(Radiographics. 2001;21:911-924.)
© RSNA, 2001
Radiologic Findings of Behçet Syndrome Involving the Gastrointestinal Tract1
Soo Yoon Chung, MD, 2,
Hyun Kwon Ha, MD,
Jung Hoon Kim, MD,
Ki Whang Kim, MD,
Nariyah Cho, MD,
Kyung Sik Cho, MD,
Yong Suck Lee, MD,
Dong Jin Chung, MD,
Hwoon-Yong Jung, MD,
Suk-Kyun Yang, MD and
Yong Il Min, MD
1 From the Departments of Diagnostic Radiology (S.Y.C., H.K.H., J.H.K., K.S.C., Y.S.L., D.J.C.) and Internal Medicine (H.Y.J., S.K.Y., Y.I.M.), Asan Medical Center, University of Ulsan College of Medicine, 388-1 Poongnap-Dong, Songpa-Ku, Seoul 138-736, Korea; and the Department of Radiology, Yongdong Severance Hospital, Yonsei University College of Medicine, Seoul, Korea (K.W.K., N.C.). Presented as a scientific exhibit at the 1999 RSNA scientific assembly. Received March 1, 2000; revision requested April 12; final revision received March 26, 2001; accepted April 2. Address correspondence to H.K.H. (e-mail: hkha@www.amc.seoul.kr).
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Abstract
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Behçet syndrome is characterized by the histopathologic finding of nonspecific vasculitis in multiple organs. The diagnosis is usually made on the basis of the combination of clinical signs and symptoms. This disease involves the gastrointestinal tract in 10%–50% of patients, and the terminal ileum and cecum are chiefly affected. Barium study is useful in demonstrating the characteristic radiographic features of Behçet syndrome involving the gastrointestinal tract. The presence of deep, penetrating ulcers results in a high rate of complications, such as perforation, fistula, hemorrhage, and peritonitis. Furthermore, recurrence of disease adjacent to or at the surgical anastomosis is common. Computed tomography is useful in determining the extent of the lesions and in identifying cases in which complications are likely to occur. Familiarity with the various radiologic findings of Behçet syndrome involving the gastrointestinal tract helps in making an early diagnosis, as well as in establishing an appropriate treatment strategy.
Index Terms: Behçet disease, 95.629 • Esophagus, diseases, 71.629 • Gastrointestinal tract, diseases, 70.629 • Intestines, diseases, 74.629, 75.629
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LEARNING OBJECTIVES
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After reading this article and taking the test, the reader will be able to:
- List the histopathologic and epidemiologic features of intestinal Behçet syndrome.
- Recognize the radiographic and CT features of Behçet syndrome involving the gastrointestinal tract.
- Describe the complications of intestinal Behçet syndrome.
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Introduction
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Behçet syndrome is a chronic, recurring, systemic disorder characterized by the histopathologic finding of nonspecific vasculitis in multiple organs. It primarily affects male patients between 11 and 30 years of age. This disease is found worldwide but has been reported to be most commonly seen in the countries of the eastern Mediterranean and the eastern rim of Asia (1,2); clinical features and gastrointestinal manifestations appear to be more common and more severe in Eastern patients than in North American and British patients (1). The prevalence has been reported to be 1 in 10,000 in Eastern countries (2) and 1 in 15,000 in North America (3).
Although the exact cause of Behçet syndrome remains unknown, it has been speculated that viral infection, environmental factors, and autoimmune mechanisms might play a role (4,5). Also, there are some associations with particular human leukocyte antigens in Eastern countries (1). In contrast to Reiter disease, in which the frequency of HLA-B27 is high, HLA-B5 and HLA-B51 are associated with Behçet syndrome (6).
The diagnosis is based primarily on clinical criteria because of the nonspecificity of the histopathologic findings. Previous sets of diagnostic criteria included a long list of minor symptoms or signs and included clinical features with insufficient frequency (7). Therefore, new diagnostic criteria that were simple and more specific and excluded rarer manifestations appeared to be necessary. As a result, the International Study Group for Behçet’s Disease proposed new diagnostic criteria in 1990 (8). These criteria require the presence of oral ulcers plus any two of the following: genital ulcers, typical eye lesions, typical skin lesions, or a positive result of a pathergy test (ie, a sterile pustule developing after 24–48 hours at the site of a needle prick to the skin) (Table 1).
However, many diseases produce symptoms similar to those of Behçet syndrome, including Reiter disease, Stevens-Johnson syndrome, systemic lupus erythematosus, Crohn disease, and ulcerative colitis. At histopathologic analysis, there is evidence of perivasculitis with lymphocytic infiltration of the small veins and venules (9) (Fig 1). Granulomas are usually absent. Therefore, differentiation between Behçet syndrome and Crohn disease with histopathologic analysis may be very difficult in the absence of granulomas, and granulomas are found in only 50% of biopsy specimens in Crohn disease.
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Figure 1. Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows the classic microscopic features of Behçet syndrome, with perivascular lymphocytic infiltration (arrows) of the subserosal venule (arrowheads).
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Behçet syndrome involves the gastrointestinal tract in 10%–50% of patients; such involvement results from vasculitis in the small vessels of the bowel wall, more frequently in the venules (10–12). The main sites of involvement are the terminal ileum and cecum, but the upper gastrointestinal tract including the esophagus and, rarely, the stomach can be affected (13). The presence of ulcers is the radiologic and pathologic hallmark of intestinal involvement. Two types of ulceration occur: localized and diffuse. In cases of localized lesions, which are commonly seen in the ileocecal region, the mucosal ulcers are deep, often penetrating to the serosal surface with common occurrence of perforation (13). In contrast, diffuse lesions are commonly seen in the colon and may occur as multiple discrete, punched-out ulcers. The smaller such lesions appear as aphthous ulcers, simulating Crohn disease (14,15). However, there is less inflammation surrounding the ulcer in Behçet syndrome than in Crohn disease.
Barium study is useful not only in demonstrating the characteristic radiographic features of Behçet syndrome involving the gastrointestinal tract, such as deep penetrating or punched-out ulcers, but also in determining the extent of the lesions. Although it is impossible or very difficult to detect mucosal ulcers, computed tomography (CT) has advantages in demonstrating bowel wall thickening and lesions in the extraluminal space. Therefore, in patients with Behçet syndrome, CT is advocated for early detection of complications as well as for exclusion of other abdominal pathologic conditions (10,16). Furthermore, CT might allow prediction of the possibility of complications (16).
The optimal medical treatment of Behçet syndrome has not yet been well established. The natural history of exacerbation and remission makes evaluation of therapy difficult (9). Corticosteroids are the mainstay of medical therapy in Behçet syndrome. Other medical treatments such as azathioprine, colchicine, dapsone, levamisole, thalidomide, and immunosuppressive therapies have been advocated (17,18). Patients in whom medical therapy has been unsuccessful or who have extensive disease or complications such as perforation, hemorrhage, or peritonitis should be considered for surgery.
This article presents a broad spectrum of radiologic findings in 56 patients with Behçet syndrome involving the gastrointestinal tract. These patients were selected from among 123 patients in whom Behçet syndrome was diagnosed between June 1989 and June 1997, and all underwent abdominal CT or barium studies of the gastrointestinal tract. The diagnosis in all patients was made by means of the clinical criteria (8). The sites of the lesions in the 56 patients (mean age, 38 years; male-to-female ratio, 32:24) were the esophagus in seven, the small intestine in 13, the ileocecal region or large intestine in 25, and the surgical anastomosis after surgery in 11. CT scans were available in 43 patients, images from small bowel follow-through or double-contrast barium enema studies were available in 47, and esophagograms were available in seven. Complications of Behçet syndrome involving the gastrointestinal tract are also discussed and illustrated.
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Esophagus
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Esophageal involvement is uncommon and occurs more frequently in male patients (19). It causes substernal pain, dysphagia, and occasionally hematemesis. The middle portion of the esophagus is most commonly involved. There are various forms of esophageal lesions, including erosions; aphthous, linear, or perforating ulcers (Figs 2, 3); widely spreading esophagitis; dissection of the mucosa; varices; and severe stenosis (18–21). Thus, there is no specific form of esophageal involvement, unlike the typical ileocolonic ulcers of intestinal Behçet syndrome, which occur as multiple punched-out or undermining lesions. Histologic examination reveals lymphocytic or neutrophilic infiltration in an ulcerative lesion (19). In more than 50% of cases, esophageal involvement in Behçet syndrome is accompanied by other gastrointestinal manifestations, mainly jejunal and ileocolonic ulcers (4,19,22,23) (Fig 3). Because Behçet syndrome is often treated with steroids or other immunosuppressive agents, herpes esophagitis should be suspected as a more likely cause of discrete esophageal ulcers (24). Endoscopic brushing, biopsy, and culture are therefore required to differentiate this condition from viral esophagitis.
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Figure 2a. Behçet syndrome involving the esophagus in a 38-year-old man with epigastric pain and a 2-year history of recurrent oral and genital ulcers. (a) Double-contrast esophagogram shows a large longitudinal ulcer (arrows) along with thickened, converging folds. (b) Double-contrast esophagogram shows multiple ulcers (arrows), diffuse fold thickening, and nodular defects (arrowheads) in the middle and distal esophagus.
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Figure 2b. Behçet syndrome involving the esophagus in a 38-year-old man with epigastric pain and a 2-year history of recurrent oral and genital ulcers. (a) Double-contrast esophagogram shows a large longitudinal ulcer (arrows) along with thickened, converging folds. (b) Double-contrast esophagogram shows multiple ulcers (arrows), diffuse fold thickening, and nodular defects (arrowheads) in the middle and distal esophagus.
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Figure 3a. Behçet syndrome involving the esophagus in a 32-year-old man with odynophagia, oral ulcers, and uveitis. (a) Double-contrast esophagogram shows diffuse ulcers (arrows) in the midesophagus along with fold thickening and luminal narrowing of the esophagus. (b) Image from a double-contrast barium enema study shows a large, discrete ulcer (arrows) with thickened, converging mucosal folds at the terminal ileum.
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Figure 3b. Behçet syndrome involving the esophagus in a 32-year-old man with odynophagia, oral ulcers, and uveitis. (a) Double-contrast esophagogram shows diffuse ulcers (arrows) in the midesophagus along with fold thickening and luminal narrowing of the esophagus. (b) Image from a double-contrast barium enema study shows a large, discrete ulcer (arrows) with thickened, converging mucosal folds at the terminal ileum.
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Small Intestine
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In a review by Kasahara et al (13), the most common site of involvement in the small intestine was the terminal ileum. However, other parts of the small intestine can be involved, although the frequency of concomitant involvement of the ileocecal region is also high in such cases. At barium study, the characteristic radiographic findings include single or multiple discrete ulcers with considerable thickening of the surrounding mucosal folds (Figs 4–6); these ulcers are similar in appearance to peptic ulcers of the stomach or duodenum (25). However, when the ulcers are small or small-bowel follow-through images of poor quality are obtained, these ulcers are not seen, with the only evidence being nonspecific findings such as mucosal fold thickening, contour deformity, luminal narrowing, and bowel separation. A nonspecific pattern of small-bowel malabsorption can sometimes be seen (11). At CT, the involved bowel segment shows mostly concentric or masslike bowel wall thickening (Fig 6b) and enhances markedly after administration of contrast material (16).
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Figure 4. Behçet syndrome involving the small intestine in a 36-year-old man with epigastric pain and a 10-year history of recurrent oral and genital ulcers and uveitis. Image from a small-bowel follow-through study shows multiple discrete ulcers (arrows) with mucosal fold thickening in the proximal ileum.
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Figure 5. Behçet syndrome involving the small intestine in a 28-year-old man with right lower quadrant pain and a 2-year history of oral ulcers. Image from a double-contrast barium enema study shows a large ulcer (arrow) with marked polypoid mucosal fold thickening (arrowheads), which simulates a submucosal tumor.
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Figure 6a. Behçet syndrome in a 42-year-old woman with lower abdominal pain and a 3-year history of oral ulcers. (a) Image from a small-bowel follow-through study shows diffuse mucosal fold thickening (arrowheads) with suspicious multiple ulcers (arrows) in the pelvic small-bowel loops. (b) Contrast material-enhanced CT scan shows concentric bowel wall thickening (arrows) in the ileum with perienteric infiltration.
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Figure 6b. Behçet syndrome in a 42-year-old woman with lower abdominal pain and a 3-year history of oral ulcers. (a) Image from a small-bowel follow-through study shows diffuse mucosal fold thickening (arrowheads) with suspicious multiple ulcers (arrows) in the pelvic small-bowel loops. (b) Contrast material-enhanced CT scan shows concentric bowel wall thickening (arrows) in the ileum with perienteric infiltration.
