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Pneumatosis intestinalis in cystic fibrosis

M Hernanz-Schulman, J Kirkpatrick, H Shwachman

    Radiology
    |August 1, 1986
    PubMed

    Insights

    Pneumatosis intestinalis (PI) in cystic fibrosis patients has increased in incidence and age of onset. PI is linked to obstructive pulmonary disease, facilitating air dissection into the lungs, often silently.

    Area of Science:

    • Pulmonary Medicine
    • Gastroenterology
    • Radiology

    Background:

    • Pneumatosis intestinalis (PI) is a rare condition characterized by the presence of gas within the intestinal wall.
    • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and digestive system.

    Purpose of the Study:

    • To investigate the clinical and radiographic characteristics of PI in patients with cystic fibrosis.
    • To analyze trends in PI onset and incidence over time in this population.

    Main Methods:

    • Retrospective review of clinical and radiographic findings.
    • Analysis of data from 441 patients with cystic fibrosis.
    • Comparison of patients with and without PI.

    Main Results:

    • Increased age at onset and incidence of PI observed since 1944.
    • 95% of PI patients had pneumomediastinum, pneumothorax, or pulmonary interstitial emphysema, versus 62% of non-PI patients.
    • PI characteristics (type, distribution, severity) evolved over time.

    Conclusions:

    • PI in cystic fibrosis is associated with obstructive pulmonary disease, promoting air dissection into lung interstitium.
    • This air dissection can be clinically silent and self-perpetuating.
    • Findings highlight the complex interplay between CF-related lung disease and PI development.

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