Keywords
Single maxillary central incisor syndrome
development
craniofacial malformation
congenital anomaly
single incisor
development
craniofacial malformation
congenital anomaly
single incisor
How to Cite
Single maxillary central incisor syndrome: case report. (2022). Odontoestomatología, 24(40), 1-15. https://doi.org/10.22592/ode2022n40e412
Abstract
Single maxillary central incisor syndrome is a rare alteration in the development and formation of organs located mainly in the midline; which occurs early between days 35 to 38 of intrauterine life. Its etiology is unknown, although it has been associated with deletions of chromosomes 7 (7q.36.1) and 8, and mutations in the Sonic Hedgehog gene. It has a prevalence of 1/50,000 live births and although it is a rare anomaly of craniofacial development, its early diagnosis and treatment are important for general dentists or specialists since it can be a sign of other serious congenital or developmental anomalies. Therefore, the objective of this case is to report the initial phase of treatment in a child with Single maxillary central incisor syndrome who had not been previously diagnosed with this syndrome.
Clinical case: Male patient, 10 years old, from Jamundí, Valle del Cauca- Colombia. Reports absence of upper dental organ. Intraoral examination shows a single central incisor on the maxillary midline, absence of labial frenulum and incisive papilla, oval palate and mandibular retrognathism. He was treated in a first phase with maxillary functional orthopedics to improve class II and is waiting to start the second phase of orthodontic treatment.
Conclusions:Single maxillary central incisor syndrome is a rare syndrome which involves multiple conditions that interfere with the normal development and growth of anatomical structures.
References
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3.Nalawade TM, Mallikarjuna RM, Sogi HPS, Bhat KG, Kumbar VM. Solitary Median Maxillary Central Incisor: A Case Report with 3-Year Follow-Up and Literature Review. Contemp Clin Dent. 2021 Jul-Sep;12(3):324-327.
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5.Schneider UEM, Moser L. Orthodontic space closure in a young female patient with solitary median maxillary central incisor syndrome. Am J Orthod Dentofacial Orthop. 2021 Jul;160(1):132-146.
6.Ilhan O, Pekcevik Y, Akbay S, Ozdemir SA, Memur S, Kanar B, Kirbiyik O, Ozer EA. Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report. Arch Argent Pediatr. 2018 Feb 1;116(1):e130-e134.
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8.Rappaport EB, Ulstrom R, Gorlin RJ. Monosuperocentroincisivodontic dwarfism. Birth Defects Orig Artic Ser. 1976;12(5):243-5.
9.Viana Eda S, Kramer PF, Closs LQ, Scalco G. Solitary median maxillary central incisor syndrome and holoprosencephaly: a case report. Pediatr Dent. 2010 Sep-Oct;32(5):424-7.
10.Utreja A, Zahid SN, Gupta R. Solitary median maxillary central incisor in association with hemifacial microsomia: A rare case report and review of literature. Contemp Clin Dent. 2011 Oct;2(4):385-9.
11.Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006 Apr 9;1:12.
12.Chandrasekaran D, Yezdani A, Tajir F, Saravanan B, Rajasekar L. Solitary median maxillary central incisor: A case report of a rare dental anomaly. J Pharm Bioallied Sci. 2015 Apr;7(Suppl 1):S307-8.
13.Poelmans S, Kawamoto T, Cristofoli F, Politis C, Vermeesch J, Bailleul-Forestier I, Hens G, Devriendt K, Verdonck A, Carels C. Genotypic and phenotypic variation in six patients with solitary median maxillary central incisor syndrome. Am J Med Genet A. 2015 Oct;167A(10):2451-8.
14.Garcia Rodriguez R, Garcia Cruz L, Novoa Medina Y, Garcia Delgado R, Perez Gonzalez J, Palma Milla C, Lopez Siles J, Medina Castellano M, Garcia Hernandez JA, Santana Rodriguez A. The solitary median maxillary central incisor (SMMCI) syndrome: Associations, prenatal diagnosis, and outcomes. Prenat Diagn. 2019 May;39(6):415-419.
15.Machado E, Machado P, Grehs B, Grehs RA. Solitary median maxillary central incisor syndrome: Case report. Dental Press J Orthod. 2010 July-Aug;15(4):55-6
16.Szakszon K, Felszeghy E, Csízy I, Józsa T, Káposzta R, Balogh E, Oláh E, Balogh I, Berényi E, Knegt AC, Ilyés I. Endocrine and anatomical findings in a case of Solitary Median Maxillary Central Incisor Syndrome. Eur J Med Genet. 2012 Feb;55(2):109-11.
