What is Mirizzi Syndrome?
Mirizzi syndrome is a rare condition in which a blockage occurs in the main bile duct or liver duct. The blockage is caused when multiple or a single large gallstone gets stuck in a small pouch in the gallbladder, named Hartman’s pouch. The symptoms of Mirizzi syndrome are similar to other gallbladder diseases, like cholecystitis, which can make it hard to tell them apart. This can be further complicated as the condition can also cause jaundice, a yellowing of the skin, which is a symptom seen in other conditions like other bile duct stones and ascending cholangitis.
Because of these similarities, it’s often difficult and quite common for doctors to misdiagnose this condition before surgery. This syndrome is named after an Argentinean surgeon, Pablo Luis Mirizzi, who was born in 1893. Mirizzi graduated medical school from the National University of Cordoba in 1915. One important thing Mirizzi is known for is performing the first intraoperative cholangiogram in 1931, a type of X-ray that examines the bile and pancreatic ducts. He also first described the syndrome that now bears his name in a medical paper published in 1940.
What Causes Mirizzi Syndrome?
Gallstones develop when bile, a digestive fluid, doesn’t fully empty from the gallbladder and turns into a sludge-like substance then eventually hardens into stones. Gallstones can also be caused by blockages in the bile flow, which can occur due to things like narrowed bile ducts or certain types of cancer, including pancreatic cancer.
The most common type of gallstone is formed from cholesterol that solidifies into what’s called cholesterol stones. Another type of gallstone, called pigmented gallstones, are typically black and develop as a result of increased breakdown of red blood cells. This breakdown leads to higher amounts of a substance called bilirubin in the bile, which can form stones.
There are also mixed pigmented stones, which are a mix of substances like calcium carbonate or calcium phosphate, cholesterol, and bile. The last type of gallstone is primarily made of calcium and is often found in patients who have high levels of calcium in their blood.
Sometimes, if there are a lot of gallstones or one large gallstone in the lower part of the gallbladder (known as Hartman’s pouch), it can put pressure on the common bile duct or common liver duct. While we aren’t entirely sure why this happens, it seems to be related to a loose Hartman’s pouch that contains a large amount of stones. This pressure can then cause inflammation in the area and over time, might lead to abnormal connections or passages between organs (known as fistulas).
Risk Factors and Frequency for Mirizzi Syndrome
Gallstones are a health concern that requires understanding. A lot of people have gallstones but don’t have any symptoms. In the U.S., around 14 million men and 6 million women between the ages of 20 to 74 have gallstones. The risk of getting gallstones increases as you age. If you are overweight, you have a higher risk of getting gallstones, especially if you’re a woman. This is because being overweight can cause more cholesterol to be released into your bile, which plays a role in gallstone development.
Furthermore, people who lose weight very quickly or don’t eat for long periods can also have a higher risk of gallstones. This is due to a slowdown in the movement of bile. There is also a connection between hormones and gallstones. The hormone estrogen can cause more cholesterol in bile and less movement in the gallbladder. So, women of child-bearing age or those taking birth control medications that contain estrogen are twice as likely to develop gallstones than men. People with chronic illnesses like diabetes also have a higher risk of getting gallstones.
- Most gallstones don’t cause symptoms.
- Approximately 14 million men and 6 million women aged 20 to 74 in the U.S. have gallstones.
- Older age and obesity increase gallstone risk.
- Rapid weight loss and fasting can lead to gallstones.
- Estrogen increases gallstone risk in women.
- People with diabetes are more likely to get gallstones.
Mirizzi syndrome is a condition that’s not very common. Only about 0.1% of people with gallstones will develop this condition. In people who required gallbladder removal, this condition was found in about 0.7% to 25% of patients. Older people might be at a greater risk, but there’s no evidence to suggest that it affects one gender more than the other. It also doesn’t seem to be more common in any specific ethnic group.
