舌缩小矫形联合射频消融术在Beckwith-Wiedemann综合征巨舌治疗中的效果观察

Effect of macroglossia reduction combined with radiofrequency ablation in the treatment of Beckwith-Wiedemann syndrome with giant tongue

目的总结Beckwith-Wiedemann综合征(BWS)巨舌缩小矫形联合舌体射频消融术的手术效果。方法回顾性分析2019年5月至2022年7月在厦门市儿童医院耳鼻咽喉头颈外科接受治疗的BWS巨舌表现患儿的临床资料,巨舌患儿均同期或分期行舌前段V形切口块状切除+舌体侧方及舌根部针式刀头低温等离子射频消融术。术后随访观察患儿并发症发生情况、舌体形态、舌体切口恢复、舌体功能,并在第3、12个月行舌体MRI检查,观察舌体消融隧道恢复情况及舌体积,舌体是否可回纳口内,进行疗效判断(治愈和未愈)。结果共纳入70例患儿,男31例,女39例,平均年龄15.4个月(5个月~4岁)。其中68例患儿同期行巨舌缩小矫形术+舌体射频消融术,2例在外院先行巨舌缩小矫形术,术后1年舌体仍肥厚,隧于我科行舌体射频消融术。所有患儿术后平均随访18个月(6~36个月),围术期均未出现大出血、舌部血肿及舌运动障碍等并发症。术后舌尖伤口浅层裂开14例,鼻饲营养、禁经口进食5~7 d逐渐好转后恢复流质饮食,4~12周痊愈,舌尖形态恢复良好;术后舌体伤口缝线反应13例,未进行特殊处理,术后2~3周自行消退。所有患者术后舌体积均明显缩小,可完全回纳口腔,疗效判定为治愈。结论巨舌缩小矫形术联合舌体射频消融术治疗BWS舌体肥大,方法可行,效果满意,推荐应用。

Objective This study aims to summarize the surgical efficacy of macroglossia reduction combined with radiofrequency ablation of Beckwith-Wiedemann Syndrome(BWS).Methods A retrospective analysis was conducted on the clinical data of patients with macroglossia who received treatment at the Department of Otolaryngology-Head and Neck Surgery,Xiamen Children’s Hospital between May 2019 and July 2022.All patients with macroglossia underwent either one-stage or staged anterior tongue V-shaped incision massive resection,followed by needle tip low-temperature plasma radiofrequency ablation on the lateral and base of the tongue.The postoperative complications in pediatric patients were monitored,including the incision shape of the tongue body,restoration of tongue body structure and function.MRI scans of the tongue were performed at 3 and 12 months post-operation to evaluate tissue healing,changes in tongue volume,and whether there was successful retraction of the enlarged tongue into the oral cavity for evaluating treatment efficacy(cured or not cured).Results The study included 70 cases,comprising 31 male and 39 female patients,with an average age of 15.4 months(ranging from 5 months to 4 years old).Among them,68 cases underwent macroglossia reduction combined with radiofrequency ablation at the same time,and 2 cases underwent macroglossia reduction surgery first in other hospitals,and tongue body radiofrequency ablation was performed one year after the surgery.All children were followed up foran average follow-up time of 18 months(6 to 36 months).There were no serious complications such as massive hemorrhage and tongue hematoma.After the operation,14 cases of superficial rupture of tongue tip wound were gradually improved after 5-7 days of no oral eating,and recovered after 4-12 weeks of liquid diet.Thirteen cases had suture reactions in the tongue back,which were self-resolving within 2 to 3 weeks with no special treatment.All patients showed significant reduction in tongue size after the surgery,which could be completely inserted into the oral cavity,and the treatment effect was satisfactory.Conclusion Macroglossia reduction combined with radiofrequency ablation is a feasible and effective method for the treatment of BWS with macroglossia and is recommended for application.