American Surgical Association

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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, MD*1, John Magee, MD2, Mary L. Brandt, MD6, Patrick J Healey, MD*3, Greg Tiao, MD*4, Fred Ryckman, MD*4, Frederick M Karrer, MD*5, Cartland Burns, MD*11, Paul Columbani, MD15, Pat Dillon, MD*14, Annie Fecteau, MD*9, Alan Flake, MD*12, Kishore Iyer, MD*8, Hanmin Lee, MD*13, Jeff Lowell, MD*14, Richard Ricketts, MD*16, Karen West, MD*10, Yun Li, MD*2, Jeffrey Moore, MD*2, Kasper S. Wang, MD*7
1Children's Memorial Hospital, Chicago, IL;2University of Michigan School of Medicine, Ann Arbor, MI;3Seattle Children's Hospital, Seattle, WA;4Cincinnati Children's Hospital, Cincinnati, OH;5The Children's Hospital, Aurora, CO;6Texas Children’s Hospital, Houston, TX;7Children's Hospital Los Angeles, Los Angeles, CA; 8 Mount Sinai School of Medicine, New York, NY; 9 The Hospital for Sick Children, Montreal, Canada; 10 Riley Hospital for Children, Indianapolis, IN; 11 Children’s Hospital Pittsburgh, Pittsburgh, PA; 12 Children’s Hospital Philadelphia, Philadelphia, PA; 13 UCSF Children’s Hospital, San Francisco, CA; 14 Washington University School of Medicine, St. Louis, MO; 15 John Hopkins School of Medicine, Baltimore, MD; 16 Children’s Healthcare Atlanta, Atlanta, GA

Objective:
The goals of this study were to describe the clinical/anatomic features of infants undergoing Kasai hepatoportoenterostomy (HPE) for biliary atresia (BA), and examine associations between these parameters and outcome.
Methods:
Infants undergoing HPE in two prospective studies from 15 centers in the Childhood Liver Disease Research and Education Network were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. The remaining patients (136/244) were studied for transplant-free survival (Kaplan-Meier) and time to transplant/death (Cox regression).
Results:
HPE was performed in 244 infants (53.7% female; mean age 64.6 ± 28.5 days; anatomic data in table). Transplant-free survival was 53.7%, 46.7%, and 44.5% at 1, 2 and 3 years post-HPE. The risk of transplant/death was significantly lower in the 45.6% of patients with a total bilirubin <2.0 mg/dl within 3 months post-HPE (HR 0.08, p<0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi Type III vs. Type I; HR 1.92, p=0.0269), non-patent CBD (Ohi Subtype b+c vs. a; HR 5.44, p=0.0068), and splenic malformations (HR 1.93, p=0.0.0249). Outcome was not associated with age at HPE, gestational age, gender, race, ethnicity, or extent of porta hepatis dissection.
Conclusion:
Anatomic pattern of BA and early postoperative jaundice clearance are significant predictors of transplant-free survival. These findings establish U.S. benchmarks for counseling families and guiding transplant evaluation.


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