Solitary median maxillary central incisor (SMMCI) syndrome
Favored term
Synonyms
Monosuperoincisivodontic dwarfism: Original term (1976)
SMMCI, short stature, choanal atresia/midnasal stenosis syndrome
Shortened to SMMCI syndrome, as other features not necessarily present in all cases
Single central incisor, single maxillary central incisor syndrome, & single incisor syndrome
These terms do not adequately describe peculiarly formed central incisor tooth
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Darcha C et al: Novel CDK10 variants with multicystic dysplastic kidney, left ventricular non-compaction, and a solitary median maxillary central incisor. Clin Genet. 100(3):348-9, 2021
Garcia Rodriguez R et al: The solitary median maxillary central incisor (SMMCI) syndrome: associations, prenatal diagnosis, and outcomes. Prenat Diagn. 39(6):415-9, 2019
Giannopoulou EZ et al: Solitary median maxillary central incisor. J Pediatr. 167(3):770.e1, 2015
Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
Poelmans S et al: Genotypic and phenotypic variation in six patients with solitary median maxillary central incisor syndrome. Am J Med Genet A. 167A(10):2451-8, 2015
Bolan M et al: Solitary median maxillary central incisor. J Dent Child (Chic). 76(1):82-6, 2009
Johnson N et al: Prenatal diagnosis of solitary median maxillary central incisor syndrome by magnetic resonance imaging. Ultrasound Obstet Gynecol. 32(1):120-2, 2008
El-Jaick KB et al: Single median maxillary central incisor: new data and mutation review. Birth Defects Res A Clin Mol Teratol. 79(8):573-80, 2007
Garavelli L et al: Solitary median maxillary central incisor syndrome: clinical case with a novel mutation of sonic hedgehog. Am J Med Genet A. 127A(1):93-5, 2004
Nanni L et al: SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 102(1):1-10, 2001
Van Den Abbeele T et al: Congenital nasal pyriform aperture stenosis: diagnosis and management of 20 cases. Ann Otol Rhinol Laryngol. 110(1):70-5, 2001
Hall RK et al: Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 84(6):651-62, 1997
Arlis H et al: Congenital nasal pyriform aperture stenosis. Isolated abnormality vs developmental field defect. Arch Otolaryngol Head Neck Surg. 118(9):989-91, 1992
Solitary median maxillary central incisor (SMMCI) syndrome
Favored term
Synonyms
Monosuperoincisivodontic dwarfism: Original term (1976)
SMMCI, short stature, choanal atresia/midnasal stenosis syndrome
Shortened to SMMCI syndrome, as other features not necessarily present in all cases
Single central incisor, single maxillary central incisor syndrome, & single incisor syndrome
These terms do not adequately describe peculiarly formed central incisor tooth
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Darcha C et al: Novel CDK10 variants with multicystic dysplastic kidney, left ventricular non-compaction, and a solitary median maxillary central incisor. Clin Genet. 100(3):348-9, 2021
Garcia Rodriguez R et al: The solitary median maxillary central incisor (SMMCI) syndrome: associations, prenatal diagnosis, and outcomes. Prenat Diagn. 39(6):415-9, 2019
Giannopoulou EZ et al: Solitary median maxillary central incisor. J Pediatr. 167(3):770.e1, 2015
Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
Poelmans S et al: Genotypic and phenotypic variation in six patients with solitary median maxillary central incisor syndrome. Am J Med Genet A. 167A(10):2451-8, 2015
Bolan M et al: Solitary median maxillary central incisor. J Dent Child (Chic). 76(1):82-6, 2009
Johnson N et al: Prenatal diagnosis of solitary median maxillary central incisor syndrome by magnetic resonance imaging. Ultrasound Obstet Gynecol. 32(1):120-2, 2008
El-Jaick KB et al: Single median maxillary central incisor: new data and mutation review. Birth Defects Res A Clin Mol Teratol. 79(8):573-80, 2007
Garavelli L et al: Solitary median maxillary central incisor syndrome: clinical case with a novel mutation of sonic hedgehog. Am J Med Genet A. 127A(1):93-5, 2004
Nanni L et al: SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 102(1):1-10, 2001
Van Den Abbeele T et al: Congenital nasal pyriform aperture stenosis: diagnosis and management of 20 cases. Ann Otol Rhinol Laryngol. 110(1):70-5, 2001
Hall RK et al: Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 84(6):651-62, 1997
Arlis H et al: Congenital nasal pyriform aperture stenosis. Isolated abnormality vs developmental field defect. Arch Otolaryngol Head Neck Surg. 118(9):989-91, 1992