A 15-year-old female with progressive systemic sclerosis presented with a three day history of fever, acute periumbilical abdominal pain and vomiting. She had a history of sexual abuse that required abdominal surgery with a colostomy. On physical exam, the temperature was 38.3°C, the abdomen was mildly distended and diffusely tender with hypoactive bowel sounds and rebound. Joints and skin had evidence of systemic sclerosis. Plain abdominal x-ray showed dilated small bowel and some features atypical of mechanical obstruction: the folds were tightly packed and the small bowel had the hidebound appearance described in systemic sclerosis and there were no air fluid levels. A small bowel series also showed tightly packed folds and a delayed transit time (8 to 10 hours). It was thought that the findings were those of systemic sclerosis rather than mechanical obstruction possibly related to adhesions. About 85% of patients with scleroderma have gastrointestinal involvement, which remains subclinical, though causing radiological abnormalities in 50%. Therefore the radiological findings in this patient did not necessarily mean that the obstruction was related to the systemic sclerosis. In fact, the obstruction resolved promptly with nasogastric aspiration and a subsequent motility study was normal. It was concluded that the intestinal obstruction had been mechanical, though only partial, but that the patient might well develop systemic sclerosis related gastrointestinal disease with time. There is no treatment proved to be effective for progressive systemic sclerosis but there is evidence that immunosuppression for severe organ involvement, treatment of hypertension with angiotensin converting enzyme inhibitors and supportive treatment for skin and gut, often result in a good clinical outcome.
|Original language||English (US)|
|Number of pages||4|
|Journal||Children's Hospital Quarterly|
|State||Published - Jan 1 2000|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health