Dento-maxillo-facial phenotype and implants-based oral rehabilitation in Ectodermal Dysplasia with WNT10A gene mutation: report of a case and literature review.
J Craniomaxillofac Surg
; 42(6): e346-51, 2014 Sep.
Article
em En
| MEDLINE
| ID: mdl-24702986
ABSTRACT
PURPOSE:
To report the dento-craniofacial phenotype of a family affected by a WNT10A HED and to describe the implant-based oral rehabilitation of a patient presenting a severe oligodontia linked to this mutation. A molecular hypothesis concerning the involvement of Wnt-ß-catenin pathway in implant osteointegration will be proposed. MATERIAL ANDMETHODS:
Patients affected by a WNT10A mutation were included from a large group of HED patients. WNT10A gene was sequenced in second intention for patients negative for EDA-EDAR-EDARADD mutations. Dento-craniofacial phenotype was described based on clinical and radiological data.RESULTS:
Severe oligodontia was observed in the patient affected by a compound heterozygous mutation of WNT10A gene. CT exams showed marked maxillary bone hypoplasia in the posterior areas with a sub-normal mandible treatment consisted in the placement of 4 mandibular implants and in 2 implant-supported bridges. In the maxilla, an autogenous bone graft was indicated. The post-operative radiological follow-up showed partial bone resorption of the grafts, treated with ramus bone shaving and a membrane, followed by the placement of 4 maxillary implants.CONCLUSION:
Patients affected by WNT10A HED require multi-disciplinary dental diagnosis and treatment. A close post-operative radiological follow-up appears necessary given the biological functions of Wnt-ß-catenin in bone repair.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Displasia Ectodérmica
/
Implantes Dentários
/
Prótese Dentária Fixada por Implante
/
Mutação de Sentido Incorreto
/
Proteínas Wnt
/
Anodontia
/
Reabilitação Bucal
Tipo de estudo:
Observational_studies
/
Prognostic_studies
Limite:
Humans
/
Male
Idioma:
En
Revista:
J Craniomaxillofac Surg
Ano de publicação:
2014
Tipo de documento:
Article