Clinical characteristics of chronic idiopathic intestinalpseudo-obstruction in adults

Background—Chronic idiopathic intestinalpseudo-obstruction, a syndrome of ineffectual motility due to a primarydisorder of enteric nerve or muscle, is rare.
Aims—To determine the clinical spectrum,underlying pathologies, response to treatments, and prognosis in aconsecutive unselected group of patients.
Methods—Cross sectional study of all patientswith clinical and radiological features of intestinal obstruction inthe absence of organic obstruction, associated with dilated smallintestine (with or without dilated large intestine), being activelymanaged in one tertiary referral centre at one time.
Results—Twenty patients (11 men and nine women,median age 43 years, range 22-67) fulfilled the diganostic criteria.Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two patients had an autosomally dominant inherited visceralmyopathy. Major presenting symptoms were pain (80%), vomiting (75%),constipation (40%), and diarrhoea (20%). Eighteen patients requiredabdominal surgery, and a further patient had a full thickness rectalbiopsy. The mean time interval from symptom onset to first operationwas 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one otherpatient small bowel motility studies were suggestive of neuropathy. Twopatients died within two years of symptom onset, one from generalisedthrombosis and the other from an inflammatory myopathy. Of theremaining 18 patients, eight were nutritionally independent ofsupplements, two had gastrostomy or jejunostomy feeds, and eight werereceiving home parenteral nutrition. Five patients were opiatedependent, only one patient had benefited from prokinetic drug therapy,and five patients required formal psychological intervention and support.
Conclusions—In a referral setting visceralmyopathy is the most common diagnosis in this heterogeneous syndrome,the course of the illness is usually prolonged, and prokinetic drugtherapies are not usually helpful. Ongoing management problems includepain relief and nutritional support.

Keywords:adult; intestinal; pseudo-obstruction; myopathy; neuropathy