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Delayed eruption of permanent teeth

MedGen UID:
340353
Concept ID:
C1849540
Finding
Synonyms: Delayed eruption of secondary teeth; Delayed permanent dentition
 
HPO: HP:0000696

Definition

Delayed tooth eruption affecting the secondary dentition. [from HPO]

Conditions with this feature

Cleidocranial dysostosis
MedGen UID:
3486
Concept ID:
C0008928
Disease or Syndrome
Cleidocranial dysplasia (CCD) spectrum disorder is a skeletal dysplasia that represents a clinical continuum ranging from classic CCD (triad of delayed closure of the cranial sutures, hypoplastic or aplastic clavicles, and dental abnormalities) to mild CCD to isolated dental anomalies without the skeletal features. Most individuals come to diagnosis because they have classic features. At birth, affected individuals typically have abnormally large, wide-open fontanelles that may remain open throughout life. Clavicular hypoplasia can result in narrow, sloping shoulders that can be opposed at the midline. Moderate short stature may be observed, with most affected individuals being shorter than their unaffected sibs. Dental anomalies may include supernumerary teeth, eruption failure of the permanent teeth, and presence of the second permanent molar with the primary dentition. Individuals with CCD spectrum disorder are at increased risk of developing recurrent sinus infections, recurrent ear infections leading to conductive hearing loss, and upper-airway obstruction. Intelligence is typically normal.
Pyknodysostosis
MedGen UID:
116061
Concept ID:
C0238402
Disease or Syndrome
Pycnodysostosis is characterized by short-limbed short stature, typical facial appearance (convex nasal ridge and small jaw with obtuse mandibular angle), osteosclerosis with increased bone fragility, acroosteolysis of the distal phalanges, delayed closure of the cranial sutures, and dysplasia of the clavicle. In affected individuals, the facial features become more prominent with age, likely due to progressive acroosteolysis of the facial bones, but can usually be appreciated from early childhood, particularly the small jaw and convex nasal ridge. Additional features include dental and nail anomalies. Intelligence is typically normal with mild psychomotor difficulties reported in some individuals.
Andersen Tawil syndrome
MedGen UID:
327586
Concept ID:
C1563715
Disease or Syndrome
Andersen-Tawil syndrome (ATS) is characterized by a triad of: episodic flaccid muscle weakness (i.e., periodic paralysis); ventricular arrhythmias and prolonged QT interval; and anomalies including low-set ears, widely spaced eyes, small mandible, fifth-digit clinodactyly, syndactyly, short stature, and scoliosis. Affected individuals present in the first or second decade with either cardiac symptoms (palpitations and/or syncope) or weakness that occurs spontaneously following prolonged rest or following rest after exertion. Mild permanent weakness is common. Mild learning difficulties and a distinct neurocognitive phenotype (i.e., deficits in executive function and abstract reasoning) have been described.
Brachydactyly type B1
MedGen UID:
349432
Concept ID:
C1862112
Congenital Abnormality
A rare subtype of brachydactyly type B characterized by hypoplasia or aplasia of the distal phalanges of digits 2-5 with or without nail dysplasia, in association with fusion of the middle and distal phalanges, a broad or bifid thumb, and occasionally distal and proximal symphalangism or syndactyly. The feet are less severely affected than the hands.
Weismann-Netter syndrome
MedGen UID:
350610
Concept ID:
C1862172
Disease or Syndrome
The diagnostic hallmarks of Weismann-Netter syndrome (WNS) are anterior bowing of the diaphyses of the tibia and fibula, broadening or 'tibialization' of the fibula, posterior cortical thickening of both bones, and short stature. The diaphyses of other long bones may be similarly affected, but usually to a milder degree. Some WNS patients have also displayed mental retardation (summary by Peippo et al., 2009).
Craniometaphyseal dysplasia, autosomal recessive
MedGen UID:
419753
Concept ID:
C2931244
Disease or Syndrome
Craniometaphyseal dysplasia is an osteochondrodysplasia characterized by hyperostosis and sclerosis of the craniofacial bones associated with abnormal modeling of the metaphyses. Sclerosis of the skull may lead to asymmetry of the mandible, as well as to cranial nerve compression, that may finally result in hearing loss and facial palsy (summary by Nurnberg et al., 1997). The delineation of separate autosomal dominant (CMDD; 123000) and autosomal recessive forms of CMD by Gorlin et al. (1969) was confirmed by reports that made it evident that the dominant form is relatively mild and comparatively common, whereas the recessive form is rare, severe, and possibly heterogeneous.
