the iris should be checked for glaucoma.
Dr. H. Ozeki, Department of Ophthalmology, Nagoya City University Medical School, 1-Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601; Japan
Classification:
9.1.3 Syndromes of Axenfeld, Rieger, Peters, aniridia (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
