Celiac-Sprue Disease

Published on 13/07/2015 by admin

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Last modified 13/07/2015

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 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 

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