Complex presentation of Bouveret syndrome: Gastric outlet obstruction and septic complications

Abstract

Bouveret syndrome, a rare form of gastric outlet obstruction, occurs when a large gallstone migrates through a cholecystoenteric fistula into the duodenum, causing obstruction. We report a 31-year-old female who initially presented with nausea, vomiting, and abdominal pain. Imaging identified a large, calcified gallstone, a hepatic abscess, and acute cholecystitis. Initial treatment involved drainage of the hepatic abscess and antibiotics, with plans for elective cholecystectomy. Three years later, she returned with recurrent symptoms, including early satiety, significant weight loss, and gastric outlet obstruction. Endoscopy confirmed duodenal obstruction, and further imaging revealed a cholecystoduodenal fistula. She underwent an open cholecystectomy with fistula excision, duodenal repair, and nasojejunal feeding tube placement. Her postoperative recovery was smooth, with a gradual return to enteral nutrition. This report presents essential diagnostic and treatment approaches for Bouveret syndrome, including the roles of imaging, endoscopy, and surgery in managing complex cases. Early recognition and a coordinated, individualized treatment plan are crucial to optimizing outcomes in this rare condition.

Introduction

Bouveret syndrome, a rare form of gastric outlet obstruction, arises from the impaction of a gallstone in the proximal duodenum after migration through a cholecystoenteric fistula [1]. Though gallstone disease is common, only a small fraction of patients develop cholecystoenteric fistulas, and fewer still experience gallstone-related bowel obstruction [2]. Among these cases, Bouveret syndrome remains exceedingly rare, accounting for less than 3% of all gallstone ileus cases [3]. The syndrome predominantly affects elderly women, reflecting the demographic most commonly afflicted by gallstone disease, and often presents with nonspecific symptoms, leading to frequent diagnostic delays [4].

The pathophysiology of Bouveret syndrome involves chronic inflammation within the gallbladder, usually in the setting of longstanding cholelithiasis, resulting in an erosion into adjacent gastrointestinal structures, most commonly the duodenum [5]. This erosion creates a fistulous tract that permits large gallstones to pass into the bowel, where they may become lodged in the duodenum or pylorus, causing an obstruction [6]. The size of the gallstone, typically over 2-3 cm, plays a crucial role in determining the likelihood of obstruction, as stones of this size rarely pass spontaneously through the bowel [6]. This obstruction presents unique challenges in diagnosis and management due to its rare occurrence and overlap in symptoms with other causes of gastric outlet obstruction [7].

Diagnostic workup generally involves imaging, with CT scans often providing the most comprehensive view of the obstructing gallstone, the presence of pneumobilia, and any fistulous connections [8]. Endoscopic evaluation may be attempted for both diagnostic confirmation and therapeutic intervention, although many cases ultimately require surgery, especially when endoscopic methods fail to remove the impacted stone [9]. While surgical intervention is generally effective, it carries increased risk in this patient population, especially among those with comorbidities or advanced age. [3] Bouveret syndrome, therefore, necessitates a highly individualized approach to achieve timely diagnosis and effective treatment, balancing the risks and benefits of conservative, endoscopic, and surgical options [8].

Case presentation

A 31-year-old woman with a history of gestational diabetes mellitus 7 months prior presented to the emergency department with nausea, vomiting, and abdominal pain that had persisted for 5 days. She denied fever, chills, chest pain, shortness of breath, or changes in bowel habits. She had no significant past medical history aside from gestational diabetes, which had been managed with insulin during her pregnancy. Her family history was notable for a mother who developed diabetes mellitus in childhood and was insulin dependent.

On examination, the patient appeared uncomfortable but was hemodynamically stable. Laboratory tests revealed a blood glucose level exceeding 400 mg/dL and an anion gap of 15. Hemoglobin was slightly low at 10.6 g/dL, but white blood cell count was within normal limits. Liver function tests showed an elevated alkaline phosphatase level of 202 U/L. She was started on intravenous fluids and a diabetic ketoacidosis (DKA) protocol.

