AUTHOR AND EDITOR INFORMATION

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• Follow-up
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INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Fistula is derived from the Latin word that means "pipe." A fistula is an abnormal connection between two epithelialized surfaces. It usually involves the gut and another hollow organ, such as the bladder, urethra, vagina, or other regions of the GI tract. Fistulas may also form between the gut and the skin or between the gut and an abscess cavity. Rarely, fistulas arise between a vessel and the gut, resulting in profound GI bleeding, which is a surgical emergency.

Most GI fistulas (75-85%) occur as a complication of abdominal surgery. However, 15-25% of fistulas evolve spontaneously and are usually the result of intra-abdominal inflammation or infection. Regardless of their cause, fistulas have a tremendous impact on patients and society. Increased morbidity and mortality rates, greater health care costs for diagnosis and treatment, prolonged hospital stays, and delayed return to work are just a few direct consequences of this condition.

Fistulas were associated with considerable mortality rates until the 1960s. In the decades following the 1960s, the introduction of ICUs and parenteral nutrition lowered the mortality rate to approximately 20%; however, prolonged hospital stays and the high cost of medical and surgical care remained unchanged. In addition, the frequency of fistula formation has not decreased because of advanced and complicated disease, complex surgical techniques, and an aging population.

Several classification systems for fistulas exist, none of which are used exclusively. The 3 most commonly used classification systems are based on anatomic, physiologic (output volume), and etiologic characteristics. Used in combination, these classifications can help to provide an integrated understanding and optimal management scheme for the fistula. Anatomically, the fistulas are named according to their participating anatomic components, and they can be divided into internal and external fistulas. Internal fistulas connect the GI tract with another internal organ, peritoneal space, retroperitoneal space, thorax, or blood vessel. External fistulas, which commonly occur postoperatively, are abnormal connections between the GI tract and the skin.

Pathophysiology

Contrary to common belief, fistulas do not necessarily develop as a consequence of downstream stenosis of the intestine.

• Gastric fistulas are iatrogenic in most cases (85%). The remainder of cases are usually a consequence of irradiation, malignancy, inflammation, and ischemia. Anastomotic leak after a gastric resection for cancer, peptic ulcer disease, or bariatric surgery can lead to leakage of intestinal or gastric juices, which initiates a cascade of events: localized infection, abscess formation, and, possibly, abscess and fistula formation.
• Nearly 80% of small bowel fistulas result from complications of abdominal surgery. These fistulas may occur from disruption of the anastomotic suture line, inadvertent iatrogenic enterotomy, or small bowel injury at the time of closure. Inadequate blood flow from devascularization or tension at the anastomotic suture lines, anastomosis of diseased bowel, or perianastomotic abscess may compromise the integrity of surgical anastomoses.
• Crohn disease, malignancy, peptic ulcer disease, and pancreatitis spontaneously cause 10-15% of small bowel fistulas. In patients with Crohn disease, fistulas arise from aphthous ulcers that progress to deep transmural fissures and inflammation, subsequently leading to adherence of the bowel to adjacent structures that eventually penetrate other structures. Microperforation with abscess formation leads to subsequent macroperforation into the adjacent organ or skin, hence, fistula formation. Crohn fistulas are more often internal and less commonly external (to the skin). Ileosigmoid fistulas, usually a complication of a diseased terminal ileum that invades the sigmoid colon, are the most common type of fistula between two loops of bowel. Enteroenteric, gastrocolic, duodenocolic, enterovesical, rectovaginal, and perianal fistulas are other potential fistulizing complications of Crohn disease. Perianal fistulas are the most common external fistulas in patients with Crohn disease.
• Colonic fistulas are primarily a consequence of intra-abdominal inflammation but can also occur after surgical intervention for an inflammatory condition. Inflammatory bowel disease (IBD), diverticulitis, malignancy, and appendicitis (especially with the presence of an appendiceal abscess requiring percutaneous drainage) are the most common inflammatory conditions that lead to colonic fistulas. See related CME at Inflammatory Bowel Disease.
• Aortoenteric fistulas most commonly occur secondarily, usually after the surgical placement of a graft. Aortoenteric fistulas can develop in several ways, as follows:
• • A suture line, most commonly the proximal one, can communicate with the intestinal tract.
  • A suture line pseudoaneurysm can erode into adjacent bowel.
  • Erosions can occur in the graft close to the suture line, resulting in the midportion of the graft eroding into adjacent bowel. Conversely, primary aortoenteric fistulas almost always result from erosion of the aneurysmal or infected aorta into surrounding areas, most commonly the bowel.

