This is a case of a girl born with citrullinemia diagnosed in her firstweek of life after presenting with hyperammonemia and encephalopathy. At 1 year of age, she received a deceased donor segmental split left LT due to recurrent and refractory metabolic decompensations despite optimal medical and dietary management. During the initial LT, a duct-to-duct anastomosis was performed. One week later, the patient required a revision of the biliary anastomosis due to a bile leak and subphrenic abscess. During revision, the end-toend anastomosis was taken down, and a bilio-enteric anastomosis was created using a Roux-en-Y hepatojejunostomy. A surgical drain remained in place for 1 month, after which a cholangiogram revealed stenosis of the anastomosis with a biliary stricture. The biliary drain
AbstractThis case report describes an 8-year-old girl who underwent a segmental LT for a primary diagnosis of citrullinemia at the age of 12 months. She presented with cholangitis secondary to stenosis of the biliary-enteric anastomosis. MRI revealed dilatation of intrahepatic bile ducts associated with multiple stones. An endoscopic approach failed to decompress the bile ducts and remove the stones. A percutaneous approach was then undertaken. After placement of a temporary external biliary drain for 12 days, a 26 French sheath was placed to access the bile ducts. Using a 14Fr flexible cystoscope, 80%-90% of the biliary stones were removed. This was followed by antegrade balloon dilatation of the biliary-enteric anastomosis. Two months later, the procedure was repeated, resulting in complete clearance of the biliary stones. An internal-external biliary drain was maintained in placed for 10 months. The patient has been asymptomatic, with no evidence of stone recurrence for 13 months after drain removal. Percutaneous biliary stone removal is commonly performed in adults with non-transplanted livers, especially in complex cases, and has also been shown to be successful in the pediatric population. However, it is rarely reported in transplanted livers in adults, and to the best of our knowledge, no pediatric cases have been reported. This case illustrates that this technique can be successfully utilized in pediatric LT patients. K E Y W O R D S biliary complications, biliary stones, citrullinemia, liver transplantation, pediatric liver transplant, percutaneous stone removal How to cite this article: Rohringer TJ, Hannick JH, Lorenzo A, Avitzur Y, Temple M, Parra DA. Percutaneous removal of biliary stones post-liver transplant in a pediatric patient: Case report and review of the literature. Pediatr Transplant.