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Similar Publications

Surgical management of pleural complications in lymphangioleiomyomatosis.

J Thorac Cardiovasc Surg

August 2024

Center for LAM and Rare Lung Diseases at Columbia University, Department of Anesthesiology, Columbia University Irving Medical Center, New York, NY. Electronic address:

Article Synopsis
  • Lymphangioleiomyomatosis (LAM) is a rare lung disease mostly affecting women, often leading to complications like pneumothorax and chylothorax, so surgical management options were examined.
  • The study reviewed records of 326 patients suspected of having LAM, revealing that 40.5% underwent surgical procedures, with significant instances of pneumothorax and associated treatments like pleurodesis.
  • Findings suggest a high occurrence of pleural disease in LAM patients, indicating a need for thoracic surgeons to be aware of these issues and highlighting the potential benefits of using tunneled indwelling pleural catheters for better management.
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A chylothorax in a young woman: The difficulties of medical management.

Respirol Case Rep

February 2024

Academic Respiratory Unit North Bristol NHS Trust Bristol UK.

Lymphangioleiomyomatosis is a rare pulmonary disease affecting women of childbearing age. Whilst chylothorax is a well-recognized complication of the condition, management strategies aren't well-defined, have low success rates and are often only available at tertiary or specialist centres. We describe a case of a young woman referred to pleural clinic with a chylous effusion found to be secondary to lymphangioleiomyomatosis.

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Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal proliferation of smooth muscle-like cells which results in the formation of thin-walled cysts and progressive lung destruction. It commonly presents with progressive dyspnea that is often associated with a history of pneumothorax or chylothorax particularly among females of reproductive age. In this report, we present a case of hydropneumothorax as the initial presentation of LAM in a 33-year-old woman, a rather rare presentation.

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Malignancy and infections are the most common causes of recurrent chylothorax. Cystic lung disease, especially sporadic pulmonary lymphangioleiomyomatosis (LAM), is a rare condition that may manifest as recurrent chylothorax. We present a case of a 42-year female who presented with dyspnea on exertion secondary to recurrent chylothorax, requiring three thoracenteses within a few weeks.

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