Celiac-Sprue Disease

 Mucosal hyperenhancement accompanies active ulceration


image Reversal of jejunoileal fold patterns (atrophied jejunal, thickened ileal)

image Submucosal edema, fat, or gas

image Small bowel intussusception

image Eccentric soft tissue density mass in bowel wall (tumor)

image Mesenteric adenopathy (may be cavitated)


• Excess fluid within SB lumen
image Conformation of flaccid SB segments

image Distends lumen and dilutes contrast medium

• Colonic luminal dilation
image Excess gas, fluid, fat within lumen

• Eccentric soft tissue density mass in bowel wall
image Strongly suggests lymphoma or carcinoma




TOP DIFFERENTIAL DIAGNOSES




• Whipple disease

• Crohn disease

• Intestinal opportunistic infections


CLINICAL ISSUES




• Common: Affects 1 in 200 in USA, but < 10% are currently diagnosed
image Most common cause of SB disease and malabsorption

• Steatorrhea, abdominal distension, flatulence
image Diarrhea, weight loss, glossitis, anemia

• Refractory disease
image Enteritis that does not respond to at least 6 months of gluten-free diet

image GI malignancies are main cause of death in celiac disease

image
(Left) Axial CECT in a 37-year-old man with painful abdominal cramps shows 1 of several sites of intussusception image, typically short segment and nonobstructing.


image
(Right) Axial CECT in the same patient demonstrates that the jejunal fold pattern seems blunted. Also noted is mesenteric lymphadenopathy image.

image
(Left) Axial CECT in the same patient shows more mesenteric lymphadenopathy image along with the abnormally blunted jejunal fold pattern.


image
(Right) Axial CECT in the same patient shows another intussusception image. There is a suggestion of abnormal fold prominence in the ileum image. The flaccid, dilated pelvic SB loops press on each other without intervening space, known as the conformation sign.


TERMINOLOGY


Synonyms




• Nontropical sprue or celiac-sprue disease, gluten-sensitive enteropathy


Definitions




• Celiac disease: Chronic intolerance of gluten that induces intestinal injury in genetically predisposed individuals

• Tropical sprue: Malabsorption seen in inhabitants of tropical countries


IMAGING


General Features




• Best diagnostic clue
image CT enterography: Evidence of reversed fold pattern, multifocal intussusception

• Location
image Celiac disease: More proximal small bowel

image Tropical sprue: Entire small bowel

• Other general features
image Most common small bowel disease producing malabsorption syndrome

image Due to sensitivity of small bowel to α-gliadin
– Component of gluten

image Has familial susceptibility with genetic basis


Radiographic Findings




• Barium small bowel follow-through (SBFT)
image Dilatation of small bowel (jejunum): > 3 cm

image Valvulae conniventes: May exhibit 5 patterns
– Valvulae look normal in most patients

– Ends at margin that are squared off rather than rounded

– Reversed jejunoileal fold pattern: ↓ number of jejunal folds and ↑ ileal folds

– Blunted or absent valvulae: “Moulage” sign (cast): Characteristic of sprue

– Thickening: In severe disease and hypoproteinemia

image “Colonization of jejunum”: Loss of jejunal folds → colon-like haustrations

image Hypersecretion-related artifacts: Due to excess fluid
– Flocculation: Coarse granular appearance of small clumps of disintegrated barium due to excess fluid; mainly in patients with steatorrhea

– Segmentation: Break up of normal continuous column of barium, creating large clumps of barium separated by string-like strands

image Transit time: May be long, short, or normal

image Nonpropulsive peristalsis (flaccid and poorly contracting bowel loops)

image Painless, transient intussusceptions often seen on fluoroscopic studies

• Fluoroscopic-guided enteroclysis
image More accurate than SBFT in diagnosing celiac disease

image Jejunal folds
– Decreased number of proximal jejunal folds (< 3/inch; normal: ≥ 5/inch)

– Increased separation and absence of folds; “ileal” appearance

image Ileal folds
– Increased number of folds in distal ileum (4-6/inch; normal: 2-4/inch)

– Increased fold thickness ≥ 1 mm: “Jejunization” of ileum in 78% of cases

image Mosaic pattern: Due to total villous atrophy
– 1-2 mm islands of mucosa surrounded by barium-filled grooves

image Duodenal changes
– Decreased number and irregular folds, especially in distal duodenum

– “Bubbly” duodenum: Nodular pattern in mucosa


CT Findings




• Excess fluid within SB lumen
image Distends lumen and dilutes positive enteric contrast medium

• SB wall may be thick or thinned
image Mucosal hyperenhancement accompanies active ulceration

image Submucosal edema; halo sign

image Submucosal fat in wall of duodenum and jejunum

image Pneumatosis has been reported (not due to ischemia)

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Celiac-Sprue Disease

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