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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 is a disorder of tooth development. This condition causes the teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth.\n\nResearchers have described three types of dentinogenesis imperfecta with similar dental abnormalities. Type I occurs in people who have osteogenesis imperfecta, a genetic condition in which bones are brittle and easily broken. Dentinogenesis imperfecta type II and type III usually occur in people without other inherited disorders. A few older individuals with type II have had progressive high-frequency hearing loss in addition to dental abnormalities, but it is not known whether this hearing loss is related to dentinogenesis imperfecta.\n\nSome researchers believe that dentinogenesis imperfecta type II and type III, along with a condition called dentin dysplasia type II, are actually forms of a single disorder. The signs and symptoms of dentin dysplasia type II are very similar to those of dentinogenesis imperfecta. However, dentin dysplasia type II affects the primary teeth much more than the permanent teeth.
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(1):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

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
Hess P, Lauridsen EF, Daugaard-Jensen J, Worsaae N, Kofod T, Hermann NV
Oral Health Prev Dent 2020 Sep 4;18(1):669-681. doi: 10.3290/j.ohpd.a45070. PMID: 32895649
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

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
Gerlach RF, Jorge J Jr, de Almeida OP, Coletta RD, Zaia AA
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998 Mar;85(3):308-13. doi: 10.1016/s1079-2104(98)90014-2. PMID: 9540089

Clinical prediction guides

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
Slootweg PJ, Meuwissen PR
J Oral Pathol 1985 Mar;14(3):256-62. doi: 10.1111/j.1600-0714.1985.tb00489.x. PMID: 3921681

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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