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Oligodontia of primary teeth

MedGen UID:
868598
•Concept ID:
C4022997
•
Finding
Synonyms: Decreased number of baby teeth; Decreased number of deciduous teeth; Decreased number of milk teeth; Decreased number of primary teeth; Failure of development of some deciduous teeth; Failure of development of some primary teeth; Fewer baby teeth than normal; Fewer deciduous teeth than normal; Fewer primary teeth than normal; Missing some baby teeth; Missing some milk teeth; Missing some primary teeth; Partial anodontia of deciduous teeth; Partial anodontia of primary teeth
 
HPO: HP:0012225

Definition

Reduced number of primary teeth. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOligodontia of primary teeth

Conditions with this feature

Cleft palate-stapes fixation-oligodontia syndrome
MedGen UID:
347795
•Concept ID:
C1859081
•
Disease or Syndrome
A rare congenital malformation syndrome characterized by cleft soft palate, severe oligodontia of the deciduous teeth, absence of the permanent dentition, bilateral conductive deafness due to fixation of the footplate of the stapes, short halluces with a wide space between the first and second toes, and fusion of carpal and tarsal bones. There have been no further descriptions in the literature since 1971.
See: Condition Record
Tooth agenesis, selective, 3
MedGen UID:
410035
•Concept ID:
C1970291
•
Disease or Syndrome
Any tooth agenesis in which the cause of the disease is a mutation in the PAX9 gene.
See: Condition Record
Cleft palate-stapes fixation-oligodontia syndrome
Tooth agenesis, selective, 3

Professional guidelines

PubMed

Clinical management of fusion in primary mandibular incisors: a systematic literature review.
Bernardi S, Bianchi S, Bernardi G, Tchorz JP, Attin T, Hellwig E, Karygianni L
Acta Odontol Scand 2020 Aug;78(6):417-424. Epub 2020 Mar 3 doi: 10.1080/00016357.2020.1734233. PMID: 32125202
Management of over-retained mandibular deciduous second molars with and without permanent successors.
Sabri R
World J Orthod 2008 Fall;9(3):209-20. PMID: 18834004
The interdisciplinary management of hypodontia: background and role of paediatric dentistry.
Nunn JH, Carter NE, Gillgrass TJ, Hobson RS, Jepson NJ, Meechan JG, Nohl FS
Br Dent J 2003 Mar 8;194(5):245-51. doi: 10.1038/sj.bdj.4809925. PMID: 12658298

Recent clinical studies

Etiology

Developmental enamel defects and dental anomalies of number and size in children with growth hormone deficiency.
Torlińska-Walkowiak N, Majewska KA, Sowińska A, Kędzia A, Opydo-Szymaczek J
Sci Rep 2023 Sep 7;13(1):14707. doi: 10.1038/s41598-023-41892-x. PMID: 37679467Free PMC Article
Infraocclusion: Prevalence, Characteristics, and Associated Dental Anomalies in Arabian Children.
Alshaya SI, Alanazi AF, Aldawish SS, Alsuhaim MM, Alomar MS, Almuaytiq YM, Alfahad SA, Almousa AAS, Alassaf A, Mallineni SK
Biomed Res Int 2022;2022:6624804. Epub 2022 Jul 23 doi: 10.1155/2022/6624804. PMID: 35915788Free PMC Article
Primary Fused Teeth and Findings in Permanent Dentition.
Açıkel H, İbiş S, Şen Tunç E
Med Princ Pract 2018;27(2):129-132. Epub 2018 Feb 1 doi: 10.1159/000487322. PMID: 29402820Free PMC Article
A catalogue of anomalies and traits of the primary dentition of southern Chinese.
King NM, Tongkoom S, Itthagarun A, Wong HM, Lee CK
J Clin Pediatr Dent 2008 Winter;32(2):139-46. doi: 10.17796/jcpd.32.2.w76653r22rnnn713. PMID: 18389680
Inherited dental anomalies and abnormalities.
Kotsomitis N, Freer TJ
ASDC J Dent Child 1997 Nov-Dec;64(6):405-8. PMID: 9466010