Frequency

United States

In developed countries, Crohn disease is the most common cause of spontaneous fistula formation. In their lifetime, as many as 40% of patients with Crohn disease develop a fistula, most of which are external or perianal.

The incidence of fistula formation in patients with diverticulitis is much lower. Fistula formation complicates diverticulitis in 1-12% of patients. Incidentally, colovesical fistulas in men and colovaginal fistulas in women are the most common types of fistulas in this population.

Fistulas can complicate radiation therapy weeks to years after treatment. Radiation therapy for malignancy is associated with fistula formation in approximately 5-10% of patients. Notably, surgery and anastomosis in previously irradiated tissue increases the risk of anastomotic leak and, subsequently, fistula formation.

International

Internationally, the frequency of various types of fistulas may vary in correlation with its prevalence in different populations.

For example, the prevalence of fistulas secondary to Crohn disease may be less prevalent in Africa primarily because the disease is less prevalent in that population. However, the prevalence of obstetric fistulas may be higher in developing countries because of obstructed labor (including malpresentation and cephalopelvic disproportion) and lack of prompt access to emergency obstetric care. Accurate prevalence rates of obstetric fistulas are unavailable, likely because of inaccurate reporting of the medical condition and the stigma of its associated symptoms.

Mortality/Morbidity

Pain, wound management, abscess formation, local infection, nutritional deficiencies, and recurrent septic states are just a few of the physical consequences of intestinal fistulas. Patients with fistulas most likely present with much more than physical discomfort and pain. The stigmas of malodorous fistula drainage, malnutrition, and emotional distress also cause significant psychological consequences. In addition, patients with postoperative fistulas have the added distress of lengthy hospital stays, associated morbidity, a delay in returning to work, and restricted social activities. Considerable mortality is associated with fistulas, primarily from sepsis.

In one study, in patients who developed fistulas after pancreaticoduodenectomy, specific factors were associated with increased mortality. These factors included fistula site, underlying disease, low hospital volume, the surgeon's experience, high intraoperative blood loss, and complications.

Race

Racial differences in patients with fistulas generally parallel those of the underlying disease or condition that predisposed the person to developing fistulas. For example, since Crohn disease is more common in whites, patients with Crohn disease who develop fistulas are more likely to be white.

Sex

Colovesical fistulas are more common in men and in women who have undergone a hysterectomy. Colovaginal fistulas occur in women. Otherwise, fistulas are equally prevalent in males and females.

Age

As with race, age parallels the etiology or underlying condition that predisposes the patient to developing fistulas.

CLINICAL

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History

Symptoms caused by fistulas that involve two segments of the bowel vary depending on the location of the fistula and the amount of bowel bypassed. For this reason, enteroenteric fistulas in which only a short segment of bowel is bypassed may be asymptomatic and diagnosed incidentally based on imaging findings or during surgery. Conversely, ileosigmoid fistula may cause diarrhea, weight loss, or abdominal pain.

• Patients with gastrocolic fistulas may present with symptoms of abdominal pain, weight loss, and feculent belching.
• Enterovesical and colovesical fistulas are easier to diagnose in patients who present with symptoms of pneumaturia, fecaluria, and recurrent urinary tract infections.
• Patients with rectovaginal and anovaginal fistulas may be asymptomatic and present with symptoms only when the bowel movements are more liquid. Possible symptoms include inadvertent passage of stool or gas, dyspareunia, and perineal pain.
• Patients with external fistulas generally present with symptoms of drainage through the skin.
• Patients with aortoenteric fistulas may report rectal bleeding.

Physical

Fluid or stool output through the skin, diarrhea, abdominal tenderness, weight loss, signs of malnutrition, and electrolyte imbalances are all possible findings in patients with fistulas.

