Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting

• Published in: Pediatric transplantation 2009-12, Vol.13 (8), p.1041-1047
• Main Authors: Singhal, Ashish, Srivastava, Ajitabh, Goyal, Neerav, Vij, Vivek, Wadhawan, Manav, Bera, Motilal, Gupta, Subash
• Format: Article
• Language: English
• Subjects: Abernethy malformation; Abnormalities, Multiple - diagnostic imaging; Arteriovenous Malformations - diagnostic imaging; Arteriovenous Malformations - surgery; biliary atresia; Biliary Atresia - diagnostic imaging; Biliary Atresia - surgery; Biological and medical sciences; Cardiology. Vascular system; Child, Preschool; congenital extrahepatic portosystemic shunt; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Echocardiography; General aspects; Heart; Heart Septal Defects, Ventricular - diagnostic imaging; Humans; intrapulmonary shunting; Liver Transplantation; living donor liver transplant; Living Donors; Male; Medical sciences; patent ductus venosus; Portal Vein - abnormalities; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tomography, X-Ray Computed; Venae Cavae - abnormalities
• ISSN: 1397-3142; 1399-3046
• DOI: 10.1111/j.1399-3046.2009.01092.x

:  Congenital portosystemic shunts are the anomalies in which the mesenteric venous drainage bypasses the liver and drains directly into the systemic circulation. This is a report of a rare case of LDLT in a four‐yr old male child suffering with biliary atresia (post‐failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno‐mesentric confluence to the LHV, (ii) intrapulmonary shunts, (iii) perimembranous VSD. The left lobe graft was procured from the mother of the child. Recipient IVC and the shunt vessel were preserved during the hepatectomy, and the caval and shunt clamping were remarkably short while performing the HV and portal anastomosis. Post‐operative course was uneventful; intrapulmonary shunts regressed within three months after transplantation and currently after 18 months following transplant child is doing well with normal liver functions. CEPSh has been extensively discussed and all the published cases of liver transplantation for CEPSh were reviewed.