Paediatric Anaesthesia 1998 8: 79–82 Case report The Alagille’s syndrome and its anaesthetic considerations DINESH K. CHOUDHRY MD, FRCA*, MOHAMMED A. REHMAN MD*, ROY E. SCHWARTZ MD* AND DAVID A. PICCOLI MD† *Department of Anaesthesia and Critical Care, St. Christopher’s Hospital for Children, Erie Avenue at Front Street, Philadelphia, PA 19134-1095, USA; †Department of Gastroenterology, Children’s Hospital of Philadelphia, One Children’s Center, 34th and Civic Center, Philadelphia, PA 19104, USA Summary Alagille’s syndrome is an inherited disorder affecting multiple organ systems. Most characteristic is the paucity of the interlobular bile ducts with cholestasis. The anaesthetic plan for these patients should be based on careful preoperative preparation and attention to the issues 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 sustained a pathological fracture of the femur and was scheduled for closed reduction and application of a hip spica cast. Relevant anaesthetic issues are discussed. Keywords: Alagille’s syndrome: cholestasis; cardiac defects; growth failure Introduction with regard to issues pertinent to the anaesthesi- ologist. Syndromic bile duct paucity (SBDP), also called Alagille’s Syndrome (AS) was first recognized inde- pendently by Watson & Miller and by Alagille and co-workers in 1973 (1,2). It is characterized by a Case report paucity of the interlobular bile ducts with cholestasis, A five-and-a half-year-old, 15 kg, female with AS occurring in association with cardiac, musculo- presented with a four-day history of worsening pain skeletal, ocular, facial, and neurodevelopmental in the right thigh, subsequent to a pathological abnormalities. fracture of her right femur, and was scheduled for We discuss the anaesthetic management of a patient closed reduction and application of a hip spica cast. with Alagille’s syndrome and review the literature Her past medical history was significant for hyper- bilirubinaemia, rickets, hepatosplenomegaly, and pruritus. She had butterfly vertebrae and posterior Correspondence to: Mohamed Rehman, St. Christopher’s Hospital embryotoxon. Her past surgical history included liver for Children, Department of Anaesthesia and Critical Care, Erie Avenue at Front Street, Philadelphia, PA 19134-1095, USA. biopsy, tonsillectomy, adenoidectomy and bilateral 79  1998 Blackwell Science Ltd