Diagnosis

Pattern and prevalence of dental anomalies among a paediatric population in Lagos, Nigeria.
Olatosi OO, Oyapero A, Akinwande KO, Ayedun OS, Aladenika ET, Obe OI
Niger Postgrad Med J 2022 Apr-Jun;29(2):167-172. doi: 10.4103/npmj.npmj_23_22. PMID: 35488587
Primary Fused Teeth and Findings in Permanent Dentition.
Açıkel H, İbiş S, Şen Tunç E
Med Princ Pract 2018;27(2):129-132. Epub 2018 Feb 1 doi: 10.1159/000487322. PMID: 29402820Free PMC Article
The dentition: the outcomes of morphogenesis leading to variations of tooth number, size and shape.
Brook AH, Jernvall J, Smith RN, Hughes TE, Townsend GC
Aust Dent J 2014 Jun;59 Suppl 1:131-42. Epub 2014 Mar 20 doi: 10.1111/adj.12160. PMID: 24646162
Crouzonodermoskeletal syndrome.
Jeftha A, Stephen L, Morkel JA, Beighton P
J Clin Pediatr Dent 2004 Winter;28(2):173-6. doi: 10.17796/jcpd.28.2.72m01l5g50448548. PMID: 14969379
Survey of anomalies in primary teeth and their correlation with the permanent dentition.
Whittington BR, Durward CS
N Z Dent J 1996 Mar;92(407):4-8. PMID: 8649664

Therapy

Association between Dental Anomalies and Orofacial Clefts: A Meta-analysis.
Marzouk T, Alves IL, Wong CL, DeLucia L, McKinney CM, Pendleton C, Howe BJ, Marazita ML, Peter TK, Kopycka-Kedzierawski DT, Morrison CS, Malmstrom H, Wang H, Shope ET
JDR Clin Trans Res 2021 Oct;6(4):368-381. Epub 2020 Oct 8 doi: 10.1177/2380084420964795. PMID: 33030085Free PMC Article
Scandcleft randomized trials of primary surgery for unilateral cleft lip and palate: dental anomalies in 8-year olds.
Rizell S, Bellardie H, Karsten A, Sæle P, Mooney J, Heliövaara A, Küseler A, Brinck E, Skaare P, Mølsted K, Chalien MN, Marcusson A, Eyres P, Shaw W, Semb G
Eur J Orthod 2020 Jan 27;42(1):8-14. doi: 10.1093/ejo/cjz070. PMID: 31579919
Esthetic Rehabilitation of Retained Primary Teeth-A Conservative Approach.
Parise Gré C, Schweigert Bona V, Pedrollo Lise D, Monteiro Júnior S
J Prosthodont 2019 Jan;28(1):e41-e44. Epub 2017 Apr 11 doi: 10.1111/jopr.12602. PMID: 28398648
Orthodontic considerations in restorative management of hypodontia patients with endosseous implants.
Borzabadi-Farahani A
J Oral Implantol 2012 Dec;38(6):779-91. Epub 2011 Jul 5 doi: 10.1563/AAID-JOI-D-11-00022. PMID: 21728818
When should we extract deciduous teeth and place implants in young individuals with tooth agenesis?
Bergendal B
J Oral Rehabil 2008 Jan;35 Suppl 1:55-63. doi: 10.1111/j.1365-2842.2007.01829.x. PMID: 18181934

Prognosis

KDF1 Novel Variant Causes Unique Dental and Oral Epithelial Defects.
Yu M, Liu H, Liu Y, Zheng J, Wu J, Sun K, Feng H, Liu H, Han D
Int J Mol Sci 2022 Oct 18;23(20) doi: 10.3390/ijms232012465. PMID: 36293320Free PMC Article
Functional Effects of WNT10A Rare Variants Associated with Tooth Agenesis.
Zeng Y, Baugh E, Akyalcin S, Letra A
J Dent Res 2021 Mar;100(3):302-309. Epub 2020 Oct 9 doi: 10.1177/0022034520962728. PMID: 33034246
Natural history of X-linked hypohidrotic ectodermal dysplasia: a 5-year follow-up study.
Wohlfart S, Meiller R, Hammersen J, Park J, Menzel-Severing J, Melichar VO, Huttner K, Johnson R, Porte F, Schneider H
Orphanet J Rare Dis 2020 Jan 10;15(1):7. doi: 10.1186/s13023-019-1288-x. PMID: 31924237Free PMC Article
Implant-supported Oral Rehabilitation in Child with Ectodermal Dysplasia - 4-year Follow-up.
Cezária Triches T, Ximenes M, Oliveira de Souza JG, Rodrigues Lopes Pereira Neto A, Cardoso AC, Bolan M
Bull Tokyo Dent Coll 2017;58(1):49-56. doi: 10.2209/tdcpublication.2016-0012. PMID: 28381734
The prognosis of retained primary molars without successors: infraocclusion, root resorption and restorations in 111 patients.
Hvaring CL, Øgaard B, Stenvik A, Birkeland K
Eur J Orthod 2014 Feb;36(1):26-30. Epub 2013 Jan 12 doi: 10.1093/ejo/cjs105. PMID: 23314329

