CLINICAL CHARACTERISTICS OF CHRONIC IDIOPATHIC INTESTINAL PSEUDOOBSTRUCTION IN ADULTS

Background-Chronic idiopathic intestinal pseudo-obstruction, a syndrom e of ineffectual motility due to a primary disorder of enteric nerve o r muscle, is rare. Aims-To determine the clinical spectrum, underlying pathologies, response to treatments, and prognosis in a consecutive u nselected group of patients. Methods-Cross sectional study of all pati ents with clinical and radiological features of intestinal obstruction in the absence of organic obstruction, associated with dilated small intestine (with or without dilated large intestine), being actively ma naged in one tertiary referral centre at one time. Results-Twenty pati ents (11 men and nine women, median age 43 years, range 22-67) fulfill ed the diganostic criteria. Median age at onset of symptoms was 17 yea rs (range two weeks to 59 years). Two patients had an autosomally domi nant inherited visceral myopathy. Major presenting symptoms were pain (80%), vomiting (75%), constipation (40%), and diarrhoea (20%). Eighte en patients required abdominal surgery and a further patient had a ful l thickness rectal biopsy. The mean time interval from symptom onset t o first operation was 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one other patient small bowel motility studies were suggestive of neuropathy. Two patients died within two years of symptom onset, one f rom generalised thrombosis and the other from an inflammatory myopathy . Of the remaining 18 patients, eight were nutritionally independent o f supplements, two had gastrostomy or jejunostomy feeds, and eight wer e receiving home parenteral nutrition. Five patients were opiate depen dent, only one patient had benefited from prokinetic drug therapy, and five patients required formal psychological intervention and support. Conclusions-In a referral setting visceral myopathy is the most commo n diagnosis in this heterogeneous syndrome, the course of the illness is usually prolonged, and prokinetic drug therapies are not usually he lpful. Ongoing management problems include pain relief and nutritional support.