Clinical Characteristics of Cystic Biliary Atresia

Background & Objective: Cystic biliary atresia (BA) is a unique form of BA. Our objective was to review the clinical characteristics of cystic BA in order to better understand its nature. Methods: We retrospectively reviewed all infants diagnosed with cystic BA during January 2005 to December 2019. The diagnosis of BA was confirmed in all cases by laparotomy. Clinical data, pre-operative laboratory and imaging, intraoperative findings, and post-operative outcome were recorded. Data were expressed as mean and SD. Results: There were 14 cystic BA patients out of the 227 BA patients (6.2%) during the studied period. The mean pre-operative ultrasound size at the maximal part of the cyst was 2.1 ± 1.2 cm. Eleven of 14 patients underwent Kasai operation. The mean age at the time of Kasai operation was 95.3 ± 37.6 days. Only 1 in 11 patients (9.0%) underwent Kasai operation before 60 days. Moreover, 6 patients (54.5%) underwent Kasai operation at 61 - 90 days whereas 4 patients (36.3%) underwent Kasai operation at 113 - 154 days. Intra-op findings revealed BA type III proximal to the cysts in all cases. Based on their surgeons’ decision, 3 patients did not receive Kasai operation, only liver biopsy was performed. After Kasai operation, 8 out of 11 patients (72.7%) had no jaundice within 3 months. There were 9 patients whose gross cirrhosis were recorded by the surgeons. Seven of the 9 patients received Kasai operation. Interestingly, 5 of the 7 patients with gross cirrhosis undergoing surgery (71.4%) were jaundice-free within 3 months. Briefly, overall jaundice-free rate was achieved in 72.7% of cystic BA patients undergoing Kasai operation. In addition, 71.4% of cystic BA patients with gross cirrhosis undergoing surgery was jaundice-free. Conclusion: Cystic BA is a rare pathology to be distinguished from choledochal cyst in neonates. Awareness of the existence of cystic BA may facilitate the diagnosis and dissection around porta hepatis area during Kasai operation. This may increase the chance of better results for the patients. Prognosis of cystic BA after Kasai operation for the early outcome seems to be promising even with gross cirrhosis.