Continuing Education Activity

Gallstone ileus is one of the rarest forms of all mechanical bowel obstructions and is a complication of cholelithiasis. Patients have non-specific symptoms such as intermittent abdominal pain, distension, nausea, vomiting, and constipation as the gallstone travels through the gastrointestinal tract. This activity describes the evaluation and treatment of gallstone ileus and explains the role of the interprofessional team in managing patients with this condition.

Objectives:

• Identify the etiology of gallstone ileus.
• Describe the formation of a biliary-enteric fistula in the pathophysiology of gallstone ileus.
• Summarize the surgical interventions used to extract stones in gallstone ileus.
• Review the importance of collaboration and communication among the interprofessional team to enhance the care of patients with gallstone ileus.

Introduction

Gallstone ileus is a rare complication of cholelithiasis and is one of the rarest forms of all mechanical bowel obstructions. It is, however, a more common cause of non-strangulating mechanical small bowel obstruction, accounting for 1% to 4% in all patients and up to 25% in the elderly. The diagnosis is often delayed since symptoms may be intermittent and investigations may fail to identify the cause of the obstruction. As a result, gallstone ileus continues to be associated with relatively high rates of morbidity and mortality.[1][2][3]

The condition is managed with surgery but the type of procedure selected depends on the presentation. Many of these patients have other comorbidities like cardiac and lung disease, which also need to be considered. In some cases, bowel resection may be required.

Etiology

It was first described in 1654 by Dr. Erasmus Bartholin and is thought to be caused by the impaction of a gallstone in the gastrointestinal (GI) tract after passing through a biliary-enteric fistula. Gallstone ileus is more common in women (the ratio being 3.5 females to 1 male) and older patients, particularly those older than 60 years. Other factors that contribute to gallstone ileus are a long history of cholelithiasis, repeated episodes of acute cholecystitis, and stones greater than 2 cm. According to literature, approximately 40% to 50% of patients eventually diagnosed with gallstone ileus have a history of recent biliary colic bouts, jaundice, or acute cholecystitis.[4][5]

Epidemiology

Gallstone ileus occurs in 0.3% to 0.5% of all patients with gallstones and is one of the rarest causes of gallstone ileus, occurring in about less than 0.1% of all mechanical obstruction cases and 1% to 4% of non-strangulating mechanical small bowel obstructions. Despite 350 years of medical advances, mortality remains high, ranging from 12% to 27%, partially because of non-specific symptoms, unremarkable biochemical investigations, high misdiagnosis rate, and delayed discovery.

Pathophysiology

The etiology of gallstone ileus results from adhesions forming between an inflamed gallbladder and an adjacent GI tract followed by gallstones causing pressure necrosis or inflammation between the two tissues. The inflammation or necrosis results in erosion and formation of a cholecyst-enteric fistula. Gallstones can move from the gallbladder to the GI tract through this direct access. Fistulas can form within any part of the GI tract, with approximately 60% occurring in the duodenum due to the proximity. Less commonly, a gallstone may enter the duodenum through the common bile duct, a dilated papilla of Vater, or after an endoscopic sphincterotomy. A fistula between the gallbladder can also occur with the stomach, transverse colon, and distal small bowel. The pathology may be part of the natural course of Mirizzi syndrome.

Spillage of gallstones during laparoscopic cholecystectomy may also result in an intraabdominal abscess that can ulcerate the intestinal wall and lead to an entryway into the bowel lumen. The site of fistula formation, size of gallstone, and size of bowel lumen will determine the location of impaction. Gallstones most commonly impact at the terminal ileum and ileocecal valve due to their narrow lumen and potentially less active peristalsis. The majority of gallstones smaller than 2 cm may pass spontaneously while those larger are more likely to become impacted. The presence of diverticula, strictures, or neoplasms can also serve as impaction sites.[6][7]

When gallstone impaction occurs, there is pressure generated on the bowel walls, with proximal distension. Eventually, necrosis and even perforation can occur.

History and Physical

Unfortunately, the diagnosis is often delayed since symptoms may be non-specific, intermittent or investigations fail to identify the cause of the obstruction due to the “tumbling phenomenon” as the stone tumbles through the variable portions of the GI tract. Patients typically present 4 to 8 days after symptoms start. The signs and symptoms are usually non-specific, including crampy, intermittent abdominal pain, variable abdominal distention, nausea, vomiting, and constipation intermittently as the stone travels through the GI tract. The degree of obstruction will vary based on the location of the gallstone, and occasionally the gallstone passes through the rectum without notice. The physical examination may be non-specific, but a provider may appreciate abdominal distension, abdominal tenderness, high-pitched bowel sounds, and obstructive jaundice. Importantly, the intensity of the pain often does not correlate with the underlying anatomic alteration.

Evaluation

The diagnosis is usually made three to eight days after symptoms, and a correct preoperative diagnosis is reported in 30% to 70% of cases. As a result, a high index of suspicion is necessary. Laboratory studies are usually non-specific, as only one-third of patients present with jaundice and/or alteration of hepatic enzymes. Ultrasound can be used to demonstrate fistulas, pneumobilia, impacted gallstones, and residual cholelithiasis or choledocholithiasis, but difficulties of locating stones and distortion by bowel gas make ultrasound suboptimal. Plain abdominal radiographs can also be used for diagnosis, with Rigler’s triad being present in some cases with partial or complete intestinal obstruction, pneumobilia or contrast in the biliary tree, and an ectopic gallstone. The gallstone can change position on serial films. The sensitivity ranges from 40% to 70%.

