A 73-year-old man presents to the hospital complaining of purulent discharge from his abdomen. The patient states that he developed pain in that area starting about a week prior to his visit. A palpable mass formed that is painful to palpation and that began to drain pus 1 day ago. He denies having any fever, weight loss, nausea, or vomiting, but he does admit to increased fatigue. He also denies having dysuria or any change in urinary frequency, and his stool pattern is normal. His medical history is significant only for Crohn disease, which he has had for over 40 years; it has been well controlled with daily prednisone for the past 13 years. His surgical history is significant for a partial right hemicolectomy approximately 20 years ago for complications stemming from his Crohn disease.
On physical examination, the patient is afebrile with a pulse of 70 bpm and a blood pressure of 130/80 mm Hg. He appears well and is noted to have a regular heart rhythm without murmurs. His lungs are clear. The abdominal exam reveals a soft and nondistended abdomen with normal bowel sounds. There is no evidence of rebound or guarding, but local examination reveals an area of erythema with what appears to be a tract extending into the subcutanous tissue along a well-healed midline incision scar (see Image 1). His genital exam is unremarkable, his prostate is nontender, and his digital rectal exam reveals no tenderness or masses; a stool guaiac test is performed and is negative.
The patient is transferred to the emergency department for further workup, where serum laboratory testing is performed and is remarkable for leukocytosis, with a normal hematocrit and platelet count. A metabolic panel is taken and is unremarkable, with normal renal function; in addition, an elevated C-reactive protein and a low albumin are noted. A sample of the purulent discharge is sent for a Gram stain and culture. A barium fluoroscopy of the small bowel is performed (see Image 2), and upon request, a magnetic resonance imaging (MRI) scan of the abdomen is also obtained (see Image 3).
What is the diagnosis?
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HINT
This is a rare complication of a common disease.
Authors:
Adarsh Babu, MD, MBBS, Foundation Year 1 House Officer, Diana Princess of Wales Hospital, NLG Hospitals NHS Trust.
Stephen Moss, MD, FRCP, Consultant Physician and Gastroenterologist, Diana Princess of Wales Hospital, Grimsby, England
eMedicine Editors:
Michael J. Rest, MD, Resident Physician, Department of Emergency Medicine, Yale-New Haven Hospital, New Haven, Connecticut.
Rick G. Kulkarni, MD, Assistant Professor, Yale School of Medicine, Section of Emergency Medicine, Department of Surgery, Attending Physician, Medical Director, Department of Emergency Services, Yale-New Haven Hospital, Conn
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ANSWER
Crohn enterocutaneous fistula: The barium fluoroscopy images (see Images 2 and 3) clearly demonstrate a fistulous tract connecting the bowel lumen to the anterior abdominal wall. A follow-up MRI scan (see Image 4) was performed and showed loops of small intestine adherent to the anterior abdominal wall, with surrounding edema and inflammation of the bowel wall. The fistula was thought to be a complication of the patient’s previous abdominal surgery in conjunction with his underlying Crohn disease.
Crohn disease was first described in 1932 by Crohn, Ginzberg, and Oppenheimer, who noted its localization to segments of the ileum. Crohn disease is a chronic granulomatous inflammatory process that can involve any part of the gastrointestinal tract. Though the exact pathogenesis of the disease is unknown, the condition is believed to be caused by an imbalance between proinflammatory and anti-inflammatory mediators, leading to autoimmune destruction of the mucosal cells. The characteristic presentation of Crohn disease is variable, but it is frequently associated with abdominal pain, diarrhea, weight loss, and anorexia. Unpredictable flare-ups and remissions characterize the long-term course of the disease.
