Pulmonary Lymphangiectasia

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
Complete pulmonary venous atresia leads to pulmonary venous obstruction which results in engorged pulmonary lymphatics and capillaries. Heart size is normal since flow through the left side of the heart is decreased. The condition usually is fatal. It is classified into 3 types: (1) Pulmonary lymphangiectasia associated with congenital heart lesions characterized by obstruction of pulmonary venous return [totally anomalous venous return below the diaphragm, pulmonary venous atresia, mitral atresia, hypoplastic left heart], (2) Pulmonary lymphangiectasia associated with systemic lymphangiectasia, and (3) Isolated pulmonary lymphangiectasia. Type (1) is the most common, Type (3) is the rarest.

Pathology:
Absence of the pulmonary venous structures, prominence of the pulmonary lymphatics and capillaries.

Imaging Findings:
Prominent bilateral, symmetrical interstitial distention of lymphatics with a general branching pattern radiating from the hila that extends to the periphery with prominence of Kerley B lines. Diffuse pulmonary hyperinflation is also seen. Less commonly a ground glass pattern can be seen.

DDX:

• Transient Tachypnea of the Newborn -

References:
See References Chapter.

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