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Late presentation of primary pulmonary lymphangiectasis
Thorax
|December 1, 1978
Summary
Primary pulmonary lymphangiectasis, typically seen in young children, can affect older individuals. This study reports the oldest cases to date, aged 13-19, highlighting its potential presence in adolescents and young adults with pulmonary cysts.
Area of Science:
- Medical Imaging
- Pulmonology
- Pediatric Radiology
Background:
- Pulmonary lymphangiectasis (PL) is a rare congenital anomaly of the lymphatic system.
- It is typically diagnosed in infancy or early childhood.
- PL can be primary or secondary to conditions causing chronic venous congestion.
Observation:
- This report details three male patients diagnosed with primary pulmonary lymphangiectasis.
- The patients' ages were 13, 16, and 19 years, making them the oldest reported cases.
- Radiological findings were confined to one or two pulmonary lobes and the mediastinum.
Findings:
- Primary pulmonary lymphangiectasis can manifest in adolescents and young adults, challenging the notion of its exclusive occurrence in young children.
- The observed cases presented with localized lymphangiectatic changes.
- These findings expand the known age range for primary pulmonary lymphangiectasis.
Implications:
- Pulmonary lymphangiectasis should be included in the differential diagnosis of "pulmonary cysts" in older children and young adults.
- Radiologists and clinicians should consider this diagnosis in non-infantile patients presenting with cystic lung lesions.
- This expands the clinical and radiological spectrum of pulmonary lymphangiectasis, aiding earlier diagnosis and management.