A 52-day female came c/o cholestatic jaundice, acholic stools, and a non-excreting hepatobiliary iminodiacetic acid (HIDA) scan. She underwent HPE and post which she developed developed multiple large biliary cystic lesions. Internal drainage of the bile lakes was accomplished using cystjejunostomy after the failure of percutaneous drainage. Post which the patient showed recovery.
A 52-day female underwent HPE for BA without any other malformationsafter she presented with cholestatic jaundice, acholic stools, and a non-excreting hepatobiliary iminodiacetic acid (HIDA) scan. Her abdominal ultrasound revealed a hypoplastic gallbladder without any evidence of intrahepatic abnormalities. At the time of her laparotomy done two days after presentation to our hospital, no identifiable gallbladder lumen was found so an intraoperative cholangiogram could not be performed. A Kasai HPE was completed, with bile drainage noted intraoperatively. Pathology revealed a fibrotic bile duct with patchy inflammatory infiltrate. The patient did well and was discharged home on the fifth postoperative day on fat soluble vitamins, ursodiol, and trimethoprim/sulfamethoxazole for cholangitis prophylaxis as per our practice.
By two months postoperatively, her total and direct bilirubin were 0.8 and 0.4 mg/dl respectively. She developed cholangitis three months after her surgery. Her abdominal ultrasound revealed diffusely prominent periportal echoes in the liver thought to represent periportal edema or inflammation as well as ascites. She was treated with a prolonged course of antibiotics. Her total and direct bilirubin levels peaked at 14.8 and 11.8 mg/dl respectively. Follow up imaging done three months after her initial cholangitis revealed possible cystic lesions at the porta hepatis. She was referred for liver transplant evaluation; however, listing was deferred due to her clinical improvement, resolution of her ascites, and improving total and direct bilirubin at that time to 5.4 and 3.7 mg/dl respectively. She was followed by her local provider for persistent cholestasis and poor weight gain in spite of nasogastric feeds and formula fortification. Her stools remained pigmented.
She presented to our institution six months after last seen with fever, cachexia, and a hard, large liver with a protuberant mass. She was admitted urgently and treated with intravenous broad spectrum antibiotics. Ultrasound suggested the presence of large intrahepatic cysts and magnetic resonance imaging (MRI) of her abdomen confirmed the presence of multiple branching fluid-filled structures throughout the liver consistent with dilated bile ducts and bile lakes. Blood cultures grew Citrobacter Freundii complex 12 h later. Percutaneous placement of two separate drains into these bile lakes resulted in resolution of her infection and marked improvement in jaundice. Biliary cultures grew the same organism. Over the next several weeks, drain output stabilized with about 200 mL of bile drainage from each side per day. She received nutritional rehabilitation and was listed for liver transplantation.
After two months of antibiotics and multiple percutaneous drain replacements, repeat abdominal imaging demonstrated improvement in her biliary dilation; however, bile lakes were persistent despite adequate drainage (total bilirubin decreased to 1.1 mg/dl) and resolution of jaundice. Given the clinical and laboratory improvement with adequate biliary drainage, we felt that internal surgical drainage would benefit our patient, so she was inactivated on the liver transplant list.
Upon re-exploration of her abdomen, moderate ascites and some features of portal hypertension were noted; the liver was firm with a micronodular appearance, which on biopsy demonstrated evidence of cholestasis and biliary-type cirrhosis. One of the bile lakes was noted on the surface of segments IVb/III where the anterior abdominal percutaneous drain was entering. The drain was removed and the cyst wall was excised. The adjacent jejunal roux limb from the previous HPE was anastomosed to the cyst wall in side-to-side configuration to create a cystjejunostomy. Of note, bile was noted both within the roux limb coming from the portoenterostomy as well as within the cyst. The other large posteriorly located bile lake that was being drained by the right flank percutaneous drain was not amenable to operative exposure without adding substantial morbidity, and was not pursued. She recovered well from this operation without apparent complications. The excised biliary cyst wall demonstrated benign fibrous tissue with no recognizable epithelial lining on pathology, consistent with a bile lake. Subsequently, her right flank percutaneous drain fell out resulting in a biliary fistula that was managed with drainage into a stoma bag until it spontaneously closed several months later.
After one cholangitis-free year, prophylactic antibiotic treatment (levofloxacin) was stopped. She developed cholangitis two months later and was retreated with antibiotics. At last follow-up, 18 months after surgery, she remains jaundice free (total and direct bilirubin pf 0.7 and 0.4 mg/dl, respectively) with adequate liver function and good growth and development.