Living donor liver transplantation in children with cholestatic liver disease: a single-center experience

Transplant Proc. 2012 Mar;44(2):469-72. doi: 10.1016/j.transproceed.2011.11.014.

Abstract

Objectives: Cholestatic liver disease (CLD) is the main indication for liver transplantation in children. This retrospective study evaluated the outcomes of living donor liver transplantation (LDLT) in children with CLD.

Methods: One hundred fifty-nine children with CLD who underwent 164 LDLT between May 2001 and May 2011 were evaluated. Their original diseases were biliary atresia (n=145, 91%), Alagille syndrome (n=8, 5%), primary sclerosing cholangitis (n=2), and the others (n=4). The mean age and body weight of the recipients at LDLT was 42±53 months and 14.0±11.0 kg, respectively.

Results: Parents were living donors in 98%. The left lateral segment was the most common type of graft (77%). There were no reoperations and no mortality in any living donor. Recipients' postoperative surgical complications consisted mainly of hepatic arterial problems (7%), hepatic vein stenosis (5%), portal vein stenosis (13%), biliary stricture (18%), intestinal perforation (3%). The overall rejection rate was 31%. Cytomegalovirus infection and Epstein-Barr virus disease were observed in 26% and 5%, respectively. Retransplantation was performed five times in four patients; the main cause was hepatic vein stenosis (n=3). Four patients died; the main cause was gastrointestinal perforation (n=2). The body height of Alagille syndrome patients less than 2 years old significantly improved compared with older patients after LDLT. The 1-, 5-, and 10-year patient survival rates were 98%, 97%, and 97%, respectively.

Conclusions: LDLT for CLD is an effective treatment with excellent long-term outcomes.

MeSH terms

  • Age Factors
  • Alagille Syndrome / mortality
  • Alagille Syndrome / surgery*
  • Biliary Atresia / mortality
  • Biliary Atresia / surgery*
  • Child
  • Child, Preschool
  • Cholangitis, Sclerosing / mortality
  • Cholangitis, Sclerosing / surgery*
  • Female
  • Graft Rejection / etiology
  • Graft Rejection / surgery
  • Hepatectomy* / adverse effects
  • Hepatectomy* / mortality
  • Hospital Mortality
  • Humans
  • Infant
  • Infant, Newborn
  • Japan
  • Kaplan-Meier Estimate
  • Liver Transplantation* / adverse effects
  • Liver Transplantation* / mortality
  • Living Donors*
  • Male
  • Postoperative Complications / etiology
  • Postoperative Complications / surgery
  • Reoperation
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome