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CT scan patterns of pulmonary alveolar proteinosis in children
V Albafouille1, N Sayegh, S De Coudenhove
1Department of Paediatric Radiology, Hôpital Necker-Enfants Malades, Paris, France.
Insights
Computed tomographic (CT) scans reveal a reticulomicronodular pattern in children with pulmonary alveolar proteinosis (PAP). Therapeutic broncho-alveolar lavage (BAL) can decrease lung infiltrates, correlating with disease severity.
Area of Science:
- Pediatric Radiology
- Pulmonology
- Medical Imaging
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disease.
- Understanding CT findings in pediatric PAP is crucial for diagnosis and management.
Observation:
- This study retrospectively analyzed CT scans of five children diagnosed with PAP.
- Patients presented with varying symptoms, including incidental bronchitis, sibling disease, or relapsing respiratory symptoms.
Findings:
- Initial CT scans consistently showed a diffuse reticulomicronodular pattern, with alveolar infiltrates in some cases.
- Asymptomatic patients without treatment showed stable or slightly improved CT findings.
- Therapeutic broncho-alveolar lavage (BAL) led to a variable reduction in lung infiltrates.
Implications:
- The reticulomicronodular pattern on CT is characteristic of PAP, representing alveoli filled with abnormal material, not interstitial infiltration.
- CT findings correlate with disease severity, aiding in treatment decisions.
- BAL is an effective therapeutic intervention for pediatric PAP, with CT monitoring showing treatment response.
Background:
To study computed tomographic (CT) findings in children with pulmonary alveolar proteinosis (PAP) more extensively.
Objective:
To describe the CT features at the time of diagnosis and after therapeutic broncho-alveolar lavage (BAL).
Materials And Methods:
We retrospectively reviewed the CT scans of five children (aged 3 months to 4 years) examined because of incidental bronchitis (n = 1), disease in a sibling (n = 1) and relapsing fever, cough and dyspnoea (n = 3). Each patient had an initial CT scan. Two asymptomatic cases were not treated but were followed up by plain chest films. The other three had BAL and follow-up CT.
Results:
Initial CT in all cases showed a diffuse reticulomicronodular pattern associated in three cases with posterior bilateral alveolar infiltrates. CT in the two asymptomatic patients remained unchanged or slightly improved without BAL. After BAL, a variable decrease of lung infiltrates was observed.
Conclusions:
Correlation between the extent of alveolar consolidation and severity of disease was found. Anatomical and pathological considerations allow us to consider that the classical reticulomicronodular pattern is not due to an interstitial infiltration but to alveoli filled with the abnormal material characteristic of PAP.
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