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Anodontia

MedGen UID:
98313
Concept ID:
C0399352
Congenital Abnormality
Synonym: Total anodontia of permanent and deciduous teeth
SNOMED CT: Developmental absence of tooth (234951001); Anodontia (26624006); Congenital absence of teeth (26624006); Anodontism (26624006); Congenital edentia (26624006); Agomphiasis (26624006); Agomphosis (26624006); Complete congenital absence of teeth (26624006)
 
HPO: HP:0000674
Monarch Initiative: MONDO:0008797
Orphanet: ORPHA99797

Definition

The absence of all teeth from the normal series by a failure to develop. [from HPO]

Conditions with this feature

Woodhouse-Sakati syndrome
MedGen UID:
83337
Concept ID:
C0342286
Disease or Syndrome
Virtually all individuals with Woodhouse-Sakati syndrome (WSS) have the endocrine findings of hypogonadism (evident at puberty) and progressive childhood-onset hair thinning that often progresses to alopecia totalis in adulthood. More than half of individuals have the neurologic findings of progressive extrapyramidal movements (dystonic spasms with dystonic posturing with dysarthria and dysphagia), moderate bilateral postlingual sensorineural hearing loss, and mild intellectual disability. To date, more than 40 families (including 33 with a molecularly confirmed diagnosis) with a total of 88 affected individuals have been reported in the literature.
Telecanthus
MedGen UID:
140836
Concept ID:
C0423113
Finding
Distance between the inner canthi more than two standard deviations above the mean (objective); or, apparently increased distance between the inner canthi.
Cranioectodermal dysplasia 1
MedGen UID:
96586
Concept ID:
C0432235
Disease or Syndrome
Cranioectodermal dysplasia (CED) is a ciliopathy with skeletal involvement (narrow thorax, shortened proximal limbs, syndactyly, polydactyly, brachydactyly), ectodermal features (widely spaced hypoplastic teeth, hypodontia, sparse hair, skin laxity, abnormal nails), joint laxity, growth deficiency, and characteristic facial features (frontal bossing, low-set simple ears, high forehead, telecanthus, epicanthal folds, full cheeks, everted lower lip). Most affected children develop nephronophthisis that often leads to end-stage kidney disease in infancy or childhood, a major cause of morbidity and mortality. Hepatic fibrosis and retinal dystrophy are also observed. Dolichocephaly, often secondary to sagittal craniosynostosis, is a primary manifestation that distinguishes CED from most other ciliopathies. Brain malformations and developmental delay may also occur.
Agenesis of permanent teeth
MedGen UID:
224851
Concept ID:
C1290511
Congenital Abnormality
A congenital defect characterized by the absence of one or more permanent teeth, including oligodontia, hypodontia, and adontia of the of permanent teeth.
Van der Woude syndrome 2
MedGen UID:
338272
Concept ID:
C1847604
Disease or Syndrome
Van der Woude syndrome (VWS) is a dominantly inherited developmental disorder characterized by pits and/or sinuses of the lower lip, and cleft lip and/or cleft palate (CL/P, CP). It is the most common cleft syndrome. For a discussion of genetic heterogeneity of van der Woude syndrome, see VWS1 (119300).
Tooth agenesis, selective, X-linked, 1
MedGen UID:
410143
Concept ID:
C1970757
Disease or Syndrome
Any tooth agenesis in which the cause of the disease is a mutation in the EDA gene.
Cleft lip/palate-ectodermal dysplasia syndrome
MedGen UID:
444067
Concept ID:
C2931488
Disease or Syndrome
Zlotogora-Ogur syndrome is an ectodermal dysplasia syndrome with characteristics of hair, skin and teeth anomalies, facial dysmorphism with cleft lip and palate, cutaneous syndactyly and, in some cases, intellectual disability.The prevalence is unknown but to date, less than 50 cases have been described in the literature. Caused by mutations in the gene PVRL1 (11q23-q24) which encodes nectin-1, the principal receptor used by alpha-herpesviruses to mediate entry into human cells. Transmission is autosomal recessive.
Ectodermal dysplasia 10B, hypohidrotic/hair/tooth type, autosomal recessive
MedGen UID:
854356
Concept ID:
C3887494
Disease or Syndrome
Hypohidrotic ectodermal dysplasia (HED) is characterized by hypotrichosis (sparseness of scalp and body hair), hypohidrosis (reduced ability to sweat), and hypodontia (congenital absence of teeth). The cardinal features of classic HED become obvious during childhood. The scalp hair is thin, lightly pigmented, and slow growing. Sweating, although present, is greatly deficient, leading to episodes of hyperthermia until the affected individual or family acquires experience with environmental modifications to control temperature. Only a few abnormally formed teeth erupt, at a later-than-average age. Physical growth and psychomotor development are otherwise within normal limits. Mild HED is characterized by mild manifestations of any or all the characteristic features.
Ectodermal dysplasia 10A, hypohidrotic/hair/nail type, autosomal dominant
MedGen UID:
854747
Concept ID:
C3888065
Disease or Syndrome
Hypohidrotic ectodermal dysplasia (HED) is characterized by hypotrichosis (sparseness of scalp and body hair), hypohidrosis (reduced ability to sweat), and hypodontia (congenital absence of teeth). The cardinal features of classic HED become obvious during childhood. The scalp hair is thin, lightly pigmented, and slow growing. Sweating, although present, is greatly deficient, leading to episodes of hyperthermia until the affected individual or family acquires experience with environmental modifications to control temperature. Only a few abnormally formed teeth erupt, at a later-than-average age. Physical growth and psychomotor development are otherwise within normal limits. Mild HED is characterized by mild manifestations of any or all the characteristic features.

