原发性淋巴水肿伴肺间质病变显微外科治疗13例  

Microsurgical treatment of primary lymphedema with interstitial lung disease:a report of 13 cases

作　　者：梁晨 夏松 常鲲 刘中 刘昕 安然 信建峰 孙宇光 沈文彬 LIANG Chen;XIA Song;CHANG Kun;LIU Zhong;LIU Xin;AN Ran;XIN Jianfeng;SUN Yuguang;SHEN Wenbin(Department of Lymphatic Surgery,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China)

机构地区：[1]首都医科大学附属北京世纪坛医院淋巴外科,北京100038

出　　处：《中华显微外科杂志》2024年第4期375-381,共7页Chinese Journal of Microsurgery

摘　　要：目的探讨原发性淋巴水肿伴肺间质病变患者的病例特点、诊断方法及胸导管和右淋巴导管成形术的疗效。方法回顾分析2018年1月-2022年12月,因原发性淋巴水肿于北京世纪坛医院淋巴外科就诊且胸部高分辨CT(HRCT)检查发现肺间质改变并行胸导管成形术或右淋巴导管成形术治疗的患者共13例,其中男7例,女6例,年龄7.3(0.7~30.0)岁。患者均以单纯淋巴水肿为首发表现,平均发病年龄1.0(0~11.0)岁;从患者出现淋巴水肿症状到检查发现肺间质病变,平均时长6.3(0.3~19.0)年。所有患者均行胸导管和右淋巴导管成形术以改善淋巴回流。9例(69.2%)术前行直接淋巴管造影(DLG),4例(44.4%)根据DLG的发现指导术中结扎异常返流支,1例(11.1%)发现乳糜经支气管纵隔干返流入肺内,除术中结扎返流支外,要求终生严格低脂饮食。术后通过门诊、电话等方式随访,评估淋巴水肿及肺淋巴回流改善程度。结果术后患者恢复良好。术后平均随访47.5(19.0~68.0)个月,2例(15.4%)水肿情况稳定,11例(84.6%)水肿较前缓解。10例(76.9%)完成术后HRCT复查,其中9例(90.0%)肺间质病变程度可见改善。结论胸导管出口梗阻导致淋巴回流障碍是原发性淋巴水肿患者肺间质病变的病因,DLG可明确病因和诊断,同时指导治疗,胸导管或右淋巴导管成形术可改善患者淋巴水肿及肺间质病变程度。ObjectiveTo investigate the characteristics and diagnostic methods for the patients with primary lymphedema and interstitial lung disease,as well as the efficacy of thoracic duct or right lymphatic duct plasty.MethodsA retrospective study was performed on 13 patients who were treated for primary lymphedema in the Department of Lymphatic Surgery,Beijing Shijitan Hospital,Capital Medical University,from January 2018 to December 2022.All patients were confirmed with interstitial lung disease by high-resolution CT(HRCT)and then underwent thoracic duct or right lymphatic duct plasty surgery.The 13 patients in this study were 7 males and 6 females aged 7.3(0.7-30.0)years old.Primary lymphedema was the first manifestation in all patients,with an average morbidity age at 1.0(0-11.0)years old.The average time from the onset of lymphedema to the confirmed interstitial lung disease was 6.3(0.3-19.0)years.All patients underwent thoracic duct or right lymphatic duct plasty for improvement of the lymphatic flow.In addition,9 cases(69.2%)received a direct lymphangiography(DLG).In which,4 cases(44.4%)received further treatment of ligation of the reflux branches,and 1(11.1%)had the treatment plans changed,because a chylous reflux into the lung through a bronchomediastinal trunk was found from DLG,therefore a ligation of the abnormal reflux branches was performed and a life-long strict low-fat diet was required.Postoperative follow-ups for evaluation of the improvements of lymphedema and pulmonary lymphatic flow were carried out by visit of outpatient clinic or via telephone interviews.ResultsAll patients recovered well after surgery with an average follow-up time of 47.5(19.0-68.0)months.Lymphedema was stable in 2 cases(15.4%)and relieved in 11 cases(84.6%).Ten cases(76.9%)had completed the postoperative HRCT reviews and 9(90.0%)were found with significant improvement in the interstitial lung disease.ConclusionThe interstitial lung disease found during the examination of patients with primary lymphedema can be explained with a ly

关 键 词：原发性淋巴水肿 肺间质病变 肺淋巴回流障碍 直接淋巴管造影 淋巴导管成形术 

分 类 号：R654.7[医药卫生—外科学]