Infantile chronic idiopathic intestinal pseudo-obstruction: the role of small intestinal manometry as a diagnostic tool and prognostic indicator

• Published in: Gut Vol. 39; no. 2; pp. 306 - 311
• Main Authors: Fell, J M, Smith, V V, Milla, P J
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Society of Gastroenterology 01.08.1996; BMJ; BMJ Publishing Group LTD
• Subjects: Biological and medical sciences; Chronic Disease; Duodenum - physiopathology; Female; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Infant; Infant, Newborn; Intestinal Pseudo-Obstruction - etiology; Intestinal Pseudo-Obstruction - physiopathology; Male; Manometry - methods; Medical sciences; Muscular Diseases - complications; Muscular Diseases - diagnosis; Nervous System Diseases - complications; Nervous System Diseases - diagnosis; Other diseases. Semiology; Predictive Value of Tests; Retrospective Studies; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Human; Performance evaluation; Manometry; Pseudoobstruction; Prognosis; Idiopathic; Gut; Chronic; Etiology; Digestive diseases; Intestinal disease; Clinical investigation; Child
• ISSN: 0017-5749; 1468-3288; 1458-3288
• DOI: 10.1136/gut.39.2.306

BACKGROUND: Chronic idiopathic intestinal pseudo-obstruction (CIIP) presenting in infancy is a rare but serious condition of heterogeneous aetiology often with an uncertain outcome. AIM: To assess whether intestinal manometry in the first two years of life can help define a neuropathic or myopathic aetiology or clinical outcome, or both, in cases of infantile CIIP. SUBJECTS AND METHODS: 14 consecutive children who presented in the first year of life with CIIP were studied histologically and by small intestinal manometry. RESULTS: Five had a myopathic disorder, four were neuropathic, and five unclassified following histological investigation of full thickness intestinal biopsy specimens. Analysis of fasting phase III activity showed four patterns: (1) (n = 4) no detectable motor activity, (2) (n = 5) low amplitude phase III activity, (3) (n = 3) poorly formed phase III complexes of short duration, (4) (n = 2) well formed cyclical phase III activity with abnormal propagation. The seven children with low amplitude phase III: motility index (MI) < 10 KPa/min, all had a poor outcome (death or dependence on parenteral nutrition) after 1-10 years follow up, compared with two of seven of those with a MI > 10 KPa/min. Of the five with myopathic histology, four had a MI < 10 KPa/min. CONCLUSION: These results show that small intestinal manometry is useful not only as an aid in diagnosing the aetiology of CIIP presenting in infancy, but also in predicting outcome.