Anaesthetic management of surgical ligation of type 2 Abernethy shunt with hepatopulmonary syndrome

Abernethy malformation is a rare congenital portosystemic shunt. A type 2 Abernethy malformation is characterised by a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava. Unmetabolised toxins that bypass hepatic circulation lead to intrapulmonary vascular dilatation. Approximately 3%–12% of portosystemic shunts present with features of hepatopulmonary syndrome, often presenting with progressive hypoxaemia and cyanosis. While endovascular device closure represents the preferred therapeutic approach for most cases, rarely, large or anatomically unfavourable shunts may necessitate surgical ligation that is met with unique anaesthetic challenges surrounding maintenance of adequate oxygenation and haemodynamic stability. We report the noteworthy anaesthetic concerns faced during successful surgical closure of Abernethy shunt in a boy in mid childhood.