THE ALAGILLES-SYNDROME AND ITS ANESTHETIC CONSIDERATIONS

Alagille's syndrome is an inherited disorder affecting multiple organ systems. Most characteristic is the paucity of the interlobular bile d ucts with cholestasis. The anaesthetic plan for these patients should be based on cartful preoperative preparation and attention to the issu es involving hepatobiliary, cardiac, neurodevelopmental, nutritional, haematological, ocular and facial abnormalities. We report the case of a five-and-a-half-year old patient with Alagille's syndrome who susta ined a pathological fracture of the femur and was scheduled for closed reduction and application of a hip spica cast. Relevant anaesthetic i ssues are discussed.