Almoosa Khalid F, Ryu Jay H, Mendez Jose, Huggins J Terrill, Young Lisa R, Sullivan Eugene J, Maurer Janet, McCormack Francis X, Sahn Steven A
Division of Pulmonary and Critical Care, University of Cincinnati, Cincinnati, OH 45267-0564, USA.
Chest. 2006 May;129(5):1274-81. doi: 10.1378/chest.129.5.1274.
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.
The LAM Foundation database of registered patients.
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.
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.
None.
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.
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.
气胸是淋巴管平滑肌瘤病（LAM）的常见并发症，其最佳治疗和预防方法尚不清楚。通常需要进行化学或手术胸膜固定术以预防复发。然而，它们在LAM中的疗效尚不清楚，并且它们是否会导致肺移植围手术期并发症也不确定。
LAM基金会注册患者数据库。
向所有至少发生过一次气胸的注册患者发送问卷，以确定复发率、复发模式及干预措施的疗效。向接受肺移植的注册LAM患者发送第二份问卷。
在395名注册患者中，260名患者（66%）报告在其一生中至少发生过一次气胸，其中193名患者（74%）完成了问卷。在单独收到问卷的85名肺移植患者中，80名患者（94%）进行了回复。
无。
在193名气胸问卷回复者中，收集到676次气胸发作的数据。82%（193名患者中的158名）在诊断LAM之前首次发生气胸。140名患者（73%）至少又发生了一次气胸，要么是同侧复发（140名患者中的99名，71%），要么是对侧气胸（140名患者中的104名，74%）。保守治疗后的复发率为66%，化学胸膜固定术后为27%，手术后为32%。在接受肺移植的患者中，80名患者中有45名（56%）曾接受过化学或手术胸膜固定术。80名患者中有14名（18%）报告了与胸膜相关的术后出血，其中13名（93%）曾接受过胸膜固定术。
化学胸膜固定术或手术在预防LAM患者气胸复发方面同样有效，且优于保守治疗。由于复发率高，对于这些患者的首次气胸应考虑采用这两种方法中的任何一种。然而，两者都会导致肺移植后围手术期出血增加，对住院时间没有影响。
