CTICasesPulmonary Lymphangiectasia
Imaging findings
Serial chest CT scans in a 30-something male with IgG subclass deficiency demonstrate chronic, shifting areas of peribronchovascular thickening, subpleural reticulation, interlobular septal lines, and a dilated thoracic duct. Bronchoscopy yields a milky, lipoproteinaceous fluid that clinically simulated alveolar proteinosis, but pathology confirms pulmonary lymphangiectasia.
Key takeaways
Pulmonary lymphangiectasia (pulmonary lymphangiectasis) is a rare congenital anomaly characterized by abnormal dilatation of the pulmonary lymphatic vessels. While classically presenting in neonates as a fatal disease, adult-onset cases can present as chronic cough and recurrent lipoproteinaceous alveolar leaks (simulating alveolar proteinosis). Diagnostic lymphangiography can help localize leaks and induce therapeutic sclerosis.
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