A rare presentation of gastric outlet obstruction (GOO) - The Bouveret's syndrome.

INTRODUCTION: Bouveret's Syndrome is a rare cause of Gastric outlet obstruction (GOO) caused by a gall stone which has migrated into the duodenal bulb from a bilioduodenal fistula. It is an uncommon condition and only a few cases have been reported in the medical literature till to date. PRESENTATION OF CASE: We report a case of a 45 year old woman who presented to us with recurrent pain in the upper abdomen for the last 8 years, and that progressed to develop symptoms of gastric outlet obstruction. An upper gastrointestinal endoscopy revealed duodenal ulceration with a stony hard lesion in the first part of duodenum. An contrast enhanced computed tomography (CECT) scan of the abdomen performed later confirmed the presence of cholecystoduodenal fistula and a gall-stone in proximal jejunum. DISCUSSION: Bouveret's syndrome is a case of gastric outlet obstruction caused by the impaction of a large gall stone in the duodenum as a result of a cholecystoduodenal fistula. It constitutes 1-3% of cases of gall stone ileus which in turn complicates only 0.3-4% cases of cholelithisasis. Bouveret's syndrome is managed surgically.
CONCLUSION: Bouveret's syndrome being an uncommon condition may pose a difficulty in diagnosis and dilemma in managing such patients. An awareness is hence essential.

Introduction

Bouveret's syndrome is a rare cause of gastric outlet obstruction owing to the impaction of a large gall stone in the duodenal bulb after migration through a bilioduodenal fistula. It is a rare variant of a relatively rare disease (gall stone ileus) with an overall incidence of 1–3% [1]. It was first described in 1896 by a French physician, Leon Bouveret, who reported two cases of gastric outlet obstruction due to gall stones in[his]- “Revue Medicale” [2]. Since then, only few cases of Bouveret's syndrome have been reported in the worldwide medical literature. Owing to its uncommon and unpredictable symptomatology, Bouveret's syndrome can pose as a diagnostic and therapeutic challenge for clinicians. The aim of reporting this case is to increase clinical awareness which will ultimately lead to early diagnosis and timely management of this rare syndrome.

Presentation of case

A 45 year old woman presented with complaints of recurrent mild pain in the upper abdomen for the last 8 years, often associated with multiple episodes of nausea and non-bilious vomiting. Pain started in epigastrium and spread to right hypochondrium, and it was not associated with fever. Patient did not have any history of jaundice or prior surgery. She did not have any other abdominal complaints. The patient also did not reveal any other significant personal or family history.

On examination, all the vital signs were within normal limits and the patient was afebrile and non icteric. Mild tenderness ellicited in epigastrium and right hypochondrium, however there was no guarding or rigidity. No lump or organomegaly was present and the bowel sounds were normal. Laboratory investigations were all within normal limits including the liver function test. An ultrasonography of the abdomen revealed a partially distended thick walled gal-bladder with multiple calculi in the lumen suggestive of chronic cholecystitis. The patient was treated conservatively as a case of calculous cholecystitis and was planned for elective cholecystectomy.

Patient returned after 15 days with complaints of abdominal pain and distension post meals associated with recurrent episodes of vomiting. On examination, the patient had distension over the epigastrium with tenderness. There was no rebound tenderness or guarding. Succussion splash was positive and bowel sounds were sluggish. Digital rectal examination did not reveal any abnormality. An upper Gastro-Intestinal endoscopy was performed which revealed duodenal ulceration with a stony hard lesion in first part of duodenum. The scope could not be negotiated beyond first part of duodenum. Multiple Biopsies were taken from the ulceration site and histopathological examination revealed only inflammatory changes. A contrast enhanced computed tomographic (CECT) scan of abdomen depicted a cholecystoduodenal fistula (in the first part of duodenum) with a gall stone in proximal jejunal loop, sludge and concretions in the gall-bladder with a distended fluid filled stomach with pneumobilia (Figs. 1 and 2).

Exploratory laparotomy was planned with a working diagnosis of gastric outlet obstruction (GOO). Operative findings were: a pulled up pylorus densely plastered at porta-hepatis with no palpable mass or scarring in duodenum or pylorus. A 5 × 4 cm calculus extracted from jejunum, 15 cm distal to duodeno-jejunal junction (Figs. 3–5). Gall bladder was contracted with sludge and concretions and a fistulous tract from gall bladder to first part of duodenum. Cholecystectomy with enterolithotomy and closure of the fistulous tract with gastro-jejunostomy was performed under general anesthesia. Post operative period was uneventful and the patient was discharged on 7th day. The patient is on a regular follow up. Patient had an uneventful recovery.

