G ASTROENTEROLOGY Copyright <0 1971 by The Williams & Wilkins C o. Vol. 60, No. I Printed in U. S. A. SPONTANEOUS UMBILICAL FISTULA IN GRANULOMATOUS (CROHN'S) DISEASE OF THE BOWEL PAUL C. HILEY, M.D., NATHANIEL COHEN, M.D., AND DANIEL H. PRESENT, M.D. Department of Medicine, Division of Gastroenterology, Mount Sinai School of Medicine, N ew York, New York Three patients with regional enteritis involving the terminal ileum and 1 with granulomatous colitis developed spontaneous umbilical fistulas in the absence of prior surgery. Two patients did well with medical management and 2 required surgery because of the severity of the basic inflammatory process. The mechanism of fistula formation to the umbilicus in the absence of previous surgery is not understood, but may occur secondary to perforation of an abscess into persistent urachal remnants. One of the characteristic features of Crohn's disease is the development of intestinal fistulae. These may occur in the rectal or perianal area, between loops of small and large bowel, to adjacent organs such as the urinary bladder and stomach and to the (anterior) abdominal wall. Abdominal wall fistulae are said to be present in 8 to 20% of cases and they usually occur through the scars of previous laparotomies. l Indeed, the development of a spontaneous fistulae to the abdominal wall without prior surgery appears not to have been documented. The purpose of this paper is to report 4 patients who developed spontaneous abdominal wall fistulae through the umbilicus without any previous surgery. Case Reports Patient 1. J. T., a 30-year-old white male, was admitted to the Mount Sinai Hospital because of umbilical drainage of 4-week duration. He reported a 12-year history of nonbloody diarrhea and a small bowel series demonstrating regional enteritis. During the year before admission he noted easy fatiguability Received April 2, 1970. Accepted August 10, 1970. Address requests for reprints to: Dr. Daniel H. Present, Mount Sinai Hospital, 100th Street and Fifth Avenue, New York, New York 10029. This work was supported by a grant from the Foundation for Ileitis and Colitis, lnc. 103 and weight loss of 25 lb. Three weeks before admission an expansile mass developed around the umbilicus which subsequently drained brownish material. On physical examination there was induration below and purulent drainage from the umbilicus. Sigmoidoscopy was normal except for a localized area of friability with polypoid excresences noted at 5 V2 inches. A biopsy from a rectal valve was normal. Laboratory studies demonstrated normal hemoglobin, hematocrit, white blood cell count, and blood chemistries. Culture of the abdominal drainage grew out Aerobacter aerogenes. A small bowel series showed ileitis with fistulization from the terminal ileum extending to the skin and involving the rectosigmoid. The patient underwent an ileotransverse colostomy without resection. At surgery a diseased area of the terminal ileum was found to be adherent to the underside of the parietal peritoneum at the region of the umbilicus. The umbilical fistula stopped draining 1 week after surgery and the patient gained 25 Ib in the subsequent year. No further drainage from the umbilicus has been noted and a barium enema performed 1 year after the surgery has shown no recurrence of disease and no fistulous communications. Patient 2. J. L., a 23-year-old white female, was admitted to the Mount Sinai Hospital with a 6-week history of a draining umbilical fistula. Five years before admission she developed abdominal pain, diarrhea, a 30-lb weight loss, and erythema nodosum. One year later the patient developed a rectal abscess and fistula which drained for 2 years despite local
104 CASE REPORTS Vol. 60, No.1 FIG. 1. Barium enema demonstrating inflammatory bowel disease with narrowing of the midtransverse colon and fistulous tract extending outside the bowel lumen. surgical intervention. Six months prior to admission the patient had experienced persistent lower abdominal pain, diarrhea, and a 25-lb weight loss despite a daily maintenance dose of 40 mg of prednisone. Physical examination demonstrated a periumbilical mass with purulent drainage from the umbilicus. Sigmoidoscopy was normal. A barium enema demonstrated narrowing of the transverse colon and extravasation of barium from the inferior surface of the colon adjacent to the umbilicus (fig. 1). The prednisone was discontinued and the patient was treated with Ampicillin and Azulfidine. The abscess decreased in size and the drainage disappeared. However, 3 months later the abdominal pain, weight loss, and umbilical drainage recurred. Laboratory data at the time of admission demonstrated a hemoglobin of 10.3 g with a normal white blood cell count and differential. Sedimentation rate was 29 mm per hr (Westergren). Culture of the abdominal wound grew out Proteus mirabilis. All other blood chemistries were normal. A repeat barium enema showed granulomatous colitis with a fistulous communication from the transverse colon to the umbilicus with some extravasation of the barium into the pericolonic tissues. The patient was explored and at surgery the midtransverse colon was drawn downward and was firmly adherent to the umbilical area by a dense inflammatory process. There was a large inflammatory mass immediately behind the parietal peritoneum of the abdominal wall and this induration extended downward in the lower midline halfway to the pubis. An ileosigmoidostomy without resection was performed. One year after surgery the patient has had no further recur-
