Alagille syndrome (AGS) is one of the most common hereditary disorder that leads to chronic liver disease in children. It is an autosomal dominant disease. Frequency is 1/100 000. Jagged 1 gene mutation which causes microdeletion of twentieth chromosome can be detected in more than 90% of patients. As a result of examinations to a 3 years old patient admitted to our hospital due to dysuria, the patient is prepared for surgery since the results show bladder stone and ureter. Characteristic dysmorphic facial features, butterfly vertebrae, and posterior embriyotoxon as ocular findings were seen with patient. Hepatomegaly and splenomegaly were detected with physical examination and ultrasonography. Uretero cystoscopy and laser lithotripsy was performed to the patient undergoing general anesthesia. We emphasized that our experience in the anesthetic management of AGS is presented.
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