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Delayed eruption of primary teeth

MedGen UID:
341477
Concept ID:
C1849538
Finding
Synonyms: Delayed deciduous tooth eruption; Delayed eruption of deciduous teeth; Delayed primary teeth eruption
 
HPO: HP:0000680

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Delayed eruption of primary teeth

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.
Cockayne syndrome type 2
MedGen UID:
155487
Concept ID:
C0751038
Disease or Syndrome
Cockayne syndrome (referred to as CS in this GeneReview) spans a continuous phenotypic spectrum that includes: CS type I, the "classic" or "moderate" form; CS type II, a more severe form with symptoms present at birth; this form overlaps with cerebrooculofacioskeletal (COFS) syndrome; CS type III, a milder and later-onset form; COFS syndrome, a fetal form of CS. CS type I is characterized by normal prenatal growth with the onset of growth and developmental abnormalities in the first two years. By the time the disease has become fully manifest, height, weight, and head circumference are far below the fifth percentile. Progressive impairment of vision, hearing, and central and peripheral nervous system function leads to severe disability; death typically occurs in the first or second decade. CS type II is characterized by growth failure at birth, with little or no postnatal neurologic development. Congenital cataracts or other structural anomalies of the eye may be present. Affected children have early postnatal contractures of the spine (kyphosis, scoliosis) and joints. Death usually occurs by age five years. CS type III is a phenotype in which major clinical features associated with CS only become apparent after age two years; growth and/or cognition exceeds the expectations for CS type I. COFS syndrome is characterized by very severe prenatal developmental anomalies (arthrogryposis and microphthalmia).
Cockayne syndrome type 1
MedGen UID:
155488
Concept ID:
C0751039
Disease or Syndrome
Cockayne syndrome (referred to as CS in this GeneReview) spans a continuous phenotypic spectrum that includes: CS type I, the "classic" or "moderate" form; CS type II, a more severe form with symptoms present at birth; this form overlaps with cerebrooculofacioskeletal (COFS) syndrome; CS type III, a milder and later-onset form; COFS syndrome, a fetal form of CS. CS type I is characterized by normal prenatal growth with the onset of growth and developmental abnormalities in the first two years. By the time the disease has become fully manifest, height, weight, and head circumference are far below the fifth percentile. Progressive impairment of vision, hearing, and central and peripheral nervous system function leads to severe disability; death typically occurs in the first or second decade. CS type II is characterized by growth failure at birth, with little or no postnatal neurologic development. Congenital cataracts or other structural anomalies of the eye may be present. Affected children have early postnatal contractures of the spine (kyphosis, scoliosis) and joints. Death usually occurs by age five years. CS type III is a phenotype in which major clinical features associated with CS only become apparent after age two years; growth and/or cognition exceeds the expectations for CS type I. COFS syndrome is characterized by very severe prenatal developmental anomalies (arthrogryposis and microphthalmia).
Oculodental syndrome, Rutherfurd type
MedGen UID:
163222
Concept ID:
C0796140
Disease or Syndrome
Oculodental syndrome, Rutherfurd type is a rare genetic disorder that is primarily characterized by the classical triad of gingival fibromatosis, non-eruption of tooth and corneal dystrophy (bilateral corneal vascularization and opacity). Abnormally shaped teeth have also been reported. The syndrome is transmitted as an autosomal dominant trait.
Emanuel syndrome
MedGen UID:
323030
Concept ID:
C1836929
Disease or Syndrome
Emanuel syndrome is characterized by pre- and postnatal growth deficiency, microcephaly, hypotonia, severe developmental delays, ear anomalies, preauricular tags or pits, cleft or high-arched palate, congenital heart defects, kidney abnormalities, and genital abnormalities in males.
Mandibulofacial dysostosis with alopecia
MedGen UID:
898794
Concept ID:
C4225349
Disease or Syndrome
A rare mandibulofacial dysostosis with the association with scalp alopecia and sparse eyebrows and eyelashes. Craniofacial dysmorphic features include zygomatic and mandibular dysplasia or hypoplasia, cleft palate, micrognathia, dental anomalies, auricular dysmorphism and eyelid anomalies among others. Patients may experience limited jaw mobility, glossoptosis, upper airway obstruction and conductive hearing loss.
Linear skin defects with multiple congenital anomalies 3
MedGen UID:
906997
Concept ID:
C4225421
Disease or Syndrome
Microphthalmia with linear skin defects (MLS) syndrome is characterized by unilateral or bilateral microphthalmia and/or anophthalmia and linear skin defects, usually involving the face and neck, which are present at birth and heal with age, leaving minimal residual scarring. Other findings can include a wide variety of other ocular abnormalities (e.g., corneal anomalies, orbital cysts, cataracts), central nervous system involvement (e.g., structural anomalies, developmental delay, infantile seizures), cardiac concerns (e.g., hypertrophic or oncocytic cardiomyopathy, atrial or ventricular septal defects, arrhythmias), short stature, diaphragmatic hernia, nail dystrophy, hearing impairment, and genitourinary malformations. Inter- and intrafamilial variability is described.
Intellectual disability, autosomal dominant 54
MedGen UID:
1614787
Concept ID:
C4540484
Mental or Behavioral Dysfunction
Marbach-Rustad progeroid syndrome
MedGen UID:
1784907
Concept ID:
C5543388
Disease or Syndrome
Marbach-Rustad progeroid syndrome (MARUPS) is characterized by progeroid appearance with little subcutaneous fat and triangular facies, growth retardation with short stature, hypoplastic mandible crowded with unerupted supernumerary teeth, and cerebellar intention tremor. Psychomotor development is normal. Although features are reminiscent of Hutchinson-Gilford progeria syndrome (HGPS; 176670), MARUPS is less severe, with a relatively good prognosis. Two patients have been reported (Marbach et al., 2019).
Cleidocranial dysplasia 2
MedGen UID:
1824016
Concept ID:
C5774243
Disease or Syndrome
Cleidocranial dysplasia-2 (CLCD2) is characterized by clavicular anomalies, ranging from unilateral 'clavicula bipartita' to bilateral clavicular aplasia, and dental anomalies, including delayed or absent eruption of deciduous teeth and supernumerary teeth. Skull abnormalities such as delayed closure of fontanels have been reported; other skeletal features include delayed bone age, short distal phalanges, and pseudoepiphyses of the metacarpals and/or metatarsals. Phenotypic variability, including intrafamilial, has been observed (Beyltjens et al., 2023). For a general phenotypic description and a discussion of genetic heterogeneity of cleidocranial dysplasia, see CLCD1 (119600).
LADD syndrome 1
MedGen UID:
1824096
Concept ID:
C5774323
Disease or Syndrome
Lacrimoauriculodentodigital syndrome-1 (LADD1) is a multiple congenital anomaly disorder mainly affecting lacrimal glands and ducts, salivary glands and ducts, ears, teeth, and distal limb segments (summary by Rohmann et al., 2006). Genetic Heterogeneity of Lacrimoauriculodentodigital Syndrome LADD syndrome-2 (LADD2; 620192) is caused by mutation in the FGFR3 gene (134934) on chromosome 4p16, and LADD syndrome-3 (LADD3; 620193) is caused by mutation in the FGF10 gene, an FGFR ligand, on chromosome 5p12.