January 1971 CASE REPORTS 105 rence of umbilical drainage, has gained 37 Ib, and has no significant bowel symptoms. Patient 3. B. M., a 16-year-old female, was admitted to Mount Sinai Hospital because of increasing abdominal pain of 3-month duration. A barium enema performed 6 years before demonstrated ileitis in the terminal 8 inches and a normal colon. During the year prior to admission an enlarging right lower quadrant mass was noted. She was maintained on prednisone (5 to 15 mg per day) but had recurrent episodes of crampy abdominal pain and fever. Two weeks before admission she noted the passage of gas and yellowish fluid with a fecal odor from the umbilicus. The drainage stopped spontaneously after 1 week. On admission the only positive physical finding was a right lower quadrant mass. Sigmoidoscopy was normal. Laboratory studies demonstrated a normal hemoglobin and hematocrit with a slightly elevated white blood cell count (11,950 per mm 3 ). The differential count was normal. Sedimentation rate was 37 mm per hr (Westergren). Blood chemistries were normal except for a slight decrease in serum albumin and slight elevation of serum globulin. Upper gastrointestinal series and barium enema demonstrated a previously noted ileitis. However, the right side of the colon was felt to be minimally involved. The patient was treated with prednisone and sulfathaladine and was discharged. During the following year the umbilical fistula spontaneously opened and closed while on maintenance doses of prednisone. At present, 2 years later, she is asymptomatic and has had no further umbilical drainage of gas or fluid, and all medications have been discontinued. The right lower quadrant mass is still palpable. Patient 4. L. D., a 13-year-old white female, was seen as an outpatient because of crampy abdominal pain, two to three loose watery stools per day, and a 25-lb weight loss of 8- month duration. Physical examination revealed fullness and tenderness in the right lower quadrant. Sigmoidoscopy was normal. An upper gastrointestinal series and small bowel follow-through demonstrated ileitis involving the terminal ileum with one fistulous tract extending from the bowel lumen. Barium enema demonstrated irritability in the cecum with an extrinsic mass. The patient was treated with Azulfidine and anticholinergics. However, she continued to lose weight and the abdominal cramps increased. After 2 months she was started on prednisone (20 mg per day) with a decrease in abdominal pain and a lo-lb weight gain. Two months after the initiation of prednisone she developed redness and tenderness around the umbilicus and a right lower quadrant mass. One week later yellow purulent drainage was noted from the umbilicus and the abdominal mass decreased in size. The patient was treated with Ampicillin and the prednisone was decreased to 15 mg per day. The drainage has persisted but is markedly decreased, and in the subsequent 3-month period she has gained 25 Ib and has experienced less abdominal pain. We interpret the intermittent drainage from the umbilicus as evidence of a communication with the gut. Discussion Abdominal wall fistulas are often noted in Crohn's disease through the sites of previous laparotomies (especially appendectomies), yet reviews of large series of patients with these diseases have failed to note the occurrence of a spontaneous fistula in the absence of prior surgery.2. 3 Crohn and Yarnis in their monograph noted that fistulae, in addition to those occurring through surgical scars, could occur in the lumbar area secondary to tracking and subsequent abscess formation. Perirectal and ischiorectal abscess or fistulization have been noted by many authors. Lockhart-Mummery and Morsor,4 in their paper on Crohn's disease of the large intestine, noted 2 patients who, after forming pericolic abscesses, developed fecal fistulas to the skin. However, they did not comment on prior surgery. Our 4 patients are unique in that they developed spontaneous umbilical fistulas without prior operative intervention. The patients in our series ranged in age from 13 to 30 years and had been ill from 9 months to 12 years prior to the appearance of the fistula. The symptoms were predominantly those of abdominal pain and the development of a superficial abdominal mass in 3 of the 4 patients which subsequently drained through the umbilicus. The drainage was purulent in all patients with a feculant odor, with cultures demonstrating colonic bacteria. The anatomic site of disease and the presumed site of fistulization were from the terminal
