Biliary atresia associated with intestinal malrotation – unusual intraoperative presentation

Abstract

Introduction Biliary atresia (BA) is an idiopathic, progressive obliterative cholangiopathy of unknown etiology. The incidence of BA is 5–10 cases per 100,000 live births. Two clinical phenotypes of BA have been described: syndromic and non-syndromic form. From 1959, Kasai procedure is a standard therapeutic procedure. However, patients in whom there is an association of BA with other structural anomalies may have a worse outcome after the procedure. The goal was to present our patient with unusual form of BA associated with intestinal malrotation.

Case report We present a two-month-old female infant hospitalized because BA was suspected. On the echosonographic examination of the abdomen the gallbladder was not visible. Intraoperative diagnosis of BA was confirmed, and the Kasai procedure was performed. During the operation, intestinal malrotation with Ladd’s bands was identified. In this case, after the complete Ladd procedure, we decided to trace the Roux coil through the mesoduodenum and then behind the duodenum towards the portal plate, where a portoenterostomy was then created in a standard way. During the “follow-up” the infant was free of complaints, the stools were normally discolored, and the values of liver function tests had a downward trend.

Conclusion Any doubt about the diagnosis of BA obliges us to determine the existence of all other possible anatomical abnormalities and associated anomalies, due to their potential importance in changing treatment plans and surgical approach, but also the impact on the outcome of treatment.

Keywords: biliary atresia; Kasai procedure; malrotation