Bouveret Syndrome: Causes, Symptoms & Treatment

Bouveret syndrome is a gallstone condition that can result in blockage of the exit of the stomach. This condition occurs rarely but is extremely life-threatening to the patient. A large gallstone migrates into the duodenum via a fistula of the gallbladder, so that it lies on the stomach outlet. This process is triggered by inflammation. The disease is named after the French physician Léon Bouveret (1850-1929), who described it classically in 1896.

What is Bouveret’s syndrome?

The formation of gallstones is related to bile, which is produced in the liver and stored in the gallbladder. This thick secretion aids in digestion, and is secreted into the intestines. It is responsible for the elimination of toxins and metabolic waste products. Bile juice consists mostly of water, which dissolves the other components of the liquid – such as cholesterol. These substances are in a very specific quantitative ratio to each other. If this balance is disturbed, crystals form in the bile, which can grow into gallstones. The same happens if the bile cannot flow out, for example due to a narrowing or even an obstruction in the so-called bile duct. A condition of this type is called Bouveret’s syndrome.

Causes

The result can be biliary colic, when a stone from the gallbladder gets into the bile duct, causing a blockage there. In an effort to remove this resulting foreign body, the muscles of the bile duct contract in spasms. This process causes severe cyclic pain known as biliary colic. In addition to the pain, the buildup of bile is a major health hazard. It may drain into the liver or even to the building salivary gland, and can thus cause dangerous inflammation throughout the abdomen. If such signs become more pronounced, a precautionary removal of the gallbladder is often the only possible rescue route. Patients are mostly women over 70 years of age who experience sudden severe pain in the abdomen, combined with strong nausea and nervous vomiting. Unnatural onset of fever and chills may accompany these symptoms. In some cases, jaundice is added, specifically yellowing of the skin. For a long time, these and other possible parallel conditions made reliable diagnosis of Bouveret’s syndrome difficult.

Symptoms, complaints, and signs

Nowadays, imaging techniques, such as sonography (ultrasound examination) or computed tomography, indicate the causes of the disease with high accuracy. Air bubbles in the bile ducts, for example, which occur in connection with the formation of fistulas in the intestinal tract, can be depicted very well. In Bouveret’s syndrome, the obstruction forms relatively high up at the stomach outlet. Thus, the inflammatory features can be localized very clearly.

Diagnosis and course

Initially, therapy is based on a gentle approach. Bouveret’s syndrome occurs far predominantly in older people, who often suffer from multiple concomitant diseases. Therefore, depending on the initial clinical condition, disintegration of the gallstone and its subsequent removal are preferable to surgery. For this purpose, the physician usually uses a simple endoscopy. If surgery is unavoidable, it should be limited, if possible, to removal of the stone and restoration of the natural passage. The risks of removing the gallbladder and closing the fistula are often disproportionately high. The gallbladder is typically inflamed in Bouveret’s syndrome, and the fistula to the duodenum is gas-filled. Endoscopy can be used to minimize the gallstone by targeting detachments at its margins. Either it has then become so small that it can be recovered orally, or it slips deeper until it is excreted by the usual routes. The fistula may subsequently regress on its own within a few days if the course is favorable and antibacterial treatment is given. If these prospects do not materialize, surgical suturing of the fistula is possible. However, statistically proven postoperative complications have been observed in one third of all cases. These are predominantly wound infections. The greatest safety is achieved when only the gallstone obstructing the stomach is removed.Surgeons now regard this as a sensible alternative to more extensive remediation, for example of the fistula. A large gallstone can reach dimensions of around seven by three centimeters. Thus, in an emergency of Bouveret’s syndrome, gastric emptying is massively blocked.

Complications

Bouveret’s syndrome occurs as a very rare complication of bile duct obstruction caused by a stone. In this case, the stone enters the duodenum through a junction (fistula) and can block the gastric outlet (pylorus), causing stenosis. This complication is characterized by sudden onset of nausea and vomiting and severe pain in the upper abdomen. Stenosis of the gastric outlet can result in the most serious metabolic derailments, which can even be life-threatening if not treated surgically. Frequent vomiting causes metabolic alkalosis in the body, as well as the risk of dehydration, since a lot of fluid is lost and hardly any is absorbed. If the operation is not clean, the wound can become infected and put additional stress on the body. In addition, internal bleeding may occur. Furthermore, the stomach can become inflamed, and in the worst case, the inflammation can spread systemically, causing sepsis. Sepsis is a life-threatening condition and must be treated immediately with medication, as it can quickly lead to the death of the patient. In general, gallstones increase the risk of inflammation of the bile ducts and gallbladder, which can also result in sepsis. The stone can also penetrate the walls and cause inflammation of the peritoneum (peritonitis).

