Bouveret syndrome: a rare complication of gallstone disease
- 1 Bristol Medical School, University of Bristol, Bristol, UK
- 2 Intensive Care, Chelsea and Westminster Healthcare NHS Trust, London, UK
- 3 General Surgery, North Bristol NHS Trust, Westbury on Trym, Bristol, UK
- Correspondence to Dr Omar Ghandour; omar.ghandour@nbt.nhs.uk
Abstract
A Caucasian man in his late 80s was admitted with central abdominal pain, abdominal distension and continuous vomiting, on the background of a recent admission for acute cholecystitis. The patient was managed for subacute bowel obstruction and was admitted to general surgery for further investigation. His blood tests showed raised inflammatory markers and deranged liver function tests. A CT scan showed the migration of a large gallstone, previously seen in the neck of the gallbladder on prior admission, to the proximal duodenum causing a degree of gastric outlet obstruction. A diagnosis of Bouveret syndrome was made, and although initially managed conservatively, the patient ultimately underwent surgery to remove the gallstone which had relocated again to the terminal ileum. Our case highlights the importance of considering rare complications such as Bouveret syndrome in patients presenting with bowel obstruction, particularly in the context of recent or chronic cholecystitis.
Background
Bouveret syndrome, first described by Leon Bouveret in 1896, is an extremely rare complication of a common disease. Although the prevalence of gallstone disease is 10%–15%, incidence reports demonstrate that 0.3%–4% of cholecystitis results in the complication of a gallstone ileus.1 Furthermore, Bouveret syndrome is estimated to account for just 1%–3% of cases of gallstone ileus.2–4 With a globally ageing population and the rising epidemic of obesity worldwide, the incidence of gallstone disease and thus its related complications are likely to continue increasing.1
With approximately 300 cases reported in the literature, case reports on the matter provide an important source of learning for all clinicians.5 Coupled with the high morbidity (60%) and mortality (30%) of Bouveret syndrome,5 it is vital to continue to share clinical experiences regarding this condition. Here we present a case of acute cholecystitis with readmission for Bouveret syndrome, which ultimately required surgical intervention after a trial of conservative management.
Case presentation
A Caucasian man in his late 80s was admitted with right-sided abdominal pain which had been progressively worsening for the past 2 weeks. The pain was exacerbated by eating, and there was a single episode of vomiting with no weight loss. He had a background of significant cardiovascular disease, chronic kidney disease, an endovascularly repaired abdominal aortic aneurysm and bilateral inguinal hernia repair. His observations were stable, and abdominal examination was unremarkable. Given the patient’s surgical history, the initial concern was an aortic aneurysm leak, and therefore, a CT angiogram was performed. His abdominal aorta was intact; however, a large gallstone in the neck of the gallbladder was noted. In light of this, the patient was managed as acute cholecystitis and discharged with oral antibiotics.
Four days later, the patient was readmitted to the surgical assessment unit with continuing abdominal pain and new distension. The abdominal pain was central and colicky in nature and accompanied by a constant feeling of satiety. He also reported ongoing nausea associated with continuous vomiting, which was dark brown, acidic and foul smelling in nature. The patient had not opened his bowels for 2 days despite passing small volumes of flatus. He was otherwise systemically well with stable observations. On examination, he appeared dehydrated, with a grossly distended abdomen and generalised abdominal tenderness most notable in the epigastric region. A provisional diagnosis of subacute bowel obstruction was made, and the patient was admitted to general surgery for further investigation.
Investigations
Following initial management for bowel obstruction, the patient was investigated with blood tests and imaging. His blood panel showed raised inflammatory markers (C-reactive protein: 48 mg/L and WBCs 14.87×109/L) and deranged liver function tests (ALT: 101 U/L and ALP: 230 U/L) but normal bilirubin. A plain film abdominal X-ray was reported to show a mildly distended stomach without other radiological signs of bowel obstruction (figure 1). Overnight, the nasogastric tube drained over 2 L of dark fluid; therefore, a non-contrast CT was arranged. The scan showed a large lamellated gallstone, which was previously seen in the neck of the gallbladder (figure 2), and had migrated to the proximal duodenum (D1) (figure 3). A fistula was visible extending from the neck of the gallbladder to the duodenum with pneumobilia. The gallstone was causing a degree of gastric outlet obstruction just beyond the pylorus, with the gastric lumen fluid filled and mildly distended. The observations therefore completed Rigler’s triad, and a diagnosis of Bouveret syndrome was made.