Signs and Symptoms of Mirizzi Syndrome
Mirizzi syndrome is a health issue that usually appears similar to acute or chronic cholecystitis, a type of gallbladder inflammation, but with added signs such as yellowing of the skin and eyes (jaundice). People who have chronic cholecystitis often feel a dull pain in the upper right area of their abdomen, and this discomfort can extend to the middle of their back or the tip of their right shoulder blade. The pain usually comes on after eating fatty foods. Additional issues can include regular feelings of nausea and sometimes vomiting, as well as increased bloating and flatulence, which often occur in the evening. These less immediate symptoms can stretch out over weeks or even months. With more prolonged chronic symptoms, acute flare-ups (known as acute biliary colic) may occur more often and with more intensity. A usual physical exam will reveal pain in the upper right of the abdomen when it’s deeply pressed (this is known as Murphy’s sign). Most patients don’t typically look acutely ill but might seem distinctly uncomfortable. Jaundice is usually present and, in some instances, a significant increase in the levels of a substance called bilirubin, which causes the jaundice, can be identified.
- Appearance of acute or chronic cholecystitis but with jaundice
- Dull pain in the upper right abdomen, extending to mid-back or right shoulder blade
- Pain often related to fatty food consumption
- Frequent nausea, occasional vomiting
- Increased bloating and flatulence, often in the evening
- Symptoms can spread over weeks or months
- More severe or frequent acute biliary colic with prolonged chronic symptoms
- Abdominal pain with deep palpation (Murphy’s sign)
- Pain even though patients do not look acutely ill
- Jaundice and potentially elevated bilirubin levels
Testing for Mirizzi Syndrome
If your doctor suspects that you might have cholecystitis (inflammation of the gallbladder), they will usually start by running some tests. The most effective way to diagnose gallstones and cholecystitis is by doing an ultrasound scan of your upper abdomen. This test is particularly reliable, with a 90% success rate for correctly identifying the condition. Additionally, depending on the level of skill of the person performing the ultrasound procedure, even tiny gallstones as small as 2 mm can be detected alongside other abnormalities such as gallbladder polyps and sludge.
There are certain indications on an ultrasound scan that may suggest you have cholecystitis, as opposed to only gallstones. These include a thicker wall of the gallbladder (more than 3 mm thick), fluid around the gallbladder, and a positive sonographic Murphy’s sign, which means pain near the gallbladder when pressure is applied during the ultrasound .
Apart from an ultrasound scan, gallstones can sometimes be observed on CT scans and MRIs, but these methods are less effective when it comes to specifically diagnosing cholecystitis. About 10% of gallstones can be identified on simple x-rays due to their high calcium content. If you have a condition called enteric fistula (an abnormal connection between your intestine and other organs), this can also be seen in these x-rays as air in the bile ducts.
If your ultrasound scan results suggest that there might be a stone stuck in the common bile duct (a duct that carries bile from the liver to the small intestine), the next step is usually to undergo a type of MRI called a magnetic resonance cholangiopancreatogram (MRCP). If a stone is indeed found in the common bile duct through MRCP, the most accurate way to confirm this is by having an endoscopic retrograde cholangiopancreatogram (ERCP). This is a procedure performed by a specialist (gastroenterologist) using a flexible, lighted tube to check the tubes (ducts) that drain the liver, gallbladder, and pancreas. In cases where an ERCP is not achievable, a percutaneous transhepatic cholangiogram (PTHC) – a procedure that takes x-ray pictures of the bile ducts, can be handy in diagnosing the presence of stones in the common bile duct.
However, sometimes the diagnosis of a condition called Mirizzi’s syndrome (a rare complication of gallstones) is either mistaken for a simple common bile duct stone or completely missed during the initial testing.
Treatment Options for Mirizzi Syndrome
The best way to treat Mirizzi syndrome is with a surgery called a cholecystectomy, which involves removing the gallbladder. Doctors usually prefer to do this procedure laparoscopically, which means they use small incisions and a special camera to see inside the body and conduct the surgery. This method is usually preferred because it generally involves less pain, less scarring, and quicker recovery for the patient.
However, if Mirizzi syndrome is advanced, a more complex surgery might be needed. In such cases, doctors might perform an open cholecystectomy, which involves a larger incision and a more direct view of the organs.
