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

TL;DR

A rare lung disease called LAM was diagnosed in a woman through imaging and biopsy, and treated with surgery to prevent recurring lung collapse.

Contribution

This case report highlights the use of pleurodesis as a management option for LAM without extra-pulmonary manifestations.

Findings

A case of pulmonary LAM with no extra-pulmonary features was diagnosed via imaging and biopsy.

Mechanical and chemical pleurodesis was successfully used to manage recurrent hydropneumothorax in this patient.

Low VEGF-D levels can complicate the diagnosis of LAM, emphasizing the need for tailored approaches.

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.

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