Idiopathic intestinal pseudo‐obstruction: A familial visceral neuropathy

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Abstract
Four individuals with idiopathic intestinal pseudoobstruction (IIP), a familial visceral neuropathy with autosomal dominant inheritance, are presented. The disorder is characterized by abdominal colic, abdominal distension and diarrhea, and is of a progressive nature with relapses and remissions. The main feature is a disturbance of motility in the affected intestine, with histological evidence of a neuronal disorder characterized by hyperplasia and eventual atrophy. Management of the condition is difficult; initially, treatment should be conservative by nasogastric suction with i.v. nutrition. When laparotomy is undertaken to exclude mechanical obstruction, resection of the intestine is inadvisable, and ileo-colic anastomosis may be beneficial.