1/5. Unusual oral findings in dermatosparaxis (ehlers-danlos syndrome type VIIC).A 13-year-old patient with dermatosparaxis (ehlers-danlos syndrome type VIIC), an autosomal recessive disorder of procollagen-I-N-proteinase, is presented. The oral findings comprise micrognathia, hypodontia, localized microdontia, opalescent tooth discoloration, root dysplasia, pulp obliteration, severe gingival hyperplasia, frontal open bite, and severe restriction of TMJ mobility. The reported anomalies suggest the need for expanding the present phenotypic spectrum. This is the first report on oral findings in the syndrome.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
2/5. Malignant infantile osteopetrosis: dental effects in paediatric patients. case reports.AIM: Malignant Infantile osteopetrosis is a hereditary pathology caused due to osteoclastic cells which are incapable of carrying out their functions and hence do not resorb osseous tissue where required. Thus the consequence is that during growth phase, the medullary cavities and nervous tissue cavities do not undergo sufficient growth and the corresponding organs do not develop adequately. The aim of this study is to outline the role of the pediatric dentist who has to carry out protocols of primary, secondary, tertiary prevention intervening at many levels. Clinical features and dental effects are described. Two case reports are presented in this study. CONCLUSION: Oral problems of osteopetrosis are delayed tooth eruption, absence of some teeth, malformed teeth, enamel hypoplasia, disturbed dentinogenesis, hypomineralisation of enamel and dentin, propensity for tooth decay, defects of the periodontal membrane, thickened lamina dura, mandibular protrusion, and the presence of odontomas. Tooth removal should be limited as it may induce bone fractures and osteomyelitis. The role of the pediatric dentist is defined.- - - - - - - - - - ranking = 2keywords = tooth (Clic here for more details about this article) |
3/5. Orofacial features of Scheie (Hurler-Scheie) syndrome (alpha-L-iduronidase deficiency).Scheie syndrome is a rare inborn error of metabolism, a mucopolysaccharidosis in which deficiency of the lysosomal enzyme alpha-L-iduronidase leads to tissue accumulation of mucopolysaccharides. Scheie syndrome is a forme fruste of Hurler syndrome (gargoylism), and some patients have the phenotype of Hurler-Scheie compound syndrome. The craniofacial abnormalities include coarse facies, mandibular condylar hypoplasia, retarded tooth eruption, and cystic jaw radiolucencies--particularly about the molars. Corneal clouding may lead to blindness but, in contrast to some other mucopolysaccharidoses, mental handicap is rare in Scheie syndrome. Cardiac valve incompetence is common, as are recurrent respiratory infections. Two brothers with Hurler-Scheie syndrome are presented and the oral and systemic complications each patient had described. The syndrome is discussed with particular reference to the orofacial features.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
4/5. orthognathic surgery: a significant contribution to facial and dental esthetics.Esthetic dentistry was once a specialty of the family dentist and the orthodontist. Today it is a field for the prosthodontist, periodontist, oral and maxillofacial surgeon, and others. When jawbones are malpositioned, unesthetic facial contours can result, and dental compensations occur that can be additionally unattractive. Orthodontic attempts to correct malocclusion or other disharmonies of the dentition in affected individuals without surgical intervention can cause tooth instability and result in less than satisfactory cosmetic results. The treatment of orthognathic surgeons is especially well suited for full-face esthetic appearance--the picture the patient views each day in the mirror.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
5/5. Primary surgery of skeletal dysgnathias.Three case studies from the patient population of the University Hospital of Aachen (RWTH) are used to describe indications for primary surgical intervention in skeletal dysgnathia. Such preconditions may apply in the case of mandibulo-alveolar protrusion, anomalies where there is little or no option to fixing orthodontic appliances (enamel hypoplasia or shortened crowns), severe transversal discrepancies and skeletal dysgnathia with pronounced malpositioning in the alveolar process and the teeth. The advantages are improved compliance (through the patient experiencing success at the outset of treatment) and limitation of postoperative orthodontic treatment to occlusive fine adjustments of the occlusion, resulting in an appreciable reduction in both the degree and duration of tooth movement and tissue damage.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |