Surg Today (2003) 33:383–386

Sigmoid Colon Perforation as an Unusual Complication ofBehçet’s Syndrome: Report of a Case

Mustafa Turan1, Metin Sen

1, Ayhan Koyuncu1, Cengiz Aydin

1, and Sema Arici2

Departments of 1General Surgery and 2Pathology, Cumhuriyet University Faculty of Medicine, 58140 Sivas, Turkey

skin lesions. Minor criteria include arthritis, epididymi-tis, and gastrointestinal, vascular, or central nervous sys-tem lesions. Behçet’s disease is defined as “complete” ifthe patient has four major symptoms, and as “incom-plete” if the patient has three major or two major andtwo minor symptoms.

This disease mainly affects young adults,9 but itsetiology and pathogenesis remain obscure. Although itis sometimes accompanied by gastrointestinal symp-toms such as nausea, vomiting, and abdominal pain,intestinal ulcers are rarely found.10,11 If intestinal ulcersare confirmed by radiography, endoscopy, or surgery,the disease is then referred to as “intestinal” Behçet’sdisease.10,11 Surgery is often performed for intestinalulcers because they tend to perforate, and there is nomedical treatment that appreciably influences thenatural course of the disorder.11,12

We report a case of sigmoid colon perforation due tointestinal involvement of Behçet’s disease.

Case Report

A 47-year-old man was admitted to our EmergencyDepartment with a 3-day history of severe and diffuseabdominal pain with nausea and vomiting, which hadincreased in severity with each day. He had been suffer-ing from Behçet’s disease with involvement of the eyes,mouth, and genitalia for 25 years. At the age of 22 years,hypopyon iritis was noted, at 24 years, acne vulgarisappeared, and by the age of 26 years, he had multipleoral and genital ulcerations. He was treated with colchi-cine, 3mg/day in the Department of Internal Medicine,and was discharged after his symptoms improved.However, the oral and genital ulceration recurred andhe was again being managed by the Department ofInternal Medicine.

On admission to the Emergency Department, he hadoral and genital ulcerations. Small pustules appeared

AbstractA 47-year-old man with long-standing Behçet’s syn-drome presented with an acute abdomen, and wasfound to have perforation of the sigmoid colon. Laparo-tomy revealed gangrenous changes in the sigmoid colonand perforation in the center of the affected segment.This is a very rare complication of Behçet’s disease, andwe report this case to stress the importance of perform-ing careful abdominal examination while evaluatingpatients with Behçet’s disease.

Key words Behçet’s disease · Sigmoid colon perfora-tion · Inflammatory bowel disease

Introduction

Behçet’s syndrome is a multisystem disorder manifest-ing as recurrent oral and genital ulcerations, as well asuveitis often leading to blindness. Behçet originallydescribed a condition with three signs involving themouth, genitalia, and eyes, in the form of hypopyoniritis.1,2 The disease is now known to affect many organs,with great variation in the appearance of signs. Therecan be involvement of the central nervous system andvascular system, each with a frequency of about 10%.3

Other recently reported manifestations include pancre-atitis, peripheral neuropathy, renal involvement, per-foration of the palate, diffuse myositis, and ventricularaneurysm.4–8 The diagnosis rests on a combination of“major” and “minor” clinical criteria.9,10 Major criteriainclude oral and genital ulcers, ocular symptoms, and

Reprint requests to: M. Turan, Gokce Bostan Mah. 2. Sok.Hurriyet, Apt. Daire No:2, 58050 Sivas, TurkeyReceived: August 17, 2001 / Accepted: September 3, 2002

384 M. Turan et al.: Sigmoid Colon Perforation in Behçet’s Disease

after pricking the skin lesions with a needle. On physicalexamination he had rebound tenderness and muscularrigidity, especially in the left lower quadrant. A hemato-logical screen showed a white blood cell count of 17000/mm3, and hemoglobin of 14g/dl. Liver function tests,blood urea nitrogen, serum creatinine, and blood glu-cose levels were normal. Air-fluid levels were seen onabdominal X-rays (Fig. 1). Abdominal ultrasonographyshowed a 5 � 6 � 8-cm mass in the left lower quadrant.A laparotomy was performed the same day under adiagnosis of acute abdomen, which revealed gangre-nous changes in 7cm of the sigmoid colon and a per-foration 1cm in diameter in the center of this affected

segment (Fig. 2). Localized purulent discharge in theabdominal cavity was cleaned and sigmoid resectionwas performed with a primary anastomosis. His post-operative course was uneventful and he was discharged7 days postoperatively.

The operative specimen had hemorrhagic areas onthe serosal surface, perforation, and localized edema,evident by the mucosal swelling (Fig. 2). Histopatho-logic examination revealed nonspecific inflammatorycell infiltrate surrounding capillaries and venules, espe-cially in the submucosa and serosa (Fig. 3). The extrava-sation of erythrocytes, infiltration of the vascular wallsby inflammatory cells, and necrosis of the endotheliumwith fibrinoid necrosis of the vessel walls were ob-served. In some sections the mucosa was focally ulcer-ated (Fig. 4).