Discussion

Bouveret's syndrome is an uncommon cause of GOO owing to intraluminal duodenal obstruction by gall stone which has migrated through a bilio-enteric fistula. Gall stone ileus is the more commonly heard complication of cholelithiasis (0.3–4%) [3]. The most common level of obstruction is the terminal ileum (60–70%), followed by the proximal ileum (25%), distal ileum (10%), jejunum (9%), colon (4%), rectum (2%), and lastly the duodenum (1–3%) [3].

This syndrome was first described by Beaussier in 1770 but it has been named after the French physician Leon Bouveret after his publication of 2 case reports in the Revue Medicale in 18962. Around 1% of gall stone cases develop bilio-enteric fistulas [4]. Types of bilio-enteric fistulas include cholecystoduodenal (60%), cholecystocolic (17%), cholecystogastric (5%), choledochoduodenal (5%) [4]. Fistula formation is thought to occur as a result of adhesions between the gall bladder and the bowel wall secondary to chronic inflammation, impaired arterial blood supply and decreased venous drainage [5]. Ensuing fistula formation can occur from pressure necrosis and compression of the gall stone against the gall bladder wall. The subsequent passage of the gall stones can occur via the fistula and the stone may get enlarged due to accumulation of salts and fecal matter on its surface.

Risk Factors for Bouveret's syndrome are age more than 70 years, female gender, gall stones more than 2.5 cm in size, post surgical altered GI anatomy [6]. Symptoms include vomiting (87%), abdominal pain (71%), hematemesis (15%), recent weight loss (14%), anorexia (13%) [7]. Common signs encompass abdominal tenderness (44%), signs of dehydration (31%), abdominal distension (26%) [7]. Extrinsic duodenal compression by a massively enlarged gall bladder containing multiple calculi may also cause GOO (pseudo-Bouveret's syndrome) [8]. Bouveret's syndrome results in complications such as duodenal perforation, hematemesis (Mallory-Weiss tear), distal oesophageal rupture (Boerhaave's syndrome), gastric bezoar [7].

Abdominal X-ray can show Rigler's triad (pneumobilia, small bowel obstruction and gall stones) in 30–35% cases [9]. Ultrasonography abdomen is helpful in 60% of cases and can reveal fluid filled distended stomach, features suggestive of cholecystitis, ectopic gall stone, pneumobilia. CECT abdomen has a sensitivity of 93%, specificity of 100% and diagnostic accuracy of 99% [10]. It can demonstrate the exact site of obstruction, fistula and gall bladder status [11]. MRCP has main utility in patients intolerant to oral contrast. It can differentiate between gall stone and surrounding bile (CT scan cannot in 15–25% cases) [11]. UGI endoscopy is both diagnostic and therapeutic. The common findings are stone causing obstruction (69%), obstruction without visualized stone or fistula (31%), partially visualized through duodenal wall (<1%), inflammation, edema or ulcer at impacted site, retained food or fluid in stomach [7].

Treatment options available are Endoscopic, Open Surgery and Laparoscopic Approach. Endoscopic approaches include endoscopic extraction, endoscopic laser lithotripsy, extracorporeal shockwave lithotripsy and intracorporeal electrohydraulic lithotripsy. The first successful endoscopic extraction has been described in 1985 by Bedogni et al. [12]. Subsequently, a number of case reports have been published describing successful endoscopic management of Bouveret's syndrome [3]. Endoscopic management often requires the use of different sized and shaped snares, grasping forceps, retrieval baskets and nets, biliary balloons, and sometimes even a side-viewing endoscope and hence can be technically challenging, time-consuming, and success rates in case series have been previously reportedly to be less than 10% [3]. While the majority of patients tolerate attempted endoscopic treatment, pulseless electrical activity (PEA) can occur during mechanical retrieval due to the gall stone getting lodged in the esophagus. The PEA abruptly resolved when the stone was pushed back into the stomach [13]. Indications for open surgery [3,14] are stone size greater than 2.5 cm, residual stones in GB, multiple stones in intestinal lumen, sepsis, perforation, stricture and failure of endoscopic approach. Open surgery involves enterolithotomy with cholecystectomy with fistula repair. Different authors have recommended different approaches for management of Bouveret's syndrome as shown in Table 1. Laparoscopy is also an additional option for surgical treatment. However it is difficult and has a long learning curve. Sica et al. reported, in 2005, the first case of uneventful stone removal and cholecystectomy by laparoscopy [15]. Usually enterolithotomy is performed at the site of impaction. A study by Capell & Davis in 2006 showed bile leak and duodenal leak in patients underwent enterolithotomy alone or enterolithotomy + cholecystectomy. Keeping this in mind, instead of doing enterotomy at the site of impaction which may have underlying mucosal ischemia, certain case reports mention milking the stone down along the jejunum and enterolithotomy or milking proximally into the stomach [16].

