肺移植治疗肺淋巴管肌瘤病9例临床分析

Lung transplantation for pulmonary lymphangioleiomyomatosis: a report of 9 cases

目的探讨肺移植治疗肺淋巴管肌瘤病的手术适应证、围术期的处理及疗效。方法回顾性分析2013年6月至2016年12月9例在南京医科大学附属无锡市人民医院胸外科接受肺移植治疗的肺淋巴管肌瘤病患者的临床资料。9例均为女性,年龄23~52岁,术前均诊断为肺淋巴管肌瘤病。移植前胸部CT检查均提示双肺多发大小不等薄壁囊状影。9例患者均行续贯式双肺移植。术后病理提示:异常增生的平滑肌样细胞围绕支气管、血管、淋巴管弥漫性生长,使肺泡间隔增宽,符合淋巴管肌瘤病病理改变。观察手术前、后患者氧合指数、1秒用力呼气量(FEV1)以及一氧化碳弥散量(DLCO)%预计值指标变化。采用配对t检验比较上述指标,P<0.05为差异有统计学意义。结果9例患者肺移植手术后氧合指数由(210±55)mmHg(1mmHg=0.133kPa)上升至(400±80)mmHg,FEV1由(1.0±0.2)L上升至(2.8±0.4)L,DLCO%预计值由(27.5±2.5)%上升至(75.0±10.0)%,3项指标手术前、后差异均有统计学意义(P<0.05)。8例康复出院,随访至收集资料时,心肺功能良好,长期生活质量佳。1例术后出现感染伴急性排异反应,术后13天死亡。结论肺移植是治疗终末期肺淋巴管肌瘤病的唯一有效手段,双肺移植术是首选手术方式。

Objective To investigate the selection of recipients, operative technique, and perioperative management of lung transplantation. Methods From June 2013 to September 2016, 9 patients with pulmonary lymphangioleiomyomatosis underwent lung transplantation at Affiliated Wuxi People' s Hospital. There were 9 females aged from 23 to 52 years. Chest enhancement of CT in 9 patients prior to transplantation suggests multiple different sized thin-walled cystic shadows in whole lung. Nine patients received bilateral sequence lung transplantation(BSLT). The pathological findings after the operation indicated that cells of the abnormal proliferation of smooth muscle diffused around the bronchi, vessels and lymphatics, and widened the alveolar septum, which was consistent with the pathological changes in the patients with PLAM. We analyzed the differences of oxygenation index, forcedexpiratory volume at 1 sec(FEV1), and DLCO% pred. Using paired t test to compare these indicators, P<0.05 was considered statistically significant. Results The oxygenation index was increased from(210±55)mmHg(1 mmHg=0.133 kPa) to(400±80)mmHg after treatment, the difference was statistically significant(P< 0.05). The FEV1 was increased from(1.0±0.2)L to(2.8±0.4)L, and the difference was statistically significant(P<0.05). The DLCO% pred was increased from(27.5±2.5)% to(75.0±10.0)%, and the difference was statistically significant(P<0.05). Finally, 8 patients were discharged from the hospital, and the pulmonary function was good. 1 patients died of infection and acute rejection on the 13 day after operation. Conclusion Lung transplantation is effective in the treatment of chronic thromboembolic pulmonary hypertension, and long-term survival requires further investigations. Lung transplantation is the only effective means for the treatment of end-stage pulmonary lymphangioleiomyomatosis. Bilateral sequence lung transplantation is the first choice.