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一例通过电视辅助胸腔镜手术诊断的肺淋巴管平滑肌瘤病早期病例。

An early case of pulmonary lymphangioleiomyomatosis diagnosed by video-assisted thoracoscopic surgery.

作者信息

Yamazaki Akio, Miyamoto Hideaki, Futagawa Toshiro, Oh Wang, Sonobe Satoshi, Takahashi Nobumasa, Izumi Hiroshi, Hirama Michiyoshi, Seyama Kuniaki, Fukuchi Yoshinosuke

机构信息

Department of General Thoracic Surgery, Juntendo University School of Medicine, Tokyo, Japan.

出版信息

Ann Thorac Cardiovasc Surg. 2005 Dec;11(6):405-7.

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease of young women that usually causes death from respiratory failure. Here we report an early case that was diagnosed by thoracoscopy. A 28-year-old woman presented to a local clinic with chest pain and her chest X-ray film showed left pneumothorax. After placement of a chest tube, the lung re-expanded fully. Following the recurrence of left pneumothorax, she was referred to our hospital and underwent video-assisted thoracoscopic surgery (VATS). However, left pneumothorax recurred again one month later. She underwent a second thoracoscopic operation, during which a bulla was recognized in the lingular segment of the left lung and was resected. On histological examination of the surgical specimen findings consistent with LAM were obtained. Taking the preoperative imaging findings and the thoracoscopic findings into consideration, a diagnosis of early lymphangioleiomyomatosis was made. Thoracoscopic management of pneumothorax in young women can facilitate the early diagnosis of this condition.

摘要

肺淋巴管平滑肌瘤病(LAM)是一种罕见的、进行性的年轻女性疾病,通常导致呼吸衰竭死亡。在此我们报告一例通过胸腔镜诊断的早期病例。一名28岁女性因胸痛就诊于当地诊所,胸部X线片显示左侧气胸。放置胸腔引流管后,肺完全复张。左侧气胸复发后,她被转诊至我院并接受了电视辅助胸腔镜手术(VATS)。然而,一个月后左侧气胸再次复发。她接受了第二次胸腔镜手术,术中在左肺舌段发现一个肺大疱并将其切除。对手术标本进行组织学检查,获得了与LAM一致的结果。综合术前影像学检查结果和胸腔镜检查结果,做出了早期淋巴管平滑肌瘤病的诊断。胸腔镜治疗年轻女性气胸有助于该病的早期诊断。

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