%0 Journal Article
%T Solitary median maxillary central incisor (SMMCI) syndrome
%A Roger K Hall
%J Orphanet Journal of Rare Diseases
%D 2006
%I BioMed Central
%R 10.1186/1750-1172-1-12
%X The name originally given to this syndrome by Hall et al. [1], "Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis syndrome", is now customarily shortened to the first part of this name: "Solitary median maxillary central incisor syndrome" or SMMCI syndrome, as the other features are not necessarily present in all cases. The use of the full description of the single incisor tooth in the name is important, as it emphasises the unique form and position of this tooth, which is the characteristic and most readily observed feature or trait of the condition.The early observations of this condition [2-8] merely referred to the congenital and hereditary absence of one central incisor. When the association with short stature was recognised in 1976, the name Monosuperoincisivodontic dwarfism was given by Rappaport et al. [9], but it was soon recognised that short stature was not always present in the disorder [10]. The names "single central incisor syndrome" or "single maxillary central incisor" or "single incisor" suggested by other authors [11-15], do not adequately describe the peculiarly formed incisor tooth.To accurately describe the characteristic tooth present in this syndrome, it is necessary to specify:£¿ Solitary: the tooth present exists as the only central incisor tooth in the maxilla.£¿ Median: this tooth is present precisely in the midline of the maxillary alveolus (a single central incisor tooth present to one or other side of the midline indicates that the contralateral tooth has been lost from trauma or disease, or did not continue to develop beyond the cellular stage, the tooth germ being resorbed).£¿ Maxillary: this characteristic tooth occurs only in the maxilla and not in the mandible.£¿ Central Incisor: the tooth is a central incisor tooth, although of unusual crown form and is not a supernumerary tooth (mesiodens).Hence, the acronym SMMCI syndrome.The following circumstances where only one central incisor tooth is pr
%U http://www.ojrd.com/content/1/1/12