medigraphic.com
ISSN 0001-0944 (Print)
Órgano Oficial de la Asociación Dental Mexicana

2023, Number 5

<< Back Next >>

Rev ADM 2023; 80 (5)

Dental treatment of a patient with Cornelia de Lange syndrome.

Lozoya-Ramírez MJ, Martínez-Rider R, Márquez-Preciado R, Ortiz-Magdaleno M, Alejandri-Gamboa V

Language: Spanish
References: 14
Page: 274-279
PDF size: 372.41 Kb.


ABSTRACT

Cornelia de Lange syndrome (CdLS) is a rare genetic disorder and is principally attributed to mutations in the NIPBL, SMC3 and SMC1A genes. The main clinical characteristics are multiple congenital anomalies, facial dimorphism, hirsutism, hypertrichosis, psychomotor retardation, intellectual disability, prenatal and postnatal growth restriction, hand and foot anomalies, as well as congenital malformations affecting different organs. In patients with CDLS, it is necessary to focus on oral hygiene due to the intellectual disability that may be present and to ensure that adequate dental valuation and hygiene is routinely performed in order to prevent oral diseases. The aim of this case report is to describe the dental management of a 10-year-old patient with CDLS and review the clinical characteristics and radiological findings that are present in the oral cavity.


REFERENCES

  1. Boyle MI, Jespersgaard C, Brondum-Nielsen K, Bisgaard AM, Tümer Z. Cornelia de Lange syndrome. Clin Genet. 2015; 88: 1-12.

  2. Cheung K, Upton J. Cornelia de Lange syndrome. J Hand Surg Am. 2015; 40: 2501-2503.

  3. Braddock SR, Lachman RS, Stoppenhagen CC, Carey JC, Ireland M, Moeschler JB et al. Radiological features in Brachmann-de Lange syndrome. Am J Med Genet. 1993; 47: 1006-1013.

  4. Jackson L, Kline AD, Barr MA, Koch S. de Lange syndrome: a clinical review of 310 individuals. Am J Med Genet. 1993; 47: 940-946.

  5. Oliver C, Arron K, Sloneem J, Hall S. Behavioural phenotype of Cornelia de Lange syndrome: case-control study. Br J Psychiatry. 2008; 193: 466-470.

  6. Sarogni P, Pallotta MM, Musio A. Cornelia de Lange syndrome: from molecular diagnosis to therapeutic approach. J Med Genet. 2020; 57: 289-295.

  7. Gupta D, Goyal S. Cornelia de-Lange syndrome. J Indian Soc Pedod Prev Dent. 2005; 23: 38-41.

  8. Grau Carbó J, López Jiménez J, Giménez Prats MJ, Sánchez Molins M. Cornelia de Lange syndrome: a case report. Med Oral Patol Oral Cir Bucal. 2007; 12: 445-448.

  9. Guadagni MG, Cetrullo N, Piana G. Cornelia de Lange syndrome: description of the orofacial features and case report. Eur J Paediatr Dent. 2008; 9 (4 Suppl): 9-13.

  10. Hei MY, Chen J, Wu LQ, Yu B, Tan YJ, Zhao LL. Cornelia de Lange syndrome: report of a case and the review of literature on 17 cases. Zhonghua Er Ke Za Zhi. 2012; 50: 606-611.

  11. Mehta DN, Bhatia R. Cornelia de-Lange syndrome: a case report. Int J Clin Pediatr Dent. 2013; 6: 115-118.

  12. Sandhu M, Nagpal M, Gulia S, Sachdev V. Dental management of Cornelia de Lange syndrome: a rare case report. J Clin Diagn Res. 2015; 9: 12-14.

  13. Doyle DJ, Hendrix JM, Garmon EH. American Society of Anesthesiologists Classification. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023. Available in: https://www.ncbi.nlm.nih.gov/books/NBK441940/

  14. Frankl S, Shiere FR, Fogels HR. Should the parent remain with the child in the dental operatory? J Dent Child. 1962; 29: 150-163.




2020     |     www.medigraphic.com