分阶段手术治疗外阴获得性淋巴管扩张症10例临床分析  

Staged operations of acquired lymphangiectasia of the vulva: 10 cases clinical analysis

作　　者：梁晨 夏松 孙宇光 常鲲 信建峰 刘昕 安然 沈文彬 Liang Chen;Xia Song;Sun Yuguang;Chang Kun;Xin Jianfeng;Liu Xin;An Ran;Shen Wenbin(Department of Lymph Surgery,Capital Medical University Affiliated Beijing Shijitan Hospital,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：《中华妇产科杂志》2024年第10期794-801,共8页Chinese Journal of Obstetrics and Gynecology

摘　　要：目的探讨外阴获得性淋巴管扩张症(ALV)的临床特点、诊断、手术方式及疗效。方法收集2009年7月—2023年7月于首都医科大学附属北京世纪坛医院因ALV住院并完成分阶段手术[指阴唇部分切除重建术+胸导管成形术和(或)会阴淋巴管-静脉吻合术]治疗的患者, 回顾性分析其临床病理资料及随访资料。术后通过门诊问卷方式采用本课题组自制的外阴淋巴管扩张症症状评分量表(以下简称症状评分量表)评估患者手术前、后会阴肿胀程度、水疱范围、漏液量及漏液频率的改善程度。结果 2009年7月—2023年7月因ALV住院治疗的患者共48例, 其中盆腔恶性肿瘤术后患者占98%(47/48), 有放疗史患者占94%(45/48)。48例ALV患者中, 完成分阶段手术治疗的ALV患者共10例, 纳入本研究。(1)临床特点及诊断:10例ALV患者的中位年龄60岁(50, 63岁), 自子宫颈癌手术及放化疗结束至出现外阴肿胀的中位间隔时间为1.5年(0.0, 2.0年), 自外阴肿胀至出现水疱及水疱破溃漏液的中位间隔时间为0.0年(0.0, 4.8年)。10例完成分阶段手术治疗的ALV患者中, 术前7例(7/10)患者有反复丹毒发作病史;7例(7/10)患者的症状评分量表中各症状评分均为最高(即会阴肿胀严重、水疱分布广泛、持续漏液且漏液量多);10例(10/10)患者的非增强磁共振淋巴显像(NCMRL)检查均表现为会阴区水肿信号, 淋巴显像(LS)检查均可见会阴区显像剂浓聚。(2)手术治疗及术后病理检查:10例完成分阶段手术治疗的ALV患者中, 6例(6/10)诊断胸导管出口梗阻, 行胸导管成形术及阴唇部分切除重建术;4例(4/10)未发现胸导管出口梗阻者分别行会阴淋巴管-静脉吻合术及阴唇部分切除重建术。10例(10/10)患者术后常规病理检查均可见真皮乳头内淋巴管扩张;其中5例患者行免疫组化检测, 淋巴管内皮细胞特异性抗体D2-40均为阳性, CD34均为阴性。(3)疗效:共有8例患者完Objective:To investigate the characteristics,diagnosis and therapeutic effect of acquired lymphangiectasia of the vulva(ALV).Methods:A retrospective analysis of clinicopathological and follow-up data was conducted on the patients treated in Capital Medical University Affiliated Beijing Shijitan Hospital due to female ALV from July 2009 to July 2023.The patients who completed the staged operations[partial labiectomy and reconstruction+thoracic ductplasty and(or)perineal lymphovenous anastomosis]were included in the study and followed up.The improvement of perineal swelling,blister range,fluid leakage volume and frequency were evaluated through outpatient visits by the symptom rating scale of ALV(hereinafter referred to as the symptom rating scale)before and after surgery.Results:A total of 48 patients were treated due to ALV from July 2009 to July 2023,of which 98%(47/48)were postoperative pelvic malignant tumors and 94%(45/48)had a history of radiotherapy.A total of 10 patients with ALV who completed the staged operations were included in this study.(1)Clinical characteristics and diagnosis:10 patients had a median age of 60 years old(50,63 years old).The median duration from cervical cancer surgery and radiation therapy to vulvar swelling was 1.5 years(0.0,2.0 years),and the median duration from vulvar swelling to blister formation and leakage was 0.0 years(0.0,4.8 years).Seven patients(7/10)had a history of recurrent erysipelas;7 patients(7/10)had the most severe symptom(widespread blisters,persistent fluid leakage,and large amount of fluid leakage);noncontrast magnetic resonance lymphography(NCMRL)showed edema signals in the perineal region of all the patients,and increase of agent in the perineal region was observed in lymphoscintigram(LS).(2)Surgical treatment and postoperative pathological examination:of the 10 ALV patients who completed staged surgical treatment,6 cases(6/10)were diagnosed with thoracic duct outlet obstruction and underwent thoracic ductplasty and partial labiectomy and reconstruction.Peri

关 键 词：淋巴管扩张 外阴疾病 水疱 胸导管 会阴 吻合术 外科 修复外科手术 

分 类 号：R654.7[医药卫生—外科学]