For people with more serious cases, a partial cholecystectomy could be considered. In this procedure, the doctors leave in place a small part of the gallbladder called Hartman’s pouch, while removing the larger part of the gallbladder and any gallstones. This can help lessen the risk of injury to the area around the liver and the bile ducts, which are tubes that transport bile, a fluid that helps with digestion.
If the patient also has a fistula, which is an abnormal connection between two body parts, the doctors might do an open cholecystectomy along with bilioenteric anastomosis. This is a procedure that helps bile flow from the liver to the intestines by creating a new connection. Sometimes, this procedure may involve a Roux-n-Y, a specific type of rerouting procedure. Studies have shown this method to be successful in treating these complex cases.
What else can Mirizzi Syndrome be?
Many health problems can look like gallbladder disease. When people come in with severe gallbladder pain, doctors often check for heart conditions first. Other common health problems that might have the same symptoms include:
- Peptic ulcer disease,
- Bowel disorders,
- Inflammatory bowel disease,
- Acid reflux,
- Blockage in the lungs (pulmonary embolism).
When people with Mirizzi syndrome show up with yellowing skin or eyes (jaundice) and other symptoms, it’s often confused with health problems like:
- Stones in the common bile duct,
- Ascending cholangitis (an infection of the bile duct),
- Bile duct cancer,
- Pancreatic tumors.
Other health conditions which might appear similar to Mirizzi syndrome are:
- Acute hepatitis,
- Ischemic liver disease,
- Drug-induced hepatitis.
What to expect with Mirizzi Syndrome
In cases where there’s no fistula (an abnormal connection between organs) present, surgery usually goes well and results in a good outcome. However, Mirizzi syndrome can complicate the anatomy of the area, leading to a high chance of needing an open gallbladder removal surgery (cholecystectomy) rather than a less intrusive method. Because of this, some experts suggest that an open surgical approach be used for everyone with Mirizzi syndrome.
The prognosis, or likely outcome, for patients with a fistula tends to involve a longer treatment with a T-tube, a special type of drain tube placed through the small to moderate-sized fistula. If the fistula is larger, a different type of surgery called a choledochoduodenostomy or a Roux-en-Y choledochojejunostomy may be done for a bile diversion. This process reroutes the flow of bile from the liver to the small intestine.
For these patients, the surgical and hospital stay may be longer, increasing their risk of complications and raising their rates of sickness and death. Additionally, gallbladder cancer has also been linked to Mirizzi syndrome. However, it is believed that this connection may be due to prolonged inflammation causing these two conditions at the same time, rather than one condition leading to the other.
In older patients with several other medical conditions and a high risk of surgical complications, non-surgical methods might be a better option to reduce the risk of sickness related to the surgery.
Possible Complications When Diagnosed with Mirizzi Syndrome
Mirizzi syndrome can have several complications, with cholecystobiliary or cholecysto-enteric fistulas being the most common. These fistulas are abnormal connections that are typically caused by long-term inflammation. There may also be surgical complications involving longer operation times, caused by thick adhesions or scar-like tissues. These complications could include damage to the bile duct and bleeding. In more complex cases, severe bleeding can happen during the surgical exploration of the area around the gallbladder called the Calot triangle. Other complications resulting from prolonged inflammation that can occur in people with Mirizzi syndrome include:
- Skin fistulas, or unnatural connections between the skin surface and the bile duct or the intestine
- Secondary biliary cirrhosis, a late stage of chronic liver disease
- Delayed onset of biliary strictures, which are abnormal narrowing of the common bile duct
Preventing Mirizzi Syndrome
It’s very important for patients to be educated about how to take care of themselves, especially if they’ve had specific procedures like a stent placement, biliary diversion, choledochoduodenostomy, or a Roux-en-Y choledochojejunostomy. These procedures relate to the gallbladder and bile ducts, which are parts of our bodies responsible for digesting fats.
When discharged from the hospital, patients need to understand how to care for their surgical wounds to avoid secondary infections and associated complications. They should also know when to see their doctor for a check-up (this is called a clinical follow-up) and the importance of following their doctor’s instructions about medication and other treatments, known as medical compliance.
If a patient needs more help with their wound care, they could be referred to a specialized nurse. This provides additional support and monitoring to help prevent infections and other complications from arising.
