Sclerosis of the intestinal tract with severe malabsorption

The clinical findings and pathological changes in a 14 year old boy who died from severe malabsorption are presented. Early signs of the illness were attacks of abdominal pain, diarrhoea, and intermittent periods of constipation. Later, severe malabsorption together with chronic ileus were apparent but no organic obstruction was found at several laparotomies. X-ray examination revealed extremly distended intestinal loops and a very slow transit time of barium. Malnutrition could not be improved by dietary, medical, or surgical therapy. Microscopic examination of the tissues obtained at autopsy showed changes in the small vessels of the lungs, heart, kidneys, and intestine. Increased fibrous tissue combined with atrophy of the muscular coat was observed in the wall of the entire intestine. Although cutaneous changes of scleroderma were not evident the deposition of collagenous material and the vascular changes seem typical of systemic sclerosis.