Bouveret’s Syndrome was first described in 1770 by Beaussier and named after French physician,
Leon Bouveret who published the first case in 1896 [1].
Cholelithiasis is a relatively common health problem. 35% of women and 20% of men develop gallstones by the age of 75 [1]. The prognosis for most is good however,
in small group (~6%),
complications occur. Rarely,
a fistula may develop secondary to adhesions,
which form between the gallbladder and bowel wall as a consequence of inflammation,
compromised arterial supply and impaired venous drainage [2,
3,
4]. Subsequent pressure necrosis and compression of a calculus against the wall of the gallbladder may result in the formation of a fistula.
The cholecystoduodenal fistula is most common type of fistulous formation followed by cholecystocolic and choledochoduodenal fistulas [4]. Most calculi pass through cholecystoduodenal fistula and the duodenum without causing obstructuion (85%) [2]. Spontaneous biliary-enteric fistulas are uncommon,
over 90% of fistulas develop as a complication of cholelithiasis or choledocholithiasis [5].
In 15%,
the calculus causes an obstruction [2].
Obstruction most commonly occurs in the terminal ileum (70%) [1,
3,
4] which is classified as a classic “gallstone ileus”.
In 1-3% of cases,
the gallstone becomes obstructed in the duodenum,
resulting in gastric outlet obstruction. This phenomenon is called Bouveret’s syndrome [1,
4]. The obstructing gallstone is usually greater than 2.5 cm [3]. Bouveret’s syndrome is thus defined as gastric outlet obstruction secondary to gallstone impaction at the level of the stomach or duodenum as it passes through a biliary-enteric fistula.
Bouveret’s syndrome occurs most commonly in elderly women with a history of biliary disease [2] – classically,
over 65 years of age [3].
Diagnosis of Bouveret’s syndrome is important as mortality may be as high as 33% [2,
4]. The high mortality is attributed to advanced age of the patients who commonly present with Bouveret’s,
associated presence of comorbidities and elevated surgical risk of such candidates.
Diagnosis
The presenting signs and symptoms of Bouveret’s Syndrome are non-specific. Most common symptoms include nausea and vomiting,
abdominal pain and,
uncommonly,
haematemesis (secondary to duodenal erosions or erosion of the coeliac axis) [4],
weight loss,
anorexia [1,
8].
Examination findings may include abdominal tenderness,
distension and dehydration [1].
There may be an obstructive pattern of deranged liver function tests with elevated bilirubin and alkaline phosphatase may be seen [4].
Diagnosis is historically based on endoscopy,
demonstrating visualization of the calculus causing obstruction [1]. This is seen in 69% of cases [1]. In 31% of cases,
no stone and or fistula can be identified. In the remaining cases,
the stone cannot be visualized or is only partly visualized as it compresses the duodenal wall [1]. Imaging with radiographs,
CT,
MR or ultrasound are increasingly contributing to early diagnosis. Identification of the calculus in the distal stomach or duodenum is highly suggestive of a diagnosis of Bouveret’s Sydrome. Detection of the fistulous tract is diagnostic.