ALAGILLE-SYNDROME (ARTERIOHEPATIC DYSPLAS IA) - FOLLOW-UP OF 23 YEARSOF STABLE OCULAR FINDINGS

Background Alagille syndrome is a arteriohepatic dysplasia which is in most cases correlated with ocular disorders. The most common ocular d efect is a posterior embryontoxon. Patient We report on a 27-year-old patient suffering from Alagille syndrome, seen in our department for 2 3 years. Within these 23 years the ophthalmological status concerning visual acuity and intraocular pressure of the patients was stable. Iri s-stroma-atrophy showed a small increase on both eyes. Conclusion In c omparision to patients with Axenfeld anomaly a development of glaucoma is not observed in these patients. Maybe the anomalies of the anterio r chamber angle are less severe or different from those in Axenfeld an omaly. The disease is also correlated with hepato- or/and splenomegali e by biliary hypoplasia, cardiac disorders etc. Cooperation of paediat ricians, internal specialists and ophthalmologists are necessary for t he treatment of these patients.