Idiopathic intestinal pseudo-obstruction in a family

Abstract

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.

Megaesophagus and Megaduodenum Found Incidentally on a Routine Chest Radiograph During a Health Examination

“…CIIP is a refractory disease characterized by chronic, irreversible progression of intestinal dysmotility, leading to malnutrition due to dilatation-associated intestinal obstructive symptoms and dysfunction of the small intestine. Many cases are sporadic, but familial occurrence has been reported as well, and the disease can affect any part of the gastrointestinal tract (4)(5)(6)(7)(8)(9)(10)(11)(12).…”