Amelogenesis imperfecta type 1G
MedGen UID:
419162
Concept ID:
C2931783
Disease or Syndrome
Amelogenesis imperfecta and gingival fibromatosis syndrome is an autosomal recessive condition characterized by mild gingival fibromatosis and dental anomalies, including hypoplastic amelogenesis imperfecta, intrapulpal calcifications, delay of tooth eruption, hypodontia/oligodontia, pericoronal radiolucencies, and unerupted teeth (Martelli-Junior et al., 2008).
Intellectual disability, autosomal dominant 15
MedGen UID:
766162
Concept ID:
C3553248
Disease or Syndrome
Coffin-Siris syndrome is a congenital malformation syndrome characterized by developmental delay, intellectual disability, coarse facial features, feeding difficulties, and hypoplastic or absent fifth fingernails and fifth distal phalanges. Other more variable features may also occur. Patients with SMARCB1 mutations may have more severe neurodevelopmental deficits including severe intellectual disability, brain structural abnormalities, and no expressive words, as well as scoliosis (summary by Kosho et al., 2014). For a general phenotypic description and a discussion of genetic heterogeneity of Coffin-Siris syndrome, see CSS1 (135900).
Coffin-Siris syndrome 10
MedGen UID:
1683634
Concept ID:
C4760583
Disease or Syndrome
Coffin-Siris syndrome (CSS) is classically characterized by aplasia or hypoplasia of the distal phalanx or nail of the fifth and additional digits, developmental or cognitive delay of varying degree, distinctive facial features, hypotonia, hirsutism/hypertrichosis, and sparse scalp hair. Congenital anomalies can include malformations of the cardiac, gastrointestinal, genitourinary, and/or central nervous systems. Other findings commonly include feeding difficulties, slow growth, ophthalmologic abnormalities, and hearing impairment.
Autosomal recessive Robinow syndrome
MedGen UID:
1770070
Concept ID:
C5399974
Disease or Syndrome
ROR2-related Robinow syndrome is characterized by distinctive craniofacial features, skeletal abnormalities, and other anomalies. Craniofacial features include macrocephaly, broad prominent forehead, low-set ears, ocular hypertelorism, prominent eyes, midface hypoplasia, short upturned nose with depressed nasal bridge and flared nostrils, large and triangular mouth with exposed incisors and upper gums, gum hypertrophy, misaligned teeth, ankyloglossia, and micrognathia. Skeletal abnormalities include short stature, mesomelic or acromesomelic limb shortening, hemivertebrae with fusion of thoracic vertebrae, and brachydactyly. Other common features include micropenis with or without cryptorchidism in males and reduced clitoral size and hypoplasia of the labia majora in females, renal tract abnormalities, and nail hypoplasia or dystrophy. The disorder is recognizable at birth or in early childhood.
Odontochondrodysplasia 2 with hearing loss and diabetes
MedGen UID:
1782909
Concept ID:
C5543275
Disease or Syndrome
Odontochondrodysplasia-2 with hearing loss and diabetes (ODCD2) is characterized by growth retardation with proportionate short stature, dentinogenesis imperfecta, sensorineural hearing loss, insulin-dependent diabetes, and mild intellectual disability (Cauwels et al., 2005; Lekszas et al., 2020). For a discussion of genetic heterogeneity of ODCD, see ODCD1 (184260).
Onychodystrophy, osteodystrophy, impaired intellectual development, and seizures syndrome
MedGen UID:
1788511
Concept ID:
C5543496
Disease or Syndrome
Onychodystrophy, osteodystrophy, impaired intellectual development, and seizures syndrome (OORS) is an autosomal recessive syndromic developmental disorder characterized by global developmental delay with impaired intellectual development, dysmorphic facial features, and hypoplastic terminal phalanges and nails. Patients have seizures or tonic posturing. The disorder is associated with a defect in GPI anchoring of membrane-bound proteins (summary by Salian et al., 2021). For a discussion of genetic heterogeneity of GPI biosynthesis defects, see GPIBD1 (610293). See also DOORS syndrome (220500), which shows some overlapping clinical features.
RECON progeroid syndrome
MedGen UID:
1841140
Concept ID:
C5830504
Disease or Syndrome
RECON progeroid syndrome (RECON) is a chromosomal instability disorder characterized by postnatal growth retardation, progeroid facial appearance, hypoplastic nose, prominent premaxilla, skin photosensitivity and xeroderma, muscle wasting with reduced subcutaneous fat, and slender elongated thumbs (Abu-Libdeh et al., 2022).

