Mirizzi syndrome type IV associated with cholecystocolic fistula: a very rare condition--report of a case

Abstract

Background: Mirizzi syndrome is a rare complication of prolonged cholelithiasis with presence of large, impacted gallstone into the Hartman's pouch, causing chronic extrinsic compression of common bile duct (CBD). Fistula formation between the CBD and the gallbladder may represent an outcome of that condition. According to Mirizzi's classification and Csendes's subclassification, Mirizzi syndrome type IV represents the most uncommon type (4%). Spontaneous biliary-enteric fistulas have also been rarely reported (1.2-5%) in a large series of cholecystectomies. Cholecystocolic fistula is the most infrequent biliary enteric fistula, causing significant morbidity and representing a diagnostic challenge.

Case presentation: We describe a very rare, to our knowledge, combination of Mirizzi syndrome type IV and cholecystocolic fistula. A 52 year old male, presented to our clinic complaining of episodic diarrhea (monthly episodes lasting 16 days), high temperature (38 degrees C-39 degrees C), right upper quadrant pain without jaundice. The definitive diagnosis was made intraoperatively. Magnetic Resonance Imaging (MRI) and Endoscopic Retrograde Cholangiopancreatography (ERCP) demonstrated the presence of Mirizzi syndrome with cholecystocolic fistula formation. The patient was operated upon, and cholecystectomy, cholecystocolic fistula excision and Roux-en-Y biliary-enteric anastomosis were undertaken with excellent post-operative course.

Conclusion: Appropriate biliary tree imaging with ERCP and MRI/MRCP is essential for the diagnosis of Mirizzi syndrome and its complications. Cholecystectomy, fistula excision and biliary-enteric anastomosis with Roux-en-Y loop appears to be the most appropriate surgical intervention in order to avoid damage to Calot's triangle anatomic elements. Particularly in our case, ERCP was a valuable diagnostic tool that Mirizzi syndrome type IV and cholecystocolic fistula.

Figure 1

Spiral CT with evidence of pneumobilia and suspicion of cholecystocolic fistula.

Figure 2

MRI:T1 and T2 weighed images with iv contrast Gadolinium- Bopta, revealing fistulous tract between the right colonic flexure and gallbladder (cholecystocolic fistula) and a large gallstone (2 cm).

Figure 3

Endoscopic retrograde cholangiopancreatography. Severe common bile duct stenosis, with wide cholecystocholedochal communication. A shrunk gallbladder with a large gallstone and a secondary formation of a fistoulous tract, the right hepatic flexure with the contrasted colonic haustration are the highlights of this image modality.

Figure 4

Schematic representation of Csendes classification for Mirizzi syndrome [4].