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Ileocecal Region and Large Intestine
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Any site in the large intestine, including the rectum, can be involved. Despite the similarity of the clinical manifestations of Behçet syndrome to those of ulcerative colitis or Crohn disease (26,27), it is generally agreed that Behçet syndrome is a distinct entity (9,28). Unlike ulcerative colitis, the usual site of Behçet syndrome is the ileocecal region or proximal ascending colon, with a low rate of rectal involvement (13,29). Furthermore, the deep, penetrating ulcers seen in Behçet syndrome differ from those seen in ulcerative colitis. The similarities between Behçet syndrome and Crohn disease are obvious. Both manifest as discrete ulcers and discontinuous bowel involvement with relative sparing of the rectum. However, certain pathologic features can help differentiate between these two conditions (13,29,30): larger and deeper ulcers, less granuloma formation, and the common occurrence of bowel perforation in Behçet syndrome (Table 2). Of course, it is necessary to carefully examine the clinical symptoms and signs, since in many cases the differential diagnosis is very difficult.
At radiologic analysis, these ulcers have been described as discrete, collar button–shaped or ring-shaped lesions with deep penetration (9,13,25,31–33) (Figs 7a, 8a, 9a). An undermining tendency results in a high frequency of perforation, fistula, and hemorrhage. The double-contrast technique is considered to be more valuable than the single-contrast technique for demonstrating small ulcerative lesions (30). Sometimes, Behçet syndrome appears as an ileocecal mass (Figs 8, 10), requiring surgical resection to differentiate an inflammatory mass from a neoplasm (16,25). In addition to the clinical symptoms of Behçet syndrome, its characteristic location across the ileocecal valve (not confined to either the cecum or terminal ileum), diffuse fold thickening in a relatively long segment, and more severe adjacent mucosal deformity may be helpful signs suggesting inflammatory bowel disease rather than a neoplastic lesion (25).
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Figure 7a. Behçet syndrome involving the ileocecal region in a 37-year-old man with right lower quadrant pain and a 3-year history of recurrent oral and genital ulcers. (a) Image from a double-contrast barium enema study shows a large ulcer (U) in the terminal ileum with convergence of thickened mucosal folds. (b) Contrast-enhanced CT scan shows polypoid bowel involvement (solid arrows) at the terminal ileum with a central ulcer (open arrow). The involved bowel segment is markedly enhanced. (Reprinted, with permission, from reference 16.)
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Figure 7b. Behçet syndrome involving the ileocecal region in a 37-year-old man with right lower quadrant pain and a 3-year history of recurrent oral and genital ulcers. (a) Image from a double-contrast barium enema study shows a large ulcer (U) in the terminal ileum with convergence of thickened mucosal folds. (b) Contrast-enhanced CT scan shows polypoid bowel involvement (solid arrows) at the terminal ileum with a central ulcer (open arrow). The involved bowel segment is markedly enhanced. (Reprinted, with permission, from reference 16.)
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Figure 8a. Behçet syndrome involving the ileocecal region in a 39-year-old man with lower abdominal pain and a 3-year history of oral and genital ulcers. (a) Image from a double-contrast barium enema study shows a polypoid masslike lesion (arrowheads) in the ileocecal region with ulcers (arrows) and thickened mucosal folds. (b) Contrast-enhanced CT scan shows marked enhancement of the thickened wall and the polypoid masslike lesion (M). (c) Photograph obtained during colonoscopy shows the large polypoid mass with an ulcer (arrows). The surrounding mucosal folds are considerably thickened.
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Figure 8b. Behçet syndrome involving the ileocecal region in a 39-year-old man with lower abdominal pain and a 3-year history of oral and genital ulcers. (a) Image from a double-contrast barium enema study shows a polypoid masslike lesion (arrowheads) in the ileocecal region with ulcers (arrows) and thickened mucosal folds. (b) Contrast-enhanced CT scan shows marked enhancement of the thickened wall and the polypoid masslike lesion (M). (c) Photograph obtained during colonoscopy shows the large polypoid mass with an ulcer (arrows). The surrounding mucosal folds are considerably thickened.
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Figure 8c. Behçet syndrome involving the ileocecal region in a 39-year-old man with lower abdominal pain and a 3-year history of oral and genital ulcers. (a) Image from a double-contrast barium enema study shows a polypoid masslike lesion (arrowheads) in the ileocecal region with ulcers (arrows) and thickened mucosal folds. (b) Contrast-enhanced CT scan shows marked enhancement of the thickened wall and the polypoid masslike lesion (M). (c) Photograph obtained during colonoscopy shows the large polypoid mass with an ulcer (arrows). The surrounding mucosal folds are considerably thickened.