Imaging studies were conducted to investigate her abdominal symptoms. A computed tomography (CT) scan of the abdomen and pelvis revealed a large, calcified gallstone lodged in the neck of a distended gallbladder, consistent with acute cholecystitis. Adjacent to the gallbladder, there was a multiloculated mass in the right lobe of the liver measuring approximately 8.5 × 5.9 cm, suggestive of a hepatic abscess (Fig. 1). An ultrasound confirmed cholelithiasis with gallbladder wall thickening, pericholecystic fluid, and sludge. Magnetic resonance imaging (MRI) further characterized the hepatic lesion as an abscess and noted a filling defect in the adjacent hepatic vein, raising concern for septic thrombophlebitis.

Fig. 1.

CT abdomen and pelvis with IV contrast (axial view) with multiloculated mass in the right lobe of the liver (red box) suggestive of a hepatic abscess.

Blood cultures grew Streptococcus anginosus, and the patient was started on intravenous antibiotics tailored to the organism. Interventional radiology placed a percutaneous drain into the hepatic abscess, yielding purulent fluid positive for the same bacteria. Surgical consultation recommended delaying cholecystectomy until resolution of the abscess. The patient was discharged home with the drain in place, prescribed oral antibiotics for 4 weeks, and scheduled for outpatient follow-up.

Approximately 3 years later, the patient returned with complaints of abdominal discomfort, early satiety, intermittent vomiting, and significant weight loss. She reported multiple daily episodes of nonbloody, nonbilious vomiting and was unable to tolerate oral intake. Physical examination revealed signs of dehydration but was otherwise unremarkable. Laboratory tests showed mild anemia with a hemoglobin level of 9.3 g/dL and elevated liver enzymes.

An esophagogastroduodenoscopy (EGD) was performed, revealing a small amount of retained food in the stomach (Fig. 2) and a complete obstruction at the first portion of the duodenum. Biopsies of the gastric antrum and duodenal bulb showed chronic inflammation but no evidence of malignancy or Helicobacter pylori infection. A nasogastric tube was placed for decompression. A repeat CT scan of the abdomen and pelvis demonstrated an abnormal gallbladder with intraluminal and intramural gas, cholelithiasis, pneumobilia, and a dilated common bile duct measuring 8.5 mm. An MRI suggested a large gallstone impacting the gallbladder neck with associated gastric outlet obstruction (Fig. 3).

Fig. 2.

An esophagogastroduodenoscopy picture of retained food in the stomach.

Fig. 3.

An MRI with a large gallstone impacting the gallbladder neck (red arrow) and associated gastric outlet obstruction.

Given the severity of her obstruction and inability to maintain adequate nutrition, the patient was admitted for further management. Nutritional support was initiated with total parenteral nutrition, and a plan was made for surgical intervention. An exploratory laparotomy revealed dense adhesions in the hepatoduodenal ligament and 2 fistulous tracts between the gallbladder and the duodenum. A subtotal cholecystectomy was performed, and the fistulas were excised with primary repair of the duodenal defects. Intraoperative cholangiography showed normal filling of the biliary tree without obstruction or leakage. A nasojejunal feeding tube was placed to provide postoperative nutritional support.

The patient's postoperative course was uneventful. She gradually advanced to enteral feeding, her abdominal pain improved, and she began passing flatus and stools. Laboratory values normalized, and the output from the surgical drain decreased significantly. She was discharged home in stable condition with instructions for follow-up care and monitoring.

Discussion

Bouveret syndrome is an uncommon cause of gastric outlet obstruction resulting from the migration of a large gallstone into the duodenum via a cholecystoenteric fistula [10]. First described by Leon Bouveret in 1896, this syndrome accounts for less than 3% of all cases of gallstone ileus, which itself represents a rare complication of cholelithiasis [11]. Most cases of Bouveret syndrome occur in older adults, particularly women, with an average age above 70 [12].

