Dentinal dysplasia type I

Dentinal dysplasia is one of the rarest hereditary disturbances of dentin formation characterized by the teeth present with short blunt roots, considerably reduced or obliterated pulp spaces, periapical abscess or cysts without an obvious causative factor, and spontaneous exfoliated. These features were observed on a patient referred for prosthodontic assessment, and an investigation of other members of the family revealed that the father and two siblings were also affected. The report described the clinical, radiographic, histologic and scanning electron microscopic features and discussed the features which separated from dentinogenesis imperfecta.     

Dentinal dysplasia type I. A subclassification

Dentinal Dysplasia Type I is a rare condition affecting the deciduous and permanent dentitions, in which the teeth present with short roots and considerably reduced or obliterated pulp spaces. These features were observed on the radiographs of a patient referred for orthodontic assessment, and an investigation of other members of the family revealed that the father and two siblings were also affected. There were variations in the lengths of the roots in those affected, and thus a subclassification of the dysplasia is proposed.     

Dentinal dysplasia, Type I. Report of a case with endodontic therapy.

The first reported case of endodontic therapy in dentinal dysplasia, Type I, is described. The first histologic diagnosis of periapical granuloma instead of periapical cyst in dentinal dysplasia, Type I, is also reported.     

Hereditary pattern for dentinal dysplasia type Id: a case report

Generalized pulpal calcifications arouse suspicion of diseases or conditions of systemic or hereditary origin. This case report describes a 45-year-old patient with generalized pulpal calcifications and bulging of the roots in areas corresponding to the pulp chambers in otherwise normal teeth. Similar findings were present in the patient's daughters and brother. This pattern of pulpal calcifications is consistent with the hereditary condition of dentinal dysplasia type Id.     

Cleidocranial dysplasia: a case report

Cleidocranial dysplasias is an autosomal dominant disorder that presents with skeletal dysplasia. The dental manifestations are mainly delayed exfoliation of primary teeth and delayed eruption of permanent teeth, with multiple impacted supernumeraries. This report addresses the complex nature of the treatment modalities. In our patient, surgical exposure of unerupted teeth was done with orthodontic traction. Post-surgical follow-up was uneventful.     

Chondroectodermal dysplasia: a case report

Chondroectodermal Dysplasia is a disease complex consisting of bilateral manual polydactyly, chondrodysplasia of long bones resulting in acromelic dwarfism, hydroitic ectodermal dysplasia affecting principally the nails, teeth and hair and congenital heart malformations. It is necessary to identify this disease at its early stage in order to render prompt treatment. The oral manifestations are characteristic for this particular disease so that a dental surgeon can identify this condition and refer the case to a cardiologist and orthopedician for corrective surgeries. In this article, a case of a 2 1/2-year old child with chondro-ectodermal dysplasia is presented along with radiographic investigations and treatment plan.     

少汗型外胚层发育不全1例

本文报道1例外胚层发育不全病例,并分析外胚层发育不全的临床表现、发病原因及遗传方面的影响因素等.     

Florid cemento-osseous dysplasia: a case report

Cemento-osseous dysplasias are a group of disorders known to originate from periodontal ligament tissues and involve, essentially, the same pathological process. They are usually classified, depending on their extent and radiographic appearances, into three main groups: periapical (surrounds the periapical region of teeth and are bilateral), florid (sclerotic symmetrical masses) and focal (single lesion) cemental dysplasias. Florid cemento-osseous dysplasia clearly appears to be a form of bone and cemental dysplasia that is limited to jaws. Patients do not have laboratory or radiologic evidence of bone disease in other parts of the skeleton. For the asymptomatic patient, the best management consists of regular recall examinations with prophylaxis and reinforcement of good home hygiene care to control periodontal disease and prevent tooth lose. Management of the symptomatic patient is more difficult. At this stage, there is an inflammatory component to the disease and the process is basically a chronic osteomyelitis involving dysplastic bone and cementum. Antibiotics may be indicated but may not be effective. A case of florid cemento-osseous dysplasia occurring in a 47-year-old Caucasian female is reported which was rare in regard to race and sex.     

Hereditary ectodermal dysplasia: a case report

Hereditary ectodermal dysplasia is an inherited disorder involving skin, hair, nails and teeth. Two main clinical forms have been described--hypohidrotic type and hidrotic type. A case of ectodermal dysplasia with absence of hypohidrosis and defective nails has been reported. The importance of early prosthetic management has been discussed.     

Facial fibrous dysplasia: report of a case

A case of facial fibrous dysplasia that involved the left frontal, temporal, malar, and mandibular regions is reported. Conservative recontouring was done several times when the patient was 16 to 23 years of age.     

Craniometaphyseal dysplasia: case report

Craniometaphyseal dysplasia is a rare genetic bone remodeling disorder characterized by undertubulation of the long bones, especially in the lower extremities, causing deformities of the metaphyses of the long bones, and sclerosis of the skull base or cranial bone hyperostosis. The authors report a case of craniometaphyseal dysplasia in an 8-year-old Brazilian child, emphasizing the importance of precocious diagnosis of this rare genetic disorder.     

Binder's maxillo-nasal dysplasia: report of a case.

Case report of a 20 year old female patient who presented with Binder's maxillonasal dysplasia. She presented with completely missing premaxilla, nasal bone and septum, there was however no associated cardiac abnormalities, mental retardation or other skeletal deformities. Her principal complaint was the associated missing incisors. The incisors and the premaxilla were satisfactorily replaced with a removable maxillofacial prosthesis.