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


METHODS
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.


CONCLUSIONS
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.
Groh
M J
MJ
Augenklinik mit Poliklinik der Universität Erlangen-Nürnberg.
Wenkel
H
H
Mayer
U M
UM
D000328:Adult; D016738:Alagille Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D001284:Atrophy; D005500:Follow-Up Studies; D006801:Humans; D007429:Intraocular Pressure / Q000502:physiology*; D007498:Iris / Q000473:pathology*; D008297:Male; D014792:Visual Acuity / Q000502:physiology*
Alagille-Syndrom (arteriohepatische Dysplasie): Stabile Augenbefunde nach 23 Jahren Krankheitsverlauf.