Orthotopic liver transplantation for congenital biliary atresia: An 11- year, single-center experience

John A. Goss, Christopher R. Shackleton, Kim Swenson, Nancy L. Satou, Barbara J. Nuesse, David K. Imagawa, Milan Kinkhabwala, Philip Seu, Jay S. Markowitz, Steven M. Rudich, Sue V. McDiarmid, Ronald W. Busuttil

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

Objective: The authors analyze a single centers 11 year experience with 190 orthotopic liver transplants for congenital biliary atresia. Summary Background Data: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patients still develop recurrent cholestasis, portal hypertension, eholangitis, and cirrhosis. Therefore, the only hope of long term survival in the majority of children with congenital biliary atresia is definitive correction with orthotopic liver transplantation. Methods: The medical records of 190 consecutive patients undergoing orthotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multivariate regression analysis to determine the statistical strength of independent associations between pretransplant covariates and patient and graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence of post transplant complications were determined, as was the quality of long term graft function. The median follow up period was 321 years. Results: The liver grafts were comprised of 155 whole organ, 24 reduced-size, and 11 living- donor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 123 kg, and 138 mg/dL, respectively. One hundred sixty four patients (86%) had undergone prior hepatic portoenterostomy. Eighty-seven patients (46%) were United Network for Organ Sharing (UNOS) status 1 or 2 at the time of liver transplantation the majority (15/24, 62%) of reduced size graft recipients were UNOS status I at the time of transplantation. One hundred fifty-nine patients (84%) received a single graft, whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial patient survival rates were 83%, 80%, and 78% respectively, whereas graft survival rates were 81%, 77%, and 76%, respectively. Cox multivariate regression analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft type significantly predicted patient survival, whereas recipient age, weight, and previous hepatic portoenterostomy did not. Current median follow up values for total bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. Conclusion: Long-term patient survival after orthotopic liver transplantation for congenital biliary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living donor graft.

Original languageEnglish (US)
Pages (from-to)276-287
Number of pages12
JournalAnnals of Surgery
Volume224
Issue number3
DOIs
StatePublished - 1996
Externally publishedYes

Fingerprint

Biliary Atresia
Liver Transplantation
Hepatic Portoenterostomy
Transplants
Graft Survival
Bilirubin
Living Donors
Weights and Measures
Survival
Multivariate Analysis
Survival Rate
Regression Analysis
Hyperbilirubinemia
Liver
Cholestasis
Portal Hypertension
Aspartate Aminotransferases
Medical Records

ASJC Scopus subject areas

  • Surgery

Cite this

Goss, J. A., Shackleton, C. R., Swenson, K., Satou, N. L., Nuesse, B. J., Imagawa, D. K., ... Busuttil, R. W. (1996). Orthotopic liver transplantation for congenital biliary atresia: An 11- year, single-center experience. Annals of Surgery, 224(3), 276-287. https://doi.org/10.1097/00000658-199609000-00004

Orthotopic liver transplantation for congenital biliary atresia : An 11- year, single-center experience. / Goss, John A.; Shackleton, Christopher R.; Swenson, Kim; Satou, Nancy L.; Nuesse, Barbara J.; Imagawa, David K.; Kinkhabwala, Milan; Seu, Philip; Markowitz, Jay S.; Rudich, Steven M.; McDiarmid, Sue V.; Busuttil, Ronald W.

In: Annals of Surgery, Vol. 224, No. 3, 1996, p. 276-287.

Research output: Contribution to journalArticle

Goss, JA, Shackleton, CR, Swenson, K, Satou, NL, Nuesse, BJ, Imagawa, DK, Kinkhabwala, M, Seu, P, Markowitz, JS, Rudich, SM, McDiarmid, SV & Busuttil, RW 1996, 'Orthotopic liver transplantation for congenital biliary atresia: An 11- year, single-center experience', Annals of Surgery, vol. 224, no. 3, pp. 276-287. https://doi.org/10.1097/00000658-199609000-00004
Goss, John A. ; Shackleton, Christopher R. ; Swenson, Kim ; Satou, Nancy L. ; Nuesse, Barbara J. ; Imagawa, David K. ; Kinkhabwala, Milan ; Seu, Philip ; Markowitz, Jay S. ; Rudich, Steven M. ; McDiarmid, Sue V. ; Busuttil, Ronald W. / Orthotopic liver transplantation for congenital biliary atresia : An 11- year, single-center experience. In: Annals of Surgery. 1996 ; Vol. 224, No. 3. pp. 276-287.
@article{78d1f6e3751540a483c6bb07e789b8f3,
title = "Orthotopic liver transplantation for congenital biliary atresia: An 11- year, single-center experience",
abstract = "Objective: The authors analyze a single centers 11 year experience with 190 orthotopic liver transplants for congenital biliary atresia. Summary Background Data: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patients still develop recurrent cholestasis, portal hypertension, eholangitis, and cirrhosis. Therefore, the only hope of long term survival in the majority of children with congenital biliary atresia is definitive correction with orthotopic liver transplantation. Methods: The medical records of 190 consecutive patients undergoing orthotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multivariate regression analysis to determine the statistical strength of independent associations between pretransplant covariates and patient and graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence of post transplant complications were determined, as was the quality of long term graft function. The median follow up period was 321 years. Results: The liver grafts were comprised of 155 whole organ, 24 reduced-size, and 11 living- donor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 123 kg, and 138 mg/dL, respectively. One hundred sixty four patients (86{\%}) had undergone prior hepatic portoenterostomy. Eighty-seven patients (46{\%}) were United Network for Organ Sharing (UNOS) status 1 or 2 at the time of liver transplantation the majority (15/24, 62{\%}) of reduced size graft recipients were UNOS status I at the time of transplantation. One hundred fifty-nine patients (84{\%}) received a single graft, whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial patient survival rates were 83{\%}, 80{\%}, and 78{\%} respectively, whereas graft survival rates were 81{\%}, 77{\%}, and 76{\%}, respectively. Cox multivariate regression analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft type significantly predicted patient survival, whereas recipient age, weight, and previous hepatic portoenterostomy did not. Current median follow up values for total bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. Conclusion: Long-term patient survival after orthotopic liver transplantation for congenital biliary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living donor graft.",
author = "Goss, {John A.} and Shackleton, {Christopher R.} and Kim Swenson and Satou, {Nancy L.} and Nuesse, {Barbara J.} and Imagawa, {David K.} and Milan Kinkhabwala and Philip Seu and Markowitz, {Jay S.} and Rudich, {Steven M.} and McDiarmid, {Sue V.} and Busuttil, {Ronald W.}",
year = "1996",
doi = "10.1097/00000658-199609000-00004",
language = "English (US)",
volume = "224",
pages = "276--287",
journal = "Annals of Surgery",
issn = "0003-4932",
publisher = "Lippincott Williams and Wilkins",
number = "3",

