Duodenal obstruction due to a gallstone (Bouveret Syndrome): Case report and literature review
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Sean S. Parsa, M.D 1*., Joy Liu, MD., 2*, Tahereh Ghaziani, MD 2, J.Augusto Bastidas, M.D. 1
* Both authors contributed equally.
Sean S. Parsa:
[email protected] Joy Liu:
[email protected] Tahereh Ghaziani:
[email protected] J. Augusto Bastidas:
[email protected]
1 National Surgical Associates 14981 National Ave. Ste 4 Los Gatos, CA 95031 United States
2 Beth Israel Deaconess Medical Center Harvard Medical School Department of Internal Medicine 330 Brookline Ave Boston, MA 02215
3
Beth Israel Deaconess Medical Center Harvard Medical School Liver Center and Liver Transplant Institute, LOMB8 110 Francis St, Boston, MA 02215 Corresponding author: Tahereh Ghaziani Beth Israel Deaconess Medical Center Lmob-8e 110 Francis Street Boston MA 02215 Phone: 617/632-1070 Fax: 617/632-1065
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Author contributions: Sean Parsa: substantial contribution to conception and design, drafting the article Joy Liu: drafting the article and revising for important intellectual content Tahereh Ghaziani: critical revision; supervision J. Augusto Bastidas: critical revision; supervision Conflicts of interest: no conflict of interest to report
Abstract: Bouveret's syndrome is a rare cause of gallstone ileus that occurs more often in the elderly and has a female predominance. In this case report we describe a patient with classic risk factors and typical presentation who underwent one-stage surgical repair without complications. Bouveret's syndrome is a diagnosis that requires a high index of suspicion; diagnosis is often delayed. Advances in imaging have improved diagnostic accuracy, but surgical exploration is required in some cases. Although non-invasive methods of treatment have been utilized with some success, surgery remains the treatment of choice for most patients. However, there is still controversy regarding best surgical approach. Choice of treatment may depend on a patient's underlying comorbidities and surgical risk factor, age, and likelihood of recurrent symptoms.
Introduction Although gallstones are a common occurrence in the general US population, gallstone ileus accounts for just 1 to 4% of bowel obstructions and only 6% of all patients with gallstones will develop complications such as fistula formation.(1, 2) Bouveret's syndrome occurs when a gallstone passes through a cholecysto-enteric fistula and impacts the duodenum at the level of the gastric outlet, causing obstruction.(2) Presenting symptoms are usually nonspecific and the diagnosis can be challenging. A high index of suspicion is essential for early diagnosis and treatment. Various interventions have been reported with variable success rates and choice of treatment may depend on surgical risk factors and severity of symptoms.
Case Presentation A 78–year-old woman presented to the emergency room with two weeks of nausea and bilious vomiting. She denied pain, but described worsening abdominal distension. There was no biliary colic, diarrhea, or other gastrointestinal symptoms. Past medical history was notable for hypertension, diabetes, hyperlipidemia and obesity. She had an intentional 60-lb weight loss. Physical exam revealed an alert, oriented, elderly
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patient in no acute distress. Initial vitals showed a temperature of 36.8°C, blood pressure of 143/64, heart rate of 90, respiratory rate of 18 and 93% oxygen saturation on room air. She had upper abdominal distention with tympany to percussion but no tenderness. Laboratory data showed a white blood cell count of 13,400 cells/mm! with 81.5% neutrophils, hemoglobin of 13.0 g/dL, platelets of 199,000/uL, potassium of 2.2 mmol/L, chloride of 88 mmol/L and normal liver function tests, amylase and lipase. Abdominal xray revealed an enlarged gastric bubble but no free air. (Figure-1) Abdominal CT scan showed pneumobilia, a large stone in the proximal duodenum, and high-grade gastric outlet obstruction. (Figure-2) Intravenous fluids were started and electrolyte repletion was administered. On hospital day 2, the patient was taken to the operating room. At surgery the gallbladder was adherent to duodenum with a cholecysto-duodenal fistula and large impacted stone obstructing the gastric outlet. Open duodenotomy with enterolithotomy was performed with fistula excision, cholecystectomy, and gastro-duodenal anastomosis. She recovered fully with no immediate post-operative complications. She had complete relief of obstructive symptoms, and was discharged home.