Professional guidelines

PubMed

Kämmerer PW, Schneider D, Schiegnitz E, Schneider S, Walter C, Frerich B, Kunkel M
Clin Oral Investig 2016 Sep;20(7):1827-35. Epub 2015 Nov 27 doi: 10.1007/s00784-015-1673-3. PMID: 26612404

Recent clinical studies

Etiology

Tsuji M, Suzuki H, Suzuki S, Moriyama K
Congenit Anom (Kyoto) 2020 Jul;60(4):106-114. Epub 2019 Oct 23 doi: 10.1111/cga.12358. PMID: 31599034Free PMC Article
Dimaisip-Nabuab J, Duijster D, Benzian H, Heinrich-Weltzien R, Homsavath A, Monse B, Sithan H, Stauf N, Susilawati S, Kromeyer-Hauschild K
BMC Pediatr 2018 Sep 14;18(1):300. doi: 10.1186/s12887-018-1277-6. PMID: 30217185Free PMC Article
Isola G, Cicciù M, Fiorillo L, Matarese G
J Craniofac Surg 2017 May;28(3):755-758. doi: 10.1097/SCS.0000000000003433. PMID: 28468159
Kämmerer PW, Schneider D, Schiegnitz E, Schneider S, Walter C, Frerich B, Kunkel M
Clin Oral Investig 2016 Sep;20(7):1827-35. Epub 2015 Nov 27 doi: 10.1007/s00784-015-1673-3. PMID: 26612404
Psoter W, Gebrian B, Prophete S, Reid B, Katz R
Community Dent Oral Epidemiol 2008 Apr;36(2):179-89. doi: 10.1111/j.1600-0528.2007.00386.x. PMID: 18333882

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
Jani P, Nguyen QC, Almpani K, Keyvanfar C, Mishra R, Liberton D, Orzechowski P, Frischmeyer-Guerrerio PA, Duverger O, Lee JS
J Med Genet 2020 Oct;57(10):699-707. Epub 2020 Mar 8 doi: 10.1136/jmedgenet-2019-106678. PMID: 32152251Free PMC Article
Tsuji M, Suzuki H, Suzuki S, Moriyama K
Congenit Anom (Kyoto) 2020 Jul;60(4):106-114. Epub 2019 Oct 23 doi: 10.1111/cga.12358. PMID: 31599034Free PMC Article
Isola G, Cicciù M, Fiorillo L, Matarese G
J Craniofac Surg 2017 May;28(3):755-758. doi: 10.1097/SCS.0000000000003433. PMID: 28468159
Dave KV, Patel SC, Dudhia BB, Panja P
Indian J Dent Res 2013 Jan-Feb;24(1):132-5. doi: 10.4103/0970-9290.114920. PMID: 23852247

Therapy

Dimaisip-Nabuab J, Duijster D, Benzian H, Heinrich-Weltzien R, Homsavath A, Monse B, Sithan H, Stauf N, Susilawati S, Kromeyer-Hauschild K
BMC Pediatr 2018 Sep 14;18(1):300. doi: 10.1186/s12887-018-1277-6. PMID: 30217185Free PMC Article
Doufexi A, Mina M, Ioannidou E
J Periodontol 2005 Jan;76(1):3-10. doi: 10.1902/jop.2005.76.1.3. PMID: 15830631

Prognosis

Alsani A, Balhaddad AA
J Contemp Dent Pract 2018 Dec 1;19(12):1434-1436. PMID: 30713169
Bhatia SK, Hunter ML, Ashley PF
Dent Update 2015 Dec;42(10):945-8, 950. doi: 10.12968/denu.2015.42.10.945. PMID: 26856001
O'Connell S, Davies J, Smallridge J, Vaidyanathan M
Eur Arch Paediatr Dent 2014 Oct;15(5):361-8. Epub 2013 Dec 19 doi: 10.1007/s40368-013-0106-8. PMID: 24353076
Alaçam A, Bani M
Dent Traumatol 2009 Apr;25(2):e25-31. doi: 10.1111/j.1600-9657.2008.00734.x. PMID: 19290890
Carter LC, Fischman SL, Mann J, Elstein D, Stabholz A, Zimran A
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998 Feb;85(2):233-9. doi: 10.1016/s1079-2104(98)90432-2. PMID: 9503462

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
Dimaisip-Nabuab J, Duijster D, Benzian H, Heinrich-Weltzien R, Homsavath A, Monse B, Sithan H, Stauf N, Susilawati S, Kromeyer-Hauschild K
BMC Pediatr 2018 Sep 14;18(1):300. doi: 10.1186/s12887-018-1277-6. PMID: 30217185Free PMC Article
Psoter W, Gebrian B, Prophete S, Reid B, Katz R
Community Dent Oral Epidemiol 2008 Apr;36(2):179-89. doi: 10.1111/j.1600-0528.2007.00386.x. PMID: 18333882
O'Connell AC, Puck JM, Grimbacher B, Facchetti F, Majorana A, Gallin JI, Malech HL, Holland SM
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000 Feb;89(2):177-85. doi: 10.1067/moe.2000.103129. PMID: 10673653

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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