Pulmonary Lymphatic Perfusion Syndrome

Mechanism: congenital anatomical lymphatic variant, with abnormal lymphatic flow within the lungs that overload the lymphatic channels. Alternatively there may be dysfunction of the valve at the junction of the thoracic duct with the left subclavian vein.

Detection: dynamic contrast-enhanced MR lymphangiography

The onset of symptoms is triggered by an increase in central venous pressure, which may follow trauma, infection or other cause.

Clinical findings:

(1) chylothorax

(2) chylopericardium

(3) plastic bronchitis

(4) chyloptysis

The leakage can be controlled by obliteration of the lymphatic vessels by glue injection.