The pathogenesis of Bouveret syndrome typically involves chronic inflammation in the gallbladder, often from longstanding cholelithiasis, which erodes the gallbladder wall and creates a fistulous connection with the adjacent duodenum or other gastrointestinal (GI) structures [13]. This cholecystoenteric fistula allows large gallstones to migrate into the bowel. Most frequently, the stone impacts at narrow regions of the bowel, like the pylorus or duodenum, causing gastric outlet obstruction [8]. Stones responsible for Bouveret syndrome generally measure over 2-3 cm, which is too large to pass spontaneously and likely to lodge in the narrow duodenal lumen [2].

Fistula formation in Bouveret syndrome results from a gradual process, typically occurring in individuals with chronic gallbladder disease [14]. Literature notes that the chronic inflammation and pressure necrosis from an impacted gallstone lead to fistula formation in up to 2%-3% of patients with gallstone disease, but only a fraction of these develop gallstone ileus or Bouveret syndrome [15].

Patients with Bouveret syndrome often present with vague and nonspecific symptoms, complicating early diagnosis [8]. Common symptoms include nausea, vomiting, early satiety, abdominal pain, and weight loss due to the gastric outlet obstruction [13]. Although the syndrome can mimic other causes of gastric outlet obstruction, some patients may have a prolonged history of gastrointestinal symptoms. Studies have highlighted that elderly patients with comorbidities, who often present late, are particularly at risk for significant complications and a challenging diagnostic process [3]. Hematemesis may occasionally occur due to mucosal erosion from the impacted stone.

Diagnosing Bouveret syndrome requires high clinical suspicion and careful imaging, particularly given the variable presentation. The combination of gastric outlet obstruction and signs of biliary disease should prompt investigation for potential Bouveret syndrome [16].

Abdominal X-ray: Although often the first imaging study, it has limited sensitivity and specificity for Bouveret syndrome. Classic findings, known as Rigler's triad, may be observed: pneumobilia, an ectopic gallstone, and signs of intestinal obstruction. However, Rigler's triad is seen in fewer than 50% of cases [17].

Computed Tomography (CT): CT imaging has become the diagnostic modality of choice due to its high sensitivity for detecting an impacted gallstone and associated findings such as pneumobilia and the fistulous connection [18]. Literature supports the use of CT for its ability to provide a clear view of the gallstone's size, location, and any surrounding inflammatory changes. In a retrospective analysis, CT demonstrated a diagnostic accuracy of over 90% in cases of suspected gallstone ileus, including Bouveret syndrome [19].

Magnetic Resonance Imaging (MRI) and Magnetic Resonance Cholangiopancreatography (MRCP): MRI and MRCP can offer additional details, particularly for evaluating biliary anatomy and detecting gallstones with associated soft tissue changes, although they are not commonly the first-line modalities [20].

Endoscopy: Endoscopy plays a diagnostic and, in some cases, therapeutic role. An upper endoscopy can directly visualize the obstructing gallstone in the duodenum and is often used to confirm the diagnosis [21]. However, endoscopic therapy has limitations, as stones large enough to cause obstruction are often too large or impacted for endoscopic removal.

Management of Bouveret syndrome is complex, and treatment must be tailored to each patient's overall condition, stone characteristics, and fistula involvement. Options range from conservative approaches to endoscopic or surgical intervention.

Endoscopic management: Although endoscopy is not always successful, it may be attempted as an initial approach, especially in patients who are not ideal surgical candidates [21]. Various endoscopic techniques have been explored, including mechanical lithotripsy, laser lithotripsy, and electrohydraulic lithotripsy, aiming to fragment the stone and enable passage [22]. The literature notes a success rate of endoscopic removal in 10%-30% of cases, with high rates of recurrence due to the residual fistula [23]. Larger stones often resist endoscopic manipulation, and successful extraction is uncommon without stone fragmentation.

Surgical intervention: Surgery is often necessary for definitive treatment, particularly when endoscopic attempts fail [24]. The standard surgical approach involves enterolithotomy, where the stone is removed via an incision in the duodenum [25]. The literature reports that additional procedures, such as cholecystectomy and fistula repair, are frequently performed to reduce the risk of recurrence and address underlying cholecystitis or fistula [26]. However, these procedures carry a higher risk in elderly or frail patients.