Professional guidelines

PubMed

Heggie C, Gartshore L
J Dent Child (Chic) 2020 Jan 15;87(1):39-43. PMID: 32151309
Wu H, Chen T, Ma Q, Xu X, Xie K, Chen Y
Sci Rep 2019 Feb 25;9(1):2645. doi: 10.1038/s41598-019-39572-w. PMID: 30804498Free PMC Article
Tieu LD, Walker SL, Major MP, Flores-Mir C
J Am Dent Assoc 2013 Jun;144(6):602-11. doi: 10.14219/jada.archive.2013.0171. PMID: 23729457

Recent clinical studies

Etiology

Seehra J, Mortaja K, Wazwaz F, Papageorgiou SN, Newton JT, Cobourne MT
Am J Orthod Dentofacial Orthop 2023 May;163(5):594-608. Epub 2023 Mar 11 doi: 10.1016/j.ajodo.2023.01.004. PMID: 36907703
Topal BG, Tanrikulu A
J Clin Pediatr Dent 2023 Mar;47(2):50-57. Epub 2023 Mar 3 doi: 10.22514/jocpd.2023.011. PMID: 36890742
Mysore ID, Nandlal B, Narayanappa D
Indian J Dent Res 2022 Oct-Dec;33(4):373-377. doi: 10.4103/ijdr.ijdr_56_22. PMID: 37005999
Turner S, Harrison JE, Sharif FN, Owens D, Millett DT
Cochrane Database Syst Rev 2021 Dec 31;12(12):CD003453. doi: 10.1002/14651858.CD003453.pub2. PMID: 34970995Free PMC Article
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

Diagnosis

Muthu MS, Vandana S, Akila G, Anusha M, Kandaswamy D, Aswath Narayanan MB
Arch Oral Biol 2024 Feb;158:105857. Epub 2023 Dec 6 doi: 10.1016/j.archoralbio.2023.105857. PMID: 38128337
Kurosaka H, Itoh S, Morita C, Tsujimoto T, Murata Y, Inubushi T, Yamashiro T
J Oral Biosci 2022 Jun;64(2):159-164. Epub 2022 Feb 26 doi: 10.1016/j.job.2022.02.005. PMID: 35231627
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
Zabeen B, Mohsin F, Taher A, Khan M, Azad K, Nahar N
Mymensingh Med J 2008 Jan;17(1):82-4. PMID: 18285740
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

