[Polysplenia syndrome associated to neonatal hepatitis]

OBJECTIVE: To report the unusual association between neonatal hepatitis and polysplenia syndrome.METHODS: Clinical, biochemical, histopathological, surgical and image analysis methods were used.RESULTS: The 36 days old patient presented hyperbilirubinemia with increase of direct reacting bilirubin and high alkaline phosphatase. The physical examination evidenced jaundice and hepatomegaly. The chest X-ray showed situs inversus and dextrocardia. Abdominal ultrasonography presented an increase in liver volume, mainly at the right lobe. There were two cystic lesions at the liver with absence of intrahepatic biliary tract dilatation. Absence of splenomegaly. Biliary scintigraphy didn't show any excretion of radioisotope to the duodenum while transoperative cholangiography presented contrast medium flowing to the duodenum lumen. During laparotomy it was possible to observe polysplenia, abdominal aorta to the right of the lower vena cava, gallbladder at the left lobe of the liver, appendix on the left side of the abdomen and sigmoid colon on the right side. The hepatic wedge biopsy was compatible with idiopathic neonatal hepatitis.CONCLUSION: The finding of polysplenia syndrome in patients with neonatal cholestasis doesn't necessarily indicate the recurrent presence of extrahepatic biliary atresia.