A Odontodisplasia Regional (ROD) é uma alteração localizada no desenvolvimento dos dentes.
Introdução
O que você precisa saber de cara
A Odontodisplasia Regional (ROD) é uma alteração localizada no desenvolvimento dos dentes.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 4 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Odontodisplasia regional
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Publicações mais relevantes
Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.
This study investigates the microstructural and mechanical characteristics of primary teeth affected by ROD. A total of two ROD-affected primary teeth from two different cases underwent clinical examinations. In addition to control samples of caries-free retained primary teeth, the affected samples were examined using micro-computed tomography (micro-CT), X-ray diffraction (XRD), scanning electron microscopy (SEM), energy-dispersive X-ray spectroscopy (EDS), transmission electron microscopy (TEM), and nanoindentation analysis. Clinical findings revealed yellowish discoloration, rough surfaces, and root resorption on the affected teeth. Radiographs indicated hypocalcified enamel, widened pulp chambers, and delayed development of permanent teeth. Micro-CT showed affected teeth with thinner and uneven enamel, disordered dentin, and reduced mineral density. XRD analysis found reduced crystallinity. SEM and TEM analyses revealed hypoplastic and loosely packed enamel crystals, whereas dentin exhibited disorganized collagen fibrils and poorly mineralized crystals. EDS analysis showed a reduced calcium/phosphorus ratio and an increased magnesium/calcium ratio in the affected enamel. Nanoindentation tests found reduced hardness and elastic modulus in ROD-affected enamel compared with control teeth. ROD-affected primary teeth display significant microstructural abnormalities and compromised mechanical properties, underscoring the need for early intervention and long-term monitoring to prevent complications.
Regional odontodysplasia: a report of unusual case with in-depth analysis.
Regional odontodysplasia (RO) is a rare developmental anomaly affecting dental tissue formation, typically confined to a single quadrant. It manifests through hypoplastic enamel, irregular dentin, short roots with open apices, and a characteristic "ghost-like" radiographic appearance. Its etiology remains unclear, with vascular, genetic and inflammatory factors proposed. In this study, we report the case of a 15-year-old female with mandibular RO affecting canine, premolars, and molars, focusing on the clinical, radiographic, and microscopic analysis of the affected teeth, including mineral density evaluation. The 2nd premolar and first and second molars (teeth 35, 36, 37) exhibited severe morphological defects and were extracted due to extreme root shortening (40 %, 90 %, and >95 % reduction), while alveolar bone height remained preserved. The canine and 1st premolar were affected to a lesser extent and were conserved upon orthodontic treatment plan. Scanning electron microscopy revealed slight decreases in the number and diameter of the dentinal tubules in the affected teeth compared with those in the control teeth. Contrary to many reported cases of RO, mineral density analysis of enamel and dentin revealed no significant differences between affected and normal teeth. The observations reported in this case suggest that resorptive changes, possibly influenced by inflammatory factors, could contribute to the observed root anomalies. The preservation of alveolar bone height provides a favourable prognosis for prosthetic rehabilitation. These findings help elucidate the structural and functional implications of ROs. This study highlights the importance of early diagnosis and multidisciplinary management for optimal outcomes.
Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.
Regional odontodysplasia (RO) is a rare developmental dental anomaly with unknown prevalence. Current knowledge is largely limited to individual case reports. This study aims to present epidemiological data, clinical features, and radiographic characteristics of pediatric and adolescent patients with RO in South Korea. In this multicenter cohort study, we prospectively and retrospectively recruited participants from 11 university dental hospitals. Patients aged < 19 years diagnosed with RO were included in this study. In prospective cases, epidemiological information, family history, and clinical and radiographic data were collected after obtaining parental consent. All data were anonymized and stored using a study-specific data management system. Age at diagnosis ranged from 2 to 12 years, with a higher incidence in males. RO was more commonly observed in the maxilla, and all patients exhibited delayed eruption and development. The treatment approaches vary widely and include observation, extraction, pulp therapy, crown restoration, prosthetic rehabilitation, and orthodontic treatment. This multicenter study provides the first comprehensive dataset on RO in Korean children and adolescents. These findings will contribute to the development of clinical guidelines and inform future healthcare policies for the management of rare dental anomalies.
Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.
Regional odontodysplasia (RO) is a rare developmental anomaly involving both mesodermal and ectodermal dental tissues. It is characterized by hypoplasia and hypomineralization of enamel and dentin affecting a group of neighboring teeth and may involve primary, permanent dentition or both. Wide pulp chambers and thin, poorly defined hard tissue outlines, described as "ghost teeth," are typical radiographic features. A case of the long-term management of a young girl suffering from RO in the left maxilla is described in the present report. The patient was 3 years old when first presented in the clinic and was treated for the subsequent 12 years by a multidisciplinary team comprised of a pediatric dentist, orthodontist, and prosthodontist. The report encompasses a detailed description of the clinical and radiographic findings of the patient. It also describes the prosthodontic treatment applied to alleviate the aesthetic handicap brought by the extraction of the affected teeth, as well as the orthodontic interventions to establish the normal eruption and settlement of the remaining dentition. A continuing and steady follow-up of RO cases is necessary in order to keep pace with the needs arising from the loss of affected teeth and to ensure the normal growth of the oral issues.
New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.
Diseases that affect teeth can change their structure and the distribution and expression of amelogenin (AMELX) and dentin-sialophosphoprotein (DSPP). This study aimed to conduct a histopathological, ultrastructural, and immunohistochemical comparison of AMELX and DSPP in teeth from patients with amelogenesis imperfecta (AI), dentinogenesis imperfecta (DI), osteogenesis imperfecta (OI), regional odontodysplasia (ROD), and control teeth. Additionally, a model of the structure of the affected primary teeth is proposed. This case series study examined 27 affected teeth with various diseases and 14 control teeth. Some teeth were analysed using light microscopy, polarised light, and scanning electron microscopy, while others underwent decalcification for histological analysis and immunohistochemistry with AMELX and DSPP antibodies. Teeth with hypoplastic AI exhibited thin enamel and abnormal or absent prismatic structure. In hypomineralised AI, the prismatic structure displayed minor alterations, while severe cases revealed remnants of enamel matrix and the presence of amelogenin. Teeth showing both types of AI and controls presented DSPP in peritubular dentin. DI and OI cases showed reduced dentinal tubule density, with loss of parallelism and varying diameters. The principal difference between DI and OI was seen with anti-DSPP antibody; in OI, a strong peritubular and intertubular immunolabeling was observed, while DI displayed minimal labeling in peritubular dentin. In ROD, enamel and dentin were thin with irregular prisms and tubules, with anti-AMELX and anti-DSPP demonstrating mild immunostaining. The study of teeth with AI, DI, OI, and ROD contributes to differentiating these pathosis, with DSPP immunostaining particularly distinguishing DI from OI.
Publicações recentes
Regional odontodysplasia: a report of unusual case with in-depth analysis.
Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.
Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.
Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.
New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.
📚 EuropePMC117 artigos no totalmostrando 41
Regional odontodysplasia: a report of unusual case with in-depth analysis.
Journal of oral biology and craniofacial researchMicrostructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.
Journal of applied oral science : revista FOBLong-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.
Case reports in dentistryEpidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.
International journal of paediatric dentistryNew Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.
International dental journalRegional Odontodysplasia in Primary Teeth: A Case Report.
CureusRegional Odontodysplasia: A Case Report Crossing the Midline with Implantoprosthetic Rehabilitation.
Journal of pharmacy & bioallied sciencesClinicial-pathologic correlations of non-trauma related Odontodysplasia in 28 dogs: 2013-2023.
Frontiers in veterinary scienceAbnormal dental follicle cells: A crucial determinant in tooth eruption disorders (Review).
Molecular medicine reportsPHACES syndrome and multi-regional odontodysplasia: a case report.
