This case presents a facially mature patient with Beckwith–Wiedemann Syndrome (BWS) who presented with severe class III malocclusion. Computed tomography imaging revealed an anterior crossbite of 19 mm and a narrow pharyngeal airway at the level of the tongue base precluding mandibular setback surgery. The patient was indicated for a LeFort III combined with a LeFort I advancement, each of 10 mm, for a 20 mm combined advancement. Stable, functional occlusion was achieved without airway compromise. This novel use of the combined LeFort III/I can restore stable class I occlusion in patients with BWS at risk for tongue base airway compromise.
Narea MatamalaGFernández Toro MdeLVillalabeitía UgarteELandaeta MendozaM. Beckwith-Wiedemann syndrome: presentation of a case report. Med Oral Patol Oral Cir Bucal. 2008;13(10):E640-E643.
2.
ChoufaniSShumanCWeksbergR. Beckwith–Wiedemann syndrome. Am J Med Genet Part C: Semin Med Genet. 2010;154C:343–354.
3.
WeksbergRShumanCBeckwithJB.
4.
GiancottiARomaniniGGirolamoRDArcuriC. A less-invasive approach with orthodontic treatment in Beckwith–Wiedemann patients. Orthod Craniofac Res. 2002;5:59–63.
5.
Cammarata-ScalisiFAvendañoAStockFCalleaMSparagoARiccioA. Beckwith-Wiedemann syndrome: clinical and etiopathogenic aspects of a model genomic imprinting entity. Arch Argent Pediatr. 2018;116:368–373.
6.
CohenJLCieloCMKupaJDuffyKAHathawayERKalishJMTaylorJA. The utility of early tongue reduction surgery for macroglossia in Beckwith-Wiedemann syndrome. Plast Reconstr Surg. 2020;145(4):803e–813e.
7.
MenardRMDelaireJSchendelSA. Treatment of the craniofacial complications of Beckwith-Wiedemann syndrome. Plast Reconstr Surg. 1995;96:27–33.
8.
MiyawakiSOyaSNoguchiHTakano-YamamotoT. Long-term changes in dentoskeletal pattern in a case with Beckwith-Wiedemann syndrome following tongue reduction and orthodontic treatment. Angle Orthod. 2000;70:326–331.
9.
AbeleiraMTSeoane-RomeroJMOutumuroMCaamañoFSuárezDCarmonaIT. A multidisciplinary approach to the treatment of oral manifestations associated with Beckwith-Wiedemann syndrome: a long-term case report. J Am Dent Assoc. 2011;142:1357–1364.
10.
HotokezakaHMatsuoTNakagawaMMizunoAKobayashiK. Severe dental open bite malocclusion with tongue reduction after orthodontic treatment. Angle Orthod. 2001;71:228–236.
11.
HikitaRKobayashiYTsujiMKawamotoTMoriyamaK. Long-term orthodontic and surgical treatment and stability of a patient with Beckwith-Wiedemann syndrome. Am J Orthod Dentofacial Orthop. 2014;145:672–684.
12.
GandedkarNHChngCKPorYCYeowVKLOwATCSeahTE. Influence of bimaxillary surgery on pharyngeal airway in class III deformities and effect on sleep apnea: a STOP-BANG questionnaire and cone-beam computed tomography study. J Oral Maxillofac Surg. 2017;75:2411–2421.
13.
PolleyJWFigueroaAA. Management of severe maxillary deficiency in childhood and adolescence through distraction osteogenesis with an external, adjustable, rigid distraction device. J Craniofac Surg. 1997;8:181–185; discussion 186.
14.
WangsrimongkolBFloresRLStaffenbergDARodriguezEDShetyePR. Skeletal and dental correction and stability following LeFort I advancement in patients with cleft lip and palate with mild, moderate, and severe maxillary hypoplasia. Cleft Palate Craniofac J. 2022;59:98–109.
15.
ChungJLimJParkHYooAKimSKooY. Correlation between speech outcomes and the amount of maxillary advancement after orthognathic surgery (Le Fort I conventional osteotomy and distraction osteogenesis) in patients with cleft lip and palate. J Craniofac Surg. 2019;30:1855–1858.
16.
TrindadeIEYamashitaRPSuguimotoRMMazzottiniRTrindadeASJr. Effects of orthognathic surgery on speech and breathing of subjects with cleft lip and palate: acoustic and aerodynamic assessment. Cleft Palate Craniofac J. 2003;40:54–64.
17.
MarguliesIGGreenbergAMTaubPJ. Surgical management of lateral orbital contour following Le Fort III distraction. J Craniofac Surg. 2020;31(1):286–288.
