Abstract
INTRODUCTION: Biliary atresia (BA) is a rare condition where the hepatic or common bile ducts get obliterated by fibrous tissue for unknown causes. It is diagnosed in 5-10 per 100,000 live births worldwide in whom liver injury and death are definite if untreated. PRESENTATION: We report a case of BA with associated malrotation and situs ambiguous discovered incidentally during Kasai procedure. The small intestine was found to be malrotated with the duodenojejunal junction to the right of the vertebral column. The liver, stomach, and spleen were in the center, right side, and left side of the abdomen respectively. The malrotation was corrected by performing a Ladd's procedure in addition to the Kasai. She had an uneventful postoperative course. At follow-up on the second, sixth and 12th weeks postoperatively, she had normal stool color and liver function. DISCUSSION: BA patients with associated structural anomalies might have a worse outcome after the Kasai procedure. Several studies demonstrated that the coexistence of malrotation with BA to be in the range of 0.3%-9.4% among BA patients. Also, the presence of situs abnormalities with BA has been reported in some studies to be present in 0.01-0.045% of BA cases. However, the presentation of BA with both situs inversus and intestinal malrotation has seldom been reported. CONCLUSION: The diagnosis of BA should prompt further investigation for other anatomical abnormalities as the presence of which might affect the management plan.