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Revista Portuguesa de Estomatologia,
Medicina Dentária e Cirurgia Maxilofacial

rev port estomatol med dent cir maxilofac. 2021;62(1):42-49

Case Report

Oral findings in Coffin-Siris syndrome: A case report

Heliton Siqueira Figueira, Pollyanna Oliveira Medina, Giorge Pessoa de Jesus,
Aida Renée Assayag Hanan, Emílio Carlos Sponchiado Júnior*, Simone Assayag Hanan

Dental School, Federal University of Amazonas, Manaus, Amazonas, Brazil

a r t i c l e i n f o a b s t r a c t
Article history: This paper reports the oral health approach of a child with Coffin-Siris syndrome. This
Received 18 August 2020 syndrome is a multisystem congenital anomaly caused by mutations in genes of BRG-
Accepted 11 March 2021 1-associated factors complex. Individuals with this syndrome have been described with
Available online xx March 2021 hypoplasia or aplasia of the fifth digit nails or phalanges. Other features include growth
deficiency, developmental and intellectual delay, and other organ-system abnormalities.
Keywords: Clinical examination revealed gingival hyperplasia in the upper arch, dental biofilm,
Case report and dental caries on the lower deciduous and permanent molars. Guidelines on oral
Coffin-Siris syndrome hygiene and dietary habits were provided to the guardians, and tooth extraction and
Dental care for children restoration with glass-ionomer cement were performed. This case suggests that these
Fifth-digit syndrome patients require home care and periodic dental consultations for preventive and sys-
tematic dental treatment and quality of life improvement. The patient is being moni-
tored, and her oral condition has improved. (Rev Port Estomatol Med Dent Cir Maxilofac.
2021;62(1):42-49)
© 2021 Sociedade Portuguesa de Estomatologia e Medicina Dentária.
Publicado por SPEMD. Este é um artigo Open Access sob uma licença CC BY-NC-ND
(http://creativecommons.org/licenses/by-nc-nd/4.0/).

* Corresponding author.
E-mail address: spemilio@ufam.edu.br (Emílio Carlos Sponchiado Júnior).
http://doi.org/10.24873/j.rpemd.2021.03.826
1646-2890/© 2021 Sociedade Portuguesa de Estomatologia e Medicina Dentária. Published by SPEMD.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).