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Published on: September 11, 2012
Infantile chronic idiopathic intestinal pseudo-obstruction: the role of small intestinal manometry as a diagnostic
J M Fell1, V V Smith, P J Milla
1Department of Gastroenterology, Great Ormond Street Hospital for Children NHS Trust, London.
Insights
Infantile chronic idiopathic intestinal pseudo-obstruction (CIIP) diagnosis is aided by small intestinal manometry. This diagnostic tool helps determine the cause and predict the clinical outcome in infants with CIIP.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Diagnostic Tools in Medicine
Background:
- Infantile chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare, severe condition with varied causes and unpredictable outcomes.
- Early diagnosis and etiological classification are crucial for managing CIIP in infants.
Observation:
- Small intestinal manometry was performed on 14 infants diagnosed with CIIP within their first year of life.
- Histological analysis identified myopathic (n=5), neuropathic (n=4), and unclassified (n=5) etiologies.
- Fasting phase III activity patterns varied, with low amplitude phase III (motility index < 10 KPa/min) observed in 7 children.
Findings:
- A motility index < 10 KPa/min correlated with poor outcomes (death or parenteral nutrition dependence) in 7 out of 7 children studied.
- Children with myopathic histology (n=5) predominantly showed a low motility index (n=4).
- Small intestinal manometry revealed distinct patterns of motor activity, aiding in etiological differentiation.
Implications:
- Small intestinal manometry is a valuable tool for diagnosing the etiology of infantile CIIP.
- Manometry findings can predict the clinical course and long-term outcome in infants with CIIP.
- This study supports the integration of manometry into the diagnostic workup for suspected infantile CIIP.
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.
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