Plain film abdominal X-ray. Although initially reported to show only mild gastric distension, on further inspection, a gallstone can be seen (marked by arrow).
Axial and coronal views of contrast CT on original admission with acute cholecystitis. Calcified calculus clearly visualised within the confines of the gallbladder (marked by arrow).
Axial and coronal views of non-contrast CT after readmission with subacute bowel obstruction. Calcified calculus clearly visualised in D1 with localised oedematous and inflammatory changes (marked by arrow).
Treatment
The patient was managed as per the protocol for bowel obstruction, with nasogastric decompression and intravenous fluids. Intravenous antibiotics were given followed by a peripherally inserted central catheter for delivery of total parenteral nutrition to meet the patient’s requirements. The patient was managed in this way, with regular aspiration of the nasogastric tube, for 2 weeks.
During this time, there were several discussions regarding potential management options for the patient. Given his significant comorbidities and poor nutritional status, it was felt that exploratory laparoscopy with enterotomy would be too high risk considering the possibility of open conversion. Naturally, the next consideration was given to endoscopic retrieval of the stone using the basket and lithotripsy method. However, again the general anaesthetic risk for this patient combined with potential complications such as oesophageal damage was deemed too great to proceed at this time.
To resolve the dilemma, a contrast swallow was performed (figure 4) to assess the severity of gastric outflow obstruction. This showed that the stone was residing in the gastric antrum with a reasonable volume passing beyond into the duodenum. The patient was therefore trialled on a normal diet which he tolerated well with no nausea or vomiting. His clinical condition improved and was deemed medically stable. This conservative strategy avoided invasive intervention in the acute phase of illness, allowing the patient to be optimised for definitive management at a later date. He therefore consented for an outpatient gastroscopy+/-removal of gallstone from the duodenum+/-laparoscopic gastrojejunostomy within a month and discharged with safety-netting advice.
Oral contrast swallow showing gallstone in the gastric antrum, with reasonable flow into the duodenum and duodenojejunal flexure. Calcified calculus seen (marked by red arrow).
Outcome and follow-up
The patient ultimately re-presented with a recurrence of his abdominal pain and vomiting. A repeat CT scan showed that the gallstone had migrated from the duodenum to the terminal ileum. Given the patient had good functional and nutritional status despite his comorbidities, the decision to go forward with surgery was made.
The surgery was initially attempted laparoscopically but was converted to open due to difficulty in maintaining views among multiple dilated loops of small bowel and bleeding from the abdominal wall. The gallstone was milked proximally into the ileum with a view to remove the stone via enterotomy but disintegrated in the process. The stone fragments were deemed small enough to trial manual advancement through the ileocaecal valve which was successful, avoiding the need for enterolithotomy. The patient recovered well and was eventually discharged, with no complications over a year later.
Discussion
Bouveret syndrome is a rare form of gallstone ileus that can occur as a complication of gallstone disease. In this condition, inflammation and adherence of the gallbladder to the duodenum results in the formation of a cholecystoduodenal, choledochoduodenal or, more rarely, a cholecystogastric fistula. The pathway created allows translocation of calculi from the biliary into the digestive tract which, depending on their size, can result in obstruction. Most commonly, this causes gallstone ileus where the stone becomes lodged in the terminal ileum. In rare instances, the stone can lodge more proximally in the duodenum resulting in a gastric outlet obstruction, as seen in Bouveret syndrome.
In a review of 128 reported cases of Bouveret syndrome by Cappell et al, the mean age of onset was 74.1 years with a 1.86 women to men ratio.6 As in this case, patients predominantly present with nausea, vomiting and abdominal pain. In addition, patients typically exhibit signs of abdominal tenderness, dehydration and abdominal distension. The signs and symptoms of Bouveret syndrome are generally non-specific, and therefore, a high index of suspicion is required for diagnosis. A history of prior or concomitant gallstone disease is an important element in considering Bouveret syndrome as a differential diagnosis.