• Malnutrition is a significant cause of morbidity and mortality, especially with enterocutaneous fistulas. Typically, patients with low-output (<200 mL/24 h) fistulas should receive their full resting expenditure, 1-1.5 grams of protein per kilogram per day, and a lipid intake that accounts for approximately 30% of daily caloric intake. Patients with high-output (>500 mL/24 h) fistulas should receive 1.5-2 times their resting energy expenditure, 1.5-2.5 grams of protein per kilogram per day, and twice the recommended daily allowance of lipids.
• Rectal bleeding may be a finding in patients with a history of radiation therapy.
• Hypotension and rectal bleeding may occur in patients with aortoenteric fistulas.

Causes

• Surgical procedures to treat cancer, IBD, lysis of adhesions, or peptic ulcer disease
• IBD, such as Crohn disease and ulcerative colitis
• Diverticular disease
• Radiation
• Malignancy, especially gynecologic and pancreatic
• Appendicitis
• Perforation of duodenal ulcers
• Abdominal trauma, such as gunshot wounds, stabbing (sharp trauma), or motor vehicle accident (blunt trauma)
• Aortic aneurysm, infected aortic graft, or previous abdominal aortic surgery

DIFFERENTIALS

Section 4 of 11  

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• Clinical
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• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

WORKUP

Section 5 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Serum tests: Albumin and prealbumin levels should be obtained, as well as blood urea nitrogen (BUN), creatinine, and electrolyte concentrations. These are used to determine the patient's nutritional status and whether fluid or metabolic disturbances are present (more of a concern for high-output fistulas). Although CBC count results may be within the reference range, leukocytosis may be present if an undrained abscess or a continued inflammatory process has developed within a segment of the bowel. Anemia may be present with chronic disease or if a malignant process is involved.
• Microbiology: Abscess culture findings may be helpful, especially in the presence of sepsis or ongoing infection (the predominant organism involved being Escherichia coli). Cultures of enterocutaneous fistula output may not be of much clinical use, as normal bowel flora often predominates.
• Urinalysis or urine culture: For colovesical fistulas, urinalysis usually reveals increased WBC count and bacteria levels. Urine culture findings may help direct antibiotic therapy.

Imaging Studies

• CT scanning: Abdominal and pelvic CT scanning is the imaging method of choice to evaluate Crohn disease and possible fistulas. While identification of the fistula is not always possible, CT scanning often reveals perifistular inflammation. This provides additional information regarding the possible etiology of the fistula and the extraluminal involvement of disease. Revealing abscess cavities or excluding possible sources of sepsis is an important step in the evaluation of patients with suspected fistulas. This information may also prove helpful if surgical intervention is planned. CT angiography may be used in the diagnosis of suspected aortoenteric fistulas if the patient is stable.
• MRI: Although MRI is reported as an imaging modality that can help identify and characterize enteric fistulas, motion artifact may limit its usefulness, and MRI is not considered a routine adjunctive study in the evaluation of patients with enteric fistulae. T1-weighted images provide information relative to the inflammation in fat planes and possible extension of the fistula relative to the surrounding visceral structures. T2-weighted images can demonstrate fluid collections along the fistula tract and inflammatory changes within the surrounding muscle.
• Fistulography: Radiographic study with contrast medium (usually given at the site of fistula output) may be performed to help delineate the extent and communication of the fistula with the underlying bowel.
• Ultrasonography: Ultrasonography can be used in conjunction with physical examination to identify abscesses and fluid collections along the fistula tract.
• Barium enema and small bowel series: Contrast studies to evaluate the stomach, small intestine, and colon may reveal a fistula but may otherwise be helpful in determining the cause of fistula formation through identifying diverticular disease, Crohn disease (characteristic string sign), or evidence of malignancy.
• Cystography and CT cystography: This procedure can help evaluate for the presence of possible enterovesical fistula.
• Angiography: Angiography may assist in preoperative planning and evaluation of aortoenteric fistulas in a stable patient or determine the arterial source of bleeding in those with a less common arterioenteric fistula.

Other Tests

• Oral administration of nonabsorbable markers: Patients can be given charcoal or Congo red dye orally to verify the presence of an enterocutaneous fistula. It is not helpful in determining which portion of bowel is involved. This test is often used in postoperative patients with persistent drainage from a wound in whom an enterocutaneous fistula is suspected or in women who have persistent vaginal drainage in whom a rectovaginal fistula is suspected.

