"Mirror, Mirror on the Wall"… Pediatric liver transplantation in the case of situs inversus totalis with a disrupted inferior vena cava

Pediatr Transplant. 2018 Aug;22(5):e13218. doi: 10.1111/petr.13218. Epub 2018 May 15.

Abstract

We present the unique case of a 15-month-old male born with biliary atresia and situs inversus totalis and disrupted inferior vena cava who underwent a successful liver transplantation. The patient had previously undergone a failed Kasai procedure and presented with persistent hyperbilirubinemia. The patient was transplanted with a left lateral segment donor having standard arterial anatomy. Technical considerations included identifying completely replaced arterial anatomy in the recipient from the superior mesenteric artery and creating a branch patch between the gastroduodenal artery and HA, anastomosing the donor left hepatic vein to confluences of the donor left, middle, and right hepatic veins, using a "lazy-S" configuration of portal vein anastomosis, and suspending the allograft to the abdominal wall. Post-operatively, his liver function tests and total bilirubin normalized and he progressed to tolerating an oral diet with tube-feed supplementation.

Keywords: disrupted vena cava; pediatric liver transplantation; situs inversus totalis.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / surgery*
  • Biliary Atresia / surgery*
  • Humans
  • Infant
  • Liver Transplantation*
  • Male
  • Situs Inversus / surgery*
  • Vena Cava, Inferior / abnormalities*