Bouveret syndrome: a case report.
Article Type: Case study
Subject: Gallstones (Case studies)
Gallstones (Diseases)
Stomach diseases (Diagnosis)
Stomach diseases (Case studies)
Stomach diseases (Care and treatment)
Stomach diseases (Prognosis)
Authors: Rossi, Daniel
Khan, Uzer
McNatt, Stephen
Vaughan, Richard
Pub Date: 03/01/2010
Publication: Name: West Virginia Medical Journal Publisher: West Virginia State Medical Association Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2010 West Virginia State Medical Association ISSN: 0043-3284
Issue: Date: March-April, 2010 Source Volume: 106 Source Issue: 2
Geographic: Geographic Scope: United States Geographic Code: 1USA United States
Accession Number: 223908173
Full Text: Bouveret syndrome is characterized by the migration of a gallstone through a cholecystenteric fistula into the proximal duodenum resulting in gastric outlet obstruction. Bouveret syndrome is a rare phenomenon that most commonly occurs in females and the elderly.

A 79-year-old female presented with symptoms of gastric outlet obstruction and was diagnosed with Bouveret syndrome. This report describes the symptoms, diagnosis, and management of Bouveret syndrome, as well as its prevalence and differentiation from gallstone ileus.

Patients with Bouveret syndrome present with varied, non-specific symptoms that may include emesis, abdominal pain, anorexia, and abdominal distention. Computed tomography remains the diagnostic modality of choice. Although different techniques are reported, surgical intervention is almost always required in the treatment of Bouveret syndrome.

Introduction

Gallstone disease is an ailment of the gastrointestinal tract afflicting approximately 6.3 million men and 14.2 million women in the United States annually. It has a prevalence of approximately 10% in the Western Hemisphere. (1) Despite the large number of individuals living with this entity, only 20-30% develop symptoms from their gallstones. Biliary colic, which includes abrupt epigastric pain that reaches maximum intensity in 60 minutes and gradually resolves over 2-6 hours, is the most common presentation in those who develop symptoms. Acute cholecystitis, gallstone pancreatitis, and acute cholangitis are also known sequelae of cholelithiasis. Less common presentations and complications include the Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. (2)

Gallstone ileus is an important, although rare, cause of mechanical intestinal obstruction with intermittent signs of nausea, vomiting, and abdominal pain. Migration of a gallstone through a cholecystenteric fistula to the distal ileum leads to impaction of the stone and subsequent symptoms of intestinal obstruction. Although gallstone ileus is a cause of only 14% of intestinal obstructions in the general population, the incidence increases in the elderly accounting for 25% of nonstrangulated small bowel obstructions. (3) Females are affected up to six times more often than males which is related to the cholestatic effects of the female sex hormonal melieu.4 The site of obstruction is most commonly the terminal ileum (50-70%), where the small bowel is at its narrowest. Less common locations of impaction include the distal jejunum (9%), the colon (4%), the rectum (4%), and the duodenum (1-3%). (3)

Bouveret syndrome, in contrast to gallstone ileus, is characterized by the cephalad or proximal migration of a gallstone into the duodenum resulting in a persistent gastric outlet obstruction as originally described by the French surgeon Leon Bouveret in 1896. (2,3,5,6,14) Bouveret syndrome accounts for 1-3% of duodenal obstruction cases. (3) As in gallstone ileus, there is a higher incidence of the disease in women than in men, (4) with a female-to-male sex ratio of 1.86. It is also a disease of the elderly with a mean age of 74.1 [+ or -] 11.1 (SD) years. (15)

Case Report

A 79-year-old Caucasian female presented to our institution in transfer with a one-week history of intractable vomiting. Emesis occurred one-two hours after eating, however anorexia was denied. She denied fever, chills, or rigors, but experienced mild dyspnea with exertion. Her past medical history was significant for type II diabetes mellitus, hypertension, morbid obesity, congestive heart failure, and chronic renal insufficiency. Her past surgical history consisted of a hysterectomy.

A focused physical examination elicited a soft, non-distended abdomen with minimal tenderness throughout, but with no obvious peritoneal signs. Chest auscultation revealed audible heart sounds with regular heart rate but occasional ectopy.

