Chronic biloma after right hepatectomy for stage IV hepatoblastoma managed with Roux-en-Y biliary cystenterostomy

Abstract

We report the complex case of a 12-month-old girl with stage IV hepatoblastoma accompanied by thrombosis and cavernous transformation of the portal vein. After neoadjuvant chemotherapy, she underwent right hepatectomy, which was complicated by iatrogenic injury of her left hepatic duct, and subsequently developed a postoperative biloma and chronic biliocutaneous fistula. Concomitant with multiple percutaneous interventions to manage the biloma nonoperatively while the child completed her adjuvant chemotherapy, she progressed to develop chronic malnutrition, jaundice, and failure to thrive. Once therapy was completed and the child was deemed free of disease, she underwent exploratory laparotomy with Roux-en-Y biliary cystenterostomy for definitive management, resulting in resolution of her biliary fistula, jaundice, and marked improvement in her nutritional status. Roux-en-Y biliary cystenterostomy is a unique and efficacious management option in the highly selected patient population with chronic biliary leak refractory to minimally invasive management.

Figure 1

(A) PRETEXT II hepatoblastoma (hbl) involving the right anterior and right posterior liver sections with impingement on the middle hepatic vein (mhv) and inferior vena cava (ivc). The portal vein was not well visualized on this study. (B) Coronal view of CT scan prior to resection, but after neoadjuvant chemotherapy, demonstrates regression of hepatoblastoma (hbl) from the middle hepatic vein (mhv) and the presence of numerous portal varices (*) consistent with cavernous transformation of the portal vein. (C) Intraoperative cholangiogram prior to right hepatectomy illustrates the right hepatic duct (rhd) an abrupt cutoff in the left hepatic duct (lhd) due to suture ligation injury and patency of the common bile duct (duct) with free flow of contrast into the duodenum (duo). (D) First cholangiogram documenting a postoperative biloma (bil) in communication with the common bile duct (cbd). (E) Postoperative percutaneous transhepatic cholangiogram (catheter = ptc) demonstrating communication between the biloma (bil) and hepatic ductal system (hds), but not the common bile duct, suggesting stricture. (F) PTC after roux-en-Y biliary cyst enterostomy demonstrating flow of contrast from the hepatic ductal system (hds) into the roux limb (rl).