Pulmonary Lymphangiectasia is a condition in which there are widespread dilation of the lymphatic channels in the
lungs.  This can be a part of generalized lymphangiectasia or a primary pulmonary developmental abnormality.  Where
there is no evidence of cardiac disease this condition seems to present either at birth or later in infancy.

The cause of Pulmonary Lymphangiectasia is unknown.  The signs of PL can sometimes be seen prenatally, but most
commonly postnatal.  Many infants are stillborn.  The newborn infants show respiratory distress.  

The typical presenting symptoms include rapid breathing, and recurrent cough or wheeze.  An examination of the
patient usually reveals the presence of fine crackles throughout the lungs.

Diagnosis of pulmonary lymphangiectasia can be difficult.  Like with many respiratory symptoms the findings can be
non-specific.  Although chest x-rays are usually abnormal, the x-rays can reflect fluid in the chest cavity or lung tissue.  
They can also show over expansion of the lungs. Chest CT scans can show patchy areas of fluid and over inflation in
PL patients.  

Most patients who survive through early infancy continue to have respiratory problems (wheeze, recurrent cough, and
pneumonia) in the first several years of life.  These patients also seem to have other medical problems
(gastrointestinal reflux) common to patients with chronic lung disease.  

Evidence has shown that symptoms improved in most patients after infancy and were notably better by the age of 6
years old.  The only treatment is medical support until patient is no longer in need of support.  The evidence suggest
that primary pulmonary lymphangiectasia does not have as dismal a prognosis as in most cases and clinical findings
improve after the first year of life.
Little Leakers
Pulmonary Lymphangiectasia