The Alagille's syndrome and its anaesthetic considerations

Abstract

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.

Histiocytic Osteolysis Secondary to Hyperbilirubinaemia: A Case Report

“…One of the manifestations is hyperbilirubinaemia caused by cholestasis secondary to paucity of the interlobular bile ducts. 1,2 Patients with chronic liver disease have an increased prevalence of osteoporosis because of calcium malabsorption caused by low levels of 25hydroxy vitamin D3 and hyperbilirubinaemia. 3 Nonetheless, the underlying mechanism causing osteoporosis secondary to hyperbilirubinaemia remains unclear.…”

Alagille syndrome and pregnancy: anesthetic management for cesarean section

“…There is little documentation concerning anesthetic management of adult patients with AS with most reported cases relating to children under five years of age undergoing liver surgery. [7][8][9] For females with AS who reach adulthood, detailed management during pregnancy and childbirth have rarely been documented. 10,11 Cholestasis, resulting from intrahepatic bile duct paucity, is the main manifestation of AS and appears in the first three months of life in 45-75%, and in the remainder before three years of age.…”

Combined general and epidural anesthesia for a child with alagille syndrome: a case report

“…However, the effect was titrated to train-of-four stimulation as patients with AGS can have neuromuscular dysfunction and increased sensitivity secondary to vitamin E deficiency. Presence of neurological symptoms secondary to vitamin deficiency may also influence the choice of muscle relaxant during rapid sequence intubation if abdominal distension is present preoperatively (11). Other fat-soluble vitamin deficiencies such as vitamin D deficiency can result in significant metabolic bone disease.…”

“…Extreme care must be taken at all times during transport and positioning to avoid fractures in already weakened bones. Eye care is of paramount importance as vitamin A deficiency can result in dry eyes and corneal ulcers in exposed eyes (11).…”