2种病变内平阳霉素注射方法治疗68例儿童唇面部微囊型淋巴管畸形疗效分析

目的: 总结儿童唇面部微囊型淋巴管畸形（lymphatic malformation,LM）2种病变内平阳霉素注射方法治疗的远期疗效和安全性。方法: 回顾分析1990年1月—2013年1月临沂市肿瘤医院收治的68例儿童唇面部弥漫性微囊型LM病例。32例患儿（A组）采用同期、全层病变内平阳霉素注射治疗,即经黏膜、黏膜下层病变内注射平阳霉素并同期行唇面部全层病变内平阳霉素注射治疗;36例患儿（B组）行分期、分层次病变内平阳霉素注射治疗,即先期行经黏膜、黏膜下层病变内平阳霉素注射治疗,黏膜面病变基本消失后再行经皮、面颊部浅层病变内平阳霉素注射治疗。疗效评价采用Achauer 4级标准。记录疗程,平阳霉素总剂量,观察远期局部凹陷萎缩并发症。采用SPSS 18.0软件包对数据进行统计学分析。结果: 随访5~25年,A组病灶评价疗效Ⅰ级0例,Ⅱ级0例,Ⅲ级4例,Ⅳ级28例;B组病灶评价疗效Ⅰ级0例,Ⅱ级0例,Ⅲ级5例,Ⅳ级31例。2组Ⅳ级疗效比较无统计学差异（P>0.05）。A组疗程（5.9±1.4）次,B组疗程（6.4±1.3）次,2组比较无统计学差异（P>0.05）。A组注射平阳霉素总剂量（44.1±8.5）mg,B组注射平阳霉素总剂量（48.2±10.6）mg,2组比较无统计学差异（P>0.05）。A组20例（62.5%）出现局部凹陷萎缩,B组1例（2.8%）出现萎缩凹陷,2组差异有显著性（P<0.05）。结论: 采用分期、分层病变内平阳霉素注射治疗,与同期、全层病变内平阳霉素注射治疗儿童唇面部微囊型淋巴管畸形相比,其疗效、疗程和平阳霉素总剂量相当,但远期局部组织萎缩凹陷并发症发生率显著降低,治疗后面部外形好,患者满意度高。

Comparison of two methods of intralesional injection of pingyangmycin for the treatment of microcystic lymphatic malformations in children's lip,face

PURPOSE: To summarize the long-term clinical experience of two methods of intralesional injection of pingyangmycin for the treatment of microcystic lymphatic malformation (LM) in children's lip and face. METHODS: From January 1990 to January 2013, a total of 68 children with diffuse microcystic LM were admitted and treated. All patients received intralesional injection of pingyangmycin, and a total of 68 cases were followed up to the age of 18 years. Among 68 patients, 37 were male and 31 were female. The age at consultation ranged from 10 months to 11 years, and the median age was 3.5 years. From January 1990 to December 1998, 32 children (group A) were treated with full-layer intralesional injection of pingyangmycin at one time. From January 1999 to January 2013, 36 children (group B) were treated with in-layers intralesional injection of pingyangmycin at different times, first mucosal, and submucosa injection followed by percutaneous injection of face and lip after mucosal lesions disappeared. The therapeutic effect was evaluated according to a 4-grade system. The course of treatment, total dose of pingyangmycin were recorded, and long-term local complications were observed. SPSS 18.0 software package was used for statistical analysis. RESULTS: The patients were followed up for 5 to 25 years, there were 4 cases of grade Ⅲ and 28 cases of grade Ⅳ in group A, and 5 cases of grade Ⅲ and 31 cases of grade Ⅳ in group B. By Fisher's exact test, there was no significant difference in the efficacy rate of grade Ⅳ between group A and group B (P>0.05). The treatment course of group A was(5.9±1.4) times, and that of group B was (6.4±1.3) times. The total dose of pingyangmycin was (44.1±8.5) mg in group A and (48.2±10.6) mg in group B, respectively. In group A, 20 cases (62.5%) showed local atrophy and depression, and in group B, 1 case (2.8%) showed atrophy and depression. CONCLUSIONS: Compared with full-layer intralesional injection of pingyangmycin at one time in the treatment of microcystic lymphatic malformation, the incidence of complications of local atrophy and depression in the long term was significantly reduced when using in-layer injection at different times. After treatment, the facial appearance was good and the patients’ satisfaction was high.