J Dentofacial Anom Orthod
Volume 17, Number 1, 2014Rare disorders and Dentofacial Orthopedics
|Number of page(s)||13|
|Published online||25 September 2014|
Multidisciplinary treatment plan for multiple dental ageneses associated with hypohidrotic ectodermal dysplasias
Address for correspondence: F. CLAUSS Unité Fonctionnelle d’Odontologie Pédiatrique, Faculté de Chirurgie Dentaire de Strasbourg, Pôle de Médecine et de Chirurgie Bucco-Dentaires, francois.clauss@chru-strasbourg
Hypohidrotic ectodermal dysplasias (HED) are a heterogeneous and complex group of syndromes characterized by a dental craniofacial phenotype associated with severe oligodontia, maxillary hypoplasia with broad face and most notably facial concavity. Cephalometric analyses show insufficient maxillary sagittal growth, a protruded mandible, reduced facial and ramus heights, as well as basicranial changes. Early diagnosis and care from a multidisciplinary team are essential. In the primary dentition, initial prosthetic treatment is recommended and may possibly be combined with interceptive orthodontic treatment. In the mixed dentition, treatment of a transverse maxillary deficiency consists in installing a removable expander or a quadhelix in the case of moderate phenotypes where there is adequate anchorage support. To achieve mandibular anterior repositioning of the maxilla the patient must wear a facial mask. In the permanent dentition, the important steps are correlated with the pre-implant and pre-prosthetic orthodontic adjustments, as well as the presurgical orthodontic preparation that precedes subsequent single or double jaw orthognathic surgery. Temporary skeletal anchorage with mini-screws or plates can be used in cases of insufficient anchorage.
Key words: Hypohidrotic ectodermal dysplasia / Craniofacial phenotype / Oligodontia
© RODF / EDP Sciences
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