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Figure 4.28. Jejunal diverticulosis. Numerous diverticula fill with barium. (Courtesy of Arunas Gasparaitis, M.D., University of Chicago).

overgrowth, and resultant malabsorption develop in these patients.

Pneumatosis Intestinalis

Intramural bowel gas (and it is a gas, not air) is called pneumatosis intestinalis, an uncommon condition. These gas collections range from a

Table 4.9. Conditions associated with pneumatosis intestinalis

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linear, oval, to a round appearance, either submucosal or subserosal in location. It should be emphasized that pneumatosis intestinalis is a sign rather than a specific disease and is found in a number of benign and serious conditions (Table 4.9). Some authors, however, limit use of the term pneumatosis cystoides intestinalis to a benign condition.

The most ominous cause of pneumatosis intestinalis is bowel ischemia; in adults a poor prognosis is suggested if both pneumatosis intestinalis and portal venous gas are detected. In the pediatric age group, however, a benign course occasionally ensues for pneumatosis intestinalis associated with gas in both the portal vein and systemic veins.

Little in the imaging appearance of pneumatosis intestinalis allows one to suggest an underlying cause. Thus benign pneumatosis and pneumatosis due to bowel ischemia have a similar appearance. In a minority of patients with benign pneumatosis, imaging also reveals a pneumoperitoneum, presumably secondary to rupture of some of the gaseous blebs.

Pneumatosis intestinalis is readily diagnosed with conventional radiography. With CT, differentiation of colonic pneumatosis involving the left colon from diverticula can be difficult. In general, CT lung windows should be used to detect pneumatosis. Magnetic resonance findings consist of circumferential collections of gas next to or intramural in location; such collections are easier to see with gradient echo images.

Bowel ischemia

Adults with chronic obstructive pulmonary disease After endoscopic biopsies

Progressive systemic sclerosis and chronic granulomatous disease

Systemic amyloidosis Myotonic dystrophy

Drug therapy with steroids, chemotherapy Immunosuppression and immunodeficiency states Cryptosporidiosis infection in AIDS patients Following surgical handling of bowel

After jejunoileal bypass surgery and organ transplantation

With malignancies such as leukemia and lymphoma Associated with gastric ulcer, bowel carcinoma and

metastatic disease

In infants with necrotizing enterocolitis In infants with pyloric stenosis

Vascular Lesions (Bleeding)

If the underlying condition cannot be otherwise corrected, the therapeutic procedure of choice in controlling an acute hemorrhage often is percutaneous transcatheter vascular embolization. A variety of embolization materials are in use.

Etiology

The list of conditions responsible for lifethreatening gastrointestinal hemorrhage is legion (Table 4.10). Discussed here are only those conditions not covered in other sections.

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Table 4.10. Causes of jejunal and ileal bleeding

Trauma

Infection

Crohn’s disease

Vascular causes

Ischemia

Angiodysplasia

Telangiectasia

Arteriovenous malformation

Dieulafoy lesion

Varices

Ruptured aneurysm

Hemangioma

Meckel’s diverticulum

Neoplasm

Other

Ectopic decidua

Bleeding disorder/overcoagulation

Aneurysm

Arterial aneurysms do rupture into the small bowel. Angiography or CT should detect them and, during active bleeding, occasionally even identify contrast extravasation into the small bowel lumen.

Neoplasms

Almost any small bowel neoplasm will bleed either acutely or on a chronic basis. Some of the rarer neoplasms associated with bleeding include:

Small bowel hemangioendotheliomas: They typically manifest through bleeding or, less often, abdominal pain.

Small bowel angiosarcomas: These bleed, ulcerate, or even obstruct. Barium studies and CT should detect these tumors once they reach a certain size. Because of the intermittent nature of bleeding with these highly vascular tumors, scintigraphy may be negative.

Angiodysplasia

The prevalence of angiodysplasia increases with age; most are acquired.An increased prevalence is found in patients with renal failure, von Willebrand’s disease, aortic stenosis, pulmonary disease,and cirrhosis,although in some patients

such an association is fortuitous. The prevalence is difficult to estimate; often bleeding from an unknown site is ascribed to small bowel angiodysplasia. Angiography detects some angiodysplasia and some are identified by the pathologist, but some are undoubtedly missed because the involved vessels collapse in resected bowel.

If detected by imaging, most angiodysplasias are resected. As an aid to the pathologist, intraarterial injection of a low-viscosity barium sulfate suspension or a dye and water-soluble contrast agent mixture into a resected specimen should reveal any dilated vessels.

Telangiectasia

Telangiectasias of the small bowel are uncommon. Multiple telangiectasias should suggest Osler-Weber-Rendu disease even in the absence of characteristic skin and mucous findings. These lesions are responsible for chronic bleeding.

Varices

Small bowel varices are rare. Some are due to portal hypertension and unusual drainage pathways; thus an occasional varix is encountered in the ileum. Varices also develop secondary to an extensive arteriovenous shunt, such as a large hemangioma. Stomal varices have developed at an ileostomy.

Similar to other sites, small bowel varices are identified by superior mesenteric angiography or percutaneous transhepatic mesenteric venography if access to the site of varices can be achieved.

Small bowel varices have been treated by percutaneous transhepatic embolization, using a transjugular approach, or portosystemic shunting if they are due to portal hypertension.

Dieulafoy Lesion

Dieulafoy lesions were essentially unheard of in the small bowel until the mid-1990s, when a number of case reports were published. These patients present with massive hemorrhage, and most reports describe jejunal rather than ileal Dieulafoy lesions.

Superior mesenteric angiography simply detects a bleeding site and provides rough local-

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ization. Intraoperative small bowel endoscopy is helpful to locate the actual bleeding site.

Other

Extrauterine decidua rarely involves the bowel wall. Occasionally such decidua bleed, with bleeding usually intraperitoneal in location. Intraluminal bleeding is unusual.

Bleeding Detection

Barium studies should detect a small bowel tumor if the tumor is sufficiently large. Angiography identifies extravasation if the bleeding is brisk enough and, at times, reveals prominent vasculature, both arterial and venous.

Most physicians encounter occasional patients with intermittent bleeding who have a negative upper gastrointestinal, small bowel, and colon workup for a bleeding site. One approach in these patients is to provoke bleeding with heparin and urokinase, followed by scintigraphy and then mesenteric angiography in those with positive scintigraphy. In 10 such provocative bleeding studies, scintigraphy detected hemorrhage in four and in two of these a source was identified and treated with embolization or surgery (116).

Computed tomography is not the primary modality used to detect gastrointestinal hemorrhage. Anecdotal reports describe detection of intraluminal contrast within the small bowel during CT performed immediately after negative angiography. Such a study must be performed without the use of oral contrast.

Some spontaneous intraluminal bleeding is associated with an intramural hematoma. Computed tomography initially reveals a hematoma to be isodense to blood, but as a clot forms it gradually becomes hyperdense; in time, with clot lysis, its attenuation (density) decreases. A noncontrast (neither oral nor IV) CT is preferred if intramural hemorrhage is suspected; contrast tends to obscure a hyperdense hematoma. The involved small bowel wall is thickened and at times a halo containing a hyperdense ring is detected. Lumen obstruction ensues with sufficient intramural bleeding and the more proximal small bowel dilates.

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Intramural hemorrhage results in hyperintense signals on precontrast T1and T2weighted MR sequences.

Both colloid and red blood cell scintigraphy detect bleeding sites, although sensitivity of Tc-99m–red blood cell scintigraphy is higher. Especially if upper gastrointestinal sources of bleeding have been excluded by other tests, a Tc- 99m–red blood cell scan is very reliable in identifying small bowel bleeding. Occasionally a bleeding site is detected only on dynamic blood flow studies and a first set of static images; peristalsis prevents identification of a bleeding site on subsequent images.

