raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:83450

Regional odontodysplasia

Synonyms: Ghost teeth

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Childhood
Status
active

A rare orodental disease characterized by localized developmental anomaly of the dental tissues, with enamel and dentin hypomineralization affecting one (odontodysplasia) or several (regional odontodysplasia; ROD) teeth (deciduous and permanent, with teeth of the maxilla more frequently involved).

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enRegional odontodysplasiaGhost teeth
frOdontodysplasie régionaleDents fantômes · Dents évanescentes
deOdontodysplasie, regionale
esOdontodisplasia regionalDiente fantasma · Dientes fantasmas
itOdontodisplasia regionaleDenti fantasma
nlRegionale odontodysplasie= en
ptOdontodisplasia regionalDentes fantasmas
plMiejscowa odontodysplazjaZęby widmo
csRegionální odontodysplázie= en
ukРегіональна одонтодисплазіяЗуби привидів
zh局部牙发育不全阴影牙
trBölgesel odontodisplaziHayalet dişler

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10K00.4NTBTinclusionvalidated
ICD-11DA07.3NTBTindexvalidated
MONDO0019367Evalidated
UMLSC0206554Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families140 casesWorldwideCase(s)ValidatedORPHANET
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

24 annotations · validated 2021-02-03
HPOTerm (as annotated by Orphanet)Diagnostic
Frequent (79-30%)
HP:0011073Abnormality of dental color
HP:0000682Abnormality of dental enamel
HP:0010299Abnormality of dentin
HP:0006481Abnormality of primary teeth
HP:0006479Abnormality of the dental pulp
HP:0000670Carious teeth
HP:0000684Delayed eruption of teeth
HP:0009722Dental enamel pits
HP:0006297Enamel hypoplasia
HP:0000230Gingivitis
HP:0011084Hypocalcification of dental enamel
HP:0040264Jaw pain
HP:0000694Odontodysplasia
HP:0003771Pulp calcification
HP:0030757Tooth abscess
HP:0000706Unerupted tooth
HP:0006286Yellow-brown discoloration of the teeth
Occasional (29-5%)
HP:0009085Alveolar ridge overgrowth
HP:0000212Gingival overgrowth
HP:0000685Hypoplasia of teeth
HP:0001572Macrodontia
HP:0200025Mandibular pain
HP:0006283Multiple unerupted teeth
HP:0006336Short dental roots

Annotation source: 31337932[PMID]_22762183[PMID]_30900210[PMID]

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Sources for this card