Clinical heterogeneity of chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIP) is a disorder of the propulsion of the intestinal content, due to a disturbance in the intestinal and colonic myoelectric activity. The diagnosis is a clinical one based on signs and symptoms of intestinal obstruction in the absence of a mechanical luminal obstruction. This disorder was first described by Meyer-Ruge in 1971. It is characterized by hyperplasia of the submucosus plexus with giant intramural ganglia. Subsequently, the definition of Neuronal Intestinal Dysplasia (NID) was given describing the possible different extension of the disease that can involve either the colon only or the whole intestinal tract. Thus, the clinical features of the disease, although of primary importance, can vary for severity, onset and evolution. The confusion existing about the interpretation of this syndrome is due to the fact that intestinal propulsion is regulated by interactions between smooth muscle cells, extrinsic nervous system and hormones, and any disorder that interferes with such mechanism that can alter intestinal motility. CIP can be primitive or secondary to neurofibromatosis, chronic proctitis, anorectal abnormalities, or generally secondary to insult-producing pathologies similar to NID. Primitive forms that are not caused by a known systemic disease are generally much more severe and mainly occur in childhood. Frequently, they have a neonatal onset but symptoms can appear at any age and, as it happens in adults, the beginning can be gradual or unexpected with a variable clinical course.' We examined retrospectively our series of patients affected by a primitive form of CIP to study the clinical and therapeutical variability of this syndrome, with reference to the necessity of parenteral nutrition (PN) and its duration. Moreover, we evaluated who and how many of these patients could be considered as candidates for small bowel transplantation (SBT).