A Purely Endoscopic Management Approach for Type 5 Meritzi Syndrome These are our disclosures. We present a case of a 94-year-old female with a past medical history of type 2 diabetes, hypertension, and hypothyroidism. She was admitted to an outside hospital following a mechanical fall and was found to have acute kidney injury. On day 5 of admission, she developed a fever, abdominal pain, and cholestatic liver enzyme elevation with an alkaline phosphatase level of 1000 and total bilirubin of 5.8. Blood cultures were positive for MRSA and MRCP showed a large calcified gallstone in the cystic duct measuring 4.6 by 2.2 by 2 cm with compression of proximal common bile duct and associated common hepatic and intrahepatic duct dilation. Overall clinical picture was consistent with acute cholangitis. The following day, the patient became hemodynamically unstable and underwent urgent ERCP. Cholangiogram revealed common hepatic and intrahepatic duct dilation with many large stones in the cystic duct and gallbladder. A plastic biliary stent was placed. Sphincterotomy was not performed due to prolonged INR of 1.9. Five days later, the patient was transferred to our hospital. Due to ongoing abdominal pain and persistent elevation in liver enzymes, a repeat ERCP was performed. The patient's previously placed bile duct stent was removed with a snare and a sphincterotomy was then performed. Contrast was injected. Cholangiogram revealed a dilated cystic duct with multiple large filling defects consistent with cystic duct stones. Common bile duct was not dilated but appeared to be compressed externally from the stones. Common hepatic and intrahepatic ducts were dilated. This was suggestive of type 1 Meritzi syndrome. Due to the large stone burden, the decision was made to proceed with cholangioscopy. A sphincteroplasty was performed with an 8mm dilating balloon to facilitate passage of the cholangioscope as well as stone clearance. The cholangioscope was then advanced into the bile duct over a 0.035 inch guide wire. The cholangioscope was advanced to the hilum, revealing no stones in the common hepatic duct or proximal common bile duct. During withdrawal, a large impacted stone was seen at the cystic duct takeoff. Due to the size of the stone, the decision was made to proceed with electrohydraulic lithotripsy. EHL was performed to fragment the large stone. A total of two EHL probes were used. Occlusion cholangiogram was performed, revealing no stones in the common bile duct. There was, however, a residual stone in the cystic duct, which had significantly decreased in size following EHL. During occlusion cholangiogram, there was also evidence of extravasation of contrast from the gallbladder into the colon, suggestive of a cholecystocolonic fistula. A cholecystocolonic fistula is a communication between the gallbladder and the colon. When this occurs in conjunction with any other type of a Meritzi syndrome, such as type 1 Meritzi syndrome in our patient, this is referred to as type 5 Meritzi syndrome. Given the presence of the residual cystic duct stone, a plastic biliary stent was placed. Following ERCP, the patient's abdominal pain improved significantly. One week later, a colonoscopy was performed to evaluate the fistula. A pediatric colonoscope fitted with a plastic cap was advanced to the hepatic flexure, where several small gallstones were seen. Close inspection of the colonic mucosa revealed a small mucosal defect with intermittent extrusion of microvilli. The defect was carefully probed with an ERCP cannula. Contrast was injected, and fluoroscopic images showed collection of contrast in the gallbladder, followed by gradual appearance of a colangiogram. This confirmed correct location of the cholecystocolonic fistula. The decision was made to close the fistula using an over-the-scope clip. Argon plasma coagulation was used to deepithelialize the fistula tract. An endoclip was then placed to mark the area for easy identification. The scope was then withdrawn and fitted with a 14x6mm type T over-the-scope clip. The scope was carefully advanced to the hepatic flexure, and the previously placed endoclip was identified. The mucosal defect was found and centered in the middle of the clip. The defect was completely sectioned into the cap, and the clip was deployed. Following deployment, the clip appeared to be in good position. Contrast was injected once again and revealed no further opacification of the biliary tree, confirming complete closure of the cholecystocolonic fistula. Following the procedure, the patient's abdominal pain resolved completely. She returned 5 months later to undergo her final ERCP for both biliary stent removal and to ensure complete stone clearance. A scout film revealed the previously placed over-the-scope clip in the right upper quadrant. Chlangioscopy was then performed, revealing a residual stone at the cystic duct take-off. This was treated with EHL using one probe. Following EHL, multiple balloon sweeps yielded small stone fragments. Occlusion cholangiogram showed no further filling defects in the biliary tree, confirming complete stone clearance. Clinical Implications As depicted in this table, surgical management is recommended for all types of Murtzy syndrome, while ERCP is only considered a feasible option for type 1 Murtzy syndrome. The treatment of patients with symptomatic type 5 Murtzy syndrome consists of surgery with cholecystectomy, fistula takedown, and possible colonic resection. The role of treatment of asymptomatic patients is unclear. Conclusions Our patient was successfully treated with ERCP and EHL for management of a large impacted cystic duct stone, resulting in biliary obstruction and acute cholangitis. Endoscopic management of a concomitant cholecystocolonic fistula using an over-the-scope clip was also successful. Although prior case reports describe surgical management as the recommended treatment option for type 5 Murtzy syndrome, this case presents the safety and efficacy of endoscopic management.

acute kidney injury

cholestatic liver enzyme elevation

cholecystocolonic fistula

endoscopic management

MRCP