Laboratory testing revealed a hypochloremic, hypokalemic metabolic alkalosis, deranged liver function tests, chronic renal insufficiency, mild malnutrition, as well as a urinary tract infection.

During the course of admission, her liver and canalicular enzymes and renal function gradually improved. Her electrolyte imbalance was resolved with standard therapy. An abdominal ultrasound of the right upper quadrant on day two of admission showed multiple gallstones in a contracted gallbladder with chronic wall thickening and a common bile duct measuring 2.1 mm in diameter. A CT scan of the abdomen and pelvis showed gallstone-induced inflammatory changes about the duodenum consistent with duodenitis as well as the presence of pneumobilia (Figure 1). A 4-5 cm calcified mass was present in what appeared to be a fluid-filled sac-like structure invaginating into the duodenum and concern was raised for the presence of a gallstone within the duodenum (Figure 2). After preoperative optimization, the patient, on day five of admission, underwent esophagoduodenoscopy and exploratory laparotomy. Intraoperatively, the patient was found to have a phlegmon in her right upper quadrant involving the gallbladder, liver, duodenum, and omentum. An endoscope was passed into the esophagus and stomach, and the findings were unremarkable. However, a large gallstone could be easily visualized just distal to the pylorus, situated in the distal duodenal bulb and the first and second portions of the duodenum (Figure 3). The stone was palpable and visible through the pylorus, and an anterior gastrotomy was performed in the antrum. Following an unsuccessful attempt to withdraw the stone through the pylorus with stone-grasping forceps, the stone was crushed and retrieved in piecemeal fashion. Upon relieving the obstruction, a cholecystoduodenal fistula was palpated and also visualized via the endoscope. The endoscope was used to confirm the relief of the duodenal obstruction by passing it into the 3rd and 4th portions of the duodenum (Figure 4). Cholecystectomy and cholecystoduodenal fistula closure were not performed due to presence of the phlegmon. The gastrotomy was then closed in two layers.

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

The patient's post-operative was uncomplicated, and she was discharged to home on post-operative day eight.

Discussion

Bouveret syndrome is considered a distinct clinical entity from gallstone ileus due to the proximal site of the obstructing stone. Proximal obstruction is due to a large obstructing stone, generally measuring 2.5 cm or more. The presence of multiple smaller gallstones, however, has also been associated with duodenal obstruction especially in the presence of strictures or edema leading to a narrowing of the lumen of the duodenum. (16) The size of the obstructing gallstone in our case, 5cm, is one of the largest in reported literature. (15)

The clinical features of Bouveret syndrome can be varied and nonspecific; however, certain complaints tend to recur. Nausea and vomiting, abdominal pain, hematemesis, recent weight loss and anorexia are the most common presenting features of the syndrome. Abdominal tenderness, signs of dehydration, abdominal distension, and pyrexia are the most common findings on physical examination. (15) These signs and symptoms are all suggestive of an upper gastrointestinal obstructive pathology but none are specific for Bouveret syndrome.

[FIGURE 3 OMITTED]

[FIGURE 4 OMITTED]

Nonspecific symptoms make it necessary to use imaging modalities to assist in making a correct diagnosis. Plain abdominal flat and upright x-rays are still useful in the diagnosis of an intestinal obstruction. The confirmation of a diagnosis of Bouveret syndrome using plain abdominal films is dependent on the observation of Rigler's tetrad as delineated in his 1941 article (17) (Table 1). The classic "Rigler's triad" comprising the first three signs is seen in only 30-35% of cases. (18) However, up to 50% of abdominal radiographs may contain two of the classic three signs which would increase the index of suspicion for a gallstone ileus significantly. (3,18) In a series of 64 cases of Bouveret syndrome in which abdominal radiographs were conducted, Cappell and Davis reported the discovery of pneumobilia in 39%, a dilated stomach in 23%, and dilated loops of bowel in 14% of cases. An ectopic stone in the region of the gallbladder is also visualized in 21-38% of cases. (7,15) An upper GI series may assist with the delineation of the cause of gastric outlet obstruction. The most common findings include a filling defect or mass in the duodenum, a gallstone in the duodenum, duodenal or pyloric obstruction, cholecystoduodenal fistula, and pneumobilia. (15)

