A rare complication of chronic (long-term) gallstone disease that occurs when a large gallstone or cluster of stones gets stuck in the gallbladder or cystic duct is called Mirizzi syndrome. It causes swelling and may block the flow of bile. Usually, people who develop this complication experience jaundice (yellowing of the eyes and skin), inflammation, and infection. In most cases, doctors treat this condition with laparoscopic surgery.
In general, gallstones are certain concentrated bile crystals that appear in your gallbladder. While most of the time they do not cause symptoms, if one of them causes blockages in the bile duct, you may have symptoms. Commonly, blockages in the bile ducts occur in the following way. In normal circumstances, the gallbladder receives and stores bile that comes from the liver and releases it into the intestine through certain small passageways (ducts). In some people, these gallstones remain in the ducts and begin to grow. When they become large enough, it may lead to blockages of the bile ducts. However, in people with this syndrome, these ducts become blocked from the outside when a gallstone within the gallbladder or one of the other ducts becomes enlarged. As a result, it puts extra pressure on the bile duct.
This syndrome occurs rarely, and it shares symptoms with other common diseases. That’s why most of the time, this condition is diagnosed during a gallstone surgery. Furthermore, surgeons identify Mirizzi syndrome in about 2.5% of people who do not have the gallbladder.
Types of Mirizzi Syndrome
Doctors have divided this complication into 5 types. These include:
- Type I – In people with this type, one or more gallstones get stuck in the gallbladder or in the cystic duct (the tube that carries bile from the gallbladder). As a result, it puts pressure on the nearby bile duct, which causes blockages of the bile flow.
- Type II – In such cases, people often have a fistula (hole) in the bile duct. Usually, this hole is less than one-third of the bile duct’s circumference.
- Type III – This type also involves a fistula, but it is up to two-thirds of the bile duct’s circumference.
- Type IV – This type occurs when the fistula in the bile duct completely destroys the bile duct wall. As a result, the gallbladder fuses (sticks) to the bile duct.
- Type V – The last type of this complication involves a fistula in the bile duct, in addition to any other Mirizzi type from I to IV.
Symptoms
The symptoms are also divided into 3 categories. For example:
- Inflammation – This causes swelling and pain in the upper right area of the abdomen (belly).
- Infection – Usually, people who develop this syndrome experience fever and sometimes nausea, vomiting, and diarrhea.
- Jaundice – This is yellowing of the skin and eyes, and it may include dark-colored urine or light-colored stools.
The previous symptoms are not specific to the Mirizzi syndrome, and they are not enough to confirm this complication. Not everyone who has gallstones will develop these symptoms. Moreover, the previous symptoms may indicate a problem with the biliary system, not necessarily Mirizzi syndrome.
Causes and Risk Factors
This complication of gallstone disease occurs when the bile duct is blocked from the outside. It happens when a gallstone is stuck, or a cluster of gallstones causes swelling that puts pressure on the common bile duct (the tube that carries bile from the liver and gallbladder to the small bowel). Sometimes, these gallstones may put pressure on the hepatic duct (the tube that carries bile from the liver directly to the small intestine).
Healthcare professionals have identified some factors that may increase your risk of developing this gallstone disease complication. For example:
- Age – This complication occurs more commonly in older adults who suffer from gallstone disease.
- Sex – Mirizzi syndrome occurs more commonly in females than males.
- Anatomical differences – For instance, a long, parallel, or abnormally low-inserting cystic duct into the common hepatic duct (CHD) usually facilitates stone impaction.
- Impacted gallstones – Large stones in the gallbladder neck or cystic duct often cause chronic (long-term) compression of the CHD.
- Other chronic health conditions (such as diabetes or high cholesterol)
- Inflammatory process – Long-term (chronic) inflammation may cause fibrous tissue to form in the gallbladder and bile ducts. As a result, it may lead to fistulas.
Complications and Prevention
While Mirizzi syndrome is a complication of gallstone disease, it may also cause some complications, especially if it is left untreated. Check some of them below:
- Cholestasis – This complication causes bile backup into the liver, which often causes inflammation.
- Fistula – This is a hole (opening) that appears as a result of inflammation and buildup of bile on the bile duct’s walls.
- Malabsorption – It occurs when the bile is not flowing into the small intestine. As a result, it negatively affects the ability of the body to break down proteins and absorb nutrients.
- Biliary obstruction – This includes cholangitis and biliary cirrhosis.
- Gallstone ileus – In such cases, a fistula in the bile duct allows a large stone to cause a small bowel obstruction.
This article does not contain a complete list of complications. However, you may talk with your doctor about ways to reduce the risk of developing them.
Unfortunately there is not always possible to prevent this syndrome. However, doctors may recommend some tips to reduce the risk. For example, you should limit your intake of things that cause gallstones (such as high-cholesterol foods), stop smoking, get regular checkups, limit or avoid alcoholic drinks, and others. For more details, discuss it with your physician.
Diagnosis
Physicians often use multiple tests to diagnose this condition. However, like the diagnosis of many other disorders, it begins with a physical examination to check for abnormalities linked to the disease. Check below some tests often used to confirm or rule out this complication:
- Complete blood count (CBC)
- Liver function tests
- Abdominal ultrasound
- CT (Computerized tomography) scans
- ERCP
- HIDA scans
- MRI (magnetic resonance imaging) scans
Treatment
In most cases, this complication is treated with surgery. However, doctors use different types of surgery to treat it because they depend on several factors. For example, the severity of the syndrome, existing health problems, gender, age, and preferences. These include:
- Gallbladder removal – This surgery is often used in more severe cases and when other treatments are not an option. During this procedure, surgeons often repair the blocked duct. Sometimes, they may also remove blocked bile ducts or use stents to keep them open.
- Laparoscopic surgery – This is a minimally invasive surgery used to treat Mirizzi syndrome.
- Laparotomy (open surgery)
In addition, surgery used to treat this syndrome may cause some adverse reactions. For example, bile leakage, excessive bleeding (hemorrhage), scarring of the bile ducts, pain, an increased risk of infection, and reactions to anesthesia.
Frequently Asked Questions
When should I see my healthcare provider?
If you experience the following symptoms, immediately go to the nearest emergency room (ER) or call 911. These include sudden, severe abdominal pain, chills, nausea, fever, jaundice, fast heart rate, excessive sweating, dyspnea (shortness of breath), or chest pain.
Does Mirizzi syndrome cause cancer?
While this complication does not directly cause cancer, it is often associated with an increased risk of gallbladder cancer. According to some studies, the underlying cause of Mirizzi syndrome often causes gallbladder cancer. For instance, approximately 5% to 28% of people with Mirizzi syndrome develop this type of cancer.
Is Mirizzi syndrome fatal?
Most of the time, this complication is treatable, but the risk of death increases as long as you do not get treatment. In general, the mortality rate is between 5% and 30%, and may increase if the affected person develops systemic infection (sepsis), liver failure, or gallbladder cancer. If you have any other questions, ask your healthcare provider.
