(Radiology. 1999;213:837-838.)
© RSNA, 1999
The "Hide-bound" Bowel Sign1
Perry J. Pickhardt, MD 2
1 From the Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd, St Louis, MO 63110. Received June 29, 1998; revision requested July 29; revision received August 21; accepted November 19. Address reprint requests to P.J.P., PSC 1005, Box 36, FPO, AE 09593 (e-mail: pjpickhardt@gan10.med.navy.mil).
Index terms: Intestines, diseases, 74.613 • Scleroderma, 71.613, 74.613 • Signs in imaging
 |
APPEARANCE
|
|---|
The "hide-bound" bowel sign is seen on barium studies of the small bowel in patients with scleroderma. This sign describes a narrow separation between valvulae conniventes of normal thickness despite dilatation of the bowel lumen (Fig 1).
View larger version (132K):
[in this window]
[in a new window]
|
Figure 1. Hide-bound bowel sign in a 38-year-old woman with systemic sclerosis. Two-hour radiograph of the small bowel shows a dilated gut, which was hypomotile at fluoroscopy. The valvulae conniventes (arrows) are packed more closely than normal, given the degree of dilatation in the proximal jejunum. Megaduodenum (arrowhead) also is seen.
|
|
|
EXPLANATION
|
|---|
Asymmetric smooth muscle atrophy and fibrosis of the inner circular layer of the tunica muscularis relative to the outer longitudinal layer probably account for the appearance of the hide-bound sign. Foreshortening of the bowel caused by longitudinal smooth muscle contraction results in packing of the valvulae conniventes, which is further accentuated by the associated dilatation of the affected loops.
|
DISCUSSION
|
|---|
Scleroderma is a systemic disease of uncertain cause that is characterized by widespread collagen deposition that results in tissue fibrosis (1). Progressive systemic sclerosis, another term used to describe this disease entity, more aptly reflects the typical clinical course and multiorgan nature of scleroderma. Cutaneous involvement can be limited to the face and distal extremities, or it can be diffuse in nature. Limited skin disease is frequently associated with CREST (ie, calcinosis, Raynaud phenomenon, esophageal disease, sclerodactyly, and telangiectasia) syndrome. The prognosis for patients with diffuse cutaneous disease generally is worse because of frequent renal, cardiac, and pulmonary involvement. As the disease progresses, the skin gradually thickens, hardens, and becomes adherent to the underlying subcutaneous tissue, and this results in an appearance that has been labeled "hide-bound" to reflect the skin's superficial resemblance to skin of domestic animals such as cattle. This term was later adopted to describe analogous changes of atrophy and fibrosis in the bowel wall, as seen on barium studies (2).
Gastrointestinal involvement manifests in up to 90% of patients with systemic sclerosis and represents a substantial cause of morbidity (3). The underlying pathologic changes, which are similar regardless of their location along the gastrointestinal tract, consist of smooth muscle atrophy and fibrosis caused by collagen deposition primarily in the tunica muscularis (4). Any portion of the gut may be affected, but esophageal disease typically predominates. There is esophageal disease involvement in 50%–90% of patients with scleroderma (5). The small bowel is the next most common sight of involvement; disease in this region is present in up to 50% of cases. Colonic and gastric findings are less frequent.
Esophageal involvement with scleroderma primarily affects the distal two-thirds of the esophagus, because the proximal third contains striated muscle. Barium studies may demonstrate reduced or absent esophageal peristalsis combined with prominent gastroesophageal reflux from an incompetent lower esophageal sphincter. Imaging findings of esophagitis are frequently present, and complications of reflux, such as stricture and Barrett metaplasia, are not uncommon.
Systemic sclerosis involves the small bowel in up to 50% of cases (6). Hypomotility from smooth muscle atrophy and fibrosis, a common finding, leads to stasis, dilatation, and pseudo-obstruction. The radiographic appearance of small-bowel dilatation and air-fluid levels in patients with scleroderma can be cause for concern, and barium studies may be necessary to exclude mechanical obstruction. Similar small intestinal dilatation occurs with pseudo-obstruction from other causes—namely visceral myopathies and visceral neuropathies—and with celiac disease and true mechanical obstruction (4). However, because circular muscle involvement does not predominate, none of these conditions will demonstrate the hide-bound sign of valvular packing that is seen with scleroderma; thus, this sign is a useful discriminating finding. In one series of patients with scleroderma, Horowitz and Meyers (2) found that more than 60% of small-bowel studies demonstrated the hide-bound bowel sign, and the findings were most prominent in the jejunum and proximal ileum.
Additional gastrointestinal manifestations of scleroderma include delayed gastric emptying, megaduodenum, pneumatosis cystoides intestinalis, intestinal telangiectasia and bleeding, bacterial overgrowth, malabsorption, absent gastrocolic reflex, and constipation (5). In addition to the hide-bound sign, another characteristic barium study finding that also is related to smooth muscle fibrosis and atrophy is wide-mouthed intestinal diverticula, or sacculations. These sacculations can occur in the small bowel and esophagus, but they more commonly involve the colon, where they are accentuated on postevacuation radiographs (Fig 2).
View larger version (127K):
[in this window]
[in a new window]
|
Figure 2. Colonic sacculations in a 45-year-old woman with systemic sclerosis. Postevacuation radiograph obtained after a barium enema shows multiple wide-mouth diverticula (arrows) that have retained contrast material.
|
|
In summary, the barium study findings associated with gastrointestinal involvement in systemic sclerosis, including the hide-bound sign, are best understood in terms of the underlying pathologic process. Knowledge of the various gastrointestinal manifestations of scleroderma is important for appropriate treatment of patients with this disease.
|
Footnotes
|
|---|
2 Current address: U.S. Naval Hospital, Guantanamo Bay, Cuba. 
A trainee (resident or fellow) wishing to submit a manuscript for Signs in Imaging should first write to the Editor for approval of the sign to be prepared, to avoid duplicate preparation of the same sign.
|
References
|
|---|
-
Gilliland BC. Systemic sclerosis (scleroderma). In: Isselbacher KJ, Braunwald E, Wilson JD, et al., eds. Harrison's principles of internal medicine. 13th ed. New York, NY: McGraw-Hill, 1994; 1655-1661.
-
Horowitz AL, Meyers MA. The "hide-bound" small bowel of scleroderma: characteristic mucosal fold pattern. AJR 1973; 119:332-334.[Abstract]
-
Cohen S, Laufer I, Snape WJ, Jr, et al. The gastrointestinal manifestations of scleroderma: pathogenesis and management. Gastroenterology 1980; 79:155-166.[Medline]
-
Rohrmann CA, Ricci MT, Krishnamurthy S, Schuffler MD. Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis. AJR 1981; 143:933-941.
-
Young MA, Rose S, Reynolds JC. Gastrointestinal manifestations of scleroderma. Rheum Dis Clin North Am 1996; 22:797-823.[Medline]
-
Poirier TJ, Rankin GB. Gastrointestinal manifestations of progressive systemic scleroderma based on a review of 364 cases. Am J Gastroenterol 1972; 58:30-44.[Medline]
This article has been cited by other articles:
TOP
APPEARANCE
EXPLANATION
