Orthotopic Liver Transplantation in an Adult With Biliary Atresia, Situs Inversus, and Inferior Cava Vein Absence: A...

We report the case of a 34-year-old man who underwent Kasai portoenterostomy for biliary atresia at 6 weeks of age. In 2011, pulmonary hypertension was diagnosed and he began treatment with sildenafil. In 2012, he presented with an episode of upper gastrointestinal bleeding secondary to esophageal varices resistant to treatment. Later, he exhibited liver dysfunction. He was included on the waiting list for transplantation on May 29, 2013, with a Model for End-stage Liver Disease score of 24.

He underwent liver transplantation with an isogroup graft from a brain dead donor on June 9, 2013. Native hepatectomy was laborious owing to important collateral circulation and adhesions after previous operations, which had injured loops of the small bowel (SB). Orthotopic implantation was accomplished with direct anastomosis of the upper liver cava vein to the right atrium of the receiver. Portal and arterial anastomoses were performed as usual. Biliary reconstruction surgery by hepatojejunostomy was delayed 24 hours owing to SB loops injuries.

Graft viability was confirmed by normal hepatic function. Postoperative complications included abdominal compartment syndrome treated by decompressing laparotomy, severe pulmonary alveolar hemorrhage resolved with artery embolization and endotracheal intubation, intraabdominal abscess requiring percutaneous drain, and stroke requiring long-term rehabilitation. He is currently asymptomatic, presents normal graft function, and receives sildenafil because of pulmonary hypertension.

The association of situs inversus and biliary atresia is low. There is no consensus on the optimal operative approach to liver transplantation. An individualized assessment and multidisciplinary patient management are required.