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
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.
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).
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.
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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