Surgical management of pleural complications in lymphangioleiomyomatosis

Abstract

Objective: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease occurring primarily in women. Pneumothorax and chylothorax are common pleural complications in LAM. We aim to explore various options in the surgical management of pleural disease in LAM.

Methods: A retrospective chart review of all patients at the Center for LAM and Rare Lung Diseases at Columbia University was performed, and date, type, and indication for surgical procedure were collected. All patients with any cystic lung disease seen between January 1, 2000, and March 1, 2023, were included in the database.

Results: The charts for 326 patients with possible LAM were reviewed, including 213 with confirmed LAM and 113 women with cystic lung disease consistent suspected to be LAM were reviewed. A total of 40.5% underwent surgical procedures at our institution or at referring hospitals. A total of 15.6% of patients underwent surgical lung biopsies. A total of 16.6% had a history of pneumothoraces, of whom 79.6% underwent chemical and/or mechanical pleurodesis, 14.8% required pleurectomy, and 7.4% were discharged with tunneled indwelling pleural catheters. We found that 5.6% of confirmed LAM patients have history of chylothorax, with thoracic duct ligation, thoracic duct embolization, pleurodesis, and pleurodesis with long-term tunneled indwelling pleural catheter placement all used as treatment strategies.

Conclusions: Here we demonstrate the significant burden of pleural disease in patients with LAM. It is imperative that thoracic surgeons understand the high incidence of pneumothorax in this patient population. Tunneled indwelling pleural catheters are underutilized but provide long-term options for chylous management with long-term mechanical pleurodesis and a decrease in hospital length of stay.

Keywords: chylothorax; indwelling tunneled pleural catheter; lymphangioleiomyomatosis; pleurodesis; pneumothorax.