Intestinal Scleroderma

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 19/07/2015

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 0 (0 votes)

This article have been viewed 2081 times

 Smooth muscle is replaced by fibrous tissue

• Gastrointestinal tract: Most common internal organ system involvement (80-90%)

image Esophagus > duodenum > anorectal > small bowel > colon
• Small bowel

image Marked dilatation of small bowel, especially duodenum and jejunum
image Duodenal findings identical to SMA syndrome
image “Hidebound” small bowel: Atonic with closely spaced thin folds, sacculations (pathognomonic of scleroderma)
image Prolonged transit time with barium retention in duodenum and small bowel up to 24 hours
image ± pneumatosis intestinalis and pneumoperitoneum
image ± transient, nonobstructive intussusceptions
• Colon

image Sacculations on border of transverse and descending colon
image Loss of haustrations
image Stercoral ulceration (from retained fecal material in rectosigmoid)

TOP DIFFERENTIAL DIAGNOSES

• SMA syndrome
• Celiac-sprue disease
• Ileus

DIAGNOSTIC CHECKLIST

• Markedly dilated atonic small bowel with thin, crowded circular folds and delayed barium transit time
image
(Left) This 50-year-old man has diffuse scleroderma with progressive dysphagia & abdominal bloating. A film from the upper GI small bowel follow-through (SBFT) shows a dilated, atonic esophagus image that is slow to empty due to a distal esophageal, peptic stricture image.

image
(Right) A 90-minute film (same case & study) from SBFT shows classic scleroderma of the small bowel with dilated, atonic jejunum & closely spaced, thin transverse folds image with slow transit. “Pseudo-obstruction” is another descriptive term relevant to this case.
image
(Left) Axial CECT in a 40-year-old woman demonstrates closely packed, thin small bowel folds image and diffusely dilated lumen, classic features of scleroderma with pseudo-obstruction.

image
(Right) Coronal CECT in the same patient demonstrates the dilated small bowel with a “hidebound” appearance of closely packed, thin folds image (particularly in the jejunum), a characteristic feature of scleroderma. Also note the disproportionate dilation of the duodenum image, another common feature of scleroderma.

TERMINOLOGY

Synonyms

• Progressive systemic sclerosis

Definitions

• Multisystem disorder of small vessels and connective tissue of unknown etiology

IMAGING

General Features

• Best diagnostic clue

image Dilated, atonic small bowel with crowded folds and wide-mouthed sacculations
• Other general features

image Multisystemic disorder with immunologic and inflammatory changes
image Characterized by atrophy, fibrosis, sclerosis of skin, vessels, and organs
image Involves skin and parenchyma of multiple organs

– GI tract, lungs, heart, kidneys, and nervous system
image Gastrointestinal tract (GI) scleroderma

– 2nd most common manifestation after skin changes (80-90% of patients)
– Most common sites: Esophagus > duodenum > anorectal > small bowel > colon
– Most frequent cause of chronic intestinal pseudo-obstruction
image Scleroderma classified into 2 types

– Diffuse scleroderma
– CREST syndrome (more benign course)
image Diffuse scleroderma: Cutaneous and visceral involvement

– Severe interstitial pulmonary fibrosis
– Organ failure more likely
– Associated with antitopoisomerase I antibody (anti-Scl 70)
image CREST syndrome: Less cutaneous and visceral involvement

– C alcinosis of skin
– R aynaud phenomenon
– E sophageal dysmotility
– S clerodactyly
– T elangiectasia

Radiographic Findings

• Esophagram

image Atony, aperistalsis: Lower 2/3 of esophagus (smooth muscle)
image Mild to moderate dilation of esophagus
image Patulous lower esophageal sphincter: Early finding
image Ulcers, fusiform peptic stricture (reflux esophagitis)

– Reflux predisposes to Barrett metaplasia (present in 40%)
image Hiatal hernia
• Upper GI series

image Stomach: Gastric dilation and delayed emptying
• Small bowel follow-through

image Marked dilatation of small bowel (particularly 2nd and 3rd parts of duodenum and jejunum)

– May have “megaduodenum”: Dilation made worse by compression of 3rd portion as it passes under mesenteric vessels
– Imaging findings identical to SMA syndrome
image Pathognomonic: Hidebound sign of small bowel

Buy Membership for Radiology Category to continue reading. Learn more here