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Original Investigation
Pacific Coast Surgical Association
January 2019

Biliary-Enteric Drainage vs Primary Liver Transplant as Initial Treatment for Children With Biliary Atresia

Author Affiliations
  • 1Department of Surgery, Huntington Memorial Hospital, Pasadena, California
  • 2Division of Hepatobiliary and Liver Transplantation, Vanderbilt University Medical Center, Nashville, Tennessee
  • 3Department of Preventive Medicine, Keck Hospital, University of Southern California, Los Angeles
JAMA Surg. 2019;154(1):26-32. doi:10.1001/jamasurg.2018.3180
Key Points

Question  For infants with biliary atresia, should biliary-enteric drainage remain the initial treatment of choice and liver transplant used as salvage therapy?

Findings  In this cohort study that included 626 infants with biliary atresia, patients who underwent primary liver transplant had a markedly reduced risk of long-term mortality compared with patients initially managed with biliary-enteric drainage procedure.

Meaning  Patients treated with a primary liver transplant live longer than those initially treated with biliary-enteric drainage; a multi-institutional clinical trial is necessary to identify the initial treatment optimal for patients with biliary atresia.


Importance  Some infants with biliary atresia are treated with primary liver transplant (pLT), but most are initially treated with biliary-enteric drainage (BED) with a subsequent salvage liver transplant. Given the improvements in liver transplant outcomes, it is important to determine whether BED treatment remains the optimal surgical algorithm for patients with biliary atresia.

Objective  To compare the survival of patients with biliary atresia initially treated with BED with patients who underwent pLT.

Design, Setting, and Participants  This cohort study used deidentified records from the California Office of Statewide Health Planning and Development database to identify patients with biliary atresia (n = 1252) between January 1, 1990, through December 31, 2015. Patients were categorized into 1 of 2 cohorts: those who received BED treatment and those who underwent pLT. Excluded from the study were those born before January 1, 1995, and those without any documented operative intervention by age 5 years. Data analysis was performed from January 1, 1990, to December 31, 2015.

Main Outcomes and Measures  Overall survival was compared between the BED and pLT cohorts using the Kaplan-Meier method. The treatment’s association with treatment era was examined by comparing survival before 2002 and on or after January 1, 2002.

Results  In total, 1252 patients with biliary atresia were identified. After exclusions, 626 remained; of these patients, 351 (56.1%) were female and 275 (43.9%) were male with a median (interquartile range) age at intervention for initial BED treatment of 65 (48-81) days. Among the 626 patients studied, initial BED treatment was performed in 313 patients (50.0%), and pLT was performed in 313 patients (50.0%). Although patients who underwent pLT had a higher mortality rate within the first 3 months after the procedure, they had a reduced risk of long-term mortality compared with patients initially managed with BED treatment (hazard ratio [HR] ≥6 months after the initial procedure, 0.19; 95% CI, 0.08-0.42; P = .01). Patients requiring salvage liver transplant had a substantially higher risk of mortality than patients who received pLT (HR, 0.43; 95% CI 0.25-0.76; P = .003). Those who underwent pLT had superior survival compared with BED treatment recipients on or after 2002 (HR, 0.16; 95% CI, 0.05-0.54; P < .001), and that persisted when censoring patients who underwent salvage liver transplant (HR, 0.23; 95% CI, 0.07-0.82; P = .01).

Conclusions and Relevance  Patients who underwent pLT experienced superior long-term survival compared with patients who underwent BED treatment. Multi-institutional trials are needed to determine which initial treatment is most advantageous to patients with biliary atresia.

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