Fig. 1. Abdominal X-ray film showing air-fluid levels

Fig. 2. Macroscopic specimen showing hemorrhagic areas onthe serosal surface and perforation (arrow)

Fig. 3. Histopathologic examination showed nonspecificinflammatory cell infiltration surrounding the vessels(H&E, �25)

Fig. 4. Mucosal ulceration (arrow) (H&E, �10)

385M. Turan et al.: Sigmoid Colon Perforation in Behçet’s Disease

Discussion

Intestinal Behçet’s disease is characterized by deepulcers, most commonly located in the terminal ileum orileoceal region, which tend to perforate or penetrate theintestinal wall.10,11 However, these ulcerative changes inthe intestine are found in less than 1% of all patientswith Behçet’s disease. The abdominal complaints thatnecessitate surgery are abdominal pain (92%), abdomi-nal mass (21%), and melena (17%). In our patient,hypogastric pain developed about 3 days before hisadmission. He had been given dietary and antacidtreatment for about 1 day before admission, with noimprovement.

There are two types of intestinal ulcers in Behçet’sdisease: localized and diffuse. According to our reviewof the literature, among 114 cases of the localized typethe terminal ileum was involved in 50, the ileocecalregion in 39, and the cecum in 14, representing 76% ofall reported cases. Other sites included the ileum in 4cases, the duodenum in 2, the transverse colon in 2, andthe stomach, jejunum, and ascending colon in 1 caseeach, respectively. Among 20 diffuse ulcers, 3 involvedthe esophagus, ileum, and cecum, 2 involved the jeju-num and ileum, 6 involved ulceration extending fromthe terminal ileum to the descending colon, and 9 in-volved almost the entire colon. Multiple ulcers werefound in 73% and perforation or penetration was foundin 56%.13 Kyle et al.14 reported a case of sigmoidalperforation due to Behçet’s disease, and to our knowl-edge, the present case is only the second reported caseof perforating sigmoidal ulceration associated withBehçet’s disease.

In our patient, histopathologic changes were pro-minent in the submucosal and serosal vessels and theunderlying pathology was vasculitis. Nonspecific inflam-matory cell infiltrate surrounding the capillaries andvenules, necrosis of the endothelium, and fibrinoidnecrosis of the vessel walls were seen. Focally superfi-cial ulcerated areas were also present. These were themajor features resulting in perforation of the intestine.

The clinical manifestations of Behçet’s colitis areeasily confused with those of ulcerative and granulo-matous colitis. The extraintestinal signs of Behçet’sdisease, such as skin lesions, aphthous stomatitis, anduveitis can occur with inflammatory bowel disease. Thisoverlap casts some doubt on whether Behçet’s disease isindeed a separate entity, or merely a form of inflam-matory bowel disease with predominant systemic mani-festations. Most gastroenterologists accept Behçet’sdisease as a separate entity that sometimes falls withinthe spectrum of inflammatory bowel disease.13

Some reports stress the similarity of colitis in Behçet’sdisease and ulcerative colitis.4,15 The intestinal ulcer for-mation in Behçet’s disease, which is suggestive of ulcer-

ative colitis, is the most common finding on procto-scopic examination.13 These ulcers are characterizedby nonspecific inflammation,11 and edematous swellingwith craters around the ulcer margin (collar buttonulcers),16 and they penetrate the serosa or fascia. How-ever, the distribution pattern, including frequent rightcolonic predominance, discontinuous involvement, andoccasional rectal sparing, is clearly different from thatof ulcerative colitis. Moreover, ulcerative colitis is char-acterized by diffuse mucosal inflammation with nointervening normal mucosa in the affected segment.14

Crypt abscesses, the inflammatory tags of mucosa,restricted inflammatory cell infiltration to the mucosaand submucosa, and pseudopolyps are all importantfeatures of ulcerative colitis that are not seen inBehçet’s colitis.

Many of the features of Behçet’s colitis are suggestiveof Crohn’s colitis, but certain clinical and pathologicalfeatures distinguish them from each other.17 Transmuralinflammation, granuloma formation, plasma cells, thick-ening of the muscularis mucosae, submucosal fibrosis,architectural distortion, and skip lesions are the majorfeatures of Crohn’s disease.18–20 Furthermore, the intes-tinal wall in Behçet’s disease is of normal thickness,unlike the rigid, narrowed segments seen in Crohn’sdisease. These histopathologic changes were not seenin our patient, which ruled out inflammatory boweldisease.

The usual operative procedures for gastrointestinaldisease are resection of the ileocecal region or righthemicolectomy because multiple ulcers are usuallyfound from the terminal ileum to the cecum. We per-formed sigmoid colon resection and primary anasto-mosis in our patient, and a lower gastrointestinalseries done 6 months after the operation was normal.Although our patient has returned to work, he still hasoral ulceration and acne vulgaris, for which he is takingcolchicine.

The length of normal bowel adjacent to the ulceratedsegment, which should be resected, is controversial.Some authors recommend removal of as much as 60 cmor more of the intestine, while others advocate a moreconservative approach with removal of only the grosslyinvolved bowel.11,13 The resected segment of sigmoidcolon in our patient was relatively short.

Medical treatment for intestinal Behçet’s diseaseremains empiric and palliative. Corticosteroids, 5-aminosalicylic acid derivatives, immunomodulators,blood transfusions, thalidomide, pentoxifylline, andmore recently, antitumor necrosis factor monoclonalantibody, have all been used to treat Behçet’s diseasewith varying degrees of success.3,11,21,22 Gastrointestinalinvolvement of Behçet’s disease is relatively commonand usually mild, although occasionally it manifests as asurgical emergency with bleeding or perforation. Thus,

386 M. Turan et al.: Sigmoid Colon Perforation in Behçet’s Disease

surgeons should be aware of these complications whileevaluating patients with Behçet’s disease.

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