17.Barcelos R, Tannure P, Farinhas J, Kahn E, and Gleiser R. Solitary median maxillary central incisor in two healthy siblings: case report. Journal of Disability and Oral Health (2011) 12/3 133-135. 123. 133-135.
18.Mendoza D, Fragoso A, Yamamoto A. Síndrome del incisivo central único de la línea media del maxilar y fusión de incisivos centrales permanentes mandibulares: Reporte de un caso clínico. Revista Odontológica Mexicana a 2005;9 (1): 37-41
19.Negi A, Negi A, Mohanan M. Solitary median maxillary central incisor syndrome: A rare entity. J Oral Maxillofac Pathol. 2020 May-Aug;24(2):402.
20.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 Aug;74(8):967-969.
21.Bolan M, Derech CD, Côrrea M, Ribeiro GLU, Almeida ICS. Palatal expansion in a patient with solitary median maxillary central incisor syndrome. Am J Orthod Dentofacial Orthop. 2010 Oct;138(4):493-497.
22.De Lima Pedro R, Kchler EC, Primo LG, de Castro Costa M (2017) Solitary Median Maxillary Central Incisor Syndrome: An esthetic solution in a child. J Dent Probl Solut 4(4): 072-075.
23.Muñoz Aguilar G, Alcón Sáez J.J., Domingo Triadó I. Síndrome del incisivo central maxilar único. Acta Pediatr Esp. 2014; 72(3): e100-e104.
24.Srivathsa SH. Congenitally missing maxillary central incisor or solitary median maxillary central incisor?. Int J Orofac Res 2018;3:17-9.
25.Nanni L, Ming JE, Du Y, Hall RK, Aldred M, Bankier A, Muenke M. SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 2001 Jul 22;102(1):1-10.
26.Zatoński T, Pazdro-Zastawny K, Morawska-Kochman M, Biela M, Kołtowska A, Rydzanicz M, Rozensztrauch A, Kosińska J, Dorobisz K, Płoski R, Śmigiel R. Single median maxillary central incisor syndrome and variant in SMO gene associated with SHH pathway. Int J Pediatr Otorhinolaryngol. 2020 Jul;134:110038.
27.Nota A, Ehsani S, Pittari L, Gastaldi G, Tecco S. Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism. Head Face Med. 2021 Nov 23;17(1):49.
2.Mustafa MM, Zakirulla M, AlShahrani I, Togoo RA, Alkahtani ZM, Ain TS. Clinical Evaluation of Solitary Median Maxillary Central Incisor Syndrome. Case Rep Dent. 2019 Sep 12;2019:2637825.
3.Nalawade TM, Mallikarjuna RM, Sogi HPS, Bhat KG, Kumbar VM. Solitary Median Maxillary Central Incisor: A Case Report with 3-Year Follow-Up and Literature Review. Contemp Clin Dent. 2021 Jul-Sep;12(3):324-327.
4.Johnson N, Windrim R, Chong K, Viero S, Thompson M, Blaser S. Prenatal diagnosis of solitary median maxillary central incisor syndrome by magnetic resonance imaging. Ultrasound Obstet Gynecol. 2008 Jul;32(1):120-2.
5.Schneider UEM, Moser L. Orthodontic space closure in a young female patient with solitary median maxillary central incisor syndrome. Am J Orthod Dentofacial Orthop. 2021 Jul;160(1):132-146.
6.Ilhan O, Pekcevik Y, Akbay S, Ozdemir SA, Memur S, Kanar B, Kirbiyik O, Ozer EA. Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report. Arch Argent Pediatr. 2018 Feb 1;116(1):e130-e134.
7.Lygidakis NN, Chatzidimitriou K, Petrou N, Lygidakis NA. Solitary median maxillary central incisor syndrome (SMMCI) with congenital nasal puriform aperture stenosis: literature review and case report with comprehensive dental treatment and 14 years follow-up. Eur Arch Paediatr Dent. 2013 Dec;14(6):417-23.
8.Rappaport EB, Ulstrom R, Gorlin RJ. Monosuperocentroincisivodontic dwarfism. Birth Defects Orig Artic Ser. 1976;12(5):243-5.
