Chest
Original Research: LymphangioleiomyomatosisManagement of Pneumothorax in Lymphangioleiomyomatosis: Effects on Recurrence and Lung Transplantation Complications
Section snippets
Methods and Materials
Data on pleural complications of LAM were collected using the LAM Patient Database developed by the LAM Foundation (Cincinnati, OH). Registration with the LAM Foundation is open to any LAM patient worldwide. On registration, each new patient is sent a demographic and clinical data questionnaire (Enrollment Questionnaire) with a consent form. The LAM Pleural Disease Consensus Group was commissioned by the LAM Foundation to address the management of pneumothorax in LAM. The LAM Pleural Disease
Initial Pneumothorax
Of 395 patients who completed the LAM Foundation enrollment questionnaire, 260 patients reported at least one spontaneous pneumothorax during their lifetime (incidence, 66%), and 200 of 260 patients (77%) indicated that they had had subsequent pneumothoraces. All patients were women, and the average age of the respondents was 45 years.
The pneumothorax questionnaire was sent to the 260 patients with at least one pneumothorax, and 193 patients (74%) responded (Table 1). In the majority (136 of
Discussion
The gross appearance of the lung in LAM reveals numerous cysts ranging from a few millimeters to centimeters in diameter, often with striking involvement of the pleural surface.3, 5 Pneumothorax can occur from either direct cyst rupture into the pleural space or indirectly through alveolar wall disruption, followed by leak of air into the lung interstitium, mediastinum, and pleural space. Pneumothorax will develop in most patients with LAM during the course of their illness, and most will
Conclusion
Pneumothorax is a common complication of LAM and often recurs prior to establishing a definitive diagnosis of LAM. Due to the morbidity and cost associated with multiple recurrences, we recommend early, definitive intervention, preferably at the time of the initial pneumothorax. Although pleurodesis may be associated with an increased risk of perioperative bleeding with lung transplantation, our data suggest that complications are manageable and do not preclude successful transplantation.
acknowledgment
We are grateful to Sue Byrnes, Meg Coutinho, and Nancy Reynolds, of the LAM Foundation for their assistance and support in the data collection and preparation of this article.
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Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).
Financial support was provided by the LAM Foundation.
The views presented in this article do not necessarily reflect those of the US Food and Drug Administration.