The Journal of clinical pediatric dentistry[Research progress in regional odontodysplasia].
Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatologyRegenerative Endodontic Procedures in Immature Teeth Affected by Regional Odontodysplasia.
Journal of endodonticsRegional odontodysplasia with facial cellulitis: a case report and literature review.
Hua xi kou qiang yi xue za zhi = Huaxi kouqiang yixue zazhi = West China journal of stomatologyDevelopmental Anomalies in Human Teeth: Odontoblastic Differentiation in Hamartomatous Calcifying Hyperplastic Dental Follicles Presenting with DSP, Nestin, and HES1.
Journal of developmental biologyRegional odontodysplasia diagnosed and treated via multidisciplinary approach: a case report.
The Journal of clinical pediatric dentistryA Review of Selected Dental Anomalies With Histologic Features in the Pediatric Patient.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyRegional odontodysplasia on the left side of the mandible: report of an unusual case.
The Journal of clinical pediatric dentistryMandibular Regional Odontodysplasia in a Toddler.
Journal of dentistry for children (Chicago, Ill.)Regional Odontodysplasia with Actinomycosis Infection: A Rare Case Report.
International journal of clinical pediatric dentistryRehabilitation of Maxillary Bilateral Regional Odontodysplasia in a 7-Year-Old Child Using Modified Essix Retainer.
Contemporary clinical dentistryCone-beam computed tomography in the diagnosis of regional odontodysplasia.
Clinical case reportsRegional Odontodysplasia: A Systematic Review of Case Reports.
International journal of environmental research and public healthMandibular premolar transplantation to replace missing maxillary anterior teeth: A multidisciplinary approach.
American journal of orthodontics and dentofacial orthopedics : official publication of the American Association of Orthodontists, its constituent societies, and the American Board of OrthodonticsTreatment Strategies for Patients with Regional Odontodysplasia: A Presentation of Seven New Cases and a Review of the Literature.
Oral health & preventive dentistry[Regional odontodysplasia: a report of three cases].
Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatologyRegional odontodysplasia: An analysis of 161 cases from 1953 to 2017.
The Saudi dental journalOral rehabilitation of a case with regional odontodysplasia using a regenerative approach-A case report and a review of literature.
Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric DentistryRegional Odontodysplasia Affecting the Maxilla.
Head and neck pathologyFamilial oligodontia and regional odontodysplasia associated with a PAX9 initiation codon mutation.
Clinical oral investigationsManagement of regional odontodysplasia: a 10-year-follow-up case report and literature review.
European oral researchRegional Odontodysplasia Crossing the Midline.
Journal of dentistry for children (Chicago, Ill.)Prevalence of dental anomalies in French orthodontic patients: A retrospective study.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieMandibular Regional Odontodysplasia in an 8-year-old Boy showing Teeth Disorders, Gubernaculum Tracts, and Altered Bone Fractal Pattern.
International journal of clinical pediatric dentistryCervicofacial infection in a 3-year-old child with regional odontodysplasia: A case report and literature review.
Journal of oral and maxillofacial pathology : JOMFPRegional Odontodysplasia with Generalised Enamel Defect.
Case reports in dentistryClinical, Radiographic, and Histologic Evaluation of Regional Odontodysplasia: a Case Report with 5-year Follow-up.
Journal of dentistry (Shiraz, Iran)Role of inflammation in developmental odontogenic pathosis.
Journal of oral and maxillofacial pathology : JOMFPInflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority.
Indian journal of dentistryMalformations of the tooth root in humans.
Frontiers in physiologyGhost teeth: Regional odontodysplasia of maxillary first molar associated with eruption disorders in a 10-year-old girl.
Journal of pharmacy & bioallied sciencesRegional odontodysplasia: Report of an unusual case involving mandibular arch.
Contemporary clinical dentistryAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.
- Regional odontodysplasia: a report of unusual case with in-depth analysis.
- Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.
- Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.
- New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:83450(Orphanet)
- MONDO:0019367(MONDO)
- GARD:19036(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q3349339(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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