Professional guidelines

PubMed

Kratochvilova L, Dostalova T, Schwarz M, Macek M Jr, Marek I, Malíková M, Míšová E
Eur J Paediatr Dent 2022 Jun;23(2):140-146. doi: 10.23804/ejpd.2022.23.02.12. PMID: 35722846
Ojala-Alasuutari M, Hassan SJ, Näpänkangas R, Ylikontiola L, Lähdesmäki R
Acta Odontol Scand 2022 Jul;80(5):382-388. Epub 2021 Dec 28 doi: 10.1080/00016357.2021.2021282. PMID: 34962856
Andrade DC, Loureiro CA, Araújo VE, Riera R, Atallah AN
Orthod Craniofac Res 2013 Aug;16(3):129-36. Epub 2013 Feb 14 doi: 10.1111/ocr.12015. PMID: 23406509

Recent clinical studies

Etiology

Bilge NH, Yeşiltepe S, Törenek Ağırman K, Çağlayan F, Bilge OM
Folia Morphol (Warsz) 2018;77(2):323-328. Epub 2017 Sep 21 doi: 10.5603/FM.a2017.0087. PMID: 28933802
Schnabl D, Brock M, Puelacher W
Swiss Dent J 2016;126(11):1031-1046. PMID: 27874919
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Mensink G, van Merkesteyn R
Br Dent J 2010 Feb 13;208(3):109-11. doi: 10.1038/sj.bdj.2010.102. PMID: 20147924
Markovic M
Swed Dent J Suppl 1982;15:153-62. PMID: 6963769

Diagnosis

Schroeder DK, Schroeder MA, Vasconcelos V
Dental Press J Orthod 2022;27(1):e22spe1. Epub 2022 Jun 6 doi: 10.1590/2177-6709.27.1.e22spe1. PMID: 35674572Free PMC Article
Bilge NH, Yeşiltepe S, Törenek Ağırman K, Çağlayan F, Bilge OM
Folia Morphol (Warsz) 2018;77(2):323-328. Epub 2017 Sep 21 doi: 10.5603/FM.a2017.0087. PMID: 28933802
Schnabl D, Brock M, Puelacher W
Swiss Dent J 2016;126(11):1031-1046. PMID: 27874919
Mohamed Riyaz SS, Jayachandran S
Indian J Dent Res 2009 Oct-Dec;20(4):508-10. doi: 10.4103/0970-9290.59442. PMID: 20139583
Kjaer I
Crit Rev Oral Biol Med 1998;9(2):224-44. doi: 10.1177/10454411980090020501. PMID: 9603237

