LeeVan E, et al. - Infants with biliary atresia initially treated with biliary-enteric drainage (BED) were compared with infants who underwent primary liver transplant (pLT) in terms of their survival. Outcomes of this cohort study including 626 infants with biliary atresia showed a markedly reduced risk of long-term mortality among patients who underwent pLT vs patients initially managed with BED procedure.

Methods

• Using deidentified records from the California Office of Statewide Health Planning and Development database, researchers performed a cohort study of patients with biliary atresia (n=1,252) between January 1, 1990, through December 31, 2015.
• They categorized patients into 1 of 2 cohorts: those who received BED treatment and those who underwent pLT.
• Those born before January 1, 1995 and those without any documented operative intervention by 5 years of age were excluded.
• From January 1, 1990, to December 31, 2015, they performed data analysis.
• The BED and pLT cohorts were compared regarding overall survival using the Kaplan-Meier method.
• By comparing survival before 2002 and on or after January 1, 2002, the investigators examined the treatment’s association with treatment era.

Results

• Of the 1,252 patients with biliary atresia who were identified, 626 remained following exclusion; 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, 313 patients (50.0%) were treated with initial BED treatment, and 313 patients (50.0%) underwent pLT.
• Despite a higher mortality rate within the first 3 months after the procedure, patients who underwent pLT showed 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=0.01).
• A substantially higher risk of mortality was evident for patients requiring salvage liver transplant than patients who received pLT (HR: 0.43; 95% CI: 0.25-0.76; P=0.003).
• Patients who underwent pLT demonstrated better survival than those receiving BED treatment on or after 2002 (HR: 0.16; 95% CI: 0.05-0.54; P < 0 .001), and this persisted when censoring patients who underwent salvage liver transplant (HR: 0.23; 95% CI: 0.07-0.82; P=0.01).