Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.
We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.
Daum
R
R
Dept. of Pediatric Surgery, University of Heidelberg, FRG.
Nützenadel
W
W
Roth
H
H
Zachariou
Z
Z
D002648:Child; D005260:Female; D005355:Fibrosis / Q000473:pathology; D005767:Gastrointestinal Diseases / Q000150:complications* / Q000473:pathology; D005769:Gastrointestinal Motility; D005770:Gastrointestinal Neoplasms / Q000150:complications / Q000473:pathology; D006801:Humans; D007223:Infant; D007415:Intestinal Obstruction / Q000209:etiology* / Q000473:pathology; D009447:Neuroblastoma / Q000150:complications / Q000473:pathology