Biliary atresia with rare associations: a case report

Abstract Background It is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. This is a rare association. Case reports A 2-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and a preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy). Discussion It is important to carefully look into the relationship between the preduodenal portal vein and biliary atresia because the patient is at risk of injury from this aberrant vein during operative intervention. Conclusion The relationship between biliary atresia and other congenital anomalies like preduodenal portal vein, intestinal malrotation, and situs inversus must be taken into consideration to avoid other diverse effects during surgical intervention.