Thomas Michael Präger1, Christian Finke, Rainer-Reginald Miethke. 1. Institut für Kieferorthopädie, Orthodontie und Kinderzahnmedizin Centrum für Zahn-, Mund- und Kieferheilkunde, Charité-Universitätsmedizin Berlin, Assmannshauser Str. 4-6, 14197 Berlin, Germany. thomas-michael.praeger@charite.de
Abstract
BACKGROUND: Ectodermal dysplasia is an inherited disease causing malformations of all tissues originating from the ectoderm. The significance of this disease lies in severe hypodontia, and an accompanying hypoplasia of the alveolar process. The clinical situation is aggravated by a significant xerostomia. It was the aim of this study to document the distribution of hypodontia and tooth malformation. Furthermore, we aimed to elucidate the clinical impact of these findings. PATIENTS AND METHODS: Records of 30 patients (19 males, 11 females) suffering from ectodermal dysplasia were included. Their age ranged between 7 and 23 years. All patients had been examined clinically and radiographically. In every patient, a record was made of which teeth were missing or malformed, and which deciduous teeth persisted. Additionally, the entire treatment procedure was assessed. RESULTS: The third molars were missing in all of the patients. The number of aplastic permanent teeth ranged from 2 to 26. The maxillary lateral incisors were most frequently absent, followed by the mandibular central incisors. The most stable teeth were the central incisors of the upper jaw, and the canines and first molars in both jaws. However, the maxillary central incisors and canines were the teeth most affected by malformation. Deciduous canines and second molars were the most often persisting teeth due to agenesis of the maxillary lateral permanent incisors and mandibular second premolars. In two-thirds of the patients, missing teeth were replaced by removable dentures. Half of the patients received orthodontic treatment. CONCLUSIONS: Hypodontia and malformation are almost regular dental characteristics in patients suffering from ectodermal dysplasia. The distribution of absent teeth deviates remarkably from the general population. Treatment requires an interdisciplinary approach including orthodontics, prosthodontics and oral surgery.
BACKGROUND:Ectodermal dysplasia is an inherited disease causing malformations of all tissues originating from the ectoderm. The significance of this disease lies in severe hypodontia, and an accompanying hypoplasia of the alveolar process. The clinical situation is aggravated by a significant xerostomia. It was the aim of this study to document the distribution of hypodontia and tooth malformation. Furthermore, we aimed to elucidate the clinical impact of these findings. PATIENTS AND METHODS: Records of 30 patients (19 males, 11 females) suffering from ectodermal dysplasia were included. Their age ranged between 7 and 23 years. All patients had been examined clinically and radiographically. In every patient, a record was made of which teeth were missing or malformed, and which deciduous teeth persisted. Additionally, the entire treatment procedure was assessed. RESULTS: The third molars were missing in all of the patients. The number of aplastic permanent teeth ranged from 2 to 26. The maxillary lateral incisors were most frequently absent, followed by the mandibular central incisors. The most stable teeth were the central incisors of the upper jaw, and the canines and first molars in both jaws. However, the maxillary central incisors and canines were the teeth most affected by malformation. Deciduous canines and second molars were the most often persisting teeth due to agenesis of the maxillary lateral permanent incisors and mandibular second premolars. In two-thirds of the patients, missing teeth were replaced by removable dentures. Half of the patients received orthodontic treatment. CONCLUSIONS:Hypodontia and malformation are almost regular dental characteristics in patients suffering from ectodermal dysplasia. The distribution of absent teeth deviates remarkably from the general population. Treatment requires an interdisciplinary approach including orthodontics, prosthodontics and oral surgery.