DOI: 10.1148/rg.244035222
RadioGraphics 2004;24:1171-1175
© RSNA, 2004
Bouveret Syndrome1
G. Brooks Brennan, MD,
Robert D. Rosenberg, MD and
Sanjeev Arora, MD
1 From the Departments of Radiology (G.B.B., R.D.R.) and Internal Medicine (S.A.), University of New Mexico, 2211 Lomas Blvd, Albuquerque, NM 87106. Received November 28, 2003; revision requested January 7, 2004, and received February 18; accepted March 10. Address correspondence to G.B.B. (e-mail: gbbrennan@salud.unm.edu).
Index Terms: Bile ducts, calculi, 76.288 • Duodenum, stenosis or obstruction, 73.819 • Fistula, biliary, 76.284 • Fistula, gastrointestinal tract, 70.245 Gallbladder, calculi, 762.288 • Stomach, stenosis or obstruction, 72.819
 |
History
|
|---|
An 89-year-old woman with a history of coronary artery disease and diabetes mellitus presented to the emergency department with a 4-day history of malaise and a 2-day history of abdominal pain with nausea. The nausea worsened over the next day with development of bilious emesis. This was accompanied by substernal chest pain that radiated to the right flank and submammary regions. Nitroglycerin had very little effect. The patient was admitted from the emergency department for evaluation of small bowel obstruction. Vital signs were stable, and physical examination demonstrated only mild tenderness in the right upper quadrant. Pertinent positive laboratory values included elevated alkaline phosphatase of 446 U/L and alanine aminotransferase of 226 U/L; pertinent negative laboratory values were normal troponin, bilirubin (total and direct), and white blood cell count. Initial upright and supine radiographs of the abdomen were obtained.
|
Imaging Findings
|
|---|
Abdominal radiography demonstrated a paucity of bowel gas and multiple curvilinear calcifications in the right upper quadrant with a maximal diameter of approximately 3–4 cm (Fig 1). A paucity of air within the stomach makes assessment of gastric distention difficult. Ultrasound (US) of the right upper quadrant was recommended to evaluate for cholecystitis. Abdominal US demonstrated a curvilinear focus of increased echogenicity that measured approximately 3.5 cm with posterior acoustic shadowing (Fig 2a). Multifocal punctate areas of increased echogenicity with ring-down artifact were also noted scattered throughout the liver parenchyma and were thought to represent pneumobilia. The common bile duct was not confidently identified, but bilateral pleural effusions and a dilated, fluid-filled stomach were noted (Fig 2b, 2c). The gallbladder was not completely identified but was thought to be contracted around the shadowing stone, making assessment for gallbladder wall thickening difficult. The differential diagnosis of gastric outlet obstruction includes gallstone ileus, perforated peptic ulcer disease, pancreatitis, and malignant fistula.
View larger version (148K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1. Cholelithiasis. Upright frontal radiograph shows multiple curvilinear areas of increased opacity in the right upper quadrant (arrow). The maximal diameter is approximately 3-4 cm. Incidental note is made of a paucity of bowel gas, even in the expected region of the stomach.
|
|
View larger version (88K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2a. Bouveret syndrome. (a) Transverse sonogram obtained over the expected location of the gallbladder fossa shows a curvilinear focus of increased echogenicity with posterior shadowing, findings consistent with a large gallstone. (b) Transverse sonogram obtained over the epigastrium shows a dilated stomach (STO). (c) Longitudinal sonogram obtained over the liver shows multiple foci of increased echogenicity with ring-down artifact (arrows), findings suggestive of pneumobilia.
|
|
View larger version (97K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2b. Bouveret syndrome. (a) Transverse sonogram obtained over the expected location of the gallbladder fossa shows a curvilinear focus of increased echogenicity with posterior shadowing, findings consistent with a large gallstone. (b) Transverse sonogram obtained over the epigastrium shows a dilated stomach (STO). (c) Longitudinal sonogram obtained over the liver shows multiple foci of increased echogenicity with ring-down artifact (arrows), findings suggestive of pneumobilia.
|
|
View larger version (111K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2c. Bouveret syndrome. (a) Transverse sonogram obtained over the expected location of the gallbladder fossa shows a curvilinear focus of increased echogenicity with posterior shadowing, findings consistent with a large gallstone. (b) Transverse sonogram obtained over the epigastrium shows a dilated stomach (STO). (c) Longitudinal sonogram obtained over the liver shows multiple foci of increased echogenicity with ring-down artifact (arrows), findings suggestive of pneumobilia.
|
|
Abdominal computed tomography (CT) with oral and intravenous contrast material also demonstrated a dilated stomach. In addition, the pneumobilia was noted with a thickened, enhancing gallbladder wall (approximately 1 cm) and filling defects, each measuring approximately 3.5–4 cm, in both the gallbladder as well as the duodenum and surrounded by high-attenuation material. The Hounsfield units of the oral contrast material in the stomach and the high-attenuation material around the filling defects in the gallbladder and duodenum were as follows: 180 HU, 215 HU, and 240 HU, respectively. When dilution of contrast material in the fluid-filled stomach is taken into account, the relative similarity in Hounsfield units suggests that the hyperattenuating material was likely oral contrast material. However, no single image or Hounsfield unit measurements exclude the possibility that the hyperattenuating material represented calcium associated with the gallstones themselves. An air-filled tubular structure extending from the duodenum to the gallbladder was also noted and was presumed to be a fistulous tract (Fig 3). These findings were thought to represent a gastric outlet obstruction secondary to a large gallstone in the second part of the duodenum.
