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. 2023 Mar;43(2):152-162.
doi: 10.1111/scd.12752. Epub 2022 Jul 25.

Oral management of children/adolescents with ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome: A scoping review

Arturo Garrocho-Rangel  1 , Guadalupe Serrano-Aguilar  1 , Yolanda Hernández-Molinar  1 , Saray Aranda-Romo  1 , Vanessa Alejandri-Gamboa  1 , Amaury Pozos-Guillén  1
Affiliations

Oral management of children/adolescents with ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome: A scoping review

Arturo Garrocho-Rangel et al. Spec Care Dentist. 2023 Mar.

Abstract

Aims: EEC is a rare syndrome characterized by the triad of ectrodactyly, ectodermal dysplasia, and orofacial clefting, along with other clinical manifestations mainly in hair, skin, and teeth. The present paper aimed to perform a scoping review to collect the most relevant studies and focused on the diagnosis and oral management of EEC syndrome in the pediatric dental setting. This review also pretended to make recommendations and map the gaps in this clinical topic.

Methods: An exhaustive electronic and manual search was conducted in four databases (PubMed, EMBASE, Google Scholar, and Dentistry & Oral Sciences Source/EBSCO) according to previously established eligibility criteria, using different combinations of keywords, MeSH terms, and Boolean operators. Titles, abstracts, and full-text articles were screened and selected by precalibrated reviewers. A data charting was also accomplished for summarizing the overview of the evidence.

Results: A total of 37 references were identified, and 32 titles remained after removing duplicates; then, 25 potential full-text articles were carefully reviewed. Finally, 15 relevant and most informative studies were included. Most studies were single clinical case reports. Only one descriptive retrospective study was detected. None randomized clinical trials or comparative observational studies were found. A medical/dental multidisciplinary approach is needed for the management of EEC syndrome.

Conclusions: Diverse dental specialists must be involved. Pediatric dentists must play a principal role in the prevention and treatment of oral diseases; particularly the preservation of the primary and mixed dentitions, trying to achieve normal orofacial growth.

Keywords: EEC syndrome; adolescents; children; oral management.

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References

REFERENCES

    1. Marwaha M, Singh-Nanda KD. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome). Contemp Clin Dent. 2012;3(2):205-208.
    1. Koul M, Dwivedi R, Upadhyay V. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome). J Oral Biol Craniofac Res. 2014;4(2):135-139.
    1. Pettit S, Regener-Campbell RDH. Ectrodactyly-ectodermal dysplasia-clefting syndrome: the oral hygiene management of a patient with EEC. Spec Care Dentist. 2010;30(6):250-254.
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