Abstract
Solitary median maxillary central incisor (SMMCI) is a rare dental anomaly. It is estimated to occur in 1 : 50,000 live births. The SMMCI tooth differs from the normal central incisor in that the crown form is symmetric and it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. The presence of SMMCI with hemifacial microsomia (HFM) is a very rare clinical condition. We report a case of SMMCI in a female of African ethnic origin, who presented with SMMCI in permanent dentition with mild nasal stenosis. An early diagnosis of SMMCI is important, since it may be a sign for other severe congenital or developmental abnormalities. Therefore, systematic follow-up and close monitoring of the growth and development of SMMCI patients are crucial.
Highlights
A solitary median maxillary central incisor (SMMCI) is a rare malformation associated with defects of midline structures, including the craniofacial bones, nasal airways, and developing brain, along with an increased risk of pituitary malformation and malfunction [1]
The etiology of SMMCI is unknown, but it may be related to a disruption in the development of the maxilla, which occurs during gestation at approximately 35 to 38 days, with abnormal formation of tooth germs and alveolar bone
The name originally given to this syndrome by Hall et al [1] was “solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis syndrome.”
Summary
A solitary median maxillary central incisor (SMMCI) is a rare malformation associated with defects of midline structures, including the craniofacial bones, nasal airways (choanal atresia and nasal pyriform aperture stenosis), and developing brain (holoprosencephaly [HPE]), along with an increased risk of pituitary malformation and malfunction [1]. The etiology of SMMCI is unknown, but it may be related to a disruption in the development of the maxilla, which occurs during gestation at approximately 35 to 38 days, with abnormal formation of tooth germs and alveolar bone. The name originally given to this syndrome by Hall et al [1] was “solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis syndrome.”. It has been customarily shortened to “solitary median maxillary central incisor syndrome” or SMMCI syndrome The name originally given to this syndrome by Hall et al [1] was “solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis syndrome.” It has been customarily shortened to “solitary median maxillary central incisor syndrome” or SMMCI syndrome
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