Presented by David R. Spielberg, MD, MHSc, Texas Children’s Hospital, Houston, TX
Often the pulmonary complications of the lymphatic malformations can manifest as or mimic other common disorders: cough or wheezing, thus mimicking asthma or non-specific chronic cough. Lack of response to the usual treatments for these disorders may be a clue to the presence of some other process. This being said, anecdotally, I cared for a KLA patient whom, on top of her other problems, manifested as having asthma that required extremely aggressive treatment to control symptoms.
The most significant pulmonary complication in the lymphatic malformations is pleural effusion. This can be chylous (fatty) fluid in GLA or bloody in KLA. The effusions are challenging to manage because they tend to re-accumulate rapidly with drainage. Effusions themselves compress the lungs and can directly reduce lung function.
KLA being particularly aggressive can directly involve lung tissue or airways, which can further complicate managing these patients.
About the Presenter
Dr. Spielberg is an Assistant Professor of Pediatrics at Baylor College of Medicine and practices at Texas Children’s Hospital in Houston, Texas, where he is a member of the Vascular Anomalies Center.
He has a particular interest in complex airway problems, and in the Rare and Interstitial Lung Disease program. He is also a member of the Pediatric Lung Transplant Team.
His research interests include lung imaging (using both CT and MRI to study lung diseases) and outcomes in Aerodigestive procedures.