Liver Transplantation in a Patient with <i>Situs inversus</i> Associated with Biliary Atresia, Polysplenia and Absence of Inferior Vena Cava: A Case Report

Abstract

Biliary atresia (BA) is the result of a process of unknown etiology which can occur as an isolated lesion or in association with several congenital abnormalities and remains the main indications of liver transplantation among infants. One of the malformations associated with biliary atresia is Situs inversus totalis , which is a rare congenital abnormality found in 10% - 20% of infants with biliary atresia and presents an incidence varying from 1:5000 to 1:20,000. This case report aims to present an extremely rare case of a patient with Situs inversus associated with biliary atresia, polysplenia and abscence of inferior vena cava that underwent liver transplantation. Case Report: A 10-month-old boy was referred to our hospital with the diagnosis of cirrhosis, due to biliary atresia. Abdominal ultrasound and Doppler scan showed the liver in the left upper quadrant as well as signs of chronic liver disease. In addition, we could notice that the spleen had several lobes, suggesting polysplenia, and was positioned in the right upper quadrant. The patient underwent living donor liver transplant. His mother was the donor and the graft, segment III, was positioned in the left upper quadrant associated with a Y en Roux reconstruction of the biliary tract. During the first post-operatory day, the patient presented signs of disseminated sepsis. Laboratory and imaging diagnostic tests were performed presenting a small intestinal malrotation associated with diffuse ischemia. The patient died at the third day of PO due to complications related to this condition. Conclusion: Any patient with Situs inversus with acute abdomen should be aggressively investigated for other structural and biochemical abnormalities. If liver transplant is indicated, the procedure can be successfully performed.