The Alagille's syndrome and its anaesthetic considerations.
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.
Choudhry
D K
DK
Department of Anaesthesia and Critical Care, St. Christopher's Hospital for Children, Philadelphia, PA 19134-1095, USA.
Rehman
M A
MA
Schwartz
R E
RE
Piccoli
D A
DA
D016738:Alagille Syndrome; D000758:Anesthesia / Q000379:methods*; D002675:Child, Preschool; D005260:Female; D006801:Humans