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Figure 9a. Behçet syndrome in an 18-year-old man with fever, right lower quadrant pain, localized rebound tenderness, and oral ulcers. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrows) with diffuse mucosal fold thickening along the ascending colon and cecum. The terminal ileum (T) is similarly involved. (b) Contrast-enhanced CT scan shows multiple polypoid lesions with a thickened colon wall (arrows). Note the diffuse pericolonic infiltration. (c) Photograph obtained at colonoscopy shows diffusely scattered punched-out ulcers (arrows) in the ascending colon. The patient underwent right hemicolectomy because peritonitis was suspected. At surgery, a definite perforation site was not found, although there was evidence of local peritonitis in the pericolonic and perienteric space surrounding the ileocecal region. (Fig 9b reprinted, with permission, from reference 16.)
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Figure 9b. Behçet syndrome in an 18-year-old man with fever, right lower quadrant pain, localized rebound tenderness, and oral ulcers. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrows) with diffuse mucosal fold thickening along the ascending colon and cecum. The terminal ileum (T) is similarly involved. (b) Contrast-enhanced CT scan shows multiple polypoid lesions with a thickened colon wall (arrows). Note the diffuse pericolonic infiltration. (c) Photograph obtained at colonoscopy shows diffusely scattered punched-out ulcers (arrows) in the ascending colon. The patient underwent right hemicolectomy because peritonitis was suspected. At surgery, a definite perforation site was not found, although there was evidence of local peritonitis in the pericolonic and perienteric space surrounding the ileocecal region. (Fig 9b reprinted, with permission, from reference 16.)
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Figure 9c. Behçet syndrome in an 18-year-old man with fever, right lower quadrant pain, localized rebound tenderness, and oral ulcers. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrows) with diffuse mucosal fold thickening along the ascending colon and cecum. The terminal ileum (T) is similarly involved. (b) Contrast-enhanced CT scan shows multiple polypoid lesions with a thickened colon wall (arrows). Note the diffuse pericolonic infiltration. (c) Photograph obtained at colonoscopy shows diffusely scattered punched-out ulcers (arrows) in the ascending colon. The patient underwent right hemicolectomy because peritonitis was suspected. At surgery, a definite perforation site was not found, although there was evidence of local peritonitis in the pericolonic and perienteric space surrounding the ileocecal region. (Fig 9b reprinted, with permission, from reference 16.)
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Figure 10a. Behçet syndrome in a 28-year-old woman with right lower quadrant pain, diarrhea, and oral and genital ulcers. (a) Contrast-enhanced CT scan shows marked wall enhancement and wall thickening (arrows) in the ascending colon and terminal ileum. (b) Contrast-enhanced CT scan obtained 9 months later shows marked progression of the bowel wall thickening. The patient underwent right hemicolectomy because panperitonitis was suspected. At surgery, there were two perforation sites at the ascending colon and terminal ileum; the presence of transmural ischemic necrosis and fibrosis was confirmed.
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Figure 10b. Behçet syndrome in a 28-year-old woman with right lower quadrant pain, diarrhea, and oral and genital ulcers. (a) Contrast-enhanced CT scan shows marked wall enhancement and wall thickening (arrows) in the ascending colon and terminal ileum. (b) Contrast-enhanced CT scan obtained 9 months later shows marked progression of the bowel wall thickening. The patient underwent right hemicolectomy because panperitonitis was suspected. At surgery, there were two perforation sites at the ascending colon and terminal ileum; the presence of transmural ischemic necrosis and fibrosis was confirmed.
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CT is useful in determining the extent of the lesions and in identifying cases in which complications are likely to occur. At CT, the involved bowel segment appears as a polypoid mass (Fig 7b), a thickened bowel wall (Fig 10), or both (Figs 8b, 9b). According to a recent study (16), the polypoid, masslike lesions may reach a diameter of 8–10 cm in some cases, with central ulceration in one-third of these cases. The presence of a large, masslike lesion may suggest the possibility of a tumor. In most cases with a thickened bowel wall, the wall is unevenly thickened. The involved bowel segments show marked enhancement in many instances, presumably due to vasculitis and perivasculitis. Other CT findings include minimal lymphadenopathy and fibrofatty proliferation. Except for cases with complications, perienteric or pericolonic changes are usually minimal.
