Idiopathic intestinal pseudo-obstruction in a family.

A patient with severe manifestations of idiopathic intestinal pseudo-obstruction is presented. Three members of his family suffer from similar symptoms but in a much milder form. The disease has a wide range of clinical and radiologic presentations and its diagnosis is dependent upon the exclusion of all known causes of intestinal pseudo-obstruction. A high incidence of associated megacystis in this family as well as in some reported cases in the literature lead us to conclude that idiopathic intestinal pseudo-obstruction is not a primary disease of the gastrointestinal tract. Its etiology remains obscure.