肺淋巴管肌瘤病合并气胸7例报告

【摘要】目的 总结肺淋巴管肌瘤病(pulmonary, lymphangioleiomyomatosis,PLAM) 合并气胸患者的临床特点和外科治疗经验，提高对PLAM的认识，进一步指导临床。方法 搜集2005年至2021年复旦大学附属中山医院胸外科收治PLAM合并气胸并行外科治疗7例患者的临床资料，进行回顾性分析和文献复习。结果 7例患者均为女性；年龄分布26~68岁；所有患者均以气胸为首发症状，4例为单侧气胸，3例为双侧气胸。7例患者通过胸部高分辨CT临床诊断为PLAM合并气胸，其中5例患者病理诊断为肺淋巴管肌瘤病；5例行电视胸腔镜下肺楔形切除+胸膜固定术，2例行胸腔闭式引流术，7例患者均症状缓解后出院。其中5例PLAM患者合并肾血管平滑肌脂肪瘤（angiomyolipoma, AML）。结论 PLAM发病率极低，主要发生于育龄期女性，临床上可以气胸首发；胸腔镜手术和胸腔闭式引流术是PLAM合并气胸患者的重要治疗方法。

Pulmonary lymphangioleiomyomatosis complicated with pneumothorax: 7 cases report

[Abstract] Objective To summarize the clinical characteristics and surgical treatment experience of patients with pulmonary lymphangiomyomatosis (PLAM) combined with pneumothorax, so as to improve the understanding of PLAM.Methods The clinical data of 7 patients with PLAM combined with pneumothorax admitted to the Thoracic surgery department of Zhongshan Hospital affiliated to Fudan University from 2005 to 2021 were collected and retrospectively analyzed and reviewed.Results All the 7 patients were female.The age distribution was 26~60 years old.Pneumothorax was the first symptom in 7 patients, unilateral pneumothorax in 4 patients and bilateral pneumothorax in 3 patients.7 patients were clinically diagnosed by chest high resolution CT: pneumothorax, pulmonary lymphangiomyomatosis; 5 cases were pathologically diagnosed as pulmonary lymphangiomyomatosis.Five patients underwent video-assisted thoracoscopic pulmonary wedge resection plus pleurodesis, two underwent bilateral closed thoracic drainage.Postoperative closed thoracic drainage was performed due to repeated pneumothorax attacks. All 7 patients were discharged after their symptoms were relieved.CT diagnosis of pulmonary LAM complicated with renal angiomyolipoma (AML) in 5 patients.Conclusion The incidence of PLAM is extremely low, mainly occurring in women of childbearing age. Pneumothorax can be the first occurrence in clinical practice and can be combined with renal AML. Thoracoscopic surgery and closed thoracic drainage are important treatment methods.