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3a. Bouveret syndrome. (a) CT scan of the upper abdomen, obtained with oral and intravenous contrast material at 8-mm collimation, shows two low-attenuation gallstones in the lumina of the duodenum and gallbladder. The stone in the gallbladder is surrounded by high-attenuation material, which possibly represents oral contrast material. (b) CT scan shows a curvilinear, air-filled fistula that extends from the gallbladder fossa toward the second part of the duodenum (arrow). (c) CT scan obtained superior to a and b shows pneumobilia and gastric distention (*).
|
|
View larger version (137K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3b. Bouveret syndrome. (a) CT scan of the upper abdomen, obtained with oral and intravenous contrast material at 8-mm collimation, shows two low-attenuation gallstones in the lumina of the duodenum and gallbladder. The stone in the gallbladder is surrounded by high-attenuation material, which possibly represents oral contrast material. (b) CT scan shows a curvilinear, air-filled fistula that extends from the gallbladder fossa toward the second part of the duodenum (arrow). (c) CT scan obtained superior to a and b shows pneumobilia and gastric distention (*).
|
|
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3c. Bouveret syndrome. (a) CT scan of the upper abdomen, obtained with oral and intravenous contrast material at 8-mm collimation, shows two low-attenuation gallstones in the lumina of the duodenum and gallbladder. The stone in the gallbladder is surrounded by high-attenuation material, which possibly represents oral contrast material. (b) CT scan shows a curvilinear, air-filled fistula that extends from the gallbladder fossa toward the second part of the duodenum (arrow). (c) CT scan obtained superior to a and b shows pneumobilia and gastric distention (*).
|
|
|
Pathologic Evaluation and Clinical Course
|
|---|
Endoscopic retrograde cholangiopancreatography (ERCP) was performed the following day and demonstrated a distended stomach with a moderate amount of food and blood despite the patient’s lack of oral intake for 3 days. In the midportion of the second part of the duodenum, a large, darkly pigmented stone occluded the entire lumen. Proximal to the stone, a fistulous orifice was noted with a second large stone eroding into the duodenum (Fig 4). Multiple passes with the snare were made without success. However, the more distal stone was successfully advanced into the distal small bowel. A percutaneous cholecystostomy was performed to alleviate the biliary obstructive symptoms. The patient subsequently developed a small bowel obstruction with the stone at the terminal ileum, as demonstrated at abdominal radiography (Fig 5). She was taken to the operating room for an enterotomy and gallstone extraction. She did well postoperatively and then returned to the operating room 5 days later for cholecystectomy and repair of the choledochoduodenal fistula. The patient’s condition deteriorated after the second surgery, and she died 10 days later.
View larger version (104K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4a. Endoluminal images from ERCP. (a) A large, 4-cm-diameter gallstone is seen in the second part of the duodenum, with bile noted proximal to the stone. Multiple attempts to snare the stone were unsuccessful. (b) More proximal to the impacted stone, there is a large gallstone eroding through the lateral wall of the duodenum.
|
|
View larger version (100K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4b. Endoluminal images from ERCP. (a) A large, 4-cm-diameter gallstone is seen in the second part of the duodenum, with bile noted proximal to the stone. Multiple attempts to snare the stone were unsuccessful. (b) More proximal to the impacted stone, there is a large gallstone eroding through the lateral wall of the duodenum.
|
|
View larger version (142K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 5. Gallstone ileus. Frontal abdominal radiograph obtained several days after ERCP shows two calcific areas of increased opacity (arrows). One is in the right upper quadrant (top arrow), the same location as on the earlier radiograph (Fig 1); the other is now in the terminal ileum (bottom arrow). Incidental note is made of a percutaneous cholecystostomy drainage catheter in the right upper abdomen.
|
|
|
Discussion
|
|---|
Gallstone-induced ileus is a rare complication of cholelithiasis, and gastric outlet obstruction is an even more rare variant. Bouveret syndrome is a gastric outlet obstruction produced by a gallstone impacted in the distal stomach or proximal duodenum. It was described by Leon Bouveret in 1896 (1) and occurs most commonly in elderly women with a mean age of 68.6 years (2). The term classic gallstone ileus often refers to an obstructing stone localized to the terminal ileum. A duodenal location accounts for only 2%–3% of cases (3). Gallstone ileus occurs in 15% of patients with a biliary-enteric fistula. The presenting clinical situation is variable and nonspecific but often includes nausea, vomiting, and epigastric pain. A small minority of patients may present with hematoemesis secondary to duodenum erosions or celiac artery erosion. Laboratory studies may indicate an obstructive pattern with increased bilirubin and elevated alkaline phosphatase values.