Criticism of the use of scintigraphy exists. In a surgical study from a community hospital, scintigraphy localized an exact bleeding site in only 19% of patients (117); confirmation of an actual site of bleeding was either at surgery or endoscopy. These results do not reflect conclusions reached in most published studies.

In some patients with known bleeding and a negative scintigraphic study within the first several hours, a conservative medical management approach appears appropriate.

Even if angiography does locate a site of hemorrhage, a surgeon still has to find the site in the small bowel. In such a setting a highly selective angiographic catheter left in place after angiography, with subsequent intraoperative methylene blue dye injected through the catheter, is useful to the surgeon in locating the site of bleeding.

Immunosuppression

AIDS

A majority of AIDS patients developing diarrhea are infected with enteric pathogens. Some specific infections tend to be more common in certain geographic regions. For instance, the intracellular protozoan Leishmania donovani is common in the Mediterranean basin and is one of the infections often detected in HIV-positive patients in that region. Cryptosporidial oocyst infection involves primarily the proximal small bowel. Several AIDS patients developed pneumatosis intestinalis and cryptosporidiosis infection and the association appears to be more

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than coincidental. Mycobacterium avium intracellulare infection results in extensive adenopathy. Small bowel perforation has developed due to M. tuberculosis in HIV-infected patients. Pneumocystis carinii bowel infection leads to lumen obstruction and bleeding. Ileal actinomycosis has been described in an AIDS patient (118). Two or more coexisting organisms are common in these patients.

Gastrointestinal lymphoma in AIDS patients is an aggressive tumor associated with a poor prognosis. These lymphomas develop anywhere from the esophagus to the colon; a polyposislike appearance is not uncommon. A more unusual lymphoma presentation was as an ulcerated tumor in a Meckel’s diverticulum (119).

Patients with AIDS develop nonspecific small bowel ulcers. These ulcers, also occurring in the oropharynx and esophagus, appear to be caused directly by the HIV itself. The ulcers tend not to become stenotic, which is in contradistinction to idiopathic ulcers in non-AIDS patients. Some small bowel ulcers are also secondary to superimposed infection and necrotic tumors.

Graft-Versus-Host Disease

Two types of bowel complications predominate after bone marrow transplantation, namely either a superinfection or acute graft- versus-host disease. The presence of both complications signifies an especially ominous prognosis.

Graft-versus-host disease generally manifests within several months after allogeneic bone marrow transplantation, with the gastrointestinal tract being a primary target organ. Initially it manifests as thickening of valvulae conniventes, followed by diffuse bowel wall thickening and resultant featureless tube-like lumen narrowing, a “pipestem” appearance, and separation of bowel loops (Fig. 4.29). At times CT reveals marked contrast enhancement, a pattern also seen with some superimposed infections and other infiltrating or ischemic conditions. Acute gastrointestinal graft- versus-host disease in adult allogeneic bone marrow transplant recipients occurs with about the same frequency as gastrointestinal infections.

A B

Figure 4.29. A: Graft-versus-host disease after bone marrow transplant. B: Viral gastroenteritis in a bone marrow transplant patient. The two conditions have a similar imaging appearance.

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Occasionally massive hematochezia develops secondary to either graft-versus-host disease or viral gastroenteritis.

CT detects small bowel wall thickening, engorgement of vasa recta and mesenteric fat stranding (120); ascites, periportal edema and mucosal contrast enhancement are evident in some patients. Fluid-filled, dilated bowel is common proximal to segments of bowel wall thickening. Bowel wall thickening is related to more severe graft-versus-host disease. Some patients exhibit a thin, enhancing mucosal layer which histologically consists of vascular granulation tissue.

A rare bone marrow transplant patient exhibits prolonged oral barium sulfate adherence to small bowel mucosal, at times for several weeks a finding probably related to severe mucosal disease resulting in a denuded mucosa and barium crystals being trapped within lamina propria.

Magnetic resonance reveals diffuse bowel wall thickening and increased postcontrast bowel wall enhancement (121).

