S.M. Larabee2, S. Peiffer1,2, B. Jeon1,2, S. Vasudevan1,2, A. King1,2 1Baylor College of Medicine, Michael E. DeBakey Department Of Surgery, Houston, TEXAS, USA 2Texas Children’s Hospital, Department Of Surgery, Division Of Pediatric Surgery, Houston, TEXAS, USA
Introduction: Biliary atresia (BA) occurs in 1/15,000 live births causing progressive fibroobliteration of the extrahepatic biliary tree in the neonatal period. Management includes early surgical management with possible need for liver transplantation. We aim to characterize patient care and outcomes of children with BA in Texas.
Methods: We retrospectively reviewed a statewide hospital inpatient discharge database (2016-2022) to identify patients <18 years with BA using International Classification of Disease 10 code, excluding patients transferred to outside hospitals to avoid double-counting admissions. Descriptive statistics and chi-squared tests were performed using STATA software.
Results: 1,739 admissions with BA (<28 days 301; 28-365 days 1,011; 1-4 years 318; 5-9 years 58; 10-14 years 25; and 15-17 years 26) were identified. Demographics include 50% female and a majority white (56.8%) with 9.5% residing in rural regions and 11% from the US-Mexican border.
Newborns accounted for 29.2% of admissions with 37.4% of newborns these being discharged at <28 days of life (DOL) and 62.2% at 28-365 DOL. The majority of overall admission occur during infancy and trend down with increasing age. Of admissions of patient >1 year, a majority where classified as emergent (34.6-63.8%), though most were cared for on a pediatric floor (59.1-70.1%) as compared to ICU (27.2-36.4%). Further, while 18.3% of <28 DOL newborns are admitted to a CSV level 1 center, the proportion of patients at CSV centers increases with age (43.3-81.0%). While 33.6% of <28 DOL admissions underwent an OR procedure during their admission, all other age groups has significant OR procedures associated with their admission (59.1-77.8%). The longest median length of stay was noted in infants 28-365 days at 29 days [IQR 8-68]. There are no difference in daily costs across ages. Mortality across ages was noted to be higher in patients <1 year accounting for 91% of all inpatient hospital deaths. The most common diagnoses associated with admissions are hepatobiliary procedures, liver transplant, diabetes, and sepsis. Of the 81 liver transplants performed, a majority were for at 1-4yr (51.8%), followed by 28-356d (32.1%), with continued need for transplantation at 5-9y (7.4%), 10-14y (4.9%), and 15-17y (3.7%).
Conclusion: Across Texas, patients with BA have high initial admission rates, high burden of care and associated admissions costs. While the majority of care occurs early in life, the need for emergent and operative intervention including transplant remains throughout their pediatric care.