Multidisciplinary treatment plan for multiple dental ageneses associated with hypohidrotic ectodermal dysplasias

Abstract

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

Oral rehabilitation of a child with hypohidrotic ectodermal dysplasia: six years follow-up study

“…The treatment of the individuals with HED is very complex, engaging several specialists: neonatologist, pediatrician, otolaryngologist, pulmonologist, ophthalmologist, dermatologist, plastic surgeon, speech therapist, and psychologist. Regarding the teeth disorders, pedodontist, orthodontist, oral surgeon and prosthodontist should be engaged, as well as maxillo-facial surgeon in the cases of skeletal malocclusions, and cleft lip and/or palate 13 .…”