Solitary median maxillary central incisor (SMMCI) syndrome – the crucial role of orthodontist. Case reports and review of the literature
Journal Title: Forum Ortodontyczne - Year 2012, Vol 8, Issue 3
Abstract
Solitary median maxillary central incisor (SMMCI) is a rare, complex disorder involving mid-sagittal structures of the body, head in particular. This developmental anomaly is characterized by the presence of symmetrical, solitary central incisor erupting exactly in the middle of the maxillary dental arch, in both primary and permanent dentition. Additional midline structures: cranial base, nasal cavity with the nasal septum, and brain may be malformed and other congenital abnormalities, such as short stature, intellectual disability, congenital heart diseases, cleft lip and palate may also be noticed. The prevalence of SMMCI is about 1: 50000 live births. Some authors suggest that SMMCI is a benign form of holoprosencephaly. In this article we present two patients with late diagnosed SMMCI syndrome. In both cases characteristic features of SMMCI syndrome were noticed: presence of solitary median maxillary central incisor in either primary or permanent dentition, indistinct philtrum, arch-shaped upper lip, absence of the fraenulum of the upper lip, midpalatal ridge, V-shaped upper dental arch and neonatal breathing difficulties of unknown aetiology. The boy was diagnosed for hypospadias and mild intellectual disability, while the girl had microcephaly, moderate intellectual disability, nasal stenosis and solitary median mandibular central incisor, which is a very rare finding not reported previously as an abnormality concomitant to SMMCI syndrome. This alteration should not be considered as a simple dental anomaly, but requires further, thorough, specialized evaluation. The role of orthodontist is crucial as one may be the first to recognize SMMCI syndrome and refer the patient for further diagnosis. (Orthod. Forum 2012; 8: 124- 134). Received: 16.06.2012 Accepted: 2.09.2012
Authors and Affiliations
Julia Rosiak, Dortoa Kuśmierczyk, Agnieszka Radomska, Barbara Pietrzak-Bilińska
Keywords
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