title: Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting.
abstract: 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.
authors.affiliation: Department of Surgical Gastroenterolgy & Liver Transplant, Indraprastha Apollo Hospital, New Delhi, India.
authors.forename: Ashish
authors.initials: A
authors.lastname: Singhal
authors.forename: Ajitabh
authors.initials: A
authors.lastname: Srivastava
authors.forename: Neerav
authors.initials: N
authors.lastname: Goyal
authors.forename: Vivek
authors.initials: V
authors.lastname: Vij
authors.forename: Manav
authors.initials: M
authors.lastname: Wadhawan
authors.forename: Motilal
authors.initials: M
authors.lastname: Bera
authors.forename: Subash
authors.initials: S
authors.lastname: Gupta
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000601:surgery*; D001656:Biliary Atresia / Q000000981:diagnostic imaging / Q000601:surgery*; D002675:Child, Preschool; D004452:Echocardiography; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D014057:Tomography, X-Ray Computed; D014684:Venae Cavae / Q000002:abnormalities*