Advertisement

European Archives of Paediatric Dentistry

, Volume 14, Issue 6, pp 417–423 | Cite as

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

  • N. N. Lygidakis
  • K. Chatzidimitriou
  • N. Petrou
  • N. A. LygidakisEmail author
Case Report

Abstract

Background

Solitary median maxillary central incisor syndrome [SMMCI] is an extremely rare anomaly, especially when no other abnormalities are present. The defect is often found together with various nasal abnormalities and short stature with or without decreased levels of growth hormone. In more severe cases, SMMCI has been associated with holoprosencephaly, the CHARGE and the VACTERL association. Also, published sporadic cases have been related with rare variants of ectodermal dysplasia, chromosomal abnormalities, precocious puberty, hypothalamic hamartoma, congenital heart defects, physical/mental retardation, genital hypoplasia and ear abnormalities. For these reasons when the initial diagnosis is made by the paediatric dentist, ENT, neurological and paediatric evaluations are essential.

Case report

A 4-year-old boy with SMMCI was referred for dental treatment. Clinical/radiographic examination revealed a symmetrical primary and permanent SMMCI, a skeletal Class I and a unilateral crossbite. Medical history indicated respiratory distress and surgery soon after birth due to congenital nasal puriform aperture stenosis. Gradual orthodontic treatment started at the age of 4 years and completed at the age of 13 years. Following maxillary expansion, upper lateral segments were moved backwards and anterior space was created for accommodating a second central incisor. Retainers with a supplementary acrylic incisor were provided for aesthetic and functional replacement until the age of 16 years, when a fixed Maryland ceramic bridge was placed.

Follow-up

Two years recall, at the age of 18 years, revealed a satisfactory and stable aesthetic and functional result.

Conclusion

Successful dental management of SMMCI patients is possible, following a detailed long-lasting treatment plan requiring multidisciplinary paediatric dental, orthodontic and prosthetic approach.

Keywords

SMMCI CNPAS Dental treatment 

Notes

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical standards

The authors state that the study has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Also, the patient presented in the case and his parents have given their informed consent prior to the inclusion in the study. Finally, the authors’ contributions to the paper were: NN. Lygidakis BDS, MJDFRCS(Eng) and K. Chatzidimitriou DDS searched and reviewed the relevant literature and wrote the manuscript; N. Petrou DDS made the Maryland bridge; NA. Lygidakis DDS, MScM, MScD, PhD did the treatment and follow-up of the patient, coordinated the study and revised the manuscript.

