Pediatr Transplantation 2014: 18: E274–E279
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12364
Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report Youn JK, Lee J-M, Yi N-J, Choi YR, Suh S-W, You T, Lee K-W, Jung C-W, Lee J-w, Bae E-J, Ko JS, Kim W-H, Park K-W, Suh K-S. (2014) Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report. Pediatr Transplant, 18: E274–E279. DOI: 10.1111/petr.12364. 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.
Joong Kee Youn1, Jeong-Moo Lee1, Nam-Joon Yi1, Young Rok Choi1, Suk-Won Suh1, Tae You1, Kwang-Woong Lee1, Chul-Woo Jung2, Ji-won Lee2, Eun-Jung Bae3, Jae Sung Ko3, Woong-Han Kim4, Kwi-Won Park1 and Kyung-Suk Suh1 1
Department of Surgery, Seoul National University College of Medicine, Seoul, Korea, 2Department of Anesthisiology and Pain Medicine, Seoul National University College of Medicine, Seoul, Korea, 3 Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea, 4Department of Thoracic Surgery, Seoul National University College of Medicine, Seoul, Korea Key words: biliary atresia – left isomerism – Fontan operation – hepatopulmonary syndrome – liver transplantation Nam-Joon Yi, MD, PhD, Associate Professor, Division of HBP Surgery, Department of Surgery, Seoul National University College of Medicine, 101 Daehak-ro, Jongro-gu, 110-744, Seoul, Korea Tel:. +82 2 2072 2990 Fax: +82 2 766 3975 E-mail: [email protected] Accepted for publication 27 August 2014
Over the past few decades, LT has become the gold standard for patients with ESLD. BA, for which the Kasai operation has failed, is the most common indication of pediatric LT. BA is associated with other congenital anomalies in up to 20% of cases (1). LI is a rare birth defect characterized by the mirror-image orientation of the abdominal and/or thoracic viscera with respect to the midline, with the incidence of Abbreviations: BA, biliary atresia; CHD, congenital heart disease; CVP, central venous pressure; ESLD, end-stage liver disease; HA, hepatic artery; HPS, hepatopulmonary syndrome; HV, hepatic vein; IVC, inferior vena cava; LI, left isomerism; LT, liver transplantation; O2, oxygen; PA, pulmonary artery; PELD, pediatric end-stage liver disease; POD, post-operative day; PV, portal vein; SVC, superior vena cava.
E274
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12364
Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report Youn JK, Lee J-M, Yi N-J, Choi YR, Suh S-W, You T, Lee K-W, Jung C-W, Lee J-w, Bae E-J, Ko JS, Kim W-H, Park K-W, Suh K-S. (2014) Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report. Pediatr Transplant, 18: E274–E279. DOI: 10.1111/petr.12364. 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.
Joong Kee Youn1, Jeong-Moo Lee1, Nam-Joon Yi1, Young Rok Choi1, Suk-Won Suh1, Tae You1, Kwang-Woong Lee1, Chul-Woo Jung2, Ji-won Lee2, Eun-Jung Bae3, Jae Sung Ko3, Woong-Han Kim4, Kwi-Won Park1 and Kyung-Suk Suh1 1
Department of Surgery, Seoul National University College of Medicine, Seoul, Korea, 2Department of Anesthisiology and Pain Medicine, Seoul National University College of Medicine, Seoul, Korea, 3 Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea, 4Department of Thoracic Surgery, Seoul National University College of Medicine, Seoul, Korea Key words: biliary atresia – left isomerism – Fontan operation – hepatopulmonary syndrome – liver transplantation Nam-Joon Yi, MD, PhD, Associate Professor, Division of HBP Surgery, Department of Surgery, Seoul National University College of Medicine, 101 Daehak-ro, Jongro-gu, 110-744, Seoul, Korea Tel:. +82 2 2072 2990 Fax: +82 2 766 3975 E-mail: [email protected] Accepted for publication 27 August 2014
Over the past few decades, LT has become the gold standard for patients with ESLD. BA, for which the Kasai operation has failed, is the most common indication of pediatric LT. BA is associated with other congenital anomalies in up to 20% of cases (1). LI is a rare birth defect characterized by the mirror-image orientation of the abdominal and/or thoracic viscera with respect to the midline, with the incidence of Abbreviations: BA, biliary atresia; CHD, congenital heart disease; CVP, central venous pressure; ESLD, end-stage liver disease; HA, hepatic artery; HPS, hepatopulmonary syndrome; HV, hepatic vein; IVC, inferior vena cava; LI, left isomerism; LT, liver transplantation; O2, oxygen; PA, pulmonary artery; PELD, pediatric end-stage liver disease; POD, post-operative day; PV, portal vein; SVC, superior vena cava.
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