Introduction: Biliary atresia (BA) is a well-known entity with an incidence of 0,8-1 per 10,000 live birth. BA can present with multiple congenital anomalies, in about 20% cases, such as polysplenia, situs inversus, intestinal atresias, and cardiovascular anomalies. But, the cooccurrence of vesicoureteral reflux (VUR) and BA is very uncommon. We report the clinicopathologic findings of a patient with BA associated VUR who underwent to the liver transplantation and nephroureterectomy in the same operation.

Case Report: A twenty two-months-old female child born of non-consanguineous marriage was reported to be icteric from first day of her life. She had antenatal history of bilateral hydronephrosis. Hepatobiliary scintigraphy showed that, there was no passage to the intestine. Liver biopsy revealed findings of BA. According to the results of the analysis, she underwent the Kasai operation when she was two-months-old. She was referred to our hospital when she was nineteen-months-old because of the complaints of jaundice, itching and increasing abdominal distension. On examination, the child had icterus, club¬bing, abdominal distantion, hepatomegaly, splenomegaly and ascites. Laboratory analysis revealed anemia, thrombocytopenia, direct hyperbilirubinemia, raised liver transaminases, hypoalbumine¬mia and prolonged prothrombin time. Renal scintigraphy showed decreased cortical uptake (acute pyelonephritis?) and bigger than the normal left kidney and atrophic right kidney. There was also bilateral VUR (grade 5 at right, grade 4 at left). She underwent both of the liver transplantation and right nephroureterectomy in the same operation at the age of 22 months. Histopathological evaluation revealed that cirrhosis with bile ductular and hepatocanalicular cholestasis widely in native liver. 6g weighing atrophic kidney with multiple parancimal cysts on cut surface and highly dilated ureter on gross examination of the nephroureterectomy specimen. Microscopic examination of the nephrectomy specimen showed diffuse sclerosis of glomeruli, diffuse interstitial inflamation and fibrosis with granulomas which was thought to developed secondery to the opaque material that is likely Tamm Horsfall protein. In addition chronic pyelonephritis ureteropelvic dilatation was diagnosed in pelvicaliceal system. In following few days after the operation the patient died from extrahepatic hematoma because of persistent thrombocytopenia.

Conclusions: A child having BA must be remain under investigation for associated anomalies include VUR that must be treated as early as possible because of its ability of rapid progress to chronic pyelonephritis and end-stage renal failure.