Single-stage surgery: In some cases, surgeons may perform a 1-stage procedure involving enterolithotomy, cholecystectomy, and fistula repair [27]. This approach aims to fully resolve the source of obstruction and prevent recurrence, but it is more technically challenging and has a higher risk of complications.

Two-stage surgery: For high-risk patients, a 2-stage approach may be preferred. The initial surgery involves enterolithotomy alone, allowing the patient to recover before a second procedure addresses the fistula and cholecystectomy [28]. A 2-stage approach is associated with lower immediate morbidity but requires careful patient selection [16].

Supportive care and nutritional management: Patients often present with signs of dehydration and malnutrition, particularly if they have experienced prolonged vomiting and poor oral intake [6]. In these cases, total parenteral nutrition (TPN) or postpyloric feeding may be necessary until the obstruction is resolved, and normal gastric function is restored. Nutritional support is critical for optimizing the patient's condition before surgical intervention, especially in frail or malnourished individuals [15].

Managing malnutrition in the perioperative period is crucial for patients with Bouveret syndrome, given the extended gastric outlet obstruction and impaired nutrient absorption they often experience [13]. Preoperative nutrition assessment is essential to identify deficiencies and establish a tailored nutrition plan. Enteral feeding is preferred whenever possible, using a nasojejunal or postpyloric feeding tube to bypass the obstruction, providing both calories and essential nutrients [11]. If enteral feeding is not feasible, total parenteral nutrition (TPN) should be considered, with regular monitoring to avoid refeeding syndrome and electrolyte imbalances [28]. Optimizing protein intake is especially important to support immune function and promote wound healing. During the postoperative period, a gradual transition to oral intake is recommended, starting with clear liquids and advancing as tolerated to minimize the risk of re-obstruction or intolerance. Regular monitoring of weight, serum protein markers, and electrolyte levels ensures that the patient's nutritional status is maintained and supports a smoother recovery [29].

Bouveret syndrome is associated with high morbidity and mortality, particularly in elderly patients and those with multiple comorbidities [3]. Common complications include aspiration pneumonia, recurrent obstruction, and postoperative infections [5]. The overall mortality rate ranges from 12% to 30%, reflecting both the complex nature of the syndrome and the high prevalence of comorbidities among affected patients [11]. Early diagnosis and tailored treatment can improve outcomes, but delayed presentation and diagnostic challenges often complicate management. Studies suggest that patients undergoing surgery within 48 hours of diagnosis have improved survival rates and shorter hospital stays compared to those with delayed intervention [8].

Furthermore, cases complicated by hepatic abscesses, as in this patient's case, add a layer of complexity to the management approach. Literature suggests that hepatic abscesses secondary to biliary pathology necessitate aggressive infection control measures, including abscess drainage and targeted antibiotic therapy [30]. The presence of a hepatic abscess in Bouveret syndrome may indicate a higher likelihood of systemic infection and sepsis, requiring a coordinated, multidisciplinary approach.

Conclusion

Bouveret syndrome, though rare, presents a significant diagnostic and therapeutic challenge due to its nonspecific symptoms, complex pathophysiology, and potential for severe complications. A high index of suspicion, prompt imaging, and timely intervention are essential for optimizing outcomes in affected patients. While endoscopic approaches may be considered, surgery remains the primary treatment for most cases, especially those complicated by large, impacted stones or associated infections like hepatic abscess. This case highlights the need for a personalized approach in managing Bouveret syndrome, balancing the patient's clinical status, the availability of endoscopic techniques, and the risks associated with surgical intervention. Early diagnosis and a multidisciplinary management strategy offer the best chance for recovery in this challenging condition.

Ethical considerations

Informed consent was obtained from the patient for all treatments and procedures. Consent was also obtained for the publication of this case report, and all efforts were made to maintain patient confidentiality.

Patient consent

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