Chapter 14 - Chronic intestinal pseudo-obstruction

Abstract

First described by Dudley, chronic intestinal pseudo-obstruction (CIPO) is a rare, heterogeneous, end-stage gastrointestinal (GI) disorder marked by failure of propulsion of intestinal contents, albeit in the absence of mechanical obstruction. It is considered to be the most severe form of GI dysmotility and has been likened to intestinal pump failure as with congestive heart failure. Since Dudley’s first publication describing intestinal obstruction over 60years ago, great progress has been made in our understanding, diagnosis, and treatment of CIPO. Nevertheless, it remains a considerable clinical challenge and frustrating diagnosis for both patients and physicians alike for a variety of reasons. First, the long-standing and recurrent digestive symptoms related to CIPO are usually nonspecific which can often-times lead to delays in diagnosis, high morbidity, and a poor quality of life. In fact, it can take an average of 8years from symptom-onset to make the correct diagnosis and up to 88% of patients will undergo risky needless surgical procedures. Secondly, there is limited awareness of this disorder by most physicians, due to its rare nature. Thirdly, therapeutic options have shown limited efficacy to date. Fourthly, another challenge in making the diagnosis of CIPO is that the clinical course can be extremely variable with some patients remaining minimally symptomatic or asymptomatic between sub-occlusive episodes for prolonged periods of time yet other patients can worsen quickly and develop significant consequences such as severe malnutrition requiring parenteral nutrition and even resulting in death. In the majority of cases, the clinical course of CIPO is progressive and associated with significant morbidity and mortality with malnutrition being its most common complication.