Therapy

Seehra J, Mortaja K, Wazwaz F, Papageorgiou SN, Newton JT, Cobourne MT
Am J Orthod Dentofacial Orthop 2023 May;163(5):594-608. Epub 2023 Mar 11 doi: 10.1016/j.ajodo.2023.01.004. PMID: 36907703
Mysore ID, Nandlal B, Narayanappa D
Indian J Dent Res 2022 Oct-Dec;33(4):373-377. doi: 10.4103/ijdr.ijdr_56_22. PMID: 37005999
Garg A, Kumar G, Goswami M, Verma D
J Indian Soc Pedod Prev Dent 2022 Apr-Jun;40(2):118-123. doi: 10.4103/jisppd.jisppd_186_22. PMID: 35859402
Turner S, Harrison JE, Sharif FN, Owens D, Millett DT
Cochrane Database Syst Rev 2021 Dec 31;12(12):CD003453. doi: 10.1002/14651858.CD003453.pub2. PMID: 34970995Free PMC Article
Partyka M, Chałas R, Dunin-Wilczyńska I, Drohomyretska M, Klatka M
Ann Agric Environ Med 2018 Mar 14;25(1):60-65. Epub 2017 Feb 11 doi: 10.5604/12321966.1233573. PMID: 29575874

Prognosis

Mysore ID, Nandlal B, Narayanappa D
Indian J Dent Res 2022 Oct-Dec;33(4):373-377. doi: 10.4103/ijdr.ijdr_56_22. PMID: 37005999
Villemur Moreau L, Dicky O, Mas E, Noirrit E, Marty M, Vaysse F, Olives JP
Arch Pediatr 2021 Feb;28(2):105-110. Epub 2020 Dec 17 doi: 10.1016/j.arcped.2020.11.002. PMID: 33341334
Nelson S, Albert JM, Soderling E, Malik A, Curtan S, Geng C, Milgrom P
Eur J Oral Sci 2014 Oct;122(5):346-52. Epub 2014 Sep 3 doi: 10.1111/eos.12147. PMID: 25183438Free PMC Article
Tieu LD, Walker SL, Major MP, Flores-Mir C
J Am Dent Assoc 2013 Jun;144(6):602-11. doi: 10.14219/jada.archive.2013.0171. PMID: 23729457
Ismail AI
Spec Care Dentist 1998 Jan-Feb;18(1):40-5. doi: 10.1111/j.1754-4505.1998.tb01357.x. PMID: 9791306

Clinical prediction guides

Mysore ID, Nandlal B, Narayanappa D
Indian J Dent Res 2022 Oct-Dec;33(4):373-377. doi: 10.4103/ijdr.ijdr_56_22. PMID: 37005999
Shih-Wei Cheng E, Tsuji M, Suzuki S, Moriyama K
Eur J Orthod 2022 Dec 1;44(6):711-722. doi: 10.1093/ejo/cjac039. PMID: 35833575
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
de la Dure-Molla M, Quentric M, Yamaguti PM, Acevedo AC, Mighell AJ, Vikkula M, Huckert M, Berdal A, Bloch-Zupan A
Orphanet J Rare Dis 2014 Jun 14;9:84. doi: 10.1186/1750-1172-9-84. PMID: 24927635Free PMC Article
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

Muthu MS, Vandana S, Akila G, Anusha M, Kandaswamy D, Aswath Narayanan MB
Arch Oral Biol 2024 Feb;158:105857. Epub 2023 Dec 6 doi: 10.1016/j.archoralbio.2023.105857. PMID: 38128337
Seehra J, Mortaja K, Wazwaz F, Papageorgiou SN, Newton JT, Cobourne MT
Am J Orthod Dentofacial Orthop 2023 May;163(5):594-608. Epub 2023 Mar 11 doi: 10.1016/j.ajodo.2023.01.004. PMID: 36907703
Turner S, Harrison JE, Sharif FN, Owens D, Millett DT
Cochrane Database Syst Rev 2021 Dec 31;12(12):CD003453. doi: 10.1002/14651858.CD003453.pub2. PMID: 34970995Free PMC Article
Tieu LD, Walker SL, Major MP, Flores-Mir C
J Am Dent Assoc 2013 Jun;144(6):602-11. doi: 10.14219/jada.archive.2013.0171. PMID: 23729457
Huber KL, Suri L, Taneja P
J Clin Pediatr Dent 2008 Spring;32(3):221-30. doi: 10.17796/jcpd.32.3.m175g328l100x745. PMID: 18524273

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