An evaluation of clinical, radiological and three-dimensional dental tomography findings in ectodermal dysplasia cases

Abstract

BackgroundThis study aimed to review the results related to head and jaw disorders in cases of ectodermal dysplasia. The evaluation of ectodermal dysplasia cases was made by clincal examination and examination of the jaw and facial areas radiologically and on cone-beam 3-dimensional dental tomography (CBCT) images.Material and MethodsIn the 36 cases evaluated in the study, typical clinical findings of pure hypohidrotic ectodermal displasia (HED) were seen, such as missing teeth, dry skin, hair and nail disorders. CBCT images were obtained from 12 of the 36 cases, aged 1.5- 45 years, and orthodontic analyses were made on these images.ResultsThe clinical and radiological evaluations determined, hypodontia or oligodontia, breathing problems, sweating problems, a history of fever, sparse hair, saddle nose, skin peeling, hypopigmentation, hyperpigmentation, finger and nail deformities, conical teeth anomalies, abnormal tooth root formation, tooth resorption in the root, gingivitis, history of epilepsy, absent lachrymal canals and vision problems in the cases which included to the study.ConclusionsEctodermal dysplasia cases have a particular place in dentistry and require a professional, multi-disciplinary approach in respect of the chewing function, orthognathic problems, growth, oral and dental health. It has been understood that with data obtained from modern technologies such as three-dimensional dental tomography and the treatments applied, the quality of life of these cases can be improved. Key words: Ectodermal dysplasia, three-dimensional dental tomography.

Ectodermal dysplasia with classical clinical presentation: A rare case report

“…In the hydrotic type, which is transferred as autosomal dominant, the sweat and adipose glands are normally formed. [5][6][7][8] Although Thurnam published the first report of a patient with ectodermal dysplasia in 1848, the term ectodermal dysplasia was coined by Weech in 1929. 9 Freire-Maia and Pinheiro proposed the first classification system of ectodermal dysplasias in 1982, 10 with additional updates in 1994 and 2001.…”

“…Many patients are not diagnosed until infancy or childhood, when dental anomalies, nail abnormalities, or alopecia become apparent. 5 Abnormalities of two or more ectodermal structures such as the hair, teeth, nails, sweat glands, salivary glands, craniofacial structure, digits and other parts of the body are seen. 11 Characteristic features include the following: Diagnosis is usually based on clinical features.…”

“…In some types, mesodermal abnormalities are also present. 5 Ectodermal dysplasias can occur in any race but are much more prevalent in caucasians than any other group and especially in fair caucasians. 1 Ectodermal dysplasia rarely occurs in this region.…”

Etiología, manifestaciones clínicas y estudio por imágenes de la displasia ectodérmica: Una revisión de la literatura

“…El procedimiento del radiólogo en la realización de estudios por imágenes considerará el beneficio en cuanto al diagnóstico y tratamiento de tecnologías vanguardistas como la TCBC. Se deberá informar al paciente sobre el o los estudios a realizar, mediante el consentimiento informado, que deberá ser rubricado por el paciente, para el diagnóstico y tratamiento (48).…”

“…La CBCT es el medio de diagnóstico más exacto y confiable porque se acerca a la realidad, sus mediciones en tratamientos de ortodoncia son los más adecuados, su uso debería ser limitado en etapas María Mercedes Rodríguez iniciales del diagnóstico en pacientes de baja edad (48). En las mutaciones genéticas más severas WNT1, el CBCT está indicado en el defecto óseo grave del maxilar, permite evaluar la densidad ósea y determinar la zona que contenga mayor soporte para la colocación de implantes, y en aquellas de menor soporte para la realización de injertos óseos, con sus correspondientes controles de volumen óseo y la osteointegración de la implantología (49).…”