Long-term Results and Quality of Life Assessment in Biliary Atresia Patients: A 35-Year Experience in a Tertiary Hospital.

OBJECTIVES: To review long-term transplant-free survival and quality of life (QOL) of patients with biliary atresia (BA).

METHODS: A retrospective study reviewing all patients with Kasai operation between January 1, 1980 and December 31, 2015 was performed to evaluate the transplant-free survival. Subgroup analysis of patients older than 20 years was carried out to assess the QOL using the Short Form-36 Health Survey and incidences of disease-related complications. Comparison between patients with native and transplanted liver was performed using two-tailed independent samples t-test (P value < 0.05, significant).

RESULTS: The 20-year Kaplan-Meier transplant-free survival of the 141 patients in our study was 51%. The subgroup analysis of long-term survivors revealed a trend of increased prevalence of complications like esophageal varices, portal hypertension, and recurrent admissions in the patient groups with raised serum bilirubin (SB).Thirty-one patients were successfully contacted for QOL assessment, 26 (16 with native liver and 10 with transplanted liver) responded (76.5%). BA patients who were documented to have active complications have a significantly lower vitality score (50.7 vs 57.5, P = 0.015). There was no statistically significant difference in the scores between the transplanted group and the disease-free control group. However, the native liver group achieved a lower score in both the general health section (42.9 vs 49.6, P = 0.029) and the overall physical component (49.6 vs 54.4, P = 0.037).

CONCLUSIONS: A significant proportion of our patients survive with their native liver for more than 20 years. These long-term survivors may suffer from complications that impair their QOL. They require continuous life-long care.