Computed tomography (CT) scanning is a better entity and has a sensitivity of 93%. Balthazar et al. described a fifth sign: two air-fluid levels in the right upper quadrant on an abdominal radiograph corresponding to the duodenum and the lateral to the gallbladder, yet this sign is only present in approximately 24% of patients at the time of admission. Therefore, if a clinician has a clinical suspicion but the patient has negative x-ray findings, a CT scan should be performed. Findings consistent with gallstone ileus include gallbladder wall thickening, pneumobilia, intestinal obstruction, and obstructing gallstones. Pneumobilia, a non-specific finding, is found in approximately 30% to 60% of patients. A hepatobiliary iminodiacetic acid (HIDA) scan, magnetic resonance cholangiopancreatography (MRCP), and esophagogastroduodenoscopy (EGD) may be performed if there is still a question after CT scanning. However, gallstone ileus is more typically diagnosed intra-operatively when a patient is undergoing laparotomy for small bowel obstruction of unknown origin.[5][8][9]

Treatment / Management

Although the treatment and management of gallstone ileus are still under controversy, the main therapeutic goal is the extraction of the offending stone after resuscitation. Gallstone ileus involves three key elements: cholelithiasis, biliary-enteric fistula, and intestinal obstruction. Cholelithiasis and fistula are typically addressed by stone removal and fistula closure. Stone removal typically addresses intestinal obstruction. The current surgical options are 1) simple entero-lithotomy; 2) entero-lithotomy, cholecystectomy, and fistula closure (one-stage procedure); and 3) entero-lithotomy with cholecystectomy performed later (two-stage procedure). Most conclude that entero-lithotomy alone is the best option for most patients. Some have advocated that a one-stage procedure (cholecystectomy and fistula repair) should be considered in low-risk patients in good general condition and adequately stabilized preoperatively. Two-stage surgery is usually an option for those with persistent symptoms despite entero-lithotomy surgery. Whether interval biliary surgery should be performed at the same time as the obstruction relief (one-stage procedure), performed later, or not at all remains unanswered.[3][10][11]

Endoscopic management can be done but it depends on the location of the stone. Anecdotal reports indicate that it is difficult and can be associated with bleeding. Plus, if the bowel enterotomy is large, closure can be difficult.

Prior to surgery, patients need aggressive hydration and the replacement of electrolytes. Admission to the ICU for patients with numerous comorbidities is recommended.

Differential Diagnosis

• Acute pancreatitis
• Bile duct stones
• Cholecystitis
• Bile duct malignancy
• Peptic ulcer disease

Prognosis

Gallstone ileus is not a benign disorder and most patients also have several comorbid features. High morbidity and mortality rates have been reported for patients with gallstone ileus. An urgent repair is often associated with postoperative complications. The longer the operating time, the higher the risk of postoperative complications. The one-stage procedure has higher morbidity but the two-stage procedure means that the patient has to undergo general anesthesia again.

Complications

Complications may include bowel obstruction, infection, and pancreatitis.

Other common complications include acute renal failure, wound dehiscence, biliary fistula, sepsis, urinary tract infections, anastomotic leaks, intra-abdominal abscess, and death.

Deterrence and Patient Education

Patients with gallstone ileus should be educated regarding their disease process and the importance of close follow-up with their providers. They should be made aware of the signs and symptoms of common postoperative complications and be advised to seek care immediately should any of these develop. Patients should also be encouraged to maintain a healthy weight by eating a healthy diet and getting regular exercise.

Pearls and Other Issues

An open procedure is the "gold standard" to treat this condition. It is difficult to examine the distended bowel and find the exact location of the gallstone during laparoscopy. Plus, laparoscopy does take a longer time to perform and needs more experienced surgeons. Some reports do indicate that that the laparoscopic enterolithotomy and classic surgery can produce good results and may help with diagnosis and also be therapeutic.

Enhancing Healthcare Team Outcomes

Gallstone ileus is best managed by an interprofessional team that includes a radiologist, a gastroenterologist, and a general surgeon. Since most of these patients are frail seniors, a critical care specialist should be involved in their care before and after surgery.

The nurse plays a vital role in educating patients with symptoms of biliary colic and acute pancreatitis. The patient should be educated about the symptoms of gallstone ileus and when to seek medical help. Further, when the patient is admitted with bowel obstruction, the nurse should closely monitor the abdominal girth, urine output, and nasogastric residuals. These patients are elderly and are also at risk for deep vein thrombosis (DVT) and aspiration pneumonia- hence appropriate precautions should be taken. Finally, since obesity is a risk factor for gallstones, patients should be urged to lose weight, eat a healthy diet, and exercise regularly. [Level 5][12][13]

Outcomes

Only anecdotal reports and small case series exist on the management of gallstone ileus. If the diagnosis is delayed, it carries a mortality rate of 15%-30%. Further, these patients have a prolonged stay in the hospital and develop a wide range of complications such as a prolonged ileus, recurrent bowel obstruction, aspiration pneumonia, and fistulas. [Level 3][9][14]