Introduction: The most common congenital malformations of the bile duct are biliary atresia and choledochal cyst. In addition, the most common liver anatomical variation is the right hepatic artery aberration. The goal of this study is to characterize a patient with this disease and propose the hepatoduodenal anastomosis as surgical treatment.
Case report: One-month-old patient with suspected congenital biliary atresia due to progressive jaundice and acholia since birth. Liver tests consistent with a cholestatic pattern and brain MRI scan consistent with biliary atresia. Periods of decreased bilirubin and sporadic slight pigmentation of depositions were described. The surgical finding was a bile duct stricture due to a vascular ring caused by aberrant right hepatic artery. Resection of bile duct and hepatic-duodenal bypass were performed. The patient evolved satisfactorily from this condition.
Conclusion: There are few reports of biliary obstruction due to vascular malformations. It is important to keep in mind that not all neonatal jaundice episodes are caused by biliary atresia or choledochal cyst. The clinical course, laboratory tests and imaging should be considered and in the case of suspicion, further exploration should take place.