Because the inflammatory process is transmural and depending on the area of inflammation, the condition may be complicated by intestinal fistulization, obstruction, or both; these complications are more common in Crohn disease than in ulcerative colitis. Up to 30 % of patients will develop fistulas or abscesses, most commonly in the perianal region.4 When fistulas develop, they usually connect the ileum, sigmoid colon, or the cecum, but they also may be enterovesical, enterovaginal, or enterocutaneous. The presentation and associated complications from fistulas are variable. Cologastric fistulas manifest as feculent vomiting, whereas enterovesical fistulas manifest as recurrent urinary tract infections and pneumaturia; enterovaginal fistulas manifest as feculent vaginal discharge; and enterocutaneous fistulas manifest as feculent soiling of the skin. Coloenteric and cologastric fistulas may result in bacterial overgrowth, diarrhea, and weight loss. Enterovesical fistulas and enterovaginal fistulas are often complicated by infection, including cystitis, abscess formation, and peritonitis. Enterocutaneous fistulas frequently develop at a former surgical site.
Multiple imaging techniques can be used to establish the diagnosis of fistulous disease. Barium contrast studies can identify features such as strictures, fistulization, and submucosal edema. The fistulas can be detected by oral barium fluoroscopy or through barium injection into the opening of the suspected fistula. Computed tomography (CT) scanning using oral and rectal contrast agents can also be helpful in diagnosing and delineating fistulas, and it has the added benefit of detecting local abscess formation, hepatobiliary complications, and renal complications. MRI is also useful in detecting fistulas, and it can be superior to CT in demonstrating pelvic lesions.
Treatment of Crohn fistulas is aimed at reducing inflammation and controlling symptoms. Fistulas between bowel loops may be benign but may also cause diarrhea with malabsorption and malnutrition. Enterovesicular, enterocutaneous, cologastric, and coloduodenal fistulas are more serious. Surgical intervention is rarely required, unless fistulas are complicated by progressive obstruction or abscess formation or a large segment of bowel is bypassed, leading to severe diarrhea and malabsorption.3 Sulfasalazine (3-4 g daily) or mesalamine (4 g daily) can be used to treat bowel disease. In cases of moderate to severe disease, steroids, such as prednisone (60 mg daily), can be used. Oral metronidazole (1 g daily for 1-2 months) can also effectively treat fistulous disease. Immunosuppressive drugs, such as 6-mercaptopurine or azathioprine, are beneficial in reducing drainage and closing fistulas in 30-40% of patients.3 Additionally, prolonged bowel rest with total parenteral nutrition can be helpful in promoting healing of fistulas; they may recur when oral feeding resumes.
The patient in this case was admitted to the hospital and received a combination treatment regimen of oral prednisone, azathioprine, and metronidazole. Over the following 2 weeks, the discharge from the enterocutaneous fistula transitioned from purulent material to a more serous and feculent discharge. Conservative medical management was determined to be adequate for long-term therapy, and the patient was discharged to home.
This case was originally published by Adarsh Babu, as Eurorad case 5703 on Eurorad.org.
References:
1. Tsui BC, Cummings GE. Anorectal fistula: An unusual presentation in a Crohn's disease patient. J Emerg Med. 1997 Jan-Feb;15(1):39-43. [MEDLINE: 9017486]
2. Mirete Ferrer C, Blázquez Encinar JC, Ornia Rodríguez E, de Teresa Parreño L. [Spontaneous enterocutaneous fistula as sole manifestation of recidive in Crohn's disease]. An Med Interna. 2004 Feb;21(2):99-100. [MEDLINE: 14974900]
3. Chen, YH. Crohn Disease. eMedicine from WebMD. Updated Jan 26 2007. Available at: http://www.emedicine.com/radio/topic197.htm. Date accessed: April 2007.
4. Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. 6th ed. New York, NY: McGraw-Hill; 2004: 530-33.
5. Rentz TW Jr, Warden CS, Garcia FJ, Kovalcik PJ. Crohn's disease with spontaneous ileoumbilical and ileovesical fistulae. Dig Dis Sci. 1979 Apr;24(4):316-8. [MEDLINE: 110569]