9.Viana Eda S, Kramer PF, Closs LQ, Scalco G. Solitary median maxillary central incisor syndrome and holoprosencephaly: a case report. Pediatr Dent. 2010 Sep-Oct;32(5):424-7.
10.Utreja A, Zahid SN, Gupta R. Solitary median maxillary central incisor in association with hemifacial microsomia: A rare case report and review of literature. Contemp Clin Dent. 2011 Oct;2(4):385-9.
11.Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006 Apr 9;1:12.
12.Chandrasekaran D, Yezdani A, Tajir F, Saravanan B, Rajasekar L. Solitary median maxillary central incisor: A case report of a rare dental anomaly. J Pharm Bioallied Sci. 2015 Apr;7(Suppl 1):S307-8.
13.Poelmans S, Kawamoto T, Cristofoli F, Politis C, Vermeesch J, Bailleul-Forestier I, Hens G, Devriendt K, Verdonck A, Carels C. Genotypic and phenotypic variation in six patients with solitary median maxillary central incisor syndrome. Am J Med Genet A. 2015 Oct;167A(10):2451-8.
14.Garcia Rodriguez R, Garcia Cruz L, Novoa Medina Y, Garcia Delgado R, Perez Gonzalez J, Palma Milla C, Lopez Siles J, Medina Castellano M, Garcia Hernandez JA, Santana Rodriguez A. The solitary median maxillary central incisor (SMMCI) syndrome: Associations, prenatal diagnosis, and outcomes. Prenat Diagn. 2019 May;39(6):415-419.
15.Machado E, Machado P, Grehs B, Grehs RA. Solitary median maxillary central incisor syndrome: Case report. Dental Press J Orthod. 2010 July-Aug;15(4):55-6
16.Szakszon K, Felszeghy E, Csízy I, Józsa T, Káposzta R, Balogh E, Oláh E, Balogh I, Berényi E, Knegt AC, Ilyés I. Endocrine and anatomical findings in a case of Solitary Median Maxillary Central Incisor Syndrome. Eur J Med Genet. 2012 Feb;55(2):109-11.
17.Barcelos R, Tannure P, Farinhas J, Kahn E, and Gleiser R. Solitary median maxillary central incisor in two healthy siblings: case report. Journal of Disability and Oral Health (2011) 12/3 133-135. 123. 133-135.
18.Mendoza D, Fragoso A, Yamamoto A. Síndrome del incisivo central único de la línea media del maxilar y fusión de incisivos centrales permanentes mandibulares: Reporte de un caso clínico. Revista Odontológica Mexicana a 2005;9 (1): 37-41
19.Negi A, Negi A, Mohanan M. Solitary median maxillary central incisor syndrome: A rare entity. J Oral Maxillofac Pathol. 2020 May-Aug;24(2):402.
20.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 Aug;74(8):967-969.
21.Bolan M, Derech CD, Côrrea M, Ribeiro GLU, Almeida ICS. Palatal expansion in a patient with solitary median maxillary central incisor syndrome. Am J Orthod Dentofacial Orthop. 2010 Oct;138(4):493-497.
22.De Lima Pedro R, Kchler EC, Primo LG, de Castro Costa M (2017) Solitary Median Maxillary Central Incisor Syndrome: An esthetic solution in a child. J Dent Probl Solut 4(4): 072-075.
23.Muñoz Aguilar G, Alcón Sáez J.J., Domingo Triadó I. Síndrome del incisivo central maxilar único. Acta Pediatr Esp. 2014; 72(3): e100-e104.
24.Srivathsa SH. Congenitally missing maxillary central incisor or solitary median maxillary central incisor?. Int J Orofac Res 2018;3:17-9.
25.Nanni L, Ming JE, Du Y, Hall RK, Aldred M, Bankier A, Muenke M. SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 2001 Jul 22;102(1):1-10.
26.Zatoński T, Pazdro-Zastawny K, Morawska-Kochman M, Biela M, Kołtowska A, Rydzanicz M, Rozensztrauch A, Kosińska J, Dorobisz K, Płoski R, Śmigiel R. Single median maxillary central incisor syndrome and variant in SMO gene associated with SHH pathway. Int J Pediatr Otorhinolaryngol. 2020 Jul;134:110038.
27.Nota A, Ehsani S, Pittari L, Gastaldi G, Tecco S. Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism. Head Face Med. 2021 Nov 23;17(1):49.