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Odontodisplasia regional
ORPHA:83450CID-10 · K00.4CID-11 · DA07.3DOENÇA RARA

A Odontodisplasia Regional (ROD) é uma alteração localizada no desenvolvimento dos dentes.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Odontodisplasia Regional (ROD) é uma alteração localizada no desenvolvimento dos dentes.

Publicações científicas
136 artigos
Último publicado: 2026 Mar-Apr

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
140
pacientes catalogados
Início
Childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: K00.4
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🦷
Dentes
14 sintomas
😀
Face
2 sintomas
📏
Crescimento
2 sintomas
🦴
Ossos e articulações
1 sintomas
🫘
Rins
1 sintomas

+ 4 sintomas em outras categorias

Características mais comuns

55%prev.
Descoloração amarelo-acastanhada dos dentes
Frequente (79-30%)
55%prev.
Anormalidade da cor dentária
Frequente (79-30%)
55%prev.
Calcificação da polpa
Frequente (79-30%)
55%prev.
Odontodisplasia
Frequente (79-30%)
55%prev.
Fóssulas no esmalte dentário
Frequente (79-30%)
55%prev.
Anormalidade dos dentes decíduos
Frequente (79-30%)
24sintomas
Frequente (17)
Ocasional (7)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

Descoloração amarelo-acastanhada dos dentesYellow-brown discoloration of the teeth
Frequente (79-30%)55%
Anormalidade da cor dentáriaAbnormality of dental color
Frequente (79-30%)55%
Calcificação da polpaPulp calcification
Frequente (79-30%)55%
OdontodisplasiaOdontodysplasia
Frequente (79-30%)55%
Fóssulas no esmalte dentárioDental enamel pits
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico136PubMed
Últimos 10 anos41publicações
Pico20248 papers
Linha do tempo
2026Hoje · 2026📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Odontodisplasia regional

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

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Publicações mais relevantes

Timeline de publicações
41 papers (10 anos)
#1

Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.

Journal of applied oral science : revista FOB2026

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.

#2

Regional odontodysplasia: a report of unusual case with in-depth analysis.

Journal of oral biology and craniofacial research2026

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.

#3

Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.

International journal of paediatric dentistry2026 Jan

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.

#4

Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.

Case reports in dentistry2025

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.

#5

New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.

International dental journal2025 Oct

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

Ver todas no PubMed

📚 EuropePMC117 artigos no totalmostrando 41

2026

Regional odontodysplasia: a report of unusual case with in-depth analysis.

Journal of oral biology and craniofacial research
2026

Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.

Journal of applied oral science : revista FOB
2025

Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.

Case reports in dentistry
2026

Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.

International journal of paediatric dentistry
2025

New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.

International dental journal
2025

Regional Odontodysplasia in Primary Teeth: A Case Report.

Cureus
2024

Regional Odontodysplasia: A Case Report Crossing the Midline with Implantoprosthetic Rehabilitation.

Journal of pharmacy &amp; bioallied sciences
2024

Clinicial-pathologic correlations of non-trauma related Odontodysplasia in 28 dogs: 2013-2023.

Frontiers in veterinary science
2024

Abnormal dental follicle cells: A crucial determinant in tooth eruption disorders (Review).

Molecular medicine reports
2024

PHACES syndrome and multi-regional odontodysplasia: a case report.

The Journal of clinical pediatric dentistry
2024

[Research progress in regional odontodysplasia].

Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatology
2024

Regenerative Endodontic Procedures in Immature Teeth Affected by Regional Odontodysplasia.

Journal of endodontics
2024

Regional 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 stomatology
2024

Developmental Anomalies in Human Teeth: Odontoblastic Differentiation in Hamartomatous Calcifying Hyperplastic Dental Follicles Presenting with DSP, Nestin, and HES1.

Journal of developmental biology
2023

Regional odontodysplasia diagnosed and treated via multidisciplinary approach: a case report.

The Journal of clinical pediatric dentistry
2023

A 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 Society
2023

Regional odontodysplasia on the left side of the mandible: report of an unusual case.

The Journal of clinical pediatric dentistry
2023

Mandibular Regional Odontodysplasia in a Toddler.

