Klin Monatsbl Augenheilkd

[Alagille syndrome (arteriohepatic dysplasia): stable ocular findings after 23 years of illness]

Year 1998
Groh MJ. Wenkel H. Mayer UM.
Augenklinik mit Poliklinik der Universitat Erlangen-Nurnberg.
BACKGROUND: Alagille syndrome is a arteriohepatic dysplasia which is in most cases correlated with ocular disorders. The most common ocular defect is a posterior embryontoxon. PATIENT: We report on a 27-year-old patient suffering from Alagille syndrome, seen in our department for 23 years. Within these 23 years the ophthalmological status concerning visual acuity and intraocular pressure of the patients was stable. Iris-stroma-atrophy showed a small increase on both eyes. CONCLUSION: In comparison to patients with Axenfeld anomaly a development of glaucoma is not observed in these patients. Maybe the anomalies of the anterior chamber angle are less severe or different from those in Axenfeld anomaly. The disease is also correlated with hepato- or/and splenomegaly by biliary hypoplasia, cardiac disorders etc. Cooperation of paediatricians, internal specialists and ophthalmologists are necessary for the treatment of these patients.