The clinical study of tougne volume reduction for macroglossia patient with Beckwith-Wiedemann syndrome
10.3760/cma.j.cn114453-20200323-00177
- VernacularTitle:舌体减容术治疗Beckwith-Wiedemann综合征巨舌的临床研究
- Author:
Jun LI
1
;
Chenbin DONG
;
Xiangqi LIU
;
Xiuya YE
;
Cheng FANG
Author Information
1. 复旦大学附属儿科医院整形外科，上海 201102
- Keywords:
Beckwith-Wiedemann syndrome;
Macroglossia;
Surgery
- From:
Chinese Journal of Plastic Surgery
2021;37(5):528-533
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the surgery effect of tongue volume reduction for macroglossia patients with Beckwith-Wiedemann syndrome (BWS).Methods:The clinical data of children diagnosed as BWS based on clinic symptoms and gene tests, who received operation in Department of Plastic Surgery, Children’s Hospital of Fudan University from July 2012 to December 2019 were analyzed retrospectively. All the patients underwent dorsal tongue bell-shaped resection and ventral V-shaped resection. By pulling tongue tip, the tongue body was cut and dissected along the marking line followed by suturing the muscle layer of the central part of the tongue tissue. Intermittent mattress suture of the dorsal and ventral mucous wounds of the tongue. After operation, all patients kept the trachea intubation and were transferred to PICU for further treatment and received early nasal feeding. The short-term postoperative complications, and long-term follow-up of the tongue body shape and clinical symptoms were recorded.The clinical effect was evaluated by onesurgeon and the parents.The effect was graded into four levels: dissatisifaction, average, satisifaction and great satisifaction.Results:A total of 61 children were included in this study, including 30 males and 31 females, with an average age of 8.5 months. The length of the tongue exposed outside at the resting state was 1.34-2.62 cm, with a median of 1.87 cm. Symmetric hypertrophy was found in 52 cases (85.2%), lateral hypertrophy in 9 cases (14.8%). There were 3 cases with respiratory obstruction (4.9%), 35 cases with salivation (57.4%), 4 cases suffering difficulty of feeding and dysphagia (6.6%). All children underwent the operation successfully, and the average operation time was 35.8 min. Among the short-term complications, there were 3 ventral wounds dehiscence, none of which needed sutured again. By prolonging nasal feeding time, the wounds were all healed in the secondary stage. Respiratory obstruction occurred in 1 patient after operation, and the symptoms were relieved after tongue retraction. The mean follow-up time was 3.3 years (2 months to 7 years), and 1 patient was inadequately corrected and good condition was got after second aryoperation. The remaining children obtained satisfactory resting tongue position and appearance, and the postoperative clinical symptoms were improved. In the long-term follow-up, the comprehensive evaluation results of the resting tongue position and clinical symptoms of the children by surgeon and parents were satisfied in 10 cases (16.4%), very satisfied in 50 cases (82.0%), and average in 1 case (insufficient correction after the first operation).Conclusions:Central resection and volume reduction of tongue body is a simple, effective and safe surgical technique in children with giant tongue diagnosed as BWS.
