M. B. Liu1, X. Hoang3, T. B. Huong3, H. Nguyen3, H. T. Le4, A. Holterman2 1Stanford University School Of Medicine,Stanford, CA, USA 2University Of Illinois College Of Medicine At Peoria,Department Of Surgery/Pediatric Surgery,Peoria, IL, USA 3National Hospital Of Pediatrics,Hepatology Department,Hanoi, HANOI, Viet Nam 4National Hospital Of Pediatrics,Hanoi, HANOI, Viet Nam
Introduction: While the natural evolution of operated biliary atresia (BA) patients who undergo the Kasai portoenterostomy is well documented, untreated biliary atresia is not a common occurrence in developed countries and has not been well characterized. The objectives of this study were to further characterize unoperated biliary atresia patients and their survival course in Vietnam, a developing country.
Methods: A retrospective chart review was undertaken of the demographics and clinical characteristics of patients diagnosed with biliary atresia between January 2012 and July 2013 at one hospital in Vietnam. Patients identified as unoperated biliary atresia cases were contacted to obtain survival data.
Results: A total of 84 patients (60 patients for 2012 and 24 patients for 2013) were identified as unoperated biliary atresia cases out of a total of 178 patients who were diagnosed with biliary atresia within the same timeframe. Mean age at diagnosis for the unoperated BA patients was 100+/-84 days with a median of 69 days. The majority (54%) present within 2 months of life (10% within 45 days); 33%, 21% and 12% present after 3, 4, and 6 months of age respectively. At the time of presentation, the mean +/- SD values for total bilirubin values were 10.3+/-4.5 mg/dL (normal between 0.1-1.0 mg/dL). Those for ALT were 141+/- 88u/L (normal <42 u/L) and for PELD scores were 15+/-21 (median of 10-15). The reasons for no surgical treatment were parents’ refusal of the Kasai surgery, late diagnosis, or lack of access to primary liver transplantation. Follow-up data was limited since only 12% had at least 1 readmission at the same hospital for complications after their initial diagnosis. The remaining 88% did not return for further management. Follow-up survival and mortality data was obtained for 34 patients out of the 84 unoperated BA cases. The remaining patients could not be contacted. Of the 34 patients, 7(20%) were still alive as of August 2013. These 7 patients had been alive for an average of 9.5+/-3.6 months at the time of being contacted. The remaining 27 deceased patients had a median lifespan of 7.4 months.
Conclusion: Our data provide the most recent survival outcomes for patients with unoperated biliary atresia. They illustrate the multiple causes for the significant medical burden of patients from Vietnam, including delays in presentation, parents’ refusal of surgical treatment, and their lack of access to follow-up care. Innovative non-invasive palliative therapy may be more acceptable to these families to improve the survival and quality of life for these patients.