Historically, the diagnosis usually has been made with endoscopy, but CT and more recently magnetic resonance (MR) cholangiopancreatography are becoming more useful for the diagnosis (4). Early diagnosis is important because mortality is historically high at 33%, although it has decreased to 12% in recent years (2). The high mortality may be related to the advanced age of the typical patient as well as other comorbidities and their impact on the risks associated with surgical intervention. The decrease in morbidity in recent years likely represents the impact of endoscopic treatment options in lieu of surgery as well as early diagnosis with noninvasive imaging, such as CT and MR imaging.
Abdominal radiography is rarely the primary diagnostic tool for Bouveret syndrome. However, in approximately 30%–35% of gallstone ileus patients, the diagnosis may be suggested on thebasis of the clinical presentation and the Rigler triad: bowel obstruction, pneumobilia, and an ectopic gallstone (5). Subsequent abdominal plain radiographs may be useful to demonstrate the migration of the gallstone.
US findings may suggest the diagnosis but often present a confusing diagnostic picture. Although the gallstone will be sufficiently large to be apparent, it may be difficult to distinguish a duodenal location from an orthotopic location with a contracted gallbladder wall. If the fistulous tract is filled with fluid or air, the fistula may also be seen but can be confused with the common duct. Pneumobilia and a dilated stomach may also be seen with US.
Identification of the Rigler triad at CT is more common. Pneumobilia and a dilated stomach are easily identified. The fistula may be seen if the tract is enhanced by positive oral or air contrast material. A secondary sign that may be useful is the identification of oral contrast material within the gallbladder. The gallstone is usually apparent in the duodenum but is isoattenuating to bile and fluid in 15%–25% of cases. Again, oral contrast material improves diagnosis by surrounding the gallstone and therefore would increase the sensitivity of CT. Recently, Pickhardt et al (4) described the use of MR cholangiopancreatography for diagnosis of Bouveret syndrome with isoattenuating stones, and this may especially be true in a patient unable to tolerate oral contrast material.
Endoscopy is preferred as a therapeutic option because removal may be performed with mechanical, electrohydraulic, or laser lithotripsy (6). Surgery often is not desirable as the patients are often poor surgical candidates secondary to concomitant illnesses and advanced age. If surgery is performed, enterolithotomy alone may be adequate treatment in the elderly, and subsequent cholecystectomy may not be required (7). However, as in the case patient, a large retained gallstone in the gallbladder is an indication for cholecystectomy.
In summary, although Bouveret syndrome is a rare variant of a relatively rare disease, a timely diagnosis with imaging is possible and important. The diagnosis may be made with abdominal radiography and US, but CT and ERCP are likely the most sensitive modalities for diagnosis and ERCP has the added advantage of therapeutic options. MR cholangiopancreatography appears to be reserved for a small minority of cases with isoattenuating stones and in patients who are unable to tolerate oral contrast material. Although early diagnosis may aid in rapid extraction of an obstructing stone, mortality remains relatively high, likely secondary to the typical patient’s advanced age and comorbid conditions and their impact on surgical intervention.
|
Footnotes
|
|---|
Abbreviations: ERCP = endoscopic retrograde cholangiopancreatography
|
References
|
|---|
- Bouveret L. Stenose du pylore adherent a la vesicule. Rev Med (Paris) 1896; 16:1-16.
- Frattaroli FM, Reggio D, Gaudalaxara A, Illomei G, Lomanto D, Pappalardo G. Bouveret’s syndrome: case report and review of the literature. Hepatogastroenterology 1997; 44:1019-1022.[Medline]
- Langhorst J, Schumacher B, Deselaers T, Neuhaus H. Successful endoscopic therapy of a gastric outlet obstruction due to gallstone with intracorporeal laser lithotripsy: a case of Bouveret’s syndrome. Gastrointest Endosc 2000; 51:209-213.[CrossRef][Medline]
- Pickhardt PJ, Friedland JA, Hruza DS, Fisher AJ. CT, MR cholangio-pancreatography, and endoscopy findings in Bouveret’s syndrome. AJR Am J Roentgenol 2003; 180:1033-1035.[Free Full Text]
- Pickhardt PJ, Bhalla S, Balfe DM. Acquired gastrointestinal fistulas: classification, etiologies, and imaging evaluation. Radiology 2002; 224:9-23.[Abstract/Free Full Text]
- Schweiger F, Shinder R. Duodenal obstruction by a gallstone (Bouveret’s syndrome) managed by endoscopic stone extraction: a case report and review. Can J Gastroenterol 1997; 11:493-496.[Medline]
- Lobo DN, Jobling JC, Balfour TW. Gallstone ileus: diagnostic pitfalls and therapeutic successes. J Clin Gastroenterol 2000; 30:72-76.[CrossRef][Medline]
Top
History
Imaging Findings
