University of Maryland Medical Center Midtown Campus, MD
Yuting Huang, MD, PhD1, Gordon Robbins, MD2, Justin Brilliant, MD2; 1University of Maryland Medical Center Midtown Campus, Rockville, MD; 2University of Maryland Medical Center, Baltimore, MD
Introduction: Sump syndrome, a collection of digested food, debris, stones, bile, and bacteria in a poorly drained, distal bile duct reservoir, is a complication of Roux-en-Y hepaticojejunostomy (RYHJ). While most cases occur at the hepaticojejunostomy sites, we report a rare case that occurred in the distal remnant CBD. The patient received a living donor liver transplant and presented with recurrent bacteremia and symptoms of acute cholangitis.
Methods: A 38 y.o. woman with a history of ileocolonic Crohn’s disease in remission, end stage liver disease secondary to primary sclerosing cholangitis, history of living donor liver transplantation via RYHJ, and Klebsiella Pneumoniae bacteremia with no identified source 3 months ago, presented with sharp right upper quadrant (RUQ) abdominal pain, fevers to 101.2 F, and positive blood cultures for Klebsiella Pneumoniae. MRCP showed a biliary stricture in the remnant CBD, associated with new edema and surrounded infiltration; the diameter of the segment proximal to the stricture had been stable for 2 years (6 mm). Neither PTC and ERCP were pursued at that time. Three months later, the patient returned with 2 days of sharp epigastric and RUQ pain associated with emesis, fevers, chills, and poor appetite. Vital signs were stable. Physical examination was significant for severe tenderness over the epigastrum and RUQ. Liver function tests were at her baseline. Workup for Crohn's disease flare and transplant rejection were negative. Repeated MRCP showed an increase in the diameter of the proximal segment of the remnant CBD from 6 to 9 mm along with persistent surrounding edema. ERCP demonstrated lower third of the CBD contained a single moderate stenosis 5 mm in length, which was successfully dilated with a 6 mm balloon. Pus was swept from the cystic duct. The hepaticojejunostomy. anastomosis was intact, with no evidence of strictures or recurrence of PSC. Following successful sphincterotomy, the patient’s abdominal pain, fever, and leukocytosis resolved. Blood cultures showed no growth. The patient was discharged with a diagnosis of sump syndrome and was maintained on sulfamethoxazole-trimethoprim at discharge. Discussion: In patients with history of RYHJ who present with symptoms of acute cholangitis with normal liver function, the diagnosis of sump syndrome of the distal CBD remnant should be considered. Prompt ERCP for biliary sphincterotomy can help treat infection and provide appropriate source control.
Figure 1. Anatomy illustration for the presenting case. Patient had living donor right liver transplantation via Roux-en-Y hepaticojejunostomy. The diameter of the proximal segment of the remnant CBD from 6 to 9 mm
Figure 2. Fluoroscopy showed distal common bile duct (CBD) remnant stricture (A). CBD become patent after dilation of the stricture (B). The biliary tree via hepaticojejunostomy are intact, no strictures or recurrence of primary sclerosing cholangitis (C).
Figure 3. Pus drainage from the remnant common bile duct after balloon dilation.
Disclosures: Yuting Huang indicated no relevant financial relationships. Gordon Robbins indicated no relevant financial relationships. Justin Brilliant indicated no relevant financial relationships.