Procedures

• Endoscopy or colonoscopy: This can be helpful in determining the origin of the bowel disease that caused the fistula, but it is not a particularly helpful or necessary study to reveal a fistula. Biopsy samples may be obtained during the procedure and are useful in diagnosing IBD or Crohn disease and malignancy.
• Fistuloscopy: Reported and described, this procedure is not a widely used modality for diagnostic and therapeutic use with enteric fistulas. A small-caliber endoscope is passed into the lumen of the fistula in an attempt to identify the source of the fistula. Fistuloscopy may identify abscesses and visualize the bowel involved. Therapeutically, a drain can be placed or Fibrin glue sealant may be applied to close the fistula.
• Cystoscopy: Useful in the evaluation of suspected enterovesical fistula, cystoscopy may allow one to visualize fistulas from within the bladder.
• Dye injection: Instilling methylene blue into the rectum and examining a vaginal tampon 15 minutes after placement can often establish the presence of a rectovaginal fistula.

Histologic Findings

Histologic findings of fistula site biopsy are usually consistent with chronic inflammation. In patients with Crohn disease as the causative factor, transmural involvement with noncaseating granulomas and lymphoid aggregates throughout the bowel wall may be observed. In patients with carcinoma, inflammation adjacent to the tumor remains a typical finding. The clinical scenario and test results are usually helpful in determining the diagnosis.

Staging

Staging is appropriate when the etiology of the fistula is carcinoma.

TREATMENT

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Medical Care

Several elements are required to successfully treat patients with an intestinal fistula: adequate nutrition, control and maintenance of the fistula drainage site, appropriate treatment of infection, and avoidance of sepsis. Spontaneous closure of a proportion of GI fistulas with nonoperative management is well documented. Although dependent on the etiology of the fistula, 60% or more close if they are iatrogenic, if no distal obstruction is present, if no foreign body is involved, if the tract is long, if there is a low output, and if there is no active infection. Numerous studies have delineated the important determinants associated with both decreasing the time to closure of a fistula and decreasing a patient's overall associated morbidity and mortality.

• Conservative management of enteric fistulas has been described for periods of up to 3 months. One study demonstrated that 90% of the fistulas that spontaneously closed did so within the first month, once management of sepsis had been established. Of note, none of the fistulas spontaneously closed after 3 months. Factors to consider for fistulas that do not spontaneously close include malignancy, foreign bodies in the fistula tract, short fistula tracts with epithelialization, undrained abscess cavities, distal obstruction, radiation enteritis, active IBD of involved bowel segment, and high-output status of fistula. In these cases, surgical repair may be the definitive treatment.
• Initial fistula management should address each of the following resuscitation and stabilization issues in the patient with a GI fistula:
• • Nutrition: Total parenteral nutrition (TPN) has long been regarded as an essential therapy (especially in high-output fistulae) to decrease output and to maintain good nutritional status.
  • Skin care and drainage control: Control of enteric contents draining from the fistula continues to be a topic of ongoing research and development. Standard ostomy supplies and other methods of skin care and drainage control can be used in an attempt to reduce or eliminate the persistent tissue inflammation and infection surrounding the fistula, which can lead to sepsis. Recently, the use of the vacuum-assisted closure (VAC) device to better manage output has been reported to help improve the perifistula environment. All of these techniques and devices are used not to close the fistula, but rather to help keep the surrounding tissues healthy and to allow the fistula to heal on its own.
  • Avoidance of infection and sepsis: Use of CT scanning and ultrasonography can help determine if fluid collections or abscesses are present along the abscess tract. Identification of these fluid collections often allows for CT-guided drainage of these loci to prevent infection. Along with better drainage control and appropriate antibiotic treatment, this helps decrease the morbidity and mortality associated with enteric fistulae and allows for a safer period of conservative management. Radiologically placed catheters have been demonstrated to safely and successfully drain most abscesses.
  • Fluid volume depletion: In patients with proximal, high-output fistulas, the volume depletion associated with the drainage can be a significant problem. Whether medications, such as octreotide (a synthetic substitute of somatostatin), help to close fistulas remains unclear. Research has demonstrated both significant and nonsignificant effects on the closure of fistulas, yet these studies agree that octreotide does have the added benefit of decreasing overall fistula output.