Diagnostic ultrasound scans usually depict what is known as a "double-arch sign" which can arouse suspicion particularly if pneumobilia is seen as well. (19) Nevertheless, sonographic visualization can be difficult if the gallbladder is collapsed or air-filled. In these situations, a gallstone in the duodenum would require significant amounts of fluid surrounding the stone in the antro-duodenal lumen for adequate visualization. Otherwise, an orthotopic gallstone in the gallbladder becomes a significant differential diagnosis. (20) Ripolles et al conducted a study to compare the accuracy of ultrasonography to plain abdominal films in the detection of Rigler's triad. They discovered that ultrasound was superior in the detection of pneumobilia and ectopic gallstones but somewhat inferior in the detection of bowel obstruction. However, they discovered that when combined with a plain abdominal X-ray, clinicians were able to make at least a probable diagnosis (visualization of only an ectopic stone or only pneumobilia and intestinal obstruction) in 96% of cases. It should be noted, however, that pneumobilia was detected in 22 of 23 cases. This increases the utility of the ultrasound since pneumobilia, unlike ectopic gallstones, has very few causes aside from gallstone ileus once prior biliary surgery has been ruled out. Ectopic stones will be visualized in exactly the same manner as bezoars on ultrasonography.8 The efficacy of ultrasonography as a tool is also dependent on the skill of the user. In another series of 40 cases of Bouveret syndrome, pneumobilia was visualized in less than half. (15) Even in the case of a definitively diagnostic plain film, ultrasonography will assist the surgeon in locating the stone as well as provide information on other stones that may be present in the GI tract. (8) Difficulties with ultrasonography can arise, however, in the presence of excessive intestinal gas.

Because of the relative lack of specificity with plain abdominal radiographs and ultrasound for a bilioenteric fistula, one should also remain cognizant of other common causes of pneumobilia (Table 2). In the absence of these conditions, pneumobilia is highly suggestive of a bilioenteric fistula. (21) Because of the limitations of plain radiographs, CT scans have become routine in the evaluation of intestinal obstruction and almost every other disease process involving the abdomen. CT scanning is particularly helpful in these situations. (22) Diagnosis is also superior with this modality as all three signs of Rigler's triad are depicted 77.78% of the time. (23) Pneumobilia is visualized in 60%, gallstones in 50%, and duodenal or gastric distension in 33% of cases of Bouveret syndrome. (15)

Gastroscopy is useful in identifying the obstructing gallstone in up to 69% of cases. In the remainder of patients, an obstructing gallstone would not be appreciated due to the possibility of being deeply embedded within the mucosa. In these cases a high suspicion should be maintained for a gallstone if the mass is hard, convex, smooth, non-friable, and non-fleshy. (15) The visualization of a gallstone on gastroscopy appears to be the only significant differentiating factor between the gastric outlet obstruction of Bouveret syndrome and the jejunoileal obstruction of the classic gallstone ileus.

The management of Bouveret syndrome is similar to the paradigm established for gallstone ileus. Definitive therapy remains surgery (15), while endoscopy and lithotripsy may be reserved as options for those unable to tolerate invasive procedures and for recurrent gallstone ileus. (13,15,26) No consensus exists for the most appropriate surgical intervention. Pavlidis et al promote a one-stage procedure in low-risk patients involving enterolithotomy or gastrotomy with removal of the obstructing gallstone followed by a cholecystectomy and fistula closure during the same procedure. (11) This combination, in comparison to enterolithotomy or gastrolithotomy alone, is thought to preclude the development of a recurrent obstructive event, cholecystitis, or cholangitis. The risk of developing a carcinoma is also eliminated, as is the need for a second operation. The significant disadvantage, however, is the associated morbidity and mortality that develops in significantly ill patients. A review of a 1001 cases of gallstone ileus by Reisner et al. noted a mortality rate of 16.7% in the one-stage procedure group compared to 11.7% in the group undergoing enterolithotomy alone for the treatment of gallstone ileus. (3) Cholecystectomy can not entirely prevent a recurrence of symptoms because obstruction may occur from stones in the common bile duct migrating into the small intestine, or from those which have already advanced into the small intestine, but were unnoticed during surgery.

