Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study.
OBJECTIVE
Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Intestinal motor abnormalities have long been identified in CIIP patients. Little is known of the natural history of the disease in adults. This study evaluated the clinical course of CIIP over time.


METHODS
Fifty-nine consecutive CIIP patients without underlying collagen, vascular diseases, or mitochondrial cytopathies were evaluated between 1985 and 2001. Family history, onset of digestive symptoms, previous surgeries, episodes suggestive of subacute intestinal obstruction, digestive symptoms, body mass index, and feeding habits were recorded. Small bowel manometry was performed by a perfusion technique, and abnormal motor patterns were visually identified. Full-thickness biopsies were available in 11 cases and were processed for immunohistochemical analysis of myogenic and neurogenic components of the gut wall.


RESULTS
Patients were prospectively followed up for a median of 4.6 years (range, 1-13 years). Diagnosis was often made several years after symptom onset (median, 8 years). Thus, the majority of patients (88%) underwent useless and potentially dangerous surgeries (mean, 2.96 per patient). Manometry invariably showed abnormal motor patterns. Pathologic findings included neuropathies in all investigated cases and abnormalities of interstitial cells of Cajal in 5 of 11 cases. Long-term outcome was generally poor despite surgical and medical therapies; 4 patients died of disease-related complications, 4 underwent small bowel transplantation, almost one third required long-term home parenteral nutrition, and two thirds had some sort of nutritional limitations.


CONCLUSIONS
CIIP is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time.
Stanghellini
Vincenzo
V
Department of Internal Medicine and Gastroenterology, University of Bologna, Italy. v.stanghellini@unibo.it
Cogliandro
Rosanna F
RF
De Giorgio
Roberto
R
Barbara
Giovanni
G
Morselli-Labate
Antonio M
AM
Cogliandro
Laura
L
Corinaldesi
Roberto
R
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001342:Autonomic Nervous System Diseases / Q000473:pathology; D001706:Biopsy; D002908:Chronic Disease; D003106:Colon / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D005725:Ganglia, Autonomic / Q000473:pathology; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis* / Q000628:therapy; D007421:Intestine, Small / Q000473:pathology / Q000503:physiopathology / Q000637:transplantation; D008297:Male; D008365:Manometry; D008875:Middle Aged; D009197:Myenteric Plexus / Q000473:pathology; D009752:Nutritional Status; D011446:Prospective Studies; D011795:Surveys and Questionnaires