LAUSR

A 6-year-old boy was evaluated for abnormal liver function tests following orthotopic liver transplantation with Rouxen-Y reconstruction for biliary atresia at 3 months of life. He was known to have chronic hepatic arterial, portal venous, and inferior vena caval occlusion with extensive venous collaterals. Percutaneous liver biopsy demonstrated large duct obstruction without signs of rejection. Vital signs were notable for a weight of 19.5 kg (24th percentile weight for age), height of 108.5 cm (4th percentile height for age), heart rate of 95 beats/min, temperature of 36.7 °C, and oxygen saturation of 100% on room air. Laboratory studies were notable for thrombocytopenia (platelet count of 39,000/μL), elevation of alkaline phosphatase (196 IU/L) and bilirubin (2.1 mg/dL). Magnetic resonance cholangiopancreatography (MRCP) revealed diffusely dilated intrahepatic bile ducts with central intrahepatic stricture, marked splenomegaly, and nodularity of the liver, consistent with cirrhotic changes. MRI using gadoxetate contrast, a hepatocyte-specific agent that is excreted into the biliary tree, facilitated the acquisition of images during the hepatobiliary phase of excretion. Excretion of gadoxetate was noted on delayed phase imaging into a loop of bowel (Fig. 1). Physical examination revealed a boy who was crying, with dry mucous membranes, healed abdominal incisions consistent with his past surgical history, and no abdominal tenderness. Single-balloon enteroscopyassisted endoscopic retrograde cholangiopancreatography (ERCP) revealed gastric varices (Fig. 2), no bile in the Roux limb and no evidence of hepaticojejunostomy. Curiously, pooled bile was evident within the duodenal bulb. A combined endoscopic and interventional radiology rendezvous procedure was then undertaken. Percutaneous access into a right posterior bile duct was obtained; contrast injection into the biliary tree revealed dilated right-sided intrahepatic bile ducts with central occlusion and initially no filling of the left lobe bile ducts. A percutaneous catheter was placed centrally after which a small channel of contrast was noted to flow from the biliary tree into the duodenal bulb (Fig. 3). Initial attempts at biliary cannulation were not successful until percutaneous cholangioplasty of the left hepatic duct stricture was performed, which finally enabled a glide wire to pass through the fistula into the duodenal bulb. This wire was then exchanged for a long wire that was captured by the endoscopist. At the conclusion of the procedure, a 7 French × 3 cm plastic stent was placed across the fistula tract and stenotic central bile ducts, with its proximal tip in the right intrahepatic bile duct and distal tip in the duodenal bulb. Good drainage of bile was noted from the stent following placement. Ultrasound post-intervention revealed a significant reduction in biliary dilation relative to preprocedure sonographic evaluation in both the right and left hepatic ducts. Liver function tests normalized in the weeks post-ERCP.