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Disease Profile

Ankylosis of teeth

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Childhood

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ICD-10

K03.5

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Molar I reinclusion; Dental ankylosis; Secondary retention of permanent molars;

Categories

Congenital and Genetic Diseases; Mouth Diseases

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
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Orpha Number: 1077

Definition
A rare disorder characterized by the fusion of the tooth to the bone, preventing both eruption and orthodontic movement.

Epidemiology
The prevalence is unknown.

Clinical description
The periodontal ligament is obliterated by a 'bony bridge' and the tooth root is fused to the alveolar bone. Dