OPHTHALMIC FEATURES OF ALAGILLE SYNDROME (ARTERIOHEPATIC DYSPLASIA)

Eight patients with Alagille syndrome (AGS) are reported. In addition to previously reported findings of posterior embryotoxon, pigmentary r etinopathy, and choroidal folds, new findings include decreased axial eye lengths, small corneal diameters, and shallow anterior chambers. O ptic disc swelling was noted ophthalmoscopically and abnormally increa sed orbital subarachnoidal fluid was detected through measurements of the arachnoidal diameters with standardized echography.