Tongue reduction in Beckwith-Wiedemann syndrome: outcome and treatment algorithm

作     者：Naujokat, H. Moeller, B. Terheyden, H. Birkenfeld, F. Caliebe, D. Krause, M. F. Fischer-Brandies, H. Wiltfang, J. 

作者机构：Univ Hosp Schleswig Holstein Dept Oral & Maxillofacial Surg Campus KielArnold Heller Str 3 D-24105 Kiel Germany Univ Hosp Schleswig Holstein Dept Anesthesiol & Intens Care Med Campus Kiel Kiel Germany Univ Hosp Schleswig Holstein Dept Pediat Campus Kiel Kiel Germany Univ Hosp Schleswig Holstein Dept Orthodont Campus Kiel Kiel Germany 

出 版 物：《INTERNATIONAL JOURNAL OF ORAL AND MAXILLOFACIAL SURGERY》 (国际口腔外科学和上颌面外科学杂志)

年 卷 期：2019年第48卷第1期

页      面：9-16页

核心收录：

学科分类：1003[医学-口腔医学] 1002[医学-临床医学] 100212[医学-眼科学] 10[医学] 

主　　题：Beckwith-Wiedemann syndrome macroglossia tongue reduction treatment algorithm 

摘      要：Beckwith-Wiedemann syndrome is a rare congenital overgrowth disorder with macroglossia being one of the cardinal symptoms. In pronounced cases, macroglossia can lead to airway obstruction, musculoskeletal alterations and functional deficits. Surgical tongue reduction is performed at varying ages and with different techniques. This study evaluated perioperative complications, as well as long-term aesthetic and functional outcomes, in a large cohort. A total of 68 patients, treated either surgically or conservatively, were included. Depending on the severity of macroglossia, patients were divided into three groups to determine the treatment algorithm. Complications after surgical tongue reduction were prolonged intubation and revision due to dehiscence or haematoma. In the long term, no patient suffered from impaired sense of taste or paresthesia, although the shape of the tongue was disproportional in 85%. With the present treatment algorithm, operative tongue reduction exerts a positive influence on skeletal, dentoalveolar and functional development with sufficient long-term outcome and high grade of satisfaction of the patients. Supportive therapy in an interdisciplinary centre is of fundamental importance for both surgical and conservative treatment.