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Pneumatosis cystoides intestinalis associated with progressive systemic sclerosis

Radiology Department, Lambeth Hospital, London, S.E.1

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Pneumatosis intestinalis is an uncommon condition characterised by the presence of gas-filled cysts in the walls of the intestine. Of 213 cases reviewed by Koss (1952), the small bowel was affected in 63 per cent and the large bowel in 22 per cent of cases. Small bowel involvement is commonly associated with lesions of the pyloric area, usually a benign stenosis. Pneumatosis has been infrequently reported in association with systemic sclerosis. In such cases intestinal perforation and obstruction may be simulated (Fallon, 1967; Miercort and Merill, 1969) and severe malabsorption is common (Hughes, Gordon, Swann and Bolt, 1966; Meihoff, Hirschfield and Kern, 1968; Seaman, Fleming and Baker, 1966). The presence of pneumatosis in systemic sclerosis carries a grave prognosis, death usually occurring within six months.

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