Therapy

Cerezo-Cayuelas M, Pérez-Silva A, Serna-Muñoz C, Vicente A, Martínez-Beneyto Y, Cabello-Malagón I, Ortiz-Ruiz AJ
Orphanet J Rare Dis 2022 Oct 17;17(1):376. doi: 10.1186/s13023-022-02533-0. PMID: 36253866Free PMC Article
Conti PCR, Bonjardim LR, Stuginski-Barbosa J, Costa YM, Svensson P
J Oral Rehabil 2021 Feb;48(2):195-206. Epub 2020 Oct 26 doi: 10.1111/joor.13112. PMID: 33047362
Shum M, Mahoney E, Naysmith K, Macfarlane S, Corbett R, Narsinh M, Natarajan A, Ramadas Y, Hitchings E, Anderson H
N Z Med J 2020 Oct 9;133(1523):41-54. PMID: 33032302
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Speirs AL
Lancet 1965 Nov 20;2(7421):1074. doi: 10.1016/s0140-6736(65)90602-1. PMID: 4159144

Prognosis

Bonczek O, Krejci P, Izakovicova-Holla L, Cernochova P, Kiss I, Vojtesek B
Clin Genet 2021 Apr;99(4):493-502. Epub 2021 Feb 16 doi: 10.1111/cge.13892. PMID: 33249565
Al-Ani AH, Antoun JS, Thomson WM, Merriman TR, Farella M
Biomed Res Int 2017;2017:9378325. Epub 2017 Mar 19 doi: 10.1155/2017/9378325. PMID: 28401166Free PMC Article
Yin W, Bian Z
Oral Dis 2016 May;22(4):265-73. Epub 2016 Jan 13 doi: 10.1111/odi.12400. PMID: 26600092
Kokich VO Jr, Kinzer GA
J Esthet Restor Dent 2005;17(1):5-10. doi: 10.1111/j.1708-8240.2005.tb00076.x. PMID: 15934680
Kjaer I
Crit Rev Oral Biol Med 1998;9(2):224-44. doi: 10.1177/10454411980090020501. PMID: 9603237

Clinical prediction guides

Letra A
Curr Osteoporos Rep 2022 Dec;20(6):389-397. Epub 2022 Nov 15 doi: 10.1007/s11914-022-00761-8. PMID: 36378475Free PMC Article
Bonczek O, Krejci P, Izakovicova-Holla L, Cernochova P, Kiss I, Vojtesek B
Clin Genet 2021 Apr;99(4):493-502. Epub 2021 Feb 16 doi: 10.1111/cge.13892. PMID: 33249565
Al-Ani AH, Antoun JS, Thomson WM, Merriman TR, Farella M
Biomed Res Int 2017;2017:9378325. Epub 2017 Mar 19 doi: 10.1155/2017/9378325. PMID: 28401166Free PMC Article
Yin W, Bian Z
Oral Dis 2016 May;22(4):265-73. Epub 2016 Jan 13 doi: 10.1111/odi.12400. PMID: 26600092
Kokich VO Jr, Kinzer GA
J Esthet Restor Dent 2005;17(1):5-10. doi: 10.1111/j.1708-8240.2005.tb00076.x. PMID: 15934680

Recent systematic reviews

Minervini G, Franco R, Marrapodi MM, Mehta V, Fiorillo L, Badnjević A, Cervino G, Cicciù M
Medicina (Kaunas) 2023 Mar 28;59(4) doi: 10.3390/medicina59040670. PMID: 37109627Free PMC Article
Cerezo-Cayuelas M, Pérez-Silva A, Serna-Muñoz C, Vicente A, Martínez-Beneyto Y, Cabello-Malagón I, Ortiz-Ruiz AJ
Orphanet J Rare Dis 2022 Oct 17;17(1):376. doi: 10.1186/s13023-022-02533-0. PMID: 36253866Free PMC Article
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Carter K, Worthington S
J Dent Res 2015 Jul;94(7):886-94. Epub 2015 Apr 16 doi: 10.1177/0022034515581644. PMID: 25883107
Khalaf K, Miskelly J, Voge E, Macfarlane TV
J Orthod 2014 Dec;41(4):299-316. doi: 10.1179/1465313314Y.0000000116. PMID: 25404667

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