小儿纵隔淋巴管畸形的外科治疗

Surgical treatment of mediastinal lymphatic malformations in children

目的探讨小儿纵隔淋巴管畸形的外科治疗方法及有效性。方法回顾性分析2012年2月至2017年2月在广州市妇女儿童医疗中心行外科手术治疗的25例纵隔淋巴管畸形的临床资料。其中男17例,女8例;年龄1个月至5岁(平均15个月)。病变位于右上纵隔8例(其中累及颈部4例),位于左上纵隔7例(其中累及颈部6例),位于后纵隔2例(其中1例累及对侧),位于前纵隔5例,累及纵隔2个区域以上者3例。病变包绕血管者17例,压迫气道者14例。胸腔镜手术切除4例;传统开胸切除21例,其中根据病变部位行颈部切口2例,颈胸联合切口8例,胸外侧切口8例,前胸壁正中切口3例(其中1例劈开部分胸骨)。病变创面常规用介入治疗药物平阳霉素浸泡18例。结果术中损伤无名静脉1例,损伤膈神经1例。术后病理检查结果均为淋巴管畸形,其中大囊型14例,微囊型4例,混合型7例。2例失随访,余23例随访时间5~56个月。复发2例,1例经再次手术治疗痊愈,另1例保守观察,病变无进行性增大;淋巴液漏6例,其中3例经穿刺引流痊愈,另3例保守观察,9~12个月时明显吸收;膈膨升9例,其中6例随访1~6个月恢复,另3例手术侧膈肌抬高1~2个肋间;胸壁塌陷1例。结论外科手术是治疗小儿纵隔淋巴管畸形的有效方法。

Objective To explore the surgical approaches and efficacies for mediastinal lymphatic malformations (MLM) in children. Methods The clinical data were retrospectively analyzed for 25 MLM children from February 1, 2012 to February 28, 2017. There were 17 boys and 8 girls with an average age of 15 months. The lesion sites were right upper mediastinum (4/8 involving neck region)(n=8), left upper mediastinum (6/7 involving neck region)(n=7), posterior mediastinum (n=2), anterior mediastinum (n=5) and two or more regions (n=3). There were vascular wrapping (n=17) and airway compressing (n=14). The procedures were video-assisted thoracoscopy (n=4) and conventional thoracotomy (n=21). And the latter included cervical incision (n=2), cervical thoracic incision (n=8), posterolateral thoracic incision (n=8) and median sternotomy (n=3). Besides surgery, 18 cases received drug therapy. Results Innominate vein (n=1) and phrenic nerve (n=1) were injured intraoperatively. All lymphatic malformations were confirmed by histological examinations. And the histological types were macrocystic (n=14), microcystic (n=4) and combined (n=7). Two cases were lost to follow-ups while the remainders were followed for 5-56 months. Recurrence occurred in 2 cases while another one required re-operation. And 3/6 cases of lymph leakage underwent puncture and drainage. Among 9 cases of diaphragmatic eventration, 3 were surgically repaired by lifting 1-2 intercostal spaces while the remainders self-restored within 1-6 months. There was 1 case of chest wall deformity. Conclusions Surgery is efficacious for mediastinal lymphatic malformations in children.