The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival

Riccardo Superina, John C. Magee, Mary L. Brandt, Patrick J. Healey, Greg Tiao, Fred Ryckman, Frederick M. Karrer, Kishore Iyer, Annie Fecteau, Karen West, R. Cartland Burns, Alan Flake, Hanmin Lee, Jeff A. Lowell, Pat Dillon, Paul Colombani, Richard Ricketts, Yun Li, Jeffrey Moore, Kasper S. Wang*

*Corresponding author for this work

Research output: Contribution to journalArticle

83 Citations (Scopus)

Abstract

OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

Original languageEnglish (US)
Pages (from-to)577-585
Number of pages9
JournalAnnals of Surgery
Volume254
Issue number4
DOIs
StatePublished - Oct 1 2011

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Biliary Atresia
Jaundice
Transplants
Survival
Drainage
Liver
Survival Analysis
Bilirubin
Ascites
Bile
Gestational Age
Dissection
Liver Diseases
Education
Research

ASJC Scopus subject areas

  • Surgery

Cite this

Superina, Riccardo ; Magee, John C. ; Brandt, Mary L. ; Healey, Patrick J. ; Tiao, Greg ; Ryckman, Fred ; Karrer, Frederick M. ; Iyer, Kishore ; Fecteau, Annie ; West, Karen ; Burns, R. Cartland ; Flake, Alan ; Lee, Hanmin ; Lowell, Jeff A. ; Dillon, Pat ; Colombani, Paul ; Ricketts, Richard ; Li, Yun ; Moore, Jeffrey ; Wang, Kasper S. / The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. In: Annals of Surgery. 2011 ; Vol. 254, No. 4. pp. 577-585.
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title = "The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival",
abstract = "OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54{\%} female; mean age: 65 ± 29 days). Transplant-free survival was 53.7{\%} and 46.7{\%} at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6{\%} of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.",
author = "Riccardo Superina and Magee, {John C.} and Brandt, {Mary L.} and Healey, {Patrick J.} and Greg Tiao and Fred Ryckman and Karrer, {Frederick M.} and Kishore Iyer and Annie Fecteau and Karen West and Burns, {R. Cartland} and Alan Flake and Hanmin Lee and Lowell, {Jeff A.} and Pat Dillon and Paul Colombani and Richard Ricketts and Yun Li and Jeffrey Moore and Wang, {Kasper S.}",
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Superina, R, Magee, JC, Brandt, ML, Healey, PJ, Tiao, G, Ryckman, F, Karrer, FM, Iyer, K, Fecteau, A, West, K, Burns, RC, Flake, A, Lee, H, Lowell, JA, Dillon, P, Colombani, P, Ricketts, R, Li, Y, Moore, J & Wang, KS 2011, 'The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival', Annals of Surgery, vol. 254, no. 4, pp. 577-585. https://doi.org/10.1097/SLA.0b013e3182300950

The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. / Superina, Riccardo; Magee, John C.; Brandt, Mary L.; Healey, Patrick J.; Tiao, Greg; Ryckman, Fred; Karrer, Frederick M.; Iyer, Kishore; Fecteau, Annie; West, Karen; Burns, R. Cartland; Flake, Alan; Lee, Hanmin; Lowell, Jeff A.; Dillon, Pat; Colombani, Paul; Ricketts, Richard; Li, Yun; Moore, Jeffrey; Wang, Kasper S.

In: Annals of Surgery, Vol. 254, No. 4, 01.10.2011, p. 577-585.

Research output: Contribution to journalArticle

TY - JOUR

T1 - The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival

AU - Superina, Riccardo

AU - Magee, John C.

AU - Brandt, Mary L.

AU - Healey, Patrick J.

AU - Tiao, Greg

AU - Ryckman, Fred

AU - Karrer, Frederick M.

AU - Iyer, Kishore

AU - Fecteau, Annie

AU - West, Karen

AU - Burns, R. Cartland

AU - Flake, Alan

AU - Lee, Hanmin

AU - Lowell, Jeff A.

AU - Dillon, Pat

AU - Colombani, Paul

AU - Ricketts, Richard

AU - Li, Yun

AU - Moore, Jeffrey

AU - Wang, Kasper S.

PY - 2011/10/1

Y1 - 2011/10/1

N2 - OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

AB - OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

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