PMID- 30208381 OWN - NLM STAT- MEDLINE DCOM- 20191125 LR - 20200309 IS - 2168-6262 (Electronic) IS - 2168-6254 (Print) IS - 2168-6254 (Linking) VI - 154 IP - 1 DP - 2019 Jan 1 TI - Biliary-Enteric Drainage vs Primary Liver Transplant as Initial Treatment for Children With Biliary Atresia. PG - 26-32 LID - 10.1001/jamasurg.2018.3180 [doi] AB - 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. FAU - LeeVan, Elyse AU - LeeVan E AD - Department of Surgery, Huntington Memorial Hospital, Pasadena, California. FAU - Matsuoka, Lea AU - Matsuoka L AD - Division of Hepatobiliary and Liver Transplantation, Vanderbilt University Medical Center, Nashville, Tennessee. FAU - Cao, Shu AU - Cao S AD - Department of Preventive Medicine, Keck Hospital, University of Southern California, Los Angeles. FAU - Groshen, Susan AU - Groshen S AD - Department of Preventive Medicine, Keck Hospital, University of Southern California, Los Angeles. FAU - Alexopoulos, Sophoclis AU - Alexopoulos S AD - Division of Hepatobiliary and Liver Transplantation, Vanderbilt University Medical Center, Nashville, Tennessee. LA - eng PT - Comparative Study PT - Evaluation Study PT - Journal Article PL - United States TA - JAMA Surg JT - JAMA surgery JID - 101589553 SB - IM CIN - Hepatology. 2020 Feb;71(2):751-752. PMID: 31517392 MH - Biliary Atresia/mortality/*surgery MH - California MH - Drainage/*methods/mortality MH - Female MH - Humans MH - Infant MH - Kaplan-Meier Estimate MH - Liver Transplantation/*methods/mortality MH - Male MH - Risk Factors MH - Salvage Therapy/methods/mortality PMC - PMC6439864 COIS- Conflict of Interest Disclosures: None reported. EDAT- 2018/09/13 06:00 MHDA- 2019/11/26 06:00 PMCR- 2019/09/12 CRDT- 2018/09/13 06:00 PHST- 2018/09/13 06:00 [pubmed] PHST- 2019/11/26 06:00 [medline] PHST- 2018/09/13 06:00 [entrez] PHST- 2019/09/12 00:00 [pmc-release] AID - 2698957 [pii] AID - soi180054 [pii] AID - 10.1001/jamasurg.2018.3180 [doi] PST - ppublish SO - JAMA Surg. 2019 Jan 1;154(1):26-32. doi: 10.1001/jamasurg.2018.3180.