106 CASE REPORTS Vol. 60, No. 1 ileum in 3 patients and the midtransverse colon in 1. Internal fistulae were seen in 3 of the 4 patients and in 2 a fistulous tract was noted to be directed towards the anterior abdominal wall. In the last 2 cases, although there was no X-ray evidence of direct communication between the gut and the anterior abdominal wall, the recurrent passage of gas and fecal type drainage (with culture of coliform bacteria) must be interpreted as communication of the intestine with the umbilicus. Three of the patients were being treated with corticosteroids (dosages varying from 5 to 40 mg per day) at the time that the umbilical drainage developed. Two patients underwent surgery and had a bypass of the diseased segment without resection and have been completely asymptomatic without further umbilical drainage (follow-up 2 and 21/2 years respectively). One patient stopped draining spontaneously and 2 years later is asymptomatic, although a vague right lower quadrant mass is still palpable. Patient 4 was treated with antibiotics and the drainage has gradually decreased, although the follow-up period is only 3 months. She is markedly improved clinically at this time, having gained 25 lb. In anterior wall fistulae, it is assumed that the inflammatory process extends from the diseased bowel along fascial planes created by the surgery and finally appears in the operative scar, where an abscess forms and eventually ruptures. The mechanism for the development of umbilical fistulas in our patients without prior surgery is not clear. The umbilical cord connects the fetus with that part of the chorion that constitutes the fetal side of the placenta. After birth the cord is obliterated. However, vestigial structures still remain, even into adult life. The umbilical connections include the middle umbilical ligament (a fibrous band stretching to the pubic bone which is the embryonal remains of the urachus), the two lateral umbilical ligaments (the obliterated umbilical arteries), and the ligamentum teres (the obliterated umbilical vein which attaches to the liver).5 In rare instances, the urachus may remain patent establishing a urachal fistula through which urine may escape. Less frequently, there may be persistence of isolated epithelial remnants or blind sinuses leading from the bladder. In none of our patients was there any evidence of a patent urachus with a urachal fistula. It is possible that these patients had some unrecognized anatomic variation in the structure of the umbilicus predisposing them to fistula formation but this seems unlikely. An alternate and more reasonable hypothesis would be the formation of an abscess in the vicinity of the umbilicus secondary to extension of the inflammatory process from either the terminal ileum or the transverse colon. As the abscess increased in size it might have entered a persistent urachal remnant and thereby found a path of lowered resistance through which to drain. The appearance of a spontaneous umbilical fistula in the presence of inflammatory bowel disease is not a definite indication for surgical intervention. Although 2 of our patients underwent surgery, they both had evidence of a severe inflammatory process (as manifested by marked weight loss and abdominal pain). One patient with mild pre-existing dinical disease has continued to do well with complete cessation of drainage. The other patient has shown persistent improvement with antibiotic therapy, although there has not been a long enough followup period in which to determine the outcome. Our experience with anterior abdominal wall fistulas through prior surgical scars has demonstrated that many may be treated successfully with conservative medical management. Therefore, in this analogous situation, it would seem rational to institute antibiotic therapy for umbilical fistulization if the patient's underlying disease process has not been severe. Surgery should be performed only if there is no improvement in the clinical course. The role of steroids in the formation or management of this complication cannot be determined in view of the few cases studied.
January 1971 CASE REPORTS 107 REFERENCES 1. Bockus HL: Gastroenterology. Philadelphia, WB Saunders Co, 1964, p 254 2. Van Patter WN, Bargen JA, Dockerty MB, et al: Regional enteritis. Gastroenterology 26:347-450, 1954 3. Crohn BB, Yarnis H: Regional Enteritis. New York, Grune and Stratton, 1958, p 52 4. Lockhart-Mummery HE, Morson BC: Crohn's disease of the large intestine. Gut 5:493-509, 1964 5. Arey LB: Developmental Anatomy. Philadelphia, WB Saunders Co, 1965, p 111-114