References

Hall RK, Bankier A, Aldred MJ Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997; 84:651-662 https://doi.org/10.1016/s1079-2104(97)90368-1
Garcia Rodriguez R, Garcia Cruz L, Novoa Medina Y The solitary median maxillary central incisor (SMMCI) syndrome: Associations, prenatal diagnosis, and outcomes. Prenat Diagn. 2019; 39:415-419 https://doi.org/10.1002/pd.5451
Nanni L, Ming JE, Du Y SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 2001; 102:1-10
Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006; 1 https://doi.org/10.1186/1750-1172-1-12
Kjaer I, Becktor KB, Lisson J Face, palate, and craniofacial morphology in patients with a solitary median maxillary central incisor. Eur J Orthod. 2001; 23:63-73 https://doi.org/10.1093/ejo/23.1.63
Tabatabaie F, Sonnesen L, Kjaer I. The neurocranial and craniofacial morphology in children with solitary median maxillary central incisor (SMMCI). Orthod Craniofac Res. 2008; 11:96-104 https://doi.org/10.1111/j.1601-6343.2007.00419.x
DiBiase AT, Cobourne MT. Beware the solitary maxillary median central incisor. J Orthod. 2008; 35:16-19 https://doi.org/10.1179/146531207225022365
Matsunaga E, Shiota K. Holoprosencephaly in human embryos: epidemiologic studies of 150 cases. Teratology. 1977; 16:261-272 https://doi.org/10.1002/tera.1420160304
Garavelli L, Zanacca C, Caselli G Solitary median maxillary central incisor syndrome: clinical case with a novel mutation of sonic hedgehog. Am J Med Genet A. 2004; 127A:93-95 https://doi.org/10.1002/ajmg.a.20685
Richieri-Costa A, Ribeiro LA. Single maxillary central incisor, holoprosencephaly, and holoprosencephaly-like phenotype. Am J Med Genet A. 2006; 140:2594-2597 https://doi.org/10.1002/ajmg.a.31379

A non-invasive treatment option in solitary median maxillary central incisor syndrome

From Volume 49, Issue 10, November 2022 | Pages 830-832

Authors

Maalini Patel

BDS(Hons), MPaedDent RCSEng, FDS RCSEng

Specialty Registrar in Paediatric Dentistry, Guy's and St Thomas' NHS Foundation Trust, Bristol, UK

Articles by Maalini Patel

Louise Ormondroyd

BDS(Hons), MJDF RCSEng, DipConSed(Ncle), MOralSurg RCSEng, FHEA

Consultant in Oral Surgery, Guy's Hospital, London

Articles by Louise Ormondroyd

Abstract

Solitary median maxillary central incisor (SMMCI) syndrome (OMIM #147250) is a disorder characterized by developmental defects of midline structures with variable expressivity. It presents dentally as a single symmetrical central incisor in the midline position. This is a case report of a child with the isolated dental feature of a single maxillary central incisor, with no medical features of SMMCI syndrome. Under the management of a multidisciplinary dental team, a non-invasive approach was undertaken to improve aesthetics. This case report describes the dental options for managing SMMCI including a non-invasive option that has not previously been reported in detail.

CPD/Clinical Relevance: Dentists should be aware of SMMCI and its potential medical sequelae.

Article

The rare finding of a solitary median maxillary central incisor (SMMCI) can either be observed as an isolated dental feature or part of a wider finding of SMMCI syndrome (OMIM #147250).1,2 It presents clinically as a single central incisor in the midline position, in both the primary and permanent dentitions. Of note, both the crown and the root of the single incisor are symmetrical.3 The single incisor is the characteristic feature of SMMCI syndrome.4 In addition to this dental anomaly, SMMCI syndrome is also associated with craniofacial morphology abnormalities including a retrognathic maxilla and increased incisor inclination.5,6

SMMCI syndrome is a disorder involving developmental defects of midline structures with variable expressivity.4 It involves the midline structures of the head, the nasal airways and brain, in addition to other midline structures of the body.4 It can be diagnosed prenatally via ultrasound.2

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