Selected Articles from the Journal Abdominal Surgery

Subacute Small Bowel Obstruction due to Ileal Diaphragm Disease – Case Report and Literature Review

Shankar Raman, M.D., M.S., MRCS
Surgical Resident

John M. Cosgrove, M.D., FACS
Attending Surgeon

Pradeep S. Basnyat, FRCS*
Attending Surgeon

From the Departments of Surgery at Bronx-Lebanon
Hospital Center, Bronx, New York and
William Harvey Hospital, Ashford Kent, U.K.*

Corresponding author:
Mrs. Saundra King, C-TAGME
Administrative Program Manager
Department of Surgery
Bronx-Lebanon Hospital Center
1650 Selwyn Avenue, Suite 4A
Bronx, New York 10457
Voice: 718 960-1216
Fax: 718 960-1370
E-mail: sstaffor@bronxleb.org

ABSTRACT
Diaphragm disease (DD) is a rare complication of bowel disorders associated with the use of non steroidal antiinflammatory drugs (NSAID). This disease is difficult to diagnose because of its vague, nonspecific symptoms. Most often, DD presents as a form of a sub acute bowel obstruction and the diagnosis is confirmed only intraoperatively. Characteristics of diaphragm disease are the formation of a stricture resembling a diaphragm, which occurs as a consequent of sub mucosal fibrosis in the gastrointestinal tract. Most studies have found that DD develops while patients are taking the NSAID, but occasionally progressive damage to the bowel mucosa occurs even after cessation of the use of NSAID. In these cases, patients become symptomatic later, as in the case presented.

INTRODUCTION
Lang1 and Bjarnason^1-3 pioneered the description of diaphragm disease (DD) in 1988, noting that patients using NSAIDs sometimes develop small intestine strictures resembling a perforated diaphragm. The disorder presents with vague gastrointestinal symptoms such as abdominal pain, distention, and vomiting and weight loss.

Physical examination and imaging studies do not add usually significant data to the diagnosis, but patients deteriorating condition requires exploratory laparotomy. Even intraoperatively, a thorough examination of the intestines is necessary to find the lesion. Limited resection followed by discontinuation of NSAIDs is curative. We present a case of DD in a 78-year old male who became symptomatic after the discontinuation of NSAID and discuss the management of the disease.

CASE REPORT
A 78-year-old male was admitted to the hospital complaining of post prandial colicky abdominal pains of three months duration. During this period, he lost fifteen pounds, in spite of having a good appetite. The pains usually started approximately two hours after food intake. The patient stated that these episodes were associated with abdominal distention, followed by copious vomiting which relieved the pain. The patient had osteoarthritis and had undergone bilateral hip replacement. He had been taking diclofenac and other NSAIDs during the last two years to deal with the osteoarthritis pain, but had stopped using these medications six months earlier after hip replacement. He also had deep vein thrombosis for which he was taking warfarin.

Physical examination revealed visible intestinal peristalsis and borborygmi in an emaciated virgin abdomen. No masses were palpable. A CT scan demonstrated dilated small bowel loops without a definitive transition zone. A colonoscopy performed up to the cecum was unremarkable. Upper gastrointestinal endoscopy showed erythematous gastric mucosa and was negative for Helicobacter pylori. Among the radiographic studies, a small bowel series revealed dilated bowels, but no definite point of obstruction. The patients’ hemoglobin level was 10 g/dl; his hematocrit level was 31% and his albumin level was 3.1 g/dl. The rest of the laboratory values, including tumor markers were unremarkable.

Figure 1

The patient’s deteriorating condition prompted a laparotomy at which the jejunum and a proximal ileum were found to be dilated. A narrowing with stricture formation was noted at about 4 cm of the mid ileum, but there was no palpable mass. A 10 cm segment of ileum was resected and a primary anastomosis was done. The narrowed segment of resected ileum had a diaphragm-like stricture (FIG 1). Histopathologic examination showed a circumferential mucosal ulceration with sub mucosal fibrosis around the stricture. No granulomas were seen. The gross and microscopic appearance of the resected segment was consistent with the classical picture of NSAIDs inducted stricture. At two-year follow-up, the patient appeared to be cured.

DISCUSSION
NSAID’s are among the most commonly prescribed classes of drugs. Although it is known that gastrointestinal ulcerations and hemorrhages occur with their use, DD has not been widely reported. The reported prevalence of DD varies widely between 8.4% and 66%^{2, 3, 4}. The exact prevalence of DD is difficult to determine because of the vague symptoms associated with it. NSAIDs can cause anemia due to gastrointestinal blood loss, perforation, protein-losing enteropathy and the formation of diaphragm-like stricture^1-6. The spectrum of small bowel involvement ranges from multiple diaphragms, most often in the ileum, to broad-based strictures. It has been postulated that the increased use of enteric coated NSAIDs is responsible for the enteropathy because of the prolonged exposure of the small bowel to the drug⁷. DD also can occur in the large intestine⁸. Occasionally, as in our case, patients develop strictures in spite of the cessation of NSAID use because of the persisting effect of the drug⁹.

