International Journal of Oral and Maxillofacial Surgery
Clinical PaperCongenital Craniofacial DeformitiesSurgical treatment of macroglossia in patients with Beckwith–Wiedemann syndrome: a 20-year experience and review of the literature
Section snippets
Materials and methods
A retrospective chart review was conducted including all patients with BWS who underwent surgical tongue reduction between 1990 and 2010 at the Academic Medical Center (AMC) in Amsterdam, The Netherlands. Inclusion criteria were a diagnosis of BWS, surgical treatment of macroglossia, and minimum follow-up of 1 year. The medical record of each patient was retrieved from the archive and studied in detail. For all surgical procedures performed, the following parameters were evaluated: gender;
Results
From 1990 to 2010, 54 BWS patients presented at the authors’ craniofacial outpatient clinic with medical problems related to macroglossia. Conservative management of macroglossia was indicated in 31 patients with mild symptoms of macroglossia. Surgical treatment was indicated and performed in 23 patients with a yearly average varying from 0 to 3 subjects. In all cases, the senior author (C.M.A.M. vdH) performed the tongue reduction surgery using the anterior, V-shaped, wedge resection (AWR)
Discussion
In BWS, macroglossia is typically present at birth and may require surgical intervention in a small percentage of patients. To the best of the authors’ knowledge, this study is unique in describing the largest series of BWS patients that were surgically treated for macroglossia. 23 patients with BWS who underwent surgical tongue reduction at the authors’ institution are discussed. All procedures were performed by the same surgeon, using the same AWR technique. The literature pertinent to BWS
Funding
None.
Competing interests
None.
Ethical approval
Not required.
Acknowledgement
The authors would like to thank S.A. Steenen for his illustrative support.
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