}

TY - JOUR

T1 - Orthotopic liver transplantation for congenital biliary atresia

T2 - An 11- year, single-center experience

AU - Goss, John A.

AU - Shackleton, Christopher R.

AU - Swenson, Kim

AU - Satou, Nancy L.

AU - Nuesse, Barbara J.

AU - Imagawa, David K.

AU - Kinkhabwala, Milan

AU - Seu, Philip

AU - Markowitz, Jay S.

AU - Rudich, Steven M.

AU - McDiarmid, Sue V.

AU - Busuttil, Ronald W.

PY - 1996

Y1 - 1996

N2 - Objective: The authors analyze a single centers 11 year experience with 190 orthotopic liver transplants for congenital biliary atresia. Summary Background Data: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patients still develop recurrent cholestasis, portal hypertension, eholangitis, and cirrhosis. Therefore, the only hope of long term survival in the majority of children with congenital biliary atresia is definitive correction with orthotopic liver transplantation. Methods: The medical records of 190 consecutive patients undergoing orthotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multivariate regression analysis to determine the statistical strength of independent associations between pretransplant covariates and patient and graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence of post transplant complications were determined, as was the quality of long term graft function. The median follow up period was 321 years. Results: The liver grafts were comprised of 155 whole organ, 24 reduced-size, and 11 living- donor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 123 kg, and 138 mg/dL, respectively. One hundred sixty four patients (86%) had undergone prior hepatic portoenterostomy. Eighty-seven patients (46%) were United Network for Organ Sharing (UNOS) status 1 or 2 at the time of liver transplantation the majority (15/24, 62%) of reduced size graft recipients were UNOS status I at the time of transplantation. One hundred fifty-nine patients (84%) received a single graft, whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial patient survival rates were 83%, 80%, and 78% respectively, whereas graft survival rates were 81%, 77%, and 76%, respectively. Cox multivariate regression analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft type significantly predicted patient survival, whereas recipient age, weight, and previous hepatic portoenterostomy did not. Current median follow up values for total bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. Conclusion: Long-term patient survival after orthotopic liver transplantation for congenital biliary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living donor graft.

AB - Objective: The authors analyze a single centers 11 year experience with 190 orthotopic liver transplants for congenital biliary atresia. Summary Background Data: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patients still develop recurrent cholestasis, portal hypertension, eholangitis, and cirrhosis. Therefore, the only hope of long term survival in the majority of children with congenital biliary atresia is definitive correction with orthotopic liver transplantation. Methods: The medical records of 190 consecutive patients undergoing orthotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multivariate regression analysis to determine the statistical strength of independent associations between pretransplant covariates and patient and graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence of post transplant complications were determined, as was the quality of long term graft function. The median follow up period was 321 years. Results: The liver grafts were comprised of 155 whole organ, 24 reduced-size, and 11 living- donor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 123 kg, and 138 mg/dL, respectively. One hundred sixty four patients (86%) had undergone prior hepatic portoenterostomy. Eighty-seven patients (46%) were United Network for Organ Sharing (UNOS) status 1 or 2 at the time of liver transplantation the majority (15/24, 62%) of reduced size graft recipients were UNOS status I at the time of transplantation. One hundred fifty-nine patients (84%) received a single graft, whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial patient survival rates were 83%, 80%, and 78% respectively, whereas graft survival rates were 81%, 77%, and 76%, respectively. Cox multivariate regression analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft type significantly predicted patient survival, whereas recipient age, weight, and previous hepatic portoenterostomy did not. Current median follow up values for total bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. Conclusion: Long-term patient survival after orthotopic liver transplantation for congenital biliary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living donor graft.

UR - http://www.scopus.com/inward/record.url?scp=9544255688&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=9544255688&partnerID=8YFLogxK

U2 - 10.1097/00000658-199609000-00004

DO - 10.1097/00000658-199609000-00004

M3 - Article

C2 - 8813256

AN - SCOPUS:9544255688

VL - 224

SP - 276

EP - 287

JO - Annals of Surgery

JF - Annals of Surgery

SN - 0003-4932

IS - 3

ER -