Dental management of a child with EEC syndrome

Abstract

Summary. The EEC syndrome is a condition characterized by ectrodactyly, ectodermal dysplasia and clefting of the lip and/or palate. Here we report a case of a boy with the EEC syndrome. The clinical and radiologic aspects of this condition, as well as the dental treatment, are reviewed. Résumé. La dysplasie ectodermique, l'ectrodactyle et la fente palatine et labiale sont caractéristiques d'un syndrôme. L'aspect clinique, radiologique et le traitement dentaire d'un patient atteint de ce syndrôme, sont étudiés dans cet article. Zusammenfassung. Das EEC Syndrom ist charakterisiert durch Ektrodaktylie, ektodermale Dysplasie und durch Lippenoder und Gaumenspalten. In diesem Bericht wird ein Fall mit EEC Syndrom beschrieben, die klinischen und röntgoloischen Aspekte dieses Syndromes, ebenso die zahnärztliche Behandlung dieses Falles. Resumen. El sindrome EEC (EDF) es una condición caracterizada por ectrodactilidad, displasia ectodérmica y fisura (hendidura) del labio y/o paladar. Se reporta un caso con el síndrome EEC y los aspectos clínicos y radiográficos de la condición así como el tratmiento odontológico realizado.

Dental and Maxillofacial Characteristics of Six Japanese Individuals with Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome

“…Oral characteristics of EEC syndrome include abnormalities in tooth structure, microdontia, tooth agenesis, and taurodontia (Buss et al, 1995). In addition, individuals with EEC are frequently affected by xerostomia, accompanied by multiple caries (Tanboga et al, 1992). Data on dental abnormalities associated with EEC are sparse compared with the data on distal limb anomalies, cleft lip and/or palate, and abnormalities of the skin and hair.…”

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

“…Principal characteristics, numerical data, and findings/conclusions from each article were summarized in Table 1. [1][2][3][4][5]7,[13][14][15][16][17][18][19][20][21]…”

“…The publication dates ranged from 1992 to 2020. Principal characteristics, numerical data, and findings/conclusions from each article were summarized in Table 1 1–5,7,13–21 …”

“…14 A familial tendency of the syndrome, present most times, suggests an autosomal dominant inheritance, and there is an abnormal gene on one of the autosomal (nonsex) chromosomes from either parent, with incomplete penetrance; however, the autosomal recessive inheritance should not be discarded. 2,5,13,16 Besides, the p63 gene protein (3q27-29) expression is necessary for limb formation and epidermal morphogenesis of different organs and tissues (teeth, hair, and sweat glands). 7,19 Thus, the impairment of the DNA-binding domain or point heterozygous mutations of the gene are the principal causes of EEC syndrome.…”

“…27 Likewise, the purposes of placing a partial or total removable prosthetic denture are to improve the masticatory function and esthetic appearance and to prevent malocclusions, atypical lip/tongue habits, psychological and social inferiority complex, inadequate food selection, speech or language alterations, and atrophy of the alveolar bone. 13 Surgical closure and reconstruction of cleft lip are indicated at around 10 weeks of age, and the closure of cleft palate or palatoplasty is accomplished between 12 and 24 months of age in order to facilitate the acquisition of a normal speech. Primary bone grafting for alveolar cleft defects can be carried out before 2 years old; secondary grafting for reconstruction of residual alveolar defects is performed from 2 years old up to 15 years old.…”