Clinical characteristics of cystic biliary atresia

Abstract

Background: Cystic biliary atresia (BA) is a unique form of BA that can be mistaken for a choledochal cyst. This study reviews the clinical characteristics of cystic BA to better understand its nature.

Objective: To characterize cystic biliary atresia in terms of clinical presentation, imaging findings, and surgical outcomes, and to highlight key features that aid in differentiating it from choledochal cysts.

Methods: We retrospectively reviewed the charts of all infants diagnosed with cystic BA from January 2005 to December 2019. The diagnosis of BA was confirmed in all cases by intraoperative cholangiography. Moreover, clinical data, pre-operative laboratory and imaging, intraoperative findings, and postoperative outcome were recorded, and data were expressed as mean ± standard deviation.

Results: Out of the 227 patients with BA, there were 14 patients with cystic BA (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, and 11 of 14 patients underwent a Kasai operation. The mean age at the time of Kasai operation was 95.3 ± 37.6 days, and only 1 in 11 patients (9.0%) underwent a Kasai operation before 60 days. Moreover, 6 patients (54.5%) underwent a Kasai operation at 61–90 days, whereas 4 patients (36.3%) underwent a Kasai operation at 113– 154 days. Intraoperative findings revealed BA type III proximal to the cysts in all cases. Based on the surgeons’ decision, 3 patients did not receive a Kasai operation, and only a liver biopsy was performed. After the Kasai operation, 8 out of 11 patients (72.7%) experienced no jaundice within 3 months. In addition, gross cirrhosis was recorded by the surgeons in 9 patients, and of these patients, 7 underwent a Kasai operation. Interestingly, 5 of the 7 patients with gross cirrhosis undergoing surgery (71.4%) were jaundice-free within 3 months. Briefly, an overall jaundice-free state was achieved in 72.7% of patients with cystic BA who underwent a Kasai operation. In addition, 71.4% of patients with cystic BA and gross cirrhosis undergoing surgery were jaundice-free.

Conclusion: Cystic BA is a rare pathology that needs to be distinguished from choledochal cysts in neonates. Awareness of the existence of cystic BA may facilitate the diagnosis and dissection around the porta hepatis area during the Kasai operation. This may increase the chance of better results for the patients. Even in the presence of gross cirrhosis, the Kasai procedure may still be considered to provide an opportunity for bile drainage.