High-risk patients are recommended to undergo a two-stage procedure with enterolithotomy alone and cholecystectomy at a second stage only if symptomatic stones remain. A patient may be considered higher risk if an ASA class of 3 or 4 is identified and/or hypotension is diagnosed during pre-operative evaluation. (12) Recurrent gallstone ileus is a significant problem. The overall recurrence has been shown to be 4.7%, with 57% of the recurrences occurring within the first six months after surgery. Symptomatic biliary tract disease has also been shown to develop in 15% of patients who do not undergo cholecystectomy.

A one-stage procedure has exhibited a greater number of early post-operative complications as well as a higher mortality rate. (3,24) Studies have also reported no difference in outcomes with enterolithotomy alone in both low- and high-risk patients. The reported mortality rates associated with the one-stage procedure may be artificially low due to a selection bias in favor of performing one-stage procedures in healthier, lower-risk patients. No significant complications were observed secondary to the remnant fistula. (12,24) A one-stage operation is recommended in patients who can tolerate the extended operative time (an average of 70 minutes in the enterolithotomy alone group versus 178 minutes in the one-stage procedure group (12)) and possibly in patients with gall bladder necrosis or empyema. (24) In patients undergoing enterolithotomy alone, many of the remnant fistulae have been shown to close spontaneously if the cystic duct remains patent and residual gallstones are not present. (3) A second stage cholecystectomy, however, can be offered to patients who have persistent biliary symptoms and who have a higher life expectancy. Laparoscopic approaches to relieve the obstruction have also been described to be safe. (25) In our case a laparotomy was performed with a gastrotomy once the large stone was confirmed to be in the duodenum. Neither closure of the cholecystoduodenal fistula nor cholecystectomy was performed.

The recurrence rate for gallstone ileus is significant, with more than half of the recurrences occurring within the first six months after surgery. This underscores the importance of interval follow-up. Due to the unhealthy status of most of the individuals presenting with this disease (86% of this patient population have been shown to belong to an ASA class of 3 or 4) (10), the mortality rate of 4.5%-25% is five to ten times higher than with all other nonmalignant causes of mechanical small bowel obstruction. (3) Therefore, it must be stressed that these patients need to be adequately prepared for their operation with correction of electrolyte abnormalities and treatment of co-morbid conditions. A one-stage procedure consisting of enterolithotomy alone is recommended in this generally high-risk population due to the low risk of disease recurrence and the increased morbidity associated with staged surgical therapy.

Conclusion

Our patient had several comorbidities and electrolyte derangement on presentation. Due to the complex nature of the patient's active and past medical problems, she was resuscitated prior to surgery and electrolyte imbalances were corrected. Following cardiac evaluation, the patient underwent a semi-elective enterolithotomy procedure that was uncomplicated. As depicted in this case, the non-emergent setting and relative stability of most patients with Bouveret syndrome necessitates the adequate preparation of the patient for surgery. Since the majority of these patients are elderly and likely to have multiple comorbidities, thorough preparation, we believe, will result in decreased morbidity and mortality. Nevertheless, half of all cases of gallstone ileus/Bouveret syndrome are diagnosed during laparotomy. A high index of suspicion should, therefore, be maintained to ensure early diagnosis and effective treatment of this otherwise rare and potentially fatal condition. (3)

References

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Table 1. Rigler's Triad

Partial or complete intestinal obstructions

Pneumobilia or contrast in the biliary tree

Visualization of ectopic gallstone in the
bowel

Change in position of previously observed
stone

* In 1978 Blathazar and Schecter described a
fifth sign: Two air fluid levels in the right upper
quadrant on an abdominal X-ray due to air in
the gallbladder.

Table 2. Causes of Pneumobilia (27)

Spontaneous biliary-enteric fistula

Surgical anastomosis--Whipple's procedure,
choledochojejunostomy

Incompetent sphincter of Oddi

ERCP with papillosphincterotomy or surgical
transduodenal sphincteroplasty

Emphysematous cholecystitis

Trauma
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