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Regional odontodysplasia

MedGen UID:
104891
Concept ID:
C0206554
Congenital Abnormality
Synonym: Odontodysplasia
SNOMED CT: Regional odontodysplasia (66063001); Odontogenic dysplasia (66063001); Ghost teeth (66063001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0000694
Monarch Initiative: MONDO:0019367
Orphanet: ORPHA83450

Definition

The diagnosis odontodysplasia requires clinical and radiological exams, in which unusually large pulp chambers and large pulp room chambers with thin enamel and dentin are visible. It may affect either a single tooth or several teeth. The term regional odontodysplasia is used if several teeth are affected. It affects the deciduous and permanent dentitions in the maxilla, the mandible or both, although the maxilla is more frequently involved. A type of dental dysplasia occurring in dentinogenesis imperfecta in which the pulp chambers are enlarged and there is a reduced amount of coronal dentin. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRegional odontodysplasia

Conditions with this feature

Dentinogenesis imperfecta type 3
MedGen UID:
97995
Concept ID:
C0399378
Congenital Abnormality
Dentinogenesis imperfecta, Shields type III (DGI-III) is an autosomal dominant disorder of dentin formation. DGI presents clinically with gray to brownish-blue discoloration of the teeth and rapid attrition of the crowns, which are bulbous. There are no skeletal manifestations. Both deciduous and permanent teeth are affected (summary by MacDougall et al., 1999).
Trichodental syndrome
MedGen UID:
96068
Concept ID:
C0406724
Disease or Syndrome
A rare ectodermal dysplasia syndrome characterized by the association of sparse, fine, dry, slow growing hair with variable dental abnormalities including oligodontia, peg-shaped incisors, and shell teeth. Mild intellectual disability, microcephaly, and dysmorphic facial features have also been reported.

Professional guidelines

PubMed

Heggie C, Gartshore L
J Dent Child (Chic) 2020 Jan 15;87(1):39-43. PMID: 32151309
Nitschke Y, Rutsch F
Curr Osteoporos Rep 2017 Aug;15(4):255-270. doi: 10.1007/s11914-017-0370-3. PMID: 28585220

Recent clinical studies

Etiology

Hess P, Lauridsen EF, Daugaard-Jensen J, Worsaae N, Kofod T, Hermann NV
Oral Health Prev Dent 2020 Sep 4;18(4):669-681. doi: 10.3290/j.ohpd.a45070. PMID: 32895649
Baron C, Houchmand-Cuny M, Enkel B, Lopez-Cazaux S
Arch Pediatr 2018 Oct;25(7):426-430. Epub 2018 Sep 22 doi: 10.1016/j.arcped.2018.07.002. PMID: 30249487
Barbería E, Sanz Coarasa A, Hernández A, Cardoso-Silva C
Eur J Paediatr Dent 2012 Jun;13(2):161-6. PMID: 22762183
Thimma Reddy BV, Vinay Reddy KK, Sunil B, Pujita R, Kiran K, Kranthi KR
J Indian Soc Pedod Prev Dent 2010 Oct-Dec;28(4):315-8. doi: 10.4103/0970-4388.76166. PMID: 21273724
Ferguson JW, Geary CP
Aust Dent J 1980 Jun;25(3):148-51. doi: 10.1111/j.1834-7819.1980.tb03705.x. PMID: 6251796

Diagnosis

Jiang Z, Ji Y, Su J
Hua Xi Kou Qiang Yi Xue Za Zhi 2024 Feb 1;42(1):121-125. doi: 10.7518/hxkq.2024.2023247. PMID: 38475960Free PMC Article
Nijakowski K, Woś P, Surdacka A
Int J Environ Res Public Health 2022 Feb 1;19(3) doi: 10.3390/ijerph19031683. PMID: 35162705Free PMC Article
Barbería E, Sanz Coarasa A, Hernández A, Cardoso-Silva C
Eur J Paediatr Dent 2012 Jun;13(2):161-6. PMID: 22762183
Crawford PJ, Aldred MJ
J Oral Pathol Med 1989 May;18(5):251-63. doi: 10.1111/j.1600-0714.1989.tb00394.x. PMID: 2549236
Hovinga J, Ingenhoes R
Int J Oral Surg 1979 Dec;8(6):474-7. doi: 10.1016/s0300-9785(79)80088-5. PMID: 232092

Prognosis

Star JM, Jordan RC, Stewart RE
J Clin Pediatr Dent 2024 May;48(3):166-170. Epub 2024 May 3 doi: 10.22514/jocpd.2024.070. PMID: 38755995
Gallo Cde B, Alves FA, Junior Ddos S, Marques MM, Sugaya NN
Gen Dent 2011 Jul-Aug;59(4):e178-81. PMID: 21903559
Jensen JL, Ambjornsen E, Haanaes HR, Storhaug K
Eur Arch Paediatr Dent 2009 Dec;10(4):244-7. doi: 10.1007/BF03262691. PMID: 19995511
Gondim JO, Pretel H, Ramalho LT, Santos-Pinto LA, Giro EM
J Indian Soc Pedod Prev Dent 2009 Jul-Sep;27(3):175-8. doi: 10.4103/0970-4388.57099. PMID: 19841550
Ferguson F, Schlissel E, Kucine A, Alexander S, DeSantis A, Hendricks R, Xu L
Pediatr Dent 2009 Jul-Aug;31(4):346-9. PMID: 19722446

Clinical prediction guides

Chebath-Taub D, Slutzky-Goldberg I
J Endod 2024 Jul;50(7):1017-1021. Epub 2024 Apr 14 doi: 10.1016/j.joen.2024.04.006. PMID: 38626857
Nijakowski K, Woś P, Surdacka A
Int J Environ Res Public Health 2022 Feb 1;19(3) doi: 10.3390/ijerph19031683. PMID: 35162705Free PMC Article
Barbería E, Sanz Coarasa A, Hernández A, Cardoso-Silva C
Eur J Paediatr Dent 2012 Jun;13(2):161-6. PMID: 22762183
Spini TH, Sargenti-Neto S, Cardoso SV, Souza KC, de Souza SO, de Faria PR, Loyola AM
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007 Sep;104(3):e40-5. Epub 2007 Jul 5 doi: 10.1016/j.tripleo.2007.02.027. PMID: 17613259
Tervonen SA, Stratmann U, Mokrys K, Reichart PA
Clin Oral Investig 2004 Jun;8(2):45-51. Epub 2004 Jan 9 doi: 10.1007/s00784-003-0245-0. PMID: 14714196

Recent systematic reviews

Nijakowski K, Woś P, Surdacka A
Int J Environ Res Public Health 2022 Feb 1;19(3) doi: 10.3390/ijerph19031683. PMID: 35162705Free PMC Article

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