Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: a case report.

Abstract

LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI and CHD is a challenging issue for a transplant surgeon. Herein, we report a successful split LT on a three-yr-old boy with LI who survived after a Fontan procedure due to single ventricle, but who suffered from HPS associated with BA.

• Authors: Joong Kee Youn, Jeong-Moo Lee, Nam-Joon Yi, Young Rok Choi, Suk-Won Suh, Tae You, Kwang-Woong Lee, Chul-Woo Jung, Ji-Won Lee, Eun-Jung Bae, Jae Sung Ko, Woong-Han Kim, Kwi-Won Park, Kyung-Suk Suh
• Journal: Pediatric transplantation (Pediatr Transplant) Vol. 18 Issue 8 Pg. E274-9 (Dec 2014) ISSN: 1399-3046 [Electronic] Denmark
• PMID: 25263970 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Topics

• Biliary Atresia (complications, surgery)
• Child, Preschool
• Fontan Procedure
• Hepatopulmonary Syndrome (etiology, surgery)
• Heterotaxy Syndrome (surgery)
• Humans
• Liver Transplantation (methods)
• Male