Journal of dentistry for children (Chicago, Ill.)
2023

Regional Odontodysplasia with Actinomycosis Infection: A Rare Case Report.

International journal of clinical pediatric dentistry
2022

Rehabilitation of Maxillary Bilateral Regional Odontodysplasia in a 7-Year-Old Child Using Modified Essix Retainer.

Contemporary clinical dentistry
2022

Cone-beam computed tomography in the diagnosis of regional odontodysplasia.

Clinical case reports
2022

Regional Odontodysplasia: A Systematic Review of Case Reports.

International journal of environmental research and public health
2021

Mandibular 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 Orthodontics
2020

Treatment Strategies for Patients with Regional Odontodysplasia: A Presentation of Seven New Cases and a Review of the Literature.

Oral health &amp; preventive dentistry
2020

[Regional odontodysplasia: a report of three cases].

Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatology
2019

Regional odontodysplasia: An analysis of 161 cases from 1953 to 2017.

The Saudi dental journal
2019

Oral 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 Dentistry
2020

Regional Odontodysplasia Affecting the Maxilla.

Head and neck pathology
2019

Familial oligodontia and regional odontodysplasia associated with a PAX9 initiation codon mutation.

Clinical oral investigations
2018

Management of regional odontodysplasia: a 10-year-follow-up case report and literature review.

European oral research
2018

Regional Odontodysplasia Crossing the Midline.

Journal of dentistry for children (Chicago, Ill.)
2018

Prevalence of dental anomalies in French orthodontic patients: A retrospective study.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
2018

Mandibular Regional Odontodysplasia in an 8-year-old Boy showing Teeth Disorders, Gubernaculum Tracts, and Altered Bone Fractal Pattern.

International journal of clinical pediatric dentistry
2018

Cervicofacial infection in a 3-year-old child with regional odontodysplasia: A case report and literature review.

Journal of oral and maxillofacial pathology : JOMFP
2016

Regional Odontodysplasia with Generalised Enamel Defect.

Case reports in dentistry
2016

Clinical, Radiographic, and Histologic Evaluation of Regional Odontodysplasia: a Case Report with 5-year Follow-up.

Journal of dentistry (Shiraz, Iran)
2016

Role of inflammation in developmental odontogenic pathosis.

Journal of oral and maxillofacial pathology : JOMFP
2015

Inflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority.

Indian journal of dentistry
2015

Malformations of the tooth root in humans.

Frontiers in physiology
2015

Ghost teeth: Regional odontodysplasia of maxillary first molar associated with eruption disorders in a 10-year-old girl.

Journal of pharmacy &amp; bioallied sciences
2015

Regional odontodysplasia: Report of an unusual case involving mandibular arch.

Contemporary clinical dentistry
Ver todos os 117 no EuropePMC

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Doenças relacionadas

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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.

  1. Microstructures and mechanical characteristics of primary teeth affected by regional odontodysplasia.
    Journal of applied oral science : revista FOB· 2026· PMID 41538587mais citado
  2. Regional odontodysplasia: a report of unusual case with in-depth analysis.
    Journal of oral biology and craniofacial research· 2026· PMID 41584217mais citado
  3. Epidemiological and Clinical Features of Regional Odontodysplasia in South Korean Pediatric Patients: A Multicenter Case Series Study.
    International journal of paediatric dentistry· 2026· PMID 41017073mais citado
  4. Long-Term Multidisciplinary Management of Regional Odontodysplasia: A Case Report.
    Case reports in dentistry· 2025· PMID 41473891mais citado
  5. New Immunohistochemical Findings on Amelogenin and Dentin Sialophosphoprotein in Genetic Tooth Diseases.
    International dental journal· 2025· PMID 40712386mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:83450(Orphanet)
  2. MONDO:0019367(MONDO)
  3. GARD:19036(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. 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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Odontodisplasia regional
Compêndio · Raras BR

Odontodisplasia regional

ORPHA:83450 · MONDO:0019367
Prevalência
Unknown
Casos
140 casos conhecidos
Herança
Not applicable
CID-10
K00.4 · Distúrbios na formação dos dentes
CID-11
Início
Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0206554
EuropePMC
Wikidata
Wikipedia
Papers 10a
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