Clinical prediction guides

Analysis of congenital deciduous teeth absence and its permanent teeth phenotype.
Qiu F, Tang Z, Meng S
Hua Xi Kou Qiang Yi Xue Za Zhi 2023 Apr 1;41(2):203-207. doi: 10.7518/hxkq.2023.2021639. PMID: 37056187Free PMC Article
Infraocclusion: Prevalence, Characteristics, and Associated Dental Anomalies in Arabian Children.
Alshaya SI, Alanazi AF, Aldawish SS, Alsuhaim MM, Alomar MS, Almuaytiq YM, Alfahad SA, Almousa AAS, Alassaf A, Mallineni SK
Biomed Res Int 2022;2022:6624804. Epub 2022 Jul 23 doi: 10.1155/2022/6624804. PMID: 35915788Free PMC Article
Clinical management of fusion in primary mandibular incisors: a systematic literature review.
Bernardi S, Bianchi S, Bernardi G, Tchorz JP, Attin T, Hellwig E, Karygianni L
Acta Odontol Scand 2020 Aug;78(6):417-424. Epub 2020 Mar 3 doi: 10.1080/00016357.2020.1734233. PMID: 32125202
Primary Fused Teeth and Findings in Permanent Dentition.
Açıkel H, İbiş S, Şen Tunç E
Med Princ Pract 2018;27(2):129-132. Epub 2018 Feb 1 doi: 10.1159/000487322. PMID: 29402820Free PMC Article
Crouzonodermoskeletal syndrome.
Jeftha A, Stephen L, Morkel JA, Beighton P
J Clin Pediatr Dent 2004 Winter;28(2):173-6. doi: 10.17796/jcpd.28.2.72m01l5g50448548. PMID: 14969379

Recent systematic reviews

What is the survival rate of deciduous molars in cases with agenesis of premolar successors? A systematic review.
Dos Santos CCO, Melo DL, da Silva PP, Normando D
Angle Orthod 2022 Jan 1;92(1):110-117. doi: 10.2319/123020-1039.1. PMID: 34329385Free PMC Article
Are developmentally missing teeth a predictive risk marker of malignant diseases in non-syndromic individuals? A systematic review.
Al-Muzian L, Almuzian M, Mohammed H, Ulhaq A, Keightley AJ
J Orthod 2021 Sep;48(3):221-230. Epub 2021 Jan 18 doi: 10.1177/1465312520984166. PMID: 33455496
Association between Dental Anomalies and Orofacial Clefts: A Meta-analysis.
Marzouk T, Alves IL, Wong CL, DeLucia L, McKinney CM, Pendleton C, Howe BJ, Marazita ML, Peter TK, Kopycka-Kedzierawski DT, Morrison CS, Malmstrom H, Wang H, Shope ET
JDR Clin Trans Res 2021 Oct;6(4):368-381. Epub 2020 Oct 8 doi: 10.1177/2380084420964795. PMID: 33030085Free PMC Article
Clinical management of fusion in primary mandibular incisors: a systematic literature review.
Bernardi S, Bianchi S, Bernardi G, Tchorz JP, Attin T, Hellwig E, Karygianni L
Acta Odontol Scand 2020 Aug;78(6):417-424. Epub 2020 Mar 3 doi: 10.1080/00016357.2020.1734233. PMID: 32125202
The Current Evidence on Retaining or Prosthodontically Replacing Retained Deciduous Teeth in the Adult Hypodontia Patient: A Systematic Review.
Laverty DP, Fairbrother K, Addison O
Eur J Prosthodont Restor Dent 2018 Mar 1;26(1):2-15. doi: 10.1922/EJPRD_01721Laverty14. PMID: 29461745

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