Discussion Bouveret's syndrome is a rare condition that more often occurs in elderly, especially female, patients with co-morbidities that predispose to cholelithiasis, such as hypercholesterolemia and diabetes. After episodes of cholecystitis, gallstones may cause fistula formation through chronic inflammation from adhesions and pressure necrosis between the gallbladder and bowel wall.(2, 3) Fistulas most often occur between the gallbladder and duodenum, but may occur anywhere along the GI tract. Though uncommon for an obstructing stone to pass spontaneously, it has been reported.(1, 4) Known risk factors for Bouveret’s syndrome include: age >70 years, female gender, gallstones >2.5 cm, and surgically altered GI anatomy.(5) Nausea and vomiting are the most common symptoms and may occur in 86% of patients, while abdominal pain may occur in up to 81%.(4) Other symptoms include hematemesis, weight loss, and anorexia. Symptoms may wax and wane as the stone “tumbles” through the GI tract. The exam is also generally non-specific, often with features of bowel obstruction such as hypoactive bowel sounds, distention, and succession splash. Although our patient presented with typical features, Bouveret’s syndrome has been associated with more unusual findings such as severe esophagitis, pancreatitis, gangrenous cholecystitis, or perforation from ischemic ulceration (6, 7). Morbidity and mortality, while improved from a rate of 60% reported in the 1960s, remain high due to delay in diagnosis, patient co-morbidities at time of surgery, and post-operative complications.(8) The combination of pneumobilia, calcified right upper quadrant mass, and dilated stomach, or “Rigler’s triad,” is specific for gallstone ileus but may only be found in in
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21% of cases.(9) Abdominal ultrasound has varying sensitivity, but combined with plain X-ray may increase sensitivity by 20%.(10) However, ultrasound may be non-diagnostic when there is excessive intestinal gas.(11) Comparatively, CT has a detection rate of greater than 75%. Additional benefits include being able to characterize the level of obstruction, number and size of the calculi, and the existence of extra-luminal complications such as abscess.(12) However, if a gallstone is iso-attenuating—in about 15-25% of cases—it may be missed.(2, 13) In these cases, magnetic resonance cholangiopancreatography (MRCP) may be useful, but is practically limited by time, expense, and the patient’s clinical status. Therefore, CT may be a preferred method of imaging. In 20% of all cases the final diagnosis will only be established during surgery.(5) Initial treatment of Bouveret’s syndrome, much like any GI obstruction, begins with appropriate resuscitation and stabilization, correction of electrolyte/acid-base abnormalities and relieving the obstruction as safely as possible. EGD is considered a first-line option, despite low success rates, because it is minimally invasive.(2) Stone retrieval during endoscopy often necessitates the use of different snares, forceps, retrieval baskets and nets, biliary balloons, and sometimes even a side-viewing endoscope, and can be technically challenging.(14, 15) Other methods, such as intracorporeal laser lithotripsy (ILL), extracorporeal shockwave lithotripsy (ESWL) and intracorporeal electro-hydraulic lithotripsy (IEHL) require multiple sessions and cannot address extra-luminal complications or exclude the presence of more distal stones. Nonetheless, non-surgical approaches, or approaches combining surgery with less invasive methods, should be considered in patients who are poor surgical candidates.(16) Surgical management has success rates upwards of 80%, but higher risk of complications and mortality.(4) There are three options: enterolithotomy alone, one-stage procedure (enterolithotomy, cholecystectomy and fistula repair) or two-stage (enterolithotomy followed by cholecystectomy and fistula repair). Whether cholecystectomy and fistula repair should be performed, especially in high-risk candidates, still remains a controversial issue, since morbidity and mortality may be high.(1, 8, 17, 18) Many patients have spontaneous fistula closure, and in one case series, only 11% of patients required fistula repair.(5) On the other hand, cholecystectomy and fistula repair address potential secondary complications such as cholecystitis, cholangitis, and the risk of gallbladder carcinoma associated with persistent bilio-enteric fistula.(3, 17) Therefore, it seems reasonable to recommend one-stage repair to patients with few co-morbid conditions and longer life expectancy who could reap the benefits of preventing these complications.(19) Laparoscopic techniques, while less morbid, are associated with higher rates of conversion, especially when fistula repair is attempted.(8) The decision to use minimally invasive surgery should be made on an individual basis and operator experience.
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In conclusion, Bouveret’s syndrome is a rare cause of gallstone ileus that often occurs in the elderly and can be associated with significant morbidity and mortality if diagnosis is delayed. Imaging is an essential part of accurate and early diagnosis. Noninvasive methods such as EGD should be considered for poor surgical candidates. Enterolithotomy alone may be the best option for older high-risk patients, while the one-stage procedure (enterolithotomy, cholecystectomy and fistula repair) should be considered for patients with low risk and favorable ASA score. Laparoscopic techniques should be considered whenever possible to minimize the physiological trauma, however, higher rates of conversion are expected. However, there is still controversy on the best surgical approach for these patients, and further studies and comparison of different treatments are needed.
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.
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10. Ripolles T. M-DA, Errando J., et al. Gallstone ileus: increased diagnostic sensitivity by combining plain film and ultrasound. Abdom Imaging 2001;26:401-5. 11. Beuran M. IIVMD. Gallstone ileus- clinical and therapeutic aspects. J Med Life. 2010;3(4):365-71. 12. Gan S. Roy-Choudhury S. AS, et al. More than meets the eye: subtle but important findings in Bouveret's syndrome. Am J Roentgenol. 2008;191:182-5. 13. Pickhardt PJ, Friedland J.A., Hruza D.S., et al. CT, MR cholangiopancreatography, and endoscopy findings in Bouveret's syndrome. Am J Roentgenol. 2000;180(4):1033-5. 14. Gannavarapu B TJ, Aadam AA. Endoscopic management of gastric outlet obstruction due to Bouveret syndrome Gastrointestinal Endoscopy. 2016;83(5S):AB644. 15. Moschos J. PI, Antonopoulos Z., et al. Complicated endoscopic management of Bouveret's syndrome: a case report and review. Rom J Gastroenterol. 2005;14:75-7. 16. Stein P.H. LC, Sejpal D.V. . A rock and a hard place: successful combined endoscopic and surgical treatment of Bouveret's syndrome. Clin Gastro Hep. 2015;13(13):A25-6. 17. Clavien P.A. RJ, Burgan S., et al. Gallstone ileus. Br J Surg. 1990;77:737. 18. Rodriguez-Sanjuan J.C. CF, Fernandez M.J., et al Cholecystectomy and fistula closure versus enterolithotomy alone in gallstone ileus. Br J Surg. 1997;6:634-7. 19. Nickel F ea. Bouveret's syndrome: presentation of two cases with review of the literature and development of a surgical treatment surgery. BMC Surg. 2013(13):33.
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Figure 1: Abdominal x-ray showing gastric distention
Figure 2: Computerized tomography scan showing obstruction at the level of the gastric outlet with accompanying distention
