Sotos syndrome is a genetic disorder characterized by distinct craniofacial features, overgrowth in childhood, and impaired intellectual development. We herein report the successful orthodontic treatment of a 14-year-old boy with Sotos syndrome caused by a heterozygous mutation in the NSD1 gene. He showed severe hypodontia, impaction of the maxillary second molars and a skeletal Class III jaw-base relationship. Orthodontic management, including space control by protraction of the maxillary first molars and traction of the impacted molars, was performed using fixed appliances and miniscrews. As a result, acceptable occlusion was obtained without any discernible relapse 18 months postretention.
KurotakiNImaizumiKHaradaN, et al.Haploinsufficiency of NSD1 causes Sotos syndrome. Nat Genet. Apr 2002;30(4):365–366. doi:10.1038/ng863
2.
Tatton-BrownKRahmanN. Clinical features of NSD1-positive Sotos syndrome. Clin Dysmorphol. Oct 2004;13(4):199–204. doi:10.1097/00019605-200410000-00001
3.
ColeTRHughesHE. Sotos syndrome: A study of the diagnostic criteria and natural history. J Med Genet. Jan 1994;31(1):20–32. doi:10.1136/jmg.31.1.20
4.
TakeiKSueishiKYamaguchiHOhtawaY. Dentofacial growth in patients with Sotos syndrome. Bull Tokyo Dent Coll. May 2007;48(2):73–85. doi:10.2209/tdcpublication.48.73
5.
HiraiNMatsuneKOhashiH. Craniofacial and oral features of Sotos syndrome: Differences in patients with submicroscopic deletion and mutation of NSD1 gene. Am J Med Genet A. Dec 2011;155a(12):2933–2939. doi:10.1002/ajmg.a.33969
6.
AllansonJEColeTR. Sotos syndrome: Evolution of facial phenotype subjective and objective assessment. Am J Med Genet. Oct 2 1996;65(1):13–20. doi:10.1002/(sici)1096-8628(19961002)65:1<13::aid-ajmg2>3.0.co;2-z
7.
NagaiTMatsumotoNKurotakiN, et al.Sotos syndrome and haploinsufficiency of NSD1: Clinical features of intragenic mutations and submicroscopic deletions. J Med Genet. Apr. 2003;40(4):285–289. doi:10.1136/jmg.40.4.285
8.
CallananAPAnandPSheehyEC. Sotos syndrome with hypodontia. Int J Paediatr Dent. Mar 2006;16(2):143–146. doi:10.1111/j.1365-263X.2006.00684.x
9.
KotilainenJPohjolaPPirinenSArteSNieminenP. Premolar hypodontia is a common feature in Sotos syndrome with a mutation in the NSD1 gene. Am J Med Genet A. Nov 2009;149a(11):2409–2414. doi:10.1002/ajmg.a.33062
10.
TakanoMKasaharaKOgawaCKatadaHSueishiK. A case of Sotos syndrome treated with distraction osteogenesis in maxilla and mandible. Bull Tokyo Dent Coll. 2012;53(2):75–82. doi:10.2209/tdcpublication.53.75
11.
ShioyasonoRYoshinagaKShioyasonoA, et al.Nonsurgical orthodontic treatment for a patient with Sotos syndrome. Am J Orthod Dentofacial Orthop. 2023. doi:10.1016/j.ajodo.2021.11.021
12.
KurotakiNHaradaNShimokawaO, et al.Fifty microdeletions among 112 cases of Sotos syndrome: Low copy repeats possibly mediate the common deletion. Hum Mutat. Nov 2003;22(5):378–387. doi:10.1002/humu.10270
13.
Tatton-BrownKDouglasJColemanK, et al.Genotype-phenotype associations in Sotos syndrome: An analysis of 266 individuals with NSD1 aberrations. Am J Hum Genet. Aug 2005;77(2):193–204. doi:10.1086/432082
14.
HobkirkJABrookAH. The management of patients with severe hypodontia. J Oral Rehabil. Jul 1980;7(4):289–298. doi:10.1111/j.1365-2842.1980.tb00447.x
15.
RobertssonSMohlinB. The congenitally missing upper lateral incisor. A retrospective study of orthodontic space closure versus restorative treatment. Eur J Orthod. Dec 2000;22(6):697–710. doi:10.1093/ejo/22.6.697
16.
NiharaJGielo-PerczakKCardinalLSaitoINandaRUribeF. Finite element analysis of mandibular molar protraction mechanics using miniscrews. Eur J Orthod. Feb 2015;37(1):95–100. doi:10.1093/ejo/cju017
17.
BaikUBChunYSJungMHSugawaraJ. Protraction of mandibular second and third molars into missing first molar spaces for a patient with an anterior open bite and anterior spacing. Am J Orthod Dentofacial Orthop. Jun 2012;141(6):783–795. doi:10.1016/j.ajodo.2010.07.031
18.
KravitzNDJolleyT. Mandibular molar protraction with temporary anchorage devices. J Clin Orthod. Jun 2008;42(6):351–355. quiz 340.
