Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases.

Patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy. This disorder is inherited as an X-linked trait. This report presents three cases with HED in which the clinical evaluation (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, Faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM examination of hair demonstrated a distinctly abnormal longitudinal grooving along the entire length of each hair and a desquamation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointegrated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.

Growth analysis of a patient with ectodermal dysplasia treated with endosseous implants: 6-year follow-up.

The purpose of this article is to report the clinical course and 6-year follow-up of a child with ectodermal dysplasia who was treated with implants surgery very early. This article reports placement of mandibular endosseous implants in a 4-year-old patient with hypohidrotic ectodermal dysplasia and oligodontia. This congenital anomaly does not appear to retard healing and the osseointegration remains after 6 years and 3 months of loading. Mandibular and maxillary skeletal growth and development was normal. However, because of lack of alveolar growth, in time, patient's vertical growth pattern changed to low angle. This could be corrected by changing the vertical heights of the abutment and prosthesis. As a result, in ectodermal dysplasias cases with anadontia, early implant placement and fixed prosthesis could be a good multidisciplinary treatment option for poor cooperative child.

The effects of a modified protraction headgear on maxilla.

Protraction headgears are commonly used in the treatment of Class III malocclusion characterized by maxillary retrognathism. The upward and forward rotation of the maxilla during protraction is a major unwanted side effect. The aim of this study was to eliminate the upward and forward rotation of maxilla while protracting. Seventeen patients with Class III malocclusion as a result of maxillary retrognathism were treated for 3 months; their average age was 12.81 years. A full coverage acrylic cap splint-type rapid maxillary expansion appliance was cemented and activated twice a day for 5 days. After sutural separation, a maxillary modified protraction headgear was worn and 750 g of force was applied. Wilcoxon signed rank test was carried out to evaluate 42 parameters measured on cephalometric radiographs. The maxilla was displaced anteriorly by downward and backward rotation. The mandible was displaced downward and backward due to anterior elongation of the maxilla. Extrusion and lingual tipping of the upper incisors and intrusion of upper molars and downward and backward rotation of functional occlusal plane were observed. The aim of our study was achieved, which was to avoid upward and forward rotation while protracting the maxilla. In conclusion, maxillary modified protraction headgear (MMPH) can be used effectively in Class III patients with retrognathic maxilla and anterior open bite tendency.