Surgical Care

The period of nonoperative management of an enteric fistula, while allowing for spontaneous closure of the fistula, also provides time to optimize nutritional status and to heal the wound site from the patient's initial surgery (if enteric fistula was postoperative). Thus, definitive surgery for fistula repair is generally delayed for several months until physiologic deficits have been restored and intra-abdominal conditions are less hostile. However, if diffuse peritonitis with ongoing sepsis is observed, immediate operative exploration may be necessary to stabilize the patient.

• The preferred procedure involves excision of the fistula tract and segmental resection of involved bowel and anastomosis of the remaining bowel. If an unexpected abscess is encountered or the quality of the bowel wall is suboptimal, some surgeons may consider a primary anastomosis unsafe, instead choosing to perform a staged procedure, with exteriorization of the ends of bowel during the first procedure. A staged repair may also be more appropriate in cases in which advanced malignancy or severe radiation changes are expected. If the procedure is performed for a malignancy, preferably, the involved segment of bowel is removed to negative margins.
• Perianal abscesses should be drained and anal strictures dilated. Patients with low anal fistulas can be treated with fistulotomy. Some surgeons are in favor of a noncutting seton, especially in the presence of an active inflammation of the rectosigmoid colon. Noncutting setons may be placed in fistula tracts in patients with rectal inflammation, and endorectal advancement flap procedures for high perianal fistulas and rectovaginal fistulas may be performed in patients without rectal inflammation. If a rectovaginal fistula persists after the patient has received medical therapy and anorectal stricture or active rectal disease is not evident, then surgical repair may be performed with either (1) transanal or transvaginal advancement flaps or (2) laparotomy with primary closure or sleeve advancement flap.
• Patients with colovesical fistulas can almost always be treated with resection of the involved segment of colon and primary reanastomosis, with or without closure of the bladder defect. Healing of the bladder is usually managed easily with temporary urethral catheter drainage.
• The overall incidence of aortoenteric fistulas has changed with the advent of endovascular repairs of abdominal aortic aneurysms, but the criterion standard remains open excisional repair and extra-anatomic bypass for revascularization in the case of secondary aortoenteric fistulas (which occur after open repair of abdominal aortic aneurysms). Endovascular repair of primary aortoenteric fistulas in high-risk patients (ie, those at risk for chronic infection of the endograft) has been reported.

Consultations

• Nutritionist
• Enterostomal or wound care nurse
• Surgeon (gynecologic or urologic surgeon if fistula involves either vagina or bladder)

Diet

In the initial period, patients are maintained on TPN and are given nothing by mouth (NPO).

In patients with low-output distal fistulas, elemental diets may be initiated as long as they do not profoundly increase the fistula output.

Activity

Aggressive physical therapy provides long-term benefits to patients. Typically, patients do not require prolonged bedrest (which only adds to comorbidities) unless necessary for some other reason.

MEDICATION

Section 7 of 11  

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The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Drug Category: Somatostatin analogs

These agents inhibit the release of serotonin and secretion of many hormones involved in GI function.

Drug Category: Immunosuppressive agents

These agents inhibit the activity of key factors in the immune system.

FOLLOW-UP

Section 8 of 11  

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Further Inpatient Care

• Follow-up care is based on disease etiology. In patients with chronic inflammatory conditions, such as Crohn disease, ongoing follow-up care is required.
• If patients are simply waiting for definitive surgical therapy and being maintained on TPN, they do not need to be in the hospital. Home infusion or placement in a rehabilitation facility is perfectly acceptable. Close contact is needed, as line sepsis and other infectious complications may occur, and early and aggressive treatment is essential to ensure a good outcome.
• In patients who have undergone surgical repair, normal postoperative follow-up care is required. Once the incisions are healed and the drains are removed, the patient may be discharged from care.

Further Outpatient Care

• Patients with Crohn disease may require ongoing, outpatient maintenance therapy with medication.

