Mirizzi Syndrome – Causes, Symptoms, Treatment

Causes of Mirizzi Syndrome

Gallstones are usually formed from bile that is in stasis. When bile is not fully emptied from the gallbladder, it can precipitate as sludge and subsequently turn into stones. Biliary obstruction may also lead to gallstones including bile duct strictures and cancers, such as pancreatic cancer. The most common cause of cholelithiasis is from the precipitation of cholesterol? that subsequently forms into cholesterol stones. The second form of gallstones is pigmented gallstones which are the result of increased red blood cell destruction in the intravascular system causing increased concentrations of jaundice?. সহজ বাংলা: জন্ডিসে বাড়তে পারে এমন হলুদ রঞ্জক।" data-rx-term="bilirubin" data-rx-definition="Bilirubin is a yellow pigment that can build up in jaundice. সহজ বাংলা: জন্ডিসে বাড়তে পারে এমন হলুদ রঞ্জক।">bilirubin? which subsequently get stored in the bile. These stones are typically black. The third type of gallstones is mixed pigmented stones which are a combination of calcium substrates such as calcium carbonate or calcium phosphate, cholesterol and bile. The fourth type is made up primarily of calcium and usually found in patients with hypercalcemia. When multiple gallstones or a singular large gallstone get impacted in Hartman’s pouch (the lower outpouching of the gallbladder), external compression of the common bile duct or the common hepatic duct can occur. The exact mechanism as to why this occurs is unknown, but it is felt to be related to a floppy Hartman’s pouch containing a higher mass of stones such as with multiple stones or a single large impacted stone. This causes subsequent infection?, or irritation, often causing pain?, swelling?, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।" data-rx-term="inflammation" data-rx-definition="Inflammation is the body’s response to injury, infection, or irritation, often causing pain, swelling, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।">inflammation? of the are which can also lead to fistula formation over time.[rx]

Pathophysiology of Mirizzi Syndrome

Gallstones occur when substances in the bile reach their limits of solubility. As bile becomes concentrated in the gallbladder, it becomes supersaturated with these substances, which in time precipitate into small crystals. These crystals, in turn, become stuck in the gallbladder mucus, resulting in gallbladder sludge. Over time, these crystals grow and form large and/or multiple stones. These gallstones may cause symptoms of cholecystitis, but if they become embedded in a floppy Hartman’s pouch, they can cause additional findings of jaundice?. As this condition progresses, internal fistulas from the gallbladder into the common bile duct, common hepatic duct (CHD) and the duodenum can develop. A grading system has been developed to categorize the various stages of Mirizzi syndrome.

Type I: No Fistula Present

• Type IA: Presence of the cystic duct
• Type IB: Obliteration of the cystic duct

Types II to IV: Fistula Present

• Type II: Defect smaller than 33% of the CHD diameter
• Type III: Defect 33% to 66% of the CHD diameter
• Type IV: Defect larger than 66% of the CHD diameter[4]

Diagnosis? of Mirizzi Syndrome

Findings of acute? or chronic? cholecystitis can be found on histology. The gallbladder wall may be thickened to variable degrees, and there may be adhesions to the serosal surface. Smooth muscle hypertrophy?, especially in prolonged chronic conditions, is present. Calcium bilirubinate or cholesterol? stones are most often present and can vary in size from sand-like to filling the entire gallbladder lumen. They can be multiple or singular. The acalculous disease may reveal sludge or very viscous bile. These findings are usual precursors to gallstones and are formed from increased biliary salts or stasis. Normal appearing bile can also be present. Various species of bacteria can be found in 11% to 30% of the cases. Rokitansky-Aschoff sinuses are present 90% of the time in cholecystitis specimens. These are a herniation of intraluminal sinuses from increased pressures possibly associated with ducts of Luschka. The mucosa will exhibit varying degrees of infection?, or irritation, often causing pain?, swelling?, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।" data-rx-term="inflammation" data-rx-definition="Inflammation is the body’s response to injury, infection, or irritation, often causing pain, swelling, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।">inflammation?.

There is an increased risk of developing gallbladder cancer with Mirizzi’s syndrome. The exact etiology is unclear but is felt to be due to persistent and recurrent irritation of the area and chronic? biliary stasis. 5% to 28% of patients with Mirizzi syndrome were found to have gallbladder cancer after cholecystectomy. Virtually all diagnoses were made postoperatively with pathologic examination of the specimens.[rx]

History and Physical

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.[rx]

Evaluation

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.[rx]

Treatment of Mirizzi Syndrome

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 lienteric anastomosis possibly with a roux-n-y has been shown to be effective.[rx][rx]

References