Successful liver transplantation in two infants with biliary atresia and complex splanchnic malformations.

Background: Liver Transplantation in infants with biliary atresia (BA) associated with complex spanchnic malformations (i.e. splenic malformations in BASM Syndrome) is associated to a worse prognosis, especially due to vascular complications [1]. S290 Posters / Digestive and Liver Disease 44 Suppl. 4 (2012) S259–S292 Specific aim: Within the last decade (2002–2012), 80 liver transplantations were performed on paediatric patients at our Centre; 25 of them were infants aging <1 year and/or weighting <10 kg. We report two paediatric cases affected by BA and splanchnic complex malformations who were successfully treated with liver transplantation. Case reports: (1) An infant with BA, asplenia, intestinal malrotation and anomalous splanchnic vascularisation was surgically treated with Kasai portoenterostomy at 63 days of life. The outcome was inefficacious since the child presented impaired liver function, persistent jaundice, partially adequate nutritional status (body weight and middle brachial circumference <3rd percentile despite adequate caloric intake). An Angiographic CT detected patent superior and inferior mesenteric arteries (displaced on the right of the superior mesenteric vein and of the aorta respectively, in intestinal malrotation); thin portal vein (lumen 1.5 mm); absence of the middle hepatic vein; the inferior vena cava was interrupted at the level of its supra-renal infra-hepatic tract; the infra-renal tract of the inferior vena cava was connected to the Azygos vein. The child completed the first year vaccination schedule, before undergoing a left split liver transplantation at the age of 1.1 year. The post-operative Doppler study (day 2) evidenced patency and adequate fluxes at all vascular anastomoses. No major peri-operative complications have occurred. The liver function and hepatic tests are normal and the child is being fed regularly with normal formula, with an adequate growth. (2) An infant with Situs Viscerum Inversus (Left-sided liver, rightsided stomach, left-sided cecum, right-sided heart, interrupted inferior vena cava and Azygos continuation, pre-duodenal portal vein) was surgically treated with Kasai portoenterostomy at 55 days of life. The outcome was inefficacious and the child presented persistent cholestasis. An episode of cholangitis was associated with acute liver failure, so an urgent liver transplantation was required at the age of 6 months. Post-operative Doppler controls (day 2) were regular and no major complications have occurred. Discussion: The presence of splanchnic malformations is no longer a contraindication to liver transplantation, even in infants weighing <10 kg, in Liver Transplantation Centres with an appropriate expertise. A careful attention to major complications has nevertheless to be considered, given the anatomical and surgical complexity of these children who basically have a worse prognosis. Reference(s) [1] Sen-Oran E, Yankol Y, Tuzun B, Kocak B, Kanmaz T, Acarli K, Kalayoglu M. Cadaveric liver transplantation in biliary atresia splenic malformation syndrome with the absence of retrohepatic inferior vena cava, preduodenal portal vein, and intestinal malrotation: a case report. Transplant Proc 2008; 40(1): 313−5.