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.

backgroundSolitary 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. CASE REPORT: A 4-year-old boy with SMMCI was referred for dental treatment. Clinical/radiographic examination revealed a symmetrical primary and permanent SMMCI, a skeletal Class I and a unilateral crossbite. Medical history indicated respiratory distress and surgery soon after birth due to congenital nasal puriform aperture stenosis. Gradual orthodontic treatment started at the age of 4 years and completed at the age of 13 years. Following maxillary expansion, upper lateral segments were moved backwards and anterior space was created for accommodating a second central incisor. Retainers with a supplementary acrylic incisor were provided for aesthetic and functional replacement until the age of 16 years, when a fixed Maryland ceramic bridge was placed. FOLLOW-UP: Two years recall, at the age of 18 years, revealed a satisfactory and stable aesthetic and functional result.

conclusionSuccessful dental management of SMMCI patients is possible, following a detailed long-lasting treatment plan requiring multidisciplinary paediatric dental, orthodontic and prosthetic approach.