Intestinal Scleroderma



Intestinal Scleroderma


Michael P. Federle, MD, FACR





Key Facts


Terminology



  • Progressive systemic sclerosis


Imaging



  • Multisystemic disorder with immunologic and inflammatory changes


  • Characterized by atrophy, fibrosis, sclerosis of skin, vessels, and organs



    • Smooth muscle is replaced by fibrous tissue


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



    • Esophagus > duodenum > anorectal > small bowel > colon


  • Small bowel



    • Marked dilatation of small bowel, especially duodenum and jejunum


    • Duodenal findings identical to “SMA syndrome”


    • “Hidebound” small bowel: Atonic with closely spaced thin folds, sacculations


    • Prolonged transit time with barium retention in duodenum and small bowel up to 24 hours


    • ± pneumatosis intestinalis and pneumoperitoneum


    • ± transient, nonobstructive intussusceptions


  • Colon



    • Sacculations on border of transverse and descending colon


    • Loss of haustrations


    • Stercoral ulceration (from retained fecal material in rectosigmoid)


Top Differential Diagnoses



  • SMA syndrome


  • Sprue-celiac disease


  • Ileus






(Left) Film from a small bowel follow through shows dilation of the duodenum with an abrupt narrowing image as it crosses the spine. (Right) Delayed film from the same patient shows barium within the colon, which has a peculiar appearance of sacculations image along the mesenteric border. These reflect the muscle atrophy within the bowel wall and its replacement by collagen and fibrosis.






(Left) Small bowel follow through shows a typical dilated duodenum image and a “hidebound” fold pattern of the jejunum with closely spaced, thin transverse folds image, and an atonic, stiff appearance at fluoroscopy. Also note the pseudosacculation image. (Right) 90-minute film from a SBFT shows classic scleroderma of the small bowel with dilated, atonic jejunum and closely spaced, thin transverse folds image with slow transit. “Pseudo-obstruction” is another descriptive term relevant to this case.



TERMINOLOGY


Synonyms



  • Progressive systemic sclerosis


Definitions



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


IMAGING


General Features

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Jun 13, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Intestinal Scleroderma

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