气管周围淋巴管畸形的治疗方法

Treatment of peritracheal lymphatic malformations

目的探讨儿童气管周围淋巴管畸形的治疗方法并总结经验。方法收集2009年10月至2019年10月南京医科大学附属儿童医院烧伤整形外科收治的气管周围淋巴管畸形27例患儿的相关资料。其中,男16例,女11例;中位年龄为8个月,年龄范围为4~14个月。所有患儿均接受磁共振成像检查,提示为T1像等或高信号,T2像高信号,脂肪抑制像呈高信号。根据气管周围淋巴管畸形的病变范围和临床表现将患儿分为3型:咽后壁型13例,咽侧壁型11例,完全弥漫型3例。所有患儿中,5例行单纯口服药物治疗,17例行单纯注射治疗,5例行注射+口服药物治疗。采用4级分级标准对治疗效果进行评价:Ⅰ级(差),瘤体缩小0~25%;Ⅱ级(中),瘤体缩小26%~50%;Ⅲ级(好),瘤体缩小51%~75%;Ⅳ级(优),瘤体缩小76%~100%。结果所有患儿呼吸困难的症状均明显缓解,控制了淋巴管畸形的增长;随访时间范围为1~10年,2例痊愈,25例尚在随访中。疗效Ⅰ级0例,Ⅱ级3例,Ⅲ级9例,Ⅳ级15例。Ⅲ级疗效以咽后壁型最佳,Ⅳ级疗效以咽侧壁型最佳。完全弥漫型瘤体体积减小最明显。19例出现发热,多见于接受平阳霉素注射者,体温多<38.5℃,经对症处理24 h内恢复正常。无过敏性休克、肺纤维化病例。结论早期发现淋巴管畸形对于改善患儿症状极为重要,若延迟治疗或治疗方法不正确,淋巴管畸形增大,会使患儿症状加重甚至危及生命。

Objective To explore the treatment of peritracheal lymphatic malformations in children.Methods From October 2009 to October 2019,the relevant clinical data were collected from 27 hospitalized children with peritracheal lymphatic malformations.There were 16 boys and 11 girls with an average age of 8(4-14)months.Magnetic resonance imaging revealed isointensity or hyperintensity on T1 images,hyperintensity on T2 images and hyperintensity on fat-suppressed images.According to disease extent and clinical manifestations,they were divided into three types of posterior pharyngeal wall(n=13),lateral pharyngeal wall(n=11)and complete diffuse(n=3).Treatments included oral medication alone(n=5),injection alone(n=17)injection plus oral medication(n=5).Therapeutic efficacy was evaluated by the 4-level grading criteria:grade I(poor),tumor shrinkage 0 to 25%;gradeⅡ(moderate),tumor shrinkage 26%to 50%;gradeⅢ(decent),tumor shrinkage 51%to 75%and gradeⅣ(excellent),tumor shrinkage 76%to 100%.Results The symptoms of dyspnea lessened obviously and the growth of lymphatic malformations was controlled.The follow-up period was 1 to 10 years.Two cases recovered while the remainders were still tracked.The grade of efficacy wasⅡ(n=3),Ⅲ(n=9)andⅣ(n=15).Posterior pharyngeal wall type achieved the best gradeⅢefficacy while lateral pharyngeal wall type offered the best gradeⅣefficacy.Complete diffuse type had the most significant reduction in tumor volume.Nineteen cases developed febrile reactions of<38.5℃.Common in those recipients of pingyangmycin injection,it normalized within 24h after symptomatic measures.There was no instance of anaphylactic shock or pulmonary fibrosis.Conclusions Early detection of lymphatic malformations is extremely important for improving the symptoms of children.If treatment is delayed or incorrect,lymphatic malformations worsen and it may aggravate the symptoms of children or even endanger life.