Table 1

Different surgical procedures used to treat Bouveret's syndrome.

S.no.	Study group	Yr.	No. of cases	Management
1	Masannat Yazan A. et al.	2006	1	Enterolithotomy
2	Cappell and Davis	2006	37	Enterolithotomy
3	Cappell and Davis	2006	28	Enterolithotomy + Cholecystectomy + Fistula repair
4	Tanwar S. et al.	2008	1	Endoscopic stone extraction
5	O′ Neill C. et al.	2009	2	Enterolithotomy
6	Giese A. et al.	2010	1	Enterolithotomy + cholecystectomy + fistula repair
7	Baloyiannis I. et al.	2012	1	Enterolithotomy + cholecystectomy + fistula repair + gastrojejunostomy
8	Mavroeidis V.K. et al.	2013	1	Enterolithotomy
9	Calvo Espino P. et al.	2014	1	Enterolithotomy
10	Case by S5 at SJH	2014	1	Enterolithotomy + cholecystectomy + fistula repair + gastrojejunostomy

In our case, an open enterolithotomy with cholecystectomy with fistula repair and gastro-jejunostomy was done as the pylorus was pulled up and plastered at the porta hepatis. This resulted in an alteration in the normal anatomic outflow of gastric contents and would have eventually led to gastroparesis. The ulcer noted in first part of duodenum could have led to stricture formation. In order to spare the patient from a future surgery for GOO (this time due to a different cause) which would significantly increase his morbidity, we did a pyloric bypass via gastro-jejunostomy. As the patient was young and vitally stable with no comorbidities, enterolithotomy with cholecystectomy with fistula repair was done in single sitting.

Conclusion

High degree of suspicion is required to diagnose Bouveret syndrome pre-operatively. Deviation from the classical presentation of cholelithiasis with a tilt towards GOO should be taken as a red flag sign for Bouveret's syndrome which definitely has a high incidence with the following features if present:

gall stone more than 2.5 cms in size.

pneumobilia and fluid filled distended stomach in ultrasonogram.

incidental endoscopic finding of obstruction in later part of duodenum & proximal jejunum.

GOO can encompasse a band of etiologies varying from benign to malignant tumors to the rarer Bouveret's syndrome and gall stone ileus. A case of cholelithiasis with presentation of GOO must raise a high index of suspicion towards Bouveret's syndrome. There is no gold standard principle laid down for the management of Bouveret's syndrome as it depends on the site of lodgement of stone, the pathological outcome attributed to the presence of the stone and the anatomical distortion. The most common treatment offered is enterolithotomy with cholecystectomy with fistula repair in a stable patient, whereas in this particular case report the operative findings warranted a deviation, which included incorporation of added gastro-jejunostomy. Hence, this case report not only presents red flags but also this sort of anatomical presentation, requiring a variance of operative management from the standard.

Ethical approval

As this is not a research study but a case reporting with no mention of patient's personal details anywhere in the report, ethical approval has not been sought.

Sources of funding

None.

Author contribution

Conception and Design of case report – Dr. S.V. Arya, Dr. Dheer Singh Kalwaniya.

Drafting of article and revising it for important intellectual content – Dr. Soumya Guha.

Dr. Manigandan G.

Other contributors – Dr. Ralte, Dr. Jasneet, Dr. Ashok Sharma, Dr. Rajkumar Chejara.

Conflicts of interest

None.

Guarantor

Dr. Dheer Singh Kalwaniya.

Dr. Soumya Guha.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.