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

Abstract

Objective. 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. Material and methods. 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. Results. 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. Conclusions. 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.