Functional gastrointestinal disorders range from highly prevalent, but generally benign conditions, eg, functional dyspepsia and irritable bowel syndrome, to rare and potentially life-threatening diseases, eg, intestinal pseudo-obstruction syndromes. The term “intestinal pseudo-obstruction” was introduced in the late 1950s by Dudley and Colleagues who reviewed their own series observed over the years of thirteen cases with intestinal obstruction unexplained by any mechanical cause (notably, some of them originally referred to as “spastic ileus”). Subsequently, the existence of intestinal pseudo-obstruction syndromes has been confirmed and extended by other Authors. It is now established that pseudo-obstruction refers to a condition characterized by symptoms and signs (both clinical and radiologic) of intestinal obstruction, but without evidence of any lesion occluding the lumen of the gut (hence the prefix “pseudo”). From a clinical standpoint, pseudo-obstruction syndromes may manifest either acutely or chronically.
Acute intestinal pseudo-obstruction occurs in the vast majority of patients undergoing abdominal surgery (ie, postoperative ileus) and resolves spontaneously within a few days. It may also be caused by a number of conditions, such as peritonitis, hypokalemia, and myocardial infarction. Acute colonic pseudo-obstruction (also referred to as Ogilvie’s syndrome) belongs to these forms and is characterized by a massive colonic dilatation mainly affecting elderly people with underlying co-morbidities (eg, neurologic diseases, spinal or pelvic traumas).
Like chronic heart failure, chronic intestinal pseudo-obstruction (CIPO) can be viewed as an insufficiency of the “intestinal pump” with defective smooth muscle contractility unable to promote transit of luminal contents through the gut, due to lack of either coordination or propulsive forces. The resulting clinical picture is characterized by recurrent episodes of intestinal subocclusion, basically undistinguishable from true mechanical obstruction. This makes CIPO a clinical challenge often remaining undiagnosed or misdiagnosed for long periods. In addition to severe dysmotility and related symptoms, CIPO is also a common cause of functional intestinal failure, being responsible for up to 20% of adult cases. The reduction of the enteric absorptive surface below the minimal amount necessary for adequate digestion of nutrients makes nutritional supplementation a fundamental therapeutic measure in most patients with CIPO. The severity of the clinical picture, very often associated with disabling digestive symptoms between subocclusive episodes, together with the inability to maintain a normal body weight and the generally limited understanding of the syndrome by physicians are all factors contributing to poor quality of life and an established mortality rate in CIPO.
This article will focus on practical issues that remain a matter of debate, such as histopathologic and pathogenetic data, clinical features, diagnosis, as well as main therapeutic measures currently available for adult patients with CIPO.
Epidemiology
CIPO is a typical example of a rare (or orphan) disease/syndrome with an unknown prevalence and incidence. Information derived from pediatric CIPO estimates that approximately 100 infants are born each year in the United States with congenital forms of CIPO, which is undoubtedly an underestimate of the number of new cases per year. Overall, females appear more prone to CIPO than males as shown in major published series of adult patients. Hopefully, registries developed by national health agencies will delineate the epidemiology of this syndrome based on accurate diagnosis in dedicated tertiary referral centers.