Liver transplantation in children with biliary atresia and polysplenia syndrome

D. Falchetti, F. Brant de Carvalho, P. Clapuyt, J. de Ville de Goyet, B. de Hemptinne, D. Claus, J. B. Otte

Research output: Contribution to journalArticle

Abstract

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.

Original languageEnglish
Pages (from-to)528-531
Number of pages4
JournalJournal of Pediatric Surgery
Volume26
Issue number5
DOIs
Publication statusPublished - 1991

Keywords

  • Biliary atresia
  • liver transplantation
  • polysplenia syndrome

ASJC Scopus subject areas

  • Surgery

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    Falchetti, D., Brant de Carvalho, F., Clapuyt, P., de Ville de Goyet, J., de Hemptinne, B., Claus, D., & Otte, J. B. (1991). Liver transplantation in children with biliary atresia and polysplenia syndrome. Journal of Pediatric Surgery, 26(5), 528-531. https://doi.org/10.1016/0022-3468(91)90698-S