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Recurrence at Surgical Anastomosis
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Patients in whom medical therapy has been unsuccessful or who have extensive disease or complications should be considered for surgery. The length of normal intestine adjacent to the ulcerated segment that should be resected is controversial. Some have recommended removal of as much as 60 cm or more of the ileum (29), whereas in other series a more conservative approach seemed warranted, with removal of only the grossly involved bowel segment (26). This controversy is attributable to the fact that recurrence of disease adjacent to or at the surgical anastomosis is common (40%–45% of cases) during the postoperative period (13). There are several types of postoperative recurrence; the most common type consists of one or two deep ulcers (Fig 11), followed by multiple aphthous ulcers (Fig 12) and enterocutaneous fistulas. Most of these recurrent ulcers are noted at or near the anastomotic site and appear within 2 years after surgery (34). Because of frequent recurrence of ulceration and infection, a large, postinflammatory polyp simulating a tumor mass rarely occurs (Fig 13). Therefore, careful followup examination with barium study and endoscopy should begin as early as possible after surgery and should focus on the anastomotic site (34).
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Figure 11a. Behçet syndrome involving the surgical anastomosis in a 49-year-old man with epigastric pain and a 6-year history of recurrent oral ulcers. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 1 year earlier. (a) Image from a small-bowel follow-through study shows a large, deep ulcer (U) with swollen mucosa. (b) Contrast-enhanced CT scan shows polypoid bowel involvement (arrows) on the ileal side of the surgical anastomosis, as well as a central ulcer (arrowhead). There is diffuse vascular engorgement in the mesentery and omentum adjacent to the involved bowel segment. (Reprinted, with permission, from reference 16.)
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Figure 11b. Behçet syndrome involving the surgical anastomosis in a 49-year-old man with epigastric pain and a 6-year history of recurrent oral ulcers. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 1 year earlier. (a) Image from a small-bowel follow-through study shows a large, deep ulcer (U) with swollen mucosa. (b) Contrast-enhanced CT scan shows polypoid bowel involvement (arrows) on the ileal side of the surgical anastomosis, as well as a central ulcer (arrowhead). There is diffuse vascular engorgement in the mesentery and omentum adjacent to the involved bowel segment. (Reprinted, with permission, from reference 16.)
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Figure 12a. Behçet syndrome in a 39-year-old man with a palpable mass in the right lower abdomen and a 7-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 3 years earlier. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrow) with deformed bowel loops (arrowheads) surrounding the surgical anastomosis. (b) Contrast-enhanced CT scan shows masslike bowel wall thickening (arrows) in the area of the surgical anastomosis.
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Figure 12b. Behçet syndrome in a 39-year-old man with a palpable mass in the right lower abdomen and a 7-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 3 years earlier. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrow) with deformed bowel loops (arrowheads) surrounding the surgical anastomosis. (b) Contrast-enhanced CT scan shows masslike bowel wall thickening (arrows) in the area of the surgical anastomosis.
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Figure 13a. Behçet syndrome involving the ileal and colonic sides of a surgical anastomosis in a 32-year-old woman with right lower quadrant pain and an 8-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 7 years earlier. (a) Image from a double-contrast barium enema study shows a large, polypoid mass (M) in the colon near the anastomotic site; the mass obstructs the colonic lumen. Transverse ridges due to pericolonic infiltration are prominent just distal to the site of obstruction. (b) Contrast-enhanced CT scan shows the large mass (M) along the colonic side of the surgical anastomosis, as well as severe pericolonic infiltration. (c) Photograph of a surgical specimen shows filiform, polypoid masses in the colon. (d) Photomicrograph (original magnification, x12.5; hematoxylin-eosin stain) shows an inflammatory pseudopolyp (P) arising from the edge of the ulcer (arrows). (Fig 13a and 13b reprinted, with permission, from reference 16.)
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Figure 13b. Behçet syndrome involving the ileal and colonic sides of a surgical anastomosis in a 32-year-old woman with right lower quadrant pain and an 8-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 7 years earlier. (a) Image from a double-contrast barium enema study shows a large, polypoid mass (M) in the colon near the anastomotic site; the mass obstructs the colonic lumen. Transverse ridges due to pericolonic infiltration are prominent just distal to the site of obstruction. (b) Contrast-enhanced CT scan shows the large mass (M) along the colonic side of the surgical anastomosis, as well as severe pericolonic infiltration. (c) Photograph of a surgical specimen shows filiform, polypoid masses in the colon. (d) Photomicrograph (original magnification, x12.5; hematoxylin-eosin stain) shows an inflammatory pseudopolyp (P) arising from the edge of the ulcer (arrows). (Fig 13a and 13b reprinted, with permission, from reference 16.)