When should you go to the doctor?

Bouveret’s syndrome is a gallstone condition that causes blockage of the stomach outlet. Medical and drug treatment is urgently needed, as there may even be acute danger to life. In most cases, Bouveret’s syndrome occurs in older people who have had previous problems with existing gallstones. The difficulty with Bouveret’s syndrome is that this clinical picture is often diagnosed very late. Often, Bouveret’s syndrome is characterized by severe and prolonged abdominal pain. In addition, there is often a stabbing pain in the abdominal region, which severely restricts everyday life. In case of unexplained abdominal pain, a doctor should be consulted as soon as possible. Anyone who forgoes a visit to the doctor at this point exposes himself to a great risk. The abdominal pain increases significantly in such a case. An increased temperature and nausea are also possible accompanying symptoms that can occur in connection with the clinical picture described above. For this reason, the following applies: the Bouveret syndrome is a clinical picture that definitely needs medical attention.

Treatment and therapy

Generally, a gallstone grows to only the size of a cherry pit. It consists essentially of solidified bile. Predominantly, it forms from deposited cholesterol, so it is yellowish in color. It is estimated that around 20 percent of gallstones are so-called pigment stones. Their core also consists of cholesterol, around which, however, the bile pigment bilirubin has settled. Pigment stones are only as small as grains of sand, but they occur in larger colonies. What all the different gallstones have in common is that they calcify during life.

Outlook and prognosis

Bouveret’s syndrome is a medical emergency. Treatment must begin quickly or it can be fatal. The majority of cases involve older women over 70 years of age. In most cases, those affected also suffer from other diseases due to their age, which can further worsen the prognosis. In the case of Bouveret’s syndrome, a gallstone moves in front of the stomach outlet via a fistula that connects the bile duct and duodenum and obstructs it. The fistula forms as a result of inflammatory processes associated with bile duct disease. The sudden onset of abdominal discomfort may be accompanied by further complications, including peritonitis. Only rapid removal or disintegration of the gallstone can end the extremely life-threatening condition. After successful removal of the stone, there is usually a complete recovery, depending on the patient’s general physical condition.Since these are usually elderly patients with other concomitant diseases, the gentlest possible therapy must be used. If possible, the stone is crushed and removed with endoscopic assistance. Otherwise, surgery must be performed to remove the stone from the gastric outlet, in which case the goal should be only to restore patency. Major surgery with removal of the gallbladder should be avoided in this case to avoid increasing the surgical risk.

Prevention

In Germany, gallstones occur statistically in about one in six residents. In women they form much more frequently than in men. However, noticeable complaints have only about 25 percent of all those affected. If gallstones do not cause any symptoms, they do not need to be treated. However, the stones are probably responsible for the first pain in the upper abdomen, which can also extend to the back and right shoulder area. A cholesterol-conscious diet is the best means of prevention against gallstone symptoms.

Here’s what you can do yourself

Bouveret’s syndrome is a rare complication that requires immediate hospital treatment. After successful removal of the gallstone, those affected should initially take it easy. Bed rest and a healthy diet that is not too irritating should regulate the gastrointestinal tract again in a short time. To flush out residues of kidney or gallstone, plenty of water or kidney tea should be drunk. For pain, herbal teas and massages help, as well as warm compresses, physical exercise or a trip to the sauna. In consultation with the doctor, the causes of Bouveret’s syndrome should also be determined. Often, the complication is based on another disease, which must be treated accordingly. In most cases, the physician in charge recommends dietary measures such as abstaining from stimulants or generally advises a healthier lifestyle. Older patients may also need to change their medication. For example, various preparations help to break down gallstones and reduce the pain that can occur. Alternatively, home remedies such as warm beer or cranberry juice can be used. If, despite all measures, severe discomfort occurs, it is necessary to go to the doctor with Bouveret’s syndrome.