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Multiple unerupted teeth

MedGen UID:
870618
Concept ID:
C4025069
Finding
Synonyms: Failure of eruption of multiple teeth; Multiple non-erupting teeth
 
HPO: HP:0006283

Definition

The presence of multiple embedded tooth germs which have failed to erupt. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Multiple unerupted teeth

Conditions with this feature

Eiken syndrome
MedGen UID:
325097
Concept ID:
C1838779
Congenital Abnormality
Eiken syndrome (EKNS) is an autosomal recessive skeletal dysplasia characterized by delayed ossification of bones, epiphyseal dysplasia, and bone remodeling abnormalities. Type A1 brachydactyly (see 112500), supernumerary epiphyses of proximal phalanges and metacarpals, and failure of eruption of primary teeth have also been described. Defining radiologic features include delayed ossification of epiphyses and primary ossification centers of short tubular bones, modeling abnormalities of tubular bones, and angel-shaped phalanges (Jacob et al., 2019). See 603740 for a disorder with similar radiologic features.
Splenogonadal fusion-limb defects-micrognathia syndrome
MedGen UID:
401073
Concept ID:
C1866745
Disease or Syndrome
Splenogonadal fusion (SGF) is a rare congenital anomaly of abnormal fusion between the spleen and the gonad or the remnants of the mesonephros. In 'continuous SGF,' there is a cord-like connection between the 2 organs, whereas in 'discontinuous SGF,' there is fusion of accessory splenic tissue and the gonad without a distinct structural connection to the spleen itself. Forty-eight percent of individuals with continuous SGF have additional malformations, compared to 9% of those with discontinuous SGF (McPherson et al., 2003).

Professional guidelines

PubMed

Frota CM, Pinheiro FHSL, Caetano IM, Carvalho RM, Aiello CA
J Clin Orthod 2021 Nov;55(11):1-13. PMID: 35015712
Rhoads SG, Hendricks HM, Frazier-Bowers SA
Am J Orthod Dentofacial Orthop 2013 Aug;144(2):194-202. doi: 10.1016/j.ajodo.2013.03.015. PMID: 23910200
Rushton VE, Horner K, Worthington HV
Br Dent J 2002 Mar 9;192(5):275-9. doi: 10.1038/sj.bdj.4801354. PMID: 11924954

Recent clinical studies

Etiology

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
de la Peña VA, Valea MC
J Am Dent Assoc 2011 Aug;142(8):938-41. doi: 10.14219/jada.archive.2011.0300. PMID: 21804060
Buch B, Noffke C, de Kock S
SADJ 2001 May;56(5):242-5. PMID: 11490697

Diagnosis

Desai RS, Momin YNA, Bansal S, Karjodkar FR
J Oral Maxillofac Surg 2017 Aug;75(8):1702-1705. Epub 2017 Jan 20 doi: 10.1016/j.joms.2017.01.011. PMID: 28199821
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
Brooks JK, Nikitakis NG
Gen Dent 2008 May-Jun;56(4):393, 395-6. PMID: 19284203
Buch B, Noffke C, de Kock S
SADJ 2001 May;56(5):242-5. PMID: 11490697

Prognosis

de la Peña VA, Valea MC
J Am Dent Assoc 2011 Aug;142(8):938-41. doi: 10.14219/jada.archive.2011.0300. PMID: 21804060

Clinical prediction guides

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

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