Chest
Volume 129, Issue 5, May 2006, Pages 1274-1281
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Original Research: Lymphangioleiomyomatosis
Management of Pneumothorax in Lymphangioleiomyomatosis: Effects on Recurrence and Lung Transplantation Complications

https://doi.org/10.1378/chest.129.5.1274Get rights and content

Study objectives

Pneumothorax is a common complication of lymphangioleiomyomatosis (LAM), and the optimal approach to its treatment and prevention is unknown. Chemical or surgical pleurodesis are often required to prevent recurrence. However, their efficacy in LAM is unclear, and whether they contribute to perioperative complications during lung transplantation is uncertain.

Setting

The LAM Foundation database of registered patients.

Design

A questionnaire was sent to all registered patients who had at least one pneumothorax to determine rates and patterns of recurrence and efficacy of interventions. A second questionnaire was sent to registered LAM patients who received a lung transplant.

Patients or participants

Of 395 registered patients, 260 patients (66%) reported at least one pneumothorax during their lifetime, 193 of whom (74%) completed the questionnaire. Of the 85 lung transplant patients who were sent a separate questionnaire, 80 patients (94%) responded.

Interventions

None.

Measurements and results

Of the 193 respondents to the pneumothorax questionnaire, data on 676 episodes of pneumothorax were collected. Eighty-two percent (158 of 193 patients) had their first pneumothorax prior to a diagnosis of LAM. One hundred forty patients (73%) had at least one additional pneumothorax, either an ipsilateral recurrence (99 of 140 patients, 71%) or a contralateral pneumothorax (104 of 140 patients, 74%). Recurrence rates were 66% after conservative therapy, 27% after chemical pleurodesis, and 32% after surgery. In patients who had undergone lung transplantation, prior chemical or surgical pleurodesis was performed in 45 of 80 patients (56%). Fourteen of 80 patients (18%) reported pleural-related postoperative bleeding, 13 of whom (93%) had prior pleurodesis.

Conclusions

Chemical pleurodesis or surgery are equally effective and better than conservative therapy in preventing recurrence of pneumothorax in LAM. Due to the high recurrence rate, either procedure should be considered for the initial pneumothorax in these patients. However, both contribute to increased perioperative bleeding following lung transplantation, with no effect on length of hospital stay.

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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  • Cited by (0)

    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.

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