Complications

• Intestinal fistulas carry high morbidity and mortality rates. If medical and nonoperative treatments are not effective, the risks of surgery need to be discussed with patients and their families.
• Complications are routine, as dense fibrotic adhesions are likely to be encountered during surgery. Infection, bleeding, and injury to adjacent organs, as well as recurrence of the fistula, are all possibilities. To reduce these complications, patients must receive optimal nutrition and must be treated by an experienced surgeon.

Prognosis

• The prognosis is based on the etiology of the fistula, as well as the comorbidities of the patient.

MISCELLANEOUS

Section 9 of 11  

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Medical/Legal Pitfalls

• Since most intestinal fistulas are iatrogenic in origin, tremendous medical legal issues surround these patients. The risks, benefits, and alternatives (RBA) of the initial surgical procedure must be discussed with the patient and a family member. This must be documented in the chart, even if it is simply noted, "R/B/A discussed with patient and family and they understand." This documentation can help if informed consent is one of the issues in a malpractice suit.
• Early recognition of the diagnosis and, if needed, transfer of the patient to an experienced surgeon are essential. High morbidity and mortality rates necessitate a surgeon experienced in working with these families. If the patient is transferred, follow-up phone calls are essential to maintain the lines of communication with the family and the referring physician.

MULTIMEDIA

Section 10 of 11  

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Media file 1:  Enterocutaneous fistula after bowel injury from an incisional hernia repair, 6 weeks post injury.
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Media type:  Photo

Media file 2:  Status post pancreatic debridement for necrotizing pancreatitis. The patient had a colonic injury with attempted closure using a skin graft. The patient later underwent definitive repair.
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Media type:  Photo

Media file 3:  Psoas abscess from Crohn disease that later fistulized to the skin.
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Media type:  CT

REFERENCES

Section 11 of 11

• Authors and Editors
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• Goverman J, Yelon JA, Platz JJ, et al. The "Fistula VAC," a technique for management of enterocutaneous fistulae arising within the open abdomen: report of 5 cases. J Trauma. Feb 2006;60(2):428-31; discussion 431. [Medline].
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• Levy C, Tremaine WJ. Management of internal fistulas in Crohn's disease. Inflamm Bowel Dis. Mar 2002;8(2):106-11. [Medline].
• Lindberg E, Jarnerot G, Huitfeldt B. Smoking in Crohn's disease: effect on localisation and clinical course. Gut. Jun 1992;33(6):779-82. [Medline].
• Meissner K. Late radiogenic small bowel damage: guidelines for the general surgeon. Dig Surg. 1999;16(3):169-74. [Medline].
• Pederzoli P, Bassi C, Falconi M, et al. Conservative treatment of external pancreatic fistulas with parenteral nutrition alone or in combination with continuous intravenous infusion of somatostatin, glucagon or calcitonin. Surg Gynecol Obstet. Nov 1986;163(5):428-32. [Medline].
• Pontari MA, McMillen MA, Garvey RH, et al. Diagnosis and treatment of enterovesical fistulae. Am Surg. Apr 1992;58(4):258-63. [Medline].
• Practice parameters for treatment of fistula-in-ano--supporting documentation. The Standards Practice Task Force. The American Society of Colon and Rectal Surgeons. Dis Colon Rectum. Dec 1996;39(12):1363-72. [Medline].
• Present DH, Rutgeerts P, Targan S, et al. Infliximab for the treatment of fistulas in patients with Crohn's disease. N Engl J Med. May 6 1999;340(18):1398-405. [Medline].
• Rolandelli R, Roslyn JJ. Surgical management and treatment of sepsis associated with gastrointestinal fistulas. Surg Clin North Am. Oct 1996;76(5):1111-22. [Medline].
• Rots WI, Mokoena T. Successful endoscopic closure of a benign gastrocolonic fistula using human fibrin sealant through gastroscopic approach: a case report and review of the literature. Eur J Gastroenterol Hepatol. Dec 2003;15(12):1351-6. [Medline].
• Wedell J, Banzhaf G, Chaoui R, et al. Surgical management of complicated colonic diverticulitis. Br J Surg. Mar 1997;84(3):380-3. [Medline].

Article Last Updated: Aug 8, 2008