Infantile chronic idiopathic intestinal pseudoobstruction: The role of small intestinal manometry as a diagnostic tool and prognostic indicator

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 indicator. amplitude phase III: motility index (A II) <10 KPa/min, all had a poor outcome (death or dependence on parenteral Methods 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.