Liver transplantation in children: The initial Toronto experience

Riccardo A. Superina*, Richard H. Pearl, Eve A. Roberts, M. James Phillips, Nancy Graham, Paul D. Greig, Bernard Langer

*Corresponding author for this work

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P<0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group. Complicating factors frequently seen in patients with EHBA at transplantation were small size (mean weight, 9.7 kg) and high incidence of portal vein atresia or hypoplasia (6 of 9). Survival in patients with fulminant hepatic failure was 50%. Four of the 18 patients developed primary CMV infection, and three died. Survival in patients with primary CMV infection was significantly lower than it was in other patients. Our early experience in pediatric liver transplantation confirms that survival in children with certain risk factors may be decreased. Earlier referral for transplantation, prevention of primary CMV disease through matching for CMV status, or better treatment of established CMV infection should considerably improve survival in small pediatric liver recipients.

Original languageEnglish (US)
Pages (from-to)1013-1019
Number of pages7
JournalJournal of Pediatric Surgery
Volume24
Issue number10
DOIs
StatePublished - Jan 1 1989

Fingerprint

Liver Transplantation
Biliary Atresia
Survival
Cytomegalovirus Infections
Portal Vein
Cytomegalovirus
Thrombosis
Hepatic Encephalopathy
Acute Liver Failure
Transplantation
Pediatrics
Tyrosinemias
Glycogen Storage Disease
Weights and Measures
Warm Ischemia
Brain Death
Waiting Lists
Incidence
Primary Prevention
Fibrosis

Keywords

  • Liver transplantation
  • pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Superina, R. A., Pearl, R. H., Roberts, E. A., Phillips, M. J., Graham, N., Greig, P. D., & Langer, B. (1989). Liver transplantation in children: The initial Toronto experience. Journal of Pediatric Surgery, 24(10), 1013-1019. https://doi.org/10.1016/S0022-3468(89)80205-2
Superina, Riccardo A. ; Pearl, Richard H. ; Roberts, Eve A. ; Phillips, M. James ; Graham, Nancy ; Greig, Paul D. ; Langer, Bernard. / Liver transplantation in children : The initial Toronto experience. In: Journal of Pediatric Surgery. 1989 ; Vol. 24, No. 10. pp. 1013-1019.
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Superina, RA, Pearl, RH, Roberts, EA, Phillips, MJ, Graham, N, Greig, PD & Langer, B 1989, 'Liver transplantation in children: The initial Toronto experience', Journal of Pediatric Surgery, vol. 24, no. 10, pp. 1013-1019. https://doi.org/10.1016/S0022-3468(89)80205-2

Liver transplantation in children : The initial Toronto experience. / Superina, Riccardo A.; Pearl, Richard H.; Roberts, Eve A.; Phillips, M. James; Graham, Nancy; Greig, Paul D.; Langer, Bernard.

In: Journal of Pediatric Surgery, Vol. 24, No. 10, 01.01.1989, p. 1013-1019.

Research output: Contribution to journalArticle

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AU - Langer, Bernard

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N2 - The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P<0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group. Complicating factors frequently seen in patients with EHBA at transplantation were small size (mean weight, 9.7 kg) and high incidence of portal vein atresia or hypoplasia (6 of 9). Survival in patients with fulminant hepatic failure was 50%. Four of the 18 patients developed primary CMV infection, and three died. Survival in patients with primary CMV infection was significantly lower than it was in other patients. Our early experience in pediatric liver transplantation confirms that survival in children with certain risk factors may be decreased. Earlier referral for transplantation, prevention of primary CMV disease through matching for CMV status, or better treatment of established CMV infection should considerably improve survival in small pediatric liver recipients.

AB - The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P<0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group. Complicating factors frequently seen in patients with EHBA at transplantation were small size (mean weight, 9.7 kg) and high incidence of portal vein atresia or hypoplasia (6 of 9). Survival in patients with fulminant hepatic failure was 50%. Four of the 18 patients developed primary CMV infection, and three died. Survival in patients with primary CMV infection was significantly lower than it was in other patients. Our early experience in pediatric liver transplantation confirms that survival in children with certain risk factors may be decreased. Earlier referral for transplantation, prevention of primary CMV disease through matching for CMV status, or better treatment of established CMV infection should considerably improve survival in small pediatric liver recipients.

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