The Bouveret syndrome was first described in 1896 by a French physician, Léon Bouveret (1850-1929), who reported two cases of gastric outlet obstruction due to gall stones - “Revue Medicale” [(3)].
The typical Bouveret syndrome is characterized by a proximal small bowel gallstone ileus with gastric outlet obstruction, due to impaction of large gallstone(s) within the duodenal bulb or proximal duodenum [(4)]. Sometimes the pylorus or antrum can be affected also. Bilioenteric fistula is often caused secondary to previous cholecystitis [(5)]. Owing to its uncommon and unpredictable presentation, Bouveret's syndrome can pose a diagnostic and therapeutic challenge for clinicians [(6)].
Ultrasound, computed tomography and endoscopy have been diagnostic in our case. CT did not appreciate the underlying pathology (gallbladder carcinoma), whereas contrast enhanced ultrasound revealed typical features.
Risk Factors for Bouveret's syndrome include age over 70 years, female gender, gallstones larger than 2.5 cm and post surgical altered GI anatomy [(7)]. Common symptoms include vomiting (87 %), abdominal pain (71 %), haematemesis (15 %), recent weight loss (14 %) and anorexia (13 %) [(8)]. Bouveret's syndrome results in complications such as duodenal perforation, hematemesis (Mallory-Weiss tear), distal oesophageal rupture (Boerhaave's syndrome) and gastric bezoar [(8)] amongst others. In patients with gastric outlet obstruction primary or secondary malignancy of the duodenum and surrounding structures needs to be considered, including ductal adenocarcinoma of the pancreas, cholangiocarcinoma, lymphoma, carcinoid, metastases and, as shown in our patient, advanced gallbladder carcinoma.
Abdominal X-ray can show the so-called “Rigler's triad” of pneumobilia, small bowel obstruction and gall stones, in 30 – 35 % cases [(9)]. Abdominal ultrasound is most helpful in the hands of experienced examiners, but review of the literature revealed positive diagnosis in 60 % of cases. The sonographic findings include a fluid filled distended stomach, features suggestive of cholecystitis, ectopic gall-stones and pneumobilia. Abdomen CT with contrast has a published sensitivity of 93 %, specificity of 100 % and diagnostic accuracy of 99 % [(10)] but missed the neoplastic nature of the disease in our case. MRCP can differentiate between gall stones and surrounding bile (CT cannot in 15 – 25 % cases) [(11)]. Endoscopy is both diagnostic and therapeutic. The common findings are gall stone causing obstruction (69 %), gastric outlet obstruction without cause identified (31 %) whilst the fistula is visualized in only 13 % of cases [(8)].
Treatment options include endoscopic, open surgical and laparoscopic approaches. Endoscopic techniques include endoscopic extraction, endoscopic laser lithotripsy, extracorporeal shockwave lithotripsy and intracorporeal electrohydraulic lithotripsy [(4)]. The first successful endoscopic extraction was described in 1985 by Bedogni et al [(12)]. 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. Hence it can be technically challenging, time-consuming and the success rates reported in case series have been less than 10 % [(13)]. Endoscopy could be considered as the first treatment in the elderly patient with significant comorbidity, combined with mechanical, electrohydraulic, or laser lithotripsy. Surgical options include simple stone extraction or definitive fistula closure in either 1 or 2 stages. The former is associated with a lower mortality rate and spontaneous fistula closure is reported, whilst the latter is thought to decrease the risk of subsequent complications [(14)]. Indications for open surgery 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 [(13)]. Treatment choice should consider advanced age, comorbidity [(15)], underlying etiology and available equipment [(16)].
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