Solitary median maxillary central incisor syndrome (SMMCI) with congenital nasal puriform aperture stenosis: literature review and case report with comprehensive dental treatment and 14 years follow-up

Background Solitary median maxillary central incisor syndrome [SMMCI] is an extremely rare anomaly, especially when no other abnormalities are present. The defect is often found together with various nasal abnormalities and short stature with or without decreased levels of growth hormone. In more severe cases, SMMCI has been associated with holoprosencephaly, the CHARGE and the VACTERL association. Also, published sporadic cases have been related with rare variants of ectodermal dysplasia, chromosomal abnormalities, precocious puberty, hypothalamic hamartoma, congenital heart defects, physical/mental retardation, genital hypoplasia and ear abnormalities. For these reasons when the initial diagnosis is made by the paediatric dentist, ENT, neurological and paediatric evaluations are essential.