References

  1. Arlis H, Ward RF. Congenital nasal pyriform aperture stenosis: isolated abnormality vs developmental field defect. Arch Otolaryngol Head Neck Surg. 1992;118(9):989–91.PubMedCrossRefGoogle Scholar
  2. Aughton DJ, AlSaadi AA, Transue DJ. Single maxillary central incisor in a girl with del(18p) syndrome. J Med Genet. 1991;28:530–2.PubMedCrossRefGoogle Scholar
  3. Barcelos R, Nivoloni Tannure P, Alfredo Farinhas J, Kahn E, Gleiser R. Solitary median maxillary central incisor in two healthy siblings: case report. J Disabil Oral Health. 2011;12(3):133–5.Google Scholar
  4. Berry SA, Pierpont ME, Gorlin RJ. Single central incisor in familial holoprosencephaly. J Pediatr. 1984;104:877–80.PubMedCrossRefGoogle Scholar
  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;74:967–9.CrossRefGoogle Scholar
  6. Bolan M, Derech CD, Correa M, Ribeiro GL, Almeida IC. Palatal expansion in a patient with solitary median maxillary central incisor syndrome. Am J Orthod Dentofacial Orthop. 2010;138:493–7.PubMedCrossRefGoogle Scholar
  7. Brown OE, Myer CM, Manning SC. Congenital nasal pyriform aperture stenosis. Laryngoscope. 1989;99:86–91.PubMedCrossRefGoogle Scholar
  8. Buntinx I, Baraitser M. A single maxillary incisor as a manifestation of an ectodermal dysplasia. J Med Genet. 1989;26:648–51.PubMedCrossRefGoogle Scholar
  9. Cho SY, Drummond BK. Solitary median maxillary central incisor and normal stature: a report of three cases. Int J Paediatr Dent. 2006;16:128–34.PubMedCrossRefGoogle Scholar
  10. Cohen MM, Shiota K. Teratogenesis of holoprosencephaly. Am J Med Genet. 2002;109:1–15.PubMedCrossRefGoogle Scholar
  11. Couly G, Le Douarin NM. The fate map of the cephalic neural primordium at the presomitic to the 3-somite stage in the avian embryo. Development. 1988;103:101–13.PubMedGoogle Scholar
  12. Fleming P, Nelson J, Gorlin RJ. Single maxillary central incisor in association with mid-line anomalies. Br Dent J. 1990;168:476–9.PubMedCrossRefGoogle Scholar
  13. Garcia de Paula e Silva FW, de Carvalho FK, Diaz-Serrano KV et al. Solitary median maxillary central incisor in association with Goldenhar’s syndrome: a case report. Spec Care Dentist. 2007;27(3):105–7.Google Scholar
  14. Hall RK, Bankier A, Aldrer 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;84:651–62.CrossRefGoogle Scholar
  15. Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006;1:12–33.PubMedCrossRefGoogle Scholar
  16. Harrison M, Calvert ML, Longhurst P. Solitary maxillary central incisor as a new finding in CHARGE association: a report of two cases. Int J Paediatr Dent. 1997;7:185–9.PubMedCrossRefGoogle Scholar
  17. Heussler HS, Suri M, Young ID, Muenke M. Extreme variability of expression of a Sonic Hedgehog mutation: attention difficulties and holoprosencephaly. Arch Dis Child. 2002;86:293–6.PubMedCrossRefGoogle Scholar
  18. Kjaer I, Becktor KB, Lisson J, Gormsen C, Russel BG. Face, palate and craniofacial morphology in patients with a solitary median maxillary central incisor. Eur J Orthod. 2001;23:63–73.PubMedCrossRefGoogle Scholar
  19. Lee KS, Yang CC, Huang JK, Chen YC, Chang KC. Congenital pyriform aperture stenosis: surgery and evaluation with three-dimensional computed tomography. Laryngoscope. 2002;112:918–21.PubMedCrossRefGoogle Scholar
  20. Lertsirivorakul J, Hall RK. Solitary median maxillary central incisor syndrome occurring together with oromandibular-limb hypogenesis syndrome type 1: a case report of this previously unreported combination of syndromes. Int J Paediatr Dent. 2008;18:306–11.PubMedCrossRefGoogle Scholar
  21. Lo FS, Lee YJ, Lin SP et al. Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. Eur J Pediatr. 1998;157:39–44.PubMedCrossRefGoogle Scholar
  22. Machado E, Machado P, Grehs B, Grehs RA. Solitary median maxillary central incisor syndrome: case report. Dental Press J Orthod. 2010;15(4):55–61.CrossRefGoogle Scholar
  23. Malandris M, Mahoney EK. Aetiology, diagnosis and treatment of posterior cross-bites in the primary dentition. Int J Pediatr Dent. 2004;14:155–66.CrossRefGoogle Scholar
  24. Marini M, Cusano R, De Biasio P et al. Previously undescribed nonsense mutation in SHH caused autosomal dominant holoprosencephaly with wide intrafamilial variability. Am J Med Genet. 2003;117A:112–5.PubMedCrossRefGoogle Scholar
  25. Masuno M, Fukushima Y, Sugio Y, Ikeda M, Kuroki Y. Two unrelated cases of single maxillary central incisor with 7q terminal deletion. Jinrui Idengaku Zasshi. 1990;35(4):311–7.PubMedCrossRefGoogle Scholar
  26. McNamara T, Field D, McNamara T. A solitary maxillary central incisor treated orthodontically: a case report. Spec Care Dent. 1999;19:135–8.CrossRefGoogle Scholar
  27. Miura M, Kato N, Kojima H, Oguchi H. Triple-X syndrome accompanied by a single maxillary central incisor: case report. Pediatr Dent. 1993;15:214–7.PubMedGoogle Scholar
  28. Nanni L, Ming JE, Bocian M et al. The mutational spectrum of the Sonic Hedgehog gene in holoprosencephaly: SHH mutations cause a significant proportion of autosomal dominant holoprosencephaly. Hum Mol Genet. 1999;8:2479–88.PubMedCrossRefGoogle Scholar
  29. Nanni L, Ming JE, Du Y 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. 2001;102:1–10.PubMedCrossRefGoogle Scholar
  30. Parentin F, Perissutti P. Solitary median maxillary incisor, Duane retraction syndrome, growth hormone deficiency and duplicated thumb phalanx: a case report. Clin Dysmorphol. 2003;12:141–2.PubMedCrossRefGoogle Scholar
  31. Ramadan HH, Ortiz O. Congenital nasal pyriform aperture (bony inlet) stenosis: clinical review. Otolaryngol Head Neck Surg. 1995;113:286–9.PubMedCrossRefGoogle Scholar
  32. Rappaport EB, Ulstrom R, Gorlin RJ. Monosuperocentroincisivodontic dwarfism. Birth Defects Orig Artic Ser. 1976;12:243–5.PubMedGoogle Scholar
  33. Scott DC. Absence of upper central incisor. Br Dent J. 1958;104:247–8.Google Scholar
  34. 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;2:385–9.PubMedCrossRefGoogle Scholar
  35. Van Den Abbeele T, Triqlia JM, Francois M, Narcy P. Congenital nasal puriform aperture stenosis: diagnosis and management of 20 cases. Ann Otol Rhinol Laryngol. 2001;110:70–5.Google Scholar
  36. Velasco DMM, Ramírez JAF, Nagano AY. Incisor syndrome single central midline fusion of the maxillary and mandibular permanent central incisors: report of clinical case. Rev Odont Mex. 2005;9:37–41.Google Scholar
  37. Wesley RK, Hoffman WH, Perrin J, Delaney JR. Solitary maxillary central incisor and normal stature. Oral Surg Oral Med Oral Pathol. 1978;46:837–42.PubMedCrossRefGoogle Scholar
  38. Wong CA, Sinclair PM, Keim RG, Kennedy DB. Arch dimension changes from successful slow maxillary expansion of unilateral posterior crossbite. Angle Orthod. 2011;81(4):616–23.PubMedCrossRefGoogle Scholar
  39. Youko K, Satoshi F, Kubota K, Goto G. Clinical evaluation of a patient with single maxillary central incisor. J Clin Pediatr Dent. 2002;26:181–6.PubMedGoogle Scholar

Copyright information

© European Academy of Paediatric Dentistry 2013

Authors and Affiliations

  • N. N. Lygidakis
    • 1
  • K. Chatzidimitriou
    • 1
  • N. Petrou
    • 1
  • N. A. Lygidakis
    • 1
    Email author
  1. 1.Private Paediatric Dental PracticeAthensGreece

Personalised recommendations