胸腔镜、纤维支气管镜诊断肺淋巴管肌瘤病9例体会

Clinical Analysis of 9 Cases of Pulmonary Lymphangioleiomyomatosis Diagnosed by Video-assisted Thoracospy and Fiberoptic Bronchoscopy

目的总结肺淋巴管肌瘤病早期诊断的经验,进一步提高对本病的认识。方法回顾分析9例经胸腔镜、纤维支气管镜(纤支镜)活检病理确诊的肺淋巴管肌瘤病患者的临床影像学表现、腔镜检查结果。结果9例皆为女性,平均年龄35岁(20～49岁)。胸部CT检查可见两肺弥漫多发小囊状透光区,其中3例行腹部CT检查见腹膜后多发淋巴结肿大。9例均为肺组织病理活检确诊,其中胸腔镜手术3例,纤支镜检查6例。术后病理诊断与术前诊断符合率为44.4%(4/9)。结论肺淋巴管肌瘤病临床罕见,CT检查可提示诊断,运用胸腔镜及纤支镜活检是早期确诊的重要手段。

Objective To review the experience of using endoscopic technique in early diagnosis of pulmonary lymphangioleiomyomatosis （PLAM）. Methods The clinical symptoms, chest roentgenographic presentations, bronchoscopic and thoracospic findings of 9 cases of PLAM were retrospectively analyzed. Results All patients were female, aged 20--49 years old （average 35.0）. Chest roentgengraphic presentations of 9 cases were typically featured by multiple small pulmonary cysts. Among them, multiple retroperitoneal lymph node enlargement was found in 3 cases by abdominal CT scan. All cases were confirmed by pathology. Six cases were subjected to fiberoptie bronchoscopy, and 3 cases to video-assisted thoracospy biopsy. The accurate rate of the pre-operative diagnosis was 44.4% （4/9）. Conclusion PLAM is a rare lesion. CT scan can achieve a definite diagnosis by showing the reticular changes with multiple small round areas. An early fiberoptic bronchoseopy and video-assisted thoraeospy biopsy is the most important step to get an accurate diagnosis.