Clinical features and long-term survival in chronic intestinal pseudo-obstruction and enteric dysmotility

Chronic intestinal pseudo-obstruction (CIP) is the most severe form of intestinal dysmotility. Enteric dysmotility (ED) has been proposed as a new diagnostic label for patients with disturbed intestinal motility and severe symptoms but no radiological signs of pseudo-obstruction. The purpose of this study was to compare the clinical features, small-bowel manometry findings and long-term survival in patients with CIP and ED.

Data collected during a 16-year period from 1987 to 2002 were retrospectively analysed and followed-up through 2007 in a tertiary referral centre. The study comprised 55 patients (41 F, median age 42 years, range 23-76) with CIP and 70 patients (63 F, median age 39 years, range 18-71) with ED.

The median observation time was 9.9 years (range 5.2-20.1). Nineteen patients with CIP (35%) and 9 patients with ED (13%) died. Survival among patients with ED was significantly better (p<0.05). Patients with CIP (49%) needed parenteral nutrition more often than patients with ED (14%). Small-bowel manometry showed similar abnormalities in the two groups but absence of a fed motor response to meals was seen in 16/43 patients with CIP compared to none with ED (p<0.001), sustained periods of uncoordinated phasic activity were more common (p<0.05) in CIP patients (23/45) than in ED patients (19/70) and severe hypomotility was only seen in 7 patients with CIP.

CIP and ED differ with respect to severity of measurable physiological derangement, nutritional needs and long-term prognosis. Our findings indicate that CIP and ED are different entities that require different approaches to management.