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Figure 13c. Behçet syndrome involving the ileal and colonic sides of a surgical anastomosis in a 32-year-old woman with right lower quadrant pain and an 8-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 7 years earlier. (a) Image from a double-contrast barium enema study shows a large, polypoid mass (M) in the colon near the anastomotic site; the mass obstructs the colonic lumen. Transverse ridges due to pericolonic infiltration are prominent just distal to the site of obstruction. (b) Contrast-enhanced CT scan shows the large mass (M) along the colonic side of the surgical anastomosis, as well as severe pericolonic infiltration. (c) Photograph of a surgical specimen shows filiform, polypoid masses in the colon. (d) Photomicrograph (original magnification, x12.5; hematoxylin-eosin stain) shows an inflammatory pseudopolyp (P) arising from the edge of the ulcer (arrows). (Fig 13a and 13b reprinted, with permission, from reference 16.)
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Figure 13d. Behçet syndrome involving the ileal and colonic sides of a surgical anastomosis in a 32-year-old woman with right lower quadrant pain and an 8-year history of recurrent oral and genital ulcers and uveitis. The patient underwent right hemicolectomy for Behçet syndrome involving the ileocecal region 7 years earlier. (a) Image from a double-contrast barium enema study shows a large, polypoid mass (M) in the colon near the anastomotic site; the mass obstructs the colonic lumen. Transverse ridges due to pericolonic infiltration are prominent just distal to the site of obstruction. (b) Contrast-enhanced CT scan shows the large mass (M) along the colonic side of the surgical anastomosis, as well as severe pericolonic infiltration. (c) Photograph of a surgical specimen shows filiform, polypoid masses in the colon. (d) Photomicrograph (original magnification, x12.5; hematoxylin-eosin stain) shows an inflammatory pseudopolyp (P) arising from the edge of the ulcer (arrows). (Fig 13a and 13b reprinted, with permission, from reference 16.)
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Complications
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Because of the presence of deep, penetrating ulcers, a high rate of complications (56%), such as perforation (Fig 14), fistula (Fig 15), hemorrhage, and peritonitis (Fig 16), has been reported (12,13,26); the perforating ulcers tend to occur at multiple sites (12,13). Various complications also occur at other sites, including thrombosis in the portal, hepatic, mesenteric, and femoral veins and inferior vena cava; abdominal aortic aneurysm; hepatic abscess; and pancreatitis (35,36). CT is useful in detecting these complications (10,16). The risk of complications is higher in patients with diffuse ulcers than in those with a single large ulcer; it is also higher in patients with a thickened bowel wall than in those with a polypoid masslike lesion (16). In the detection of complications at abdominal CT, the extent or degree of perienteric or pericolonic infiltration appears to be of more value than the pattern of bowel involvement or the enhancement pattern. In addition, mesenteric vascular engorgement occurs more frequently in patients with complications (16).
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Figure 14a. Behçet syndrome in a 32-year-old woman with diffuse abdominal pain and a 5-year history of oral ulcers and uveitis. The patient underwent right hemicolectomy with resection of the terminal ileum due to panperitonitis. (a) Image from a double-contrast barium enema study shows irregular ulcers (arrowheads) in the ileocecal region and proximal ascending colon, with evidence of a small amount of barium leakage (arrows). (b) Photograph of a specimen from the right hemicolectomy shows discrete, punched-out ulcers (arrows) in the small and large intestines, as well as irregular perforations (arrowheads). C = cecum. (c) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows necrotizing vasculitis (arrows) with diffuse infiltration of lymphocytes in the vessel wall, which involves medium-sized arteries under the base of the mucosal ulcer (arrowheads). (Fig 14a and 14b reprinted, with permission, from reference 16.)
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Figure 14b. Behçet syndrome in a 32-year-old woman with diffuse abdominal pain and a 5-year history of oral ulcers and uveitis. The patient underwent right hemicolectomy with resection of the terminal ileum due to panperitonitis. (a) Image from a double-contrast barium enema study shows irregular ulcers (arrowheads) in the ileocecal region and proximal ascending colon, with evidence of a small amount of barium leakage (arrows). (b) Photograph of a specimen from the right hemicolectomy shows discrete, punched-out ulcers (arrows) in the small and large intestines, as well as irregular perforations (arrowheads). C = cecum. (c) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows necrotizing vasculitis (arrows) with diffuse infiltration of lymphocytes in the vessel wall, which involves medium-sized arteries under the base of the mucosal ulcer (arrowheads). (Fig 14a and 14b reprinted, with permission, from reference 16.)
