Mirizzi Syndrome

The presentation of Mirizzi syndrome is usually that of acute or chronic cholecystitis with the addition of jaundice. Patients with chronic cholecystitis usually present with dull right upper abdominal pain that radiates to the mid back or right scapular tip. It is usually associated with fatty food ingestion. Nausea and occasional vomiting also accompany complaints of increased bloating and flatulence. Often the symptoms occur in the evening. Prolonged less acute symptoms are usually present over weeks or months. Increased frequency and severeness of acute exacerbations (acute biliary colic) is usually seen in the presence of more prolonged chronic symptoms. The classic physical examination will demonstrate right upper abdominal pain with deep palpation (Murphy's sign). Patients are usually not acutely ill but are uncomfortable. Patients with advanced Mirizzi syndrome or of more severe acute cholecystitis may present with more pronounced symptoms and findings. Jaundice is usually present, and at times, significantly elevated bilirubin can be identified.[6]

The routine workup for cholecystitis should be initiated. The best test for diagnosing gallstones and subsequent acute cholecystitis is a right upper quadrant abdominal ultrasound. It is associated with a 90% specificity rate and depending on the ultrasound operator, can detect stones as small as 2 mm as well as sludge and gallbladder polyps. Ultrasound findings that point toward acute cholecystitis versus cholelithiasis include gallbladder wall thickening (greater than 3 mm), pericholecystic fluid and a positive sonographic Murphy's sign. Gallstones can also often be found on CT scans, and MRIs, however, these studies are not as sensitive for acute cholecystitis. Approximately 10% of gallstones may be found on routine plain films due to their high calcium content. Air in the biliary tree may also be detected on these radiographic studies if there is an enteric fistula present. If there is a suspected stone in the common bile duct based on ultrasound results, magnetic resonance cholangiopancreatography (MRCP) is the next step. If a common duct stone is identified on the MRCP, then the gold-standard test of an endoscopic retrograde cholangiopancreatogram (ERCP) should be performed by a gastroenterologist. A percutaneous transhepatic cholangiogram (PTHC) is also useful in diagnosing common bile duct stones if an ERCP is not possible. Usually, the diagnosis of Mirizzi syndrome is either mistaken for a simple common bile duct stone or is missed entirely on preoperative workup.[7]

The treatment for Mirizzi syndrome is cholecystectomy. Laparoscopic cholecystectomy is preferable, but if the condition is advanced, then a more involved surgery may be needed. An open cholecystectomy is an option. In cases of a more progressed disease, a partial cholecystectomy can be considered. This would involve leaving Hartman's pouch in place and removing the body of the gallbladder and the gallstones. This will lower the incidence of injury to the porta hepatis and bile ducts. If a fistula is present then an open cholecystectomy with bilioenteric anastomosis possibly with a roux-n-y has been shown to be effective.[8][9]

Many other conditions can mimic gallbladder disease. Patients who present with acute biliary colic are often worked up for cardiac issues. Other common conditions with similar presenting symptoms are:[8]

• Peptic ulcer disease
• Irritable bowel disease
• Inflammatory bowel disease
• Gastroesophageal reflux disease
• Pulmonary embolism

The presence of jaundice in combination with the other presenting symptoms in Mirizzi syndrome is usually mistaken for other etiologies such as:

• Common bile duct stones
• Ascending cholangitis
• Biliary cancer
• Pancreatic tumors

Other medical conditions to consider, that may mimic Mirizzi syndrome include:

• Acute hepatitis
• Ischemic liver disease
• Drug-induced hepatitis

In patients without fistula formation, the surgical repair and outcome is usually favorable. Due to the distorted anatomy and high rate of conversion to open cholecystectomy for this disease; however, some sources recommend an open surgical approach for all patients affected by Mirizzi syndrome. The prognosis for patients with fistula formation involves prolonged treatment with T-tube placement through the small-moderate sized fistula or biliary diversion with a choledochoduodenostomy or a Roux-en-Y choledochojejunostomy for larger fistulas. Prolonged surgical and hospital course for the latter group of patients increases their risk of complications and increases the morbidity and mortality rate for these patients as well. Gallbladder cancer has been associated with Mirizzi syndrome as well, although this is thought to be due to prolonged inflammation leading to both diseases, rather than a result of Mirizzi syndrome itself.[10] In elderly patients with multiple comorbidities and high risk of surgical complications, nonoperative methods should be considered to minimize morbidity associated with the surgery.[11]

The most common complication of Mirizzi syndrome is cholecystobiliary or cholecysto-enteric fistula formation due to prolonged inflammation. Surgical complications with prolonged procedure time due to dense adhesions may also occur. These include bile duct injury and hemorrhage. Massive hemorrhage during dissection of the Calot triangle can occur in complex cases. Other complications of prolonged inflammation that can be seen in patients with Mirizzi syndrome include:

• Cutaneous fistula formation
• Secondary biliary cirrhosis
• Delayed onset biliary strictures[12]

Patient education on medical compliance, clinical follow-up and wound care is essential, especially in patients who required stent placement, biliary diversion, choledochoduodenostomy, or a Roux-en-Y choledochojejunostomy. Patients should be educated intensively on discharge and referred to wound care nursing if needed to prevent secondary infections and their associated complications. 

Mirizzi syndrome often is not diagnosed preoperatively. Awareness of this condition with the knowledge of appropriate treatment is essential. The surgeon must also entertain the possibility of the presence of occult gallbladder cancer.

An interprofessional approach must be taken for this condition. Accurate diagnosis with an accurate evaluation of the extent of this disease must be concluded by all involved. The surgeon is the main specialist for diagnosis and treatment but familiarity and a high degree of suspicion of this condition must be present by the initially treating primary caregiver or emergency physician. Delay in making an adequate diagnosis and treatment could lead to the progression of this condition with increased patient morbidity and mortality.