Multidisciplinary Management of Lymphangioleiomyomatosis Presenting With Severe Bilateral Pneumothorax During...

Lymphangioleiomyomatosis (LAM) is a rare, progressive, multisystem disease primarily affecting women of reproductive age. It is characterized by the abnormal proliferation of smooth muscle-like LAM cells that infiltrate the lungs, lymphatics, and kidneys. This case highlights that the clinical value lies not in isolated features, but in the convergence of early gestational presentation, severe bilateral disease, diagnostic limitations, and the need for coordinated multidisciplinary decision-making in a resource-constrained setting.

A 23-year-old pregnant woman (G3P2L1A1) at 17 weeks of gestation presented with severe shortness of breath and bilateral shoulder pain, escalating to respiratory distress (SpO2 80%) and functional class IV within hours. Imaging revealed bilateral pneumothorax, near-complete lung collapse, and multiple small cysts in the lung parenchyma, suggestive of LAM. Chest tube placement and pulmonary consultations were performed, followed by a control CT scan confirming LAM. Because of the risk of worsening LAM during pregnancy, termination of the pregnancy was carried out, and diagnostic confirmation was achieved through video-assisted thoracoscopic surgery (VATS) with lung biopsy, showing cystic and adhesive lesions. Treatment included decortication, lesion removal, and sirolimus therapy, an mTOR inhibitor. The patient was discharged in stable condition with ongoing medical follow-up.

Management of LAM during pregnancy requires a multidisciplinary approach, emphasizing personalized care to balance maternal and fetal health. This case highlights the need for vigilance and adaptability in the care of pregnant women with rare pulmonary disorders like LAM. Early diagnosis is key to slowing the progressive decline of pulmonary function. Further research is needed to improve treatment options for LAM patients.