Diagnosing Bouveret syndrome from clinical presentation alone proves to be challenging. Blood tests often show leucocytosis and raised inflammatory markers; however, these findings are likely non-specific—making imaging crucial for diagnosis. In line with typical presenting features, the first line investigation is either an abdominal X-ray or ultrasound. In this case, an abdominal X-ray was requested, which reported only mild distension of the stomach. This owes to the fact that the components of Rigler’s triad are infrequently seen on abdominal X-ray: pneumobilia (39%), an ectopic gallstone (38%) and bowel obstruction (23%).6
Ultrasonography may also be used, with the benefits being detection of the fistula, residual cholelithiasis and pneumobilia. However, ultrasonography is rarely capable of detecting the true cause of the obstruction, and due to increased gas and dilatation of abdominal viscera, accurate assessment may be limited. As in this case and many other reports, the next logical step after X-ray or ultrasonography is the CT scan. CT is considered the most useful investigation in Bouveret syndrome for the detection of Rigler’s triad, as well as the condition of the gallbladder and anatomy of the fistula. Importantly, however, one must note that only the radio-opaque portion of the gallstone can be seen on CT, which may lead to gross underestimation of the size of the stone.7
A hotly debated bridge between the investigation and management of Bouveret syndrome is the use of endoscopy. On the surface, endoscopy would appear ideally suited as a minimally invasive procedure to identify and remove the stone. However, Cappell et al showed that although almost all endoscopies show evidence of obstruction, the gallstone is only seen in two-thirds of cases with the fistulous connection seen in 13%.6 This is further complicated by the fact that an experienced endoscopist is required, and if stone removal is attempted, there is a risk of damage or perforation of the oesophagus due to the jagged edges.8
With a limited success rate for endoscopic retrieval, surgery is the most common form of management.6 9 10 There are several surgical approaches which may be considered, most of which depend on the expertise available and location of the stone. Evola et al describe two cases of successful removal of a large stone via gastrotomy after manual relocation into the stomach.10 Mobilising the stone and performing gastrotomy, pylorotomy or duodenotomy are all modalities which can be used.8
Whether by open or laparoscopic means, surgery is often very risky due to the age and comorbidities of the patients who present with Bouveret syndrome. This was a major consideration in the presented case, where the oral contrast swallow was used to great effect. Few case reports mention the use of the contrast swallow; however, in the correct circumstances, it may prove to be very effective. As the patient was stable, it was felt that the contrast swallow would allow determination of the degree of obstruction. If significant volume could pass, the patient could be trialled on a normal diet, and if this was the case, the patient could then return home and be managed conservatively. Conservative management with follow-up endoscopy is possible as the fistulous communication does not always require repair.6 11
Our case was complicated by the fact the gallstone had relocated to the terminal ileum from the proximal duodenum. The surgical management of gallstone ileus typically involves enterolithotomy with the option of cholecystectomy and/or fistula repair in a one-stage or two-stage procedure.12 The approach used in our case mitigated the potential risks associated with enterotomy or small bowel resection, including anastomotic leak and enteric fistula.
Bouveret syndrome has been scarcely reported in the literature making this case a unique learning opportunity. The rarity of the condition, difficulty in detection and complexities of the patients mean that rigid treatment guidelines for Bouveret syndrome do not exist.9 It is clear from this case report and other similar works that the management of Bouveret syndrome requires taking into consideration a patient’s age, clinical presentation and comorbidities. Through a patient tailored approach, one can minimise the morbidity and mortality associated with this potentially fatal condition.
Learning points
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Always consider rare complications such as Bouveret syndrome in a patient presenting with signs of bowel obstruction and a recent or chronic history of cholecystitis.
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Gallstones can translocate over a short period of time, as seen in the CT images in our presented case.
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The initial radiographic signs of Bouveret syndrome can be subtle on plain films.
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Only the radio-opaque portion of the gallstone can be seen on CT, which may lead to gross underestimation of the size of the gallstone.
Ethics statements
Patient consent for publication
Acknowledgments
We would like to thank Mr Christopher Wong for his guidance and support in supervising the completion of the case report.
Footnotes
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Contributors AK and OG contributed equally to the manuscript as joint first authors. The project was supervised by CW.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
References
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