Solitary median maxillary central incisor
The solitary median maxillary central incisor (SMMCI) is a rare dental anomaly. It is usually considered as a minor manifestation of holoprosencephaly (HPE).
It is estimated to occur in 1:50,000 live births.
It is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35 - 38th day(s) from conception. The aetiology is uncertain.
A missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI 2.
- 1. Garavelli L, Zanacca C, Caselli G et-al. Solitary median maxillary central incisor syndrome: clinical case with a novel mutation of sonic hedgehog. Am. J. Med. Genet. A. 2004;127A (1): 93-5. doi:10.1002/ajmg.a.20685 - Pubmed citation
- 2. Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006;1 : 12. doi:10.1186/1750-1172-1-12 - Free text at pubmed - Pubmed citation
- 3. Hall RK, Bankier A, Aldred MJ et-al. Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;84 (6): 651-62. Oral Surg Oral Med Oral Pathol Oral Radiol Endod (link) - Pubmed citation
- 4. Gupta. Dental Diseases Differentials Diagnosis. Jaypee Brothers Medical Publishers (P) Ltd. ISBN:8184483724. Read it at Google Books - Find it at Amazon
- 5. Blackmore K, Wynne DM. A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia. Int. J. Pediatr. Otorhinolaryngol. 2010;74 (8): 967-9. - Pubmed citation
Synonyms & Alternative Spellings
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|Central incisor syndrome||✗|
|Solitary median maxillary central incisor syndrome||✗|
|solitary median maxillary central incisor (SMMCI)||✗|