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Pulmonary lymphangioleiomyomatosis (LAM) having no extra-pulmonary manifestations with chemical and mechanical pleurodesis: A case report and review of literature

Year: 2025
Type: article
Study design: Case Report
Abstract: Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease characterized by abnormal smooth muscle proliferation, leading to cystic destruction of the lung parenchyma. We present the case of a female in her early 40s who presented with intermittent chest pain for 1 month. Imaging revealed left hydropneumothorax with multiple bilateral pulmonary parenchymal cysts, and a subsequent lung biopsy confirmed the diagnosis of pulmonary LAM. The patient underwent video-assisted thoracoscopic surgery (VATS) with mechanical and chemical pleurodesis. Given her stable condition, no immediate sirolimus therapy was initiated, and close follow-up with serial imaging was planned. This case highlights the diagnostic challenges of LAM, especially with low VEGF-D levels, and underscores the role of pleurodesis as a management option in select patients. Early recognition and tailored management are essential to optimize patient outcomes.
Language: English
Subfield: Physiology
Field: Medicine
Open Access status: gold
Estimated APC paid: $600
OpenAlex ID: W4410026769
FWCI: 0.531
Cites: 33
Cited by: 1
Related to: 10