复杂性淋巴管畸形临床诊断分析及综合治疗  

Analysis of clinical diagnosis and treatment of complex lymphatic anomalies

作　　者：邱桐 周江元 龚雪 杨开颖 张梓欣 兰钰茹 张学鹏 周子龙 张赓 付建垒 陈思源[2] 吉毅[1] Qiu Tong;Zhou Jiangyuan;Gong Xue;Yang Kaiying;Zhang Zixin;Lan Yuru;Zhang Xuepeng;Zhou Zilong;Zhang Geng;Fu Jianlei;Chen Siyuan;Ji Yi(Department of Pediatric Surgery,West China Hospital of Sichuan University,Chengdu 610041,China;Department of Critical Care Medicine,West China Hospital of Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院小儿外科,成都610041 [2]四川大学华西医院重症医学科,成都610041

出　　处：《中华整形外科杂志》2023年第11期1167-1174,共8页Chinese Journal of Plastic Surgery

基　　金：国家自然科学基金(82273556);四川省科技厅重点研发项目(2022YFS0233,2022YFS0225,2022NSFSC1480);四川大学"从0到1"创新项目(2022SCUH0033);四川大学医学+信息中心交叉学科建设开放项目(YGJC004);四川大学华西医院学科卓越发展1·3·5工程临床研究孵化项目(2023HXFH004,2020HXFH048,2019HXFH056);四川大学华西医院学科卓越发展1·3·5工程交叉学科创新项目(ZYJC21060)。

摘　　要：目的分析复杂性淋巴管畸形的临床特点、鉴别诊断、治疗预后。方法回顾性分析2010年4月至2022年4月于四川大学华西医院脉管疾病团队多学科门诊就诊并进行随访的复杂性淋巴管畸形患者临床资料。所有患者均经过小儿外科、放射科、烧伤整形科、病理科、康复科等学科专家会诊后诊断为复杂性淋巴管畸形,对患者的临床表现、血常规、凝血功能、MRI影像学检查及治疗方法进行分析,并根据随访及疾病转归将患者分为好转、稳定、进展和死亡。结果纳入18例患者,男6例,女12例,初诊年龄1个月至29岁,中位年龄2.5岁。患者随访或治疗0.4~12.0年,平均3.5年,10例患者出现胸腹腔或心包积液;15例患者累及内脏,影像学检查提示内脏的累及呈多灶样改变;9例患者伴有骨质破坏,Gorham-Stout病患者的骨破坏突破骨皮质;全身性淋巴管异常累及骨髓腔内未突破骨皮质;14例患者出现不同程度的凝血功能异常,其中8例严重凝血功能障碍的患者均为卡波西样淋巴管瘤病。18例随访患者中,1例卡波西样淋巴管瘤病患者死亡,6例患者病情进展,8例患者病情稳定,3例患者病情好转。结论复杂性淋巴管畸形的临床特点呈全身性、多样性和复杂性。弥漫性淋巴管畸形伴有骨骼、内脏多器官受累,胸腹腔积液以及凝血功能障碍的临床症状应考虑此类疾病,但由于各亚型临床特点重叠,鉴别困难,疗效和预后较差,精准靶向药物是未来此类疾病的治疗研究方向。ObjectiveTo analyze the clinical features,differential diagnosis,treatment and prognosis of complex lymphatic malformations.MethodsThe clinical data of patients with complex lymphatic malformation were retrospectively analyzed from April 2010 to April 2022 in the Multidisciplinary Outpatient Department of the Vascular Disease Team of West China Hospital,Sichuan University.All patients were diagnosed with complex lymphatic malformation after consultation with multidisciplinary experts in pediatric surgery,radiology,plastic surgery,pathology,rehabilitation and other departments.The clinical manifestations,blood routine,coagulation function,magnetic resonance imaging and treatment methods of the patients were analyzed.According to the follow-up and disease results,the patients were divided into improvement,stability,progress and death.ResultsA total of 18 patients with complex lymphatic malformations were included in the study,including 6 males and 12 females.The age of first diagnosis ranged from 1 month to 29 years old,and the median age was 2.5 years old.Patients were followed up and treated for 0.4 to 12.0 years,with an average follow-up of 3.5 years.Ten patients had pleural and pericardial effusion;15 patients had visceral involvement which showed multifocal changes in imaging examinations;9 cases were accompanied by bone destruction,which in Gorham-Stout disease patients broke through the cortex while in generalized lymphatic anomalies it did not;14 patients had various degrees of coagulation abnormalities,of which 8 patients with severe coagulation dysfunction were all diagnosed as kaposiform lymphangiomatosis.Of the 18 patients,one kaposiform lymphangiomatosis patient died;six patients progressed;eight patients were stable;and three patients improved.ConclusionThe clinical characteristics of patients with complex lymphatic malformations are systemic,diverse and complex.The clinical symptoms of patients with diffuse lymphatic malformation accompanied by involvement of bone and multiple internal organs,chest a

关 键 词：淋巴管畸形 临床特点 鉴别诊断 治疗 疾病预后 

分 类 号：R726.5[医药卫生—儿科]