Chronic idiopathic intestinal pseudo-obstruction with malabsorption, a scleroderma-like disorder

A case of intestinal pseudo-obstruction in a 76-year-old man who presented with a six-year history of intermittent vomiting and abdominal pain and a 1(1/2)-year history of diarrhea is described. Investigation demonstrated aperistalsis of the lower esophagus and an unresponsive atonic small intestine. Marked malabsorption was present and appeared due to intestinal bacterial overgrowth consequent to the hypomotility. Autonomic nervous system testing failed to reveal any definite abnormality. Although this disorder is markedly similar to scleroderma involving the gastrointestinal tract, there was only minimal fibrosis of the bowel musculature evident at autopsy and the neural components appeared to be intact. These findings suggest that the basic disorder may be one of smooth muscle dysfunction. Other known causes of pseudo-obstruction could not be demonstrated.