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Figure 14c. Behçet syndrome in a 32-year-old woman with diffuse abdominal pain and a 5-year history of oral ulcers and uveitis. The patient underwent right hemicolectomy with resection of the terminal ileum due to panperitonitis. (a) Image from a double-contrast barium enema study shows irregular ulcers (arrowheads) in the ileocecal region and proximal ascending colon, with evidence of a small amount of barium leakage (arrows). (b) Photograph of a specimen from the right hemicolectomy shows discrete, punched-out ulcers (arrows) in the small and large intestines, as well as irregular perforations (arrowheads). C = cecum. (c) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows necrotizing vasculitis (arrows) with diffuse infiltration of lymphocytes in the vessel wall, which involves medium-sized arteries under the base of the mucosal ulcer (arrowheads). (Fig 14a and 14b reprinted, with permission, from reference 16.)
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Figure 15. Behçet syndrome in a 50-year-old man with lower abdominal pain and a 3-year history of recurrent oral and genital ulcers. Image from a double-contrast barium enema study shows a large ulcer (U) at the terminal ileum with a deformed ileocecal region. Note the fistulous communication (arrowheads) between the cecum and the large ulcer at the terminal ileum.
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Figure 16a. Behçet syndrome in a 41-year-old man with diffuse abdominal pain and a 2-year history of oral ulcers. The patient underwent ileocecal resection due to multiple bowel perforations in the distal ileum. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrowheads) and mucosal fold thickening in the terminal ileum. (b) Contrast-enhanced CT scan shows a small amount of free gas (arrowheads) in the peritoneal cavity. (c) Contrast-enhanced CT scan shows concentric bowel wall thickening in the distal ileum (arrows). (d) Photograph of a surgical specimen shows multiple punched-out ulcers (arrows). At surgery, bowel perforations were found in the distal ileum.
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Figure 16b. Behçet syndrome in a 41-year-old man with diffuse abdominal pain and a 2-year history of oral ulcers. The patient underwent ileocecal resection due to multiple bowel perforations in the distal ileum. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrowheads) and mucosal fold thickening in the terminal ileum. (b) Contrast-enhanced CT scan shows a small amount of free gas (arrowheads) in the peritoneal cavity. (c) Contrast-enhanced CT scan shows concentric bowel wall thickening in the distal ileum (arrows). (d) Photograph of a surgical specimen shows multiple punched-out ulcers (arrows). At surgery, bowel perforations were found in the distal ileum.
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Figure 16c. Behçet syndrome in a 41-year-old man with diffuse abdominal pain and a 2-year history of oral ulcers. The patient underwent ileocecal resection due to multiple bowel perforations in the distal ileum. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrowheads) and mucosal fold thickening in the terminal ileum. (b) Contrast-enhanced CT scan shows a small amount of free gas (arrowheads) in the peritoneal cavity. (c) Contrast-enhanced CT scan shows concentric bowel wall thickening in the distal ileum (arrows). (d) Photograph of a surgical specimen shows multiple punched-out ulcers (arrows). At surgery, bowel perforations were found in the distal ileum.
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Figure 16d. Behçet syndrome in a 41-year-old man with diffuse abdominal pain and a 2-year history of oral ulcers. The patient underwent ileocecal resection due to multiple bowel perforations in the distal ileum. (a) Image from a double-contrast barium enema study shows multiple ulcers (arrowheads) and mucosal fold thickening in the terminal ileum. (b) Contrast-enhanced CT scan shows a small amount of free gas (arrowheads) in the peritoneal cavity. (c) Contrast-enhanced CT scan shows concentric bowel wall thickening in the distal ileum (arrows). (d) Photograph of a surgical specimen shows multiple punched-out ulcers (arrows). At surgery, bowel perforations were found in the distal ileum.
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CT also helps significantly in the clinical management by providing corroborative evidence for an inflammatory process and allowing the possibility of an abscess or perforation to be excluded (10). Avoiding unnecessary intervention in this disease is important because of the frequent postoperative complications, such as wound dehiscence, infection, gastrointestinal hemorrhage, and perforation.
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Conclusions
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Behçet syndrome is not a common disorder but commonly involves the gastrointestinal tract, with radiologic manifestations similar to those of inflammatory or neoplastic diseases. Familiarity with the radiologic findings (Table 3) helps in making an early diagnosis, as well as in establishing an appropriate treatment strategy.
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Footnotes
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2 Current address: Department of Radiology, Kumi CHA Hospital, Pochon CHA University College of Medicine, Kumi, Korea. 
See the commentary by Levy and Rohrmann
following this article.
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References
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Abstract
LEARNING OBJECTIVES
