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Solitary Median Maxillary Central Incisor
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Solitary median maxillary central incisor (SMMCI) syndrome
                  • Favored term
              • Synonyms

                • Monosuperoincisivodontic dwarfism: Original term (1976)
                • Solitary median maxillary central incisor, 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

                                    1. Giannopoulou EZ et al: Solitary median maxillary central incisor. J Pediatr. 167(3):770.e1, 2015
                                    2. Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
                                    3. 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
                                    4. Bolan M et al: Solitary median maxillary central incisor. J Dent Child (Chic). 76(1):82-6, 2009
                                    5. 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
                                    6. 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
                                    7. Naudi AB et al: A child with 18p- syndrome: a case report. Spec Care Dentist. 27(1):12-4, 2007
                                    8. Hall RK: Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 1:12, 2006
                                    9. 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
                                    10. 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
                                    11. 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
                                    12. 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
                                    13. 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
                                    14. Rappaport EB et al: Monosuperocentroincisivodontic dwarfism. Birth Defects Orig Artic Ser. 12(5):243-5, 1976
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Solitary median maxillary central incisor (SMMCI) syndrome
                                                    • Favored term
                                                • Synonyms

                                                  • Monosuperoincisivodontic dwarfism: Original term (1976)
                                                  • Solitary median maxillary central incisor, 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

                                                                      1. Giannopoulou EZ et al: Solitary median maxillary central incisor. J Pediatr. 167(3):770.e1, 2015
                                                                      2. Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
                                                                      3. 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
                                                                      4. Bolan M et al: Solitary median maxillary central incisor. J Dent Child (Chic). 76(1):82-6, 2009
                                                                      5. 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
                                                                      6. 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
                                                                      7. Naudi AB et al: A child with 18p- syndrome: a case report. Spec Care Dentist. 27(1):12-4, 2007
                                                                      8. Hall RK: Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 1:12, 2006
                                                                      9. 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
                                                                      10. 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
                                                                      11. 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
                                                                      12. 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
                                                                      13. 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
                                                                      14. Rappaport EB et al: Monosuperocentroincisivodontic dwarfism. Birth Defects Orig Artic Ser. 12(5):243-5, 1976