Diffuse invasive gastrointestinal candidiasis presented as adynamic ileus after bone marrow transplantation (122).

Other

A subset of liver and kidney transplant patients develops inflammatory bowel disease while undergoing immunosuppression; histology is consistent with either ulcerative colitis or Crohn’s disease. Usually the colon is involved, but some patients have small bowel disease. Some respond to maintenance therapy, but others require a colectomy. Although inflammatory bowel disease in these patients may be fortuitous, the onset and progression of disease while already immunosuppressed is surprising.

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liver, or even several organs. The current indications for small bowel transplantation include short-bowel syndrome, severe intractable diarrhea, cancer, and severe intestinal pseudoobstruction. Many of these patients have been on prior chronic parenteral nutrition.

Graft rejection rates appear lower if combined liver and small bowel grafts are performed rather than an isolated bowel transplant.

For Obesity

Instead of a gastric bypass, pancreaticobiliary diversion is an alternative procedure for obesity. It consists of a distal gastrectomy, gastroenterostomy, and a Roux-en-Y enteroenterostomy. The enteroenterostomy is created closer to the ileocecal valve than to the stomach.

One complication is obstruction of the pancreaticobiliary loop. It results in a dilated, fluidfilled segment that is difficult to identify with conventional radiography. Computed tomography or MR should be diagnostic.

Currently a jejunoileal bypass is rarely performed. In the occasional such patient where bowel visualization is desired, either a conventional antegrade barium study or enteroclysis defines the in-continuity small bowel but not the excluded loops. With an end-to-end jejunoileostomy and an end-to-side ileosigmoid colostomy of the excluded segment, a barium enema at times defines varying lengths of excluded ileal segment. Such an approach is not feasible with an isoperistaltic end-to-side jejunoileostomy. Computed tomography with axial reconstructions appears reasonable to help define bypassed segments, but little in the literature provides guidance for such an approach.

Postoperative Changes

Small Bowel Transplantation

Small bowel transplantation is lifesaving in those unable to tolerate chronic total parenteral nutrition. Transplantation is performed either only with small bowel or together with colon,

Examination and Surgical

Complications

Ileostomy

The most common site of obstruction after ileostomy construction is at the ileostomy site. A digital examination identifies most of these

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obstructions. If needed, a retrograde barium study evaluates the extent of the obstruction.

Short Bowel Syndrome

How short must the small bowel be before a patient develops a short bowel syndrome is difficult to predict.A minimum remaining small bowel length of about 150cm appears to prevent malabsorption. The remaining length needed to prevent small bowel syndrome also depends on the duration of prior parenteral nutrition.

Isoperistaltic colon interposition is occasionally used to treat short bowel syndrome. The interposed colonic segment functions primarily by slowing intestinal transit. Of interest is that although the interposed colon shows no gross small bowel intestinalization, it adapts by taking on some small bowel features such as an increase in crypt depth and mucosal thickness.

Ileal Pouch

An ileal pouch to anal anastomosis is the operation of choice in patients undergoing a proctocolectomy either for ulcerative colitis or familial polyposis. On a long-term basis the ileal pouch mucosa undergoes metaplasia and tends to mimic colonic mucosa. A late complication is inflammation of this pouch, or pouchitis. Of interest is that pouchitis almost always occurs in patients who had prior ulcerative colitis; presumably such pouchitis represents recurrent ulcerative colitis.

Both a temporary ileostomy and an ileoanal anastomosis are created after a total proctocolectomy. Prior to subsequent ileostomy takedown, pouchography is generally performed to evaluate the ileoanal anastomotic integrity. Disruption of the ileoanal anastomosis as detected by pouchography, is a sensitive but not specific predictor of subsequent sepsis.

A rare complication of total proctocolectomy and ileal pouch formation is duodenal compression due to superior mesenteric artery syndrome.

Catheter Related

A spontaneous knot in a transgastric jejunostomy tube has been reported (123).

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