The diagnosis of DD is complicated, not only by the ambiguity of the symptoms, but also by the fact that most complete investigations are inconclusive. At times, the diaphragms can appear as exaggerated plica ciculares and they are not seen or interpreted properly on imaging studies¹⁰. Recently, Yousufi et al¹¹ pointed out that capsule endoscopy can diagnose this disorder. However, this procedure carries the risk of the capsule becoming incarcerated into the diaphragm thus, necessitating immediate surgery. The definitive diagnosis is usually made intra operatively, after an exhaustive work-up has failed to diagnose the condition. Even at laparotomy, the bland serosal appearance of bowel makes it difficult for surgeons to identify the diseased segment of the intestine. For the same reason, laparoscopy has a limited role¹².Meticulous palpation of the entire length of the bowels and intraoperative enteroscopy are helpful means of locating the affected segment of the intestine. However, the risks associated with intraoperative enteroscopy include enterotomies, small bowel inflation and mesenteric tears¹². Occasionally, strictures can be visible externally, as in our case. Once found, the diaphragms can be managed by limited resection of strictureplasty. As in Crohn’s disease, strictureplasty is useful in setting of multiple strictures or when the small bowel length needs to be optimized. The exact mechanism of DD has not been elucidated thus far.

Microscopically, diaphragm disease is characterized by the presence of sub mucosal fibrosis and the granulomas differentiate this subset of intestinal strictures from Crohn’s disease and tuberculosis. In DD, a disorganized arrangement of neural, vascular and smooth muscle elements resembling hamartoma can be present¹. Although the surgical treatment of DD is potentially curative, Lang, et al reported symptoms recurrence rate of 50%¹.

Awareness and a high index of suspicion are necessary when ambiguous obstructive symptoms occur in a patient taking, or previously on NSAIDs.

REFERENCES
1. Lang J, Price AB, Levi AJ, Burke M, Gumpel JM, Bjarnason I. Diaphragm disease: Pathology of Disease of the Small Intestine Induced by Non-Steroidal Anti- Inflammatory Drugs. J Clin Pathol. 1988 May;41(5):516-526.

2. Allison MC, Howatson AG, Torrence CJ, Lee FD, Russell RI. Gastrointestinal damage associated with the use of non-steroidal anti-inflammatory drugs. N Engl J Med. 1992;327:749-754.

3. Bjarnason I. Non-steroidal anti-inflammatory drug-induced small intestinal inflammation in man. In: Pounder RE, ed. Recent Advances in Gastroenterology. Vol 7. Edinburgh, S cotland: Churchill Livingston; 1988:23-46.

4. Shumaker DA, Bladen K, Katon RM. NSAID-induced small bowel diaphragms and strictures diagnosed with intraoperative enteroscopy. Can J Gastroenterol. 2001;15:619-623.

5. Bjarnason I, Price B, Zanelli G, et al. Clinicopathological features of non-steroidal anti-inflammatory drug-induced small intestinal strictures. Gastroenterology. 1988;94:1070-1074.

6. Kwo PY, Tremaine WJ. Non-steroidal anti-inflammatory drug-induced enteropathy: Case discussion and review of literature. Mayo Clin Proc. 1995;70:55-61.

7. Cipolla G, Crema F, Sacco S, Mor E, dePonti F, Frigo G. Non-steroidal anti-inflammatoroy drugs and inflammatory bowel disease: Current perspectives. Pharmacol Res. 2002:46:1-6.

8. Halter F, Gut A, Ruchti C. Intestinal pathology from NSAIDs. Inflammopharmacologyy. 1996:4:43-60.

9. Bjarnason I. Zanelli G, Smith T, et al. Non-steroidal anti-inflammatory drug-induced intestinal inflammation in humans. Gastroenterology. 1987;93:480-489.

10. Scholz FJ, Heiss FW, Roberts PL, Thomas C. Diaphragm like strictures of the small bowel associated with the use of non-steroidal anti-inflammatory drugs. Am J Roentgenol. 1994;162:49-50.

11. Yousufi MM, DePetris G, Leighton JA, et al. Diaphragm disease after use of non-steroidal anti-inflammatory agents: First report of diagnosis with capsule endoscopy. J Clin Gastroenterol. 2004;38:686-691.

12. Kelly ME, McMahon LE, Jaroszewski DE, Yousfi MM, DePetris G, Swain JM. Small-bowel diaphragm disease: Seven surgical cases. Arch Surg. 2005 Dec; 140(12):1162-1166.