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

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.

Prosthetic rehabilitation of a patient with ectrodactyly‐ectodermal dysplasia‐cleft lip/palate syndrome through a hybrid workflow: A case report with 2‐year follow‐up

“…In addition to restorative surgeries and orthodontic treatments, removable prosthetic appliances are preferred in early childhood due to the growth of the jawbones. Relining, rebasing and remaking of the denture are necessary to accommodate the normal growth changes 11 …”

“…Relining, rebasing and remaking of the denture are necessary to accommodate the normal growth changes. 11 Oral management of EEC syndrome in children and adolescents is quite difficult because the patient exhibits different anomalies in the oral cavity. 20,21 Removable prosthetic appliances are the most frequently reported type of dentures of EEC patients.…”

“…Two forms of EEC are categorized according to clinical perspective: cleft lip with or without cleft palate, and cleft palate alone. 11 Patients with EEC syndrome require both esthetic and functional dental rehabilitation. [16][17][18]…”

“…The prevalence of EEC syndrome has been calculated around 1.5 per 100 million live births, but the exact incidence of this disorder in the whole population is unknown 11–14 …”

“…The prevalence of EEC syndrome has been calculated around 1.5 per 100 million live births, but the exact incidence of this disorder in the whole population is unknown. [11][12][13][14] EEC syndrome affects males and females in equal numbers. According to Balci et al, 15 there are three identified types of EEC with different gene loci: (1) OMIM 129900, assigned to 7q11.2-q21.3; (2) MIM 602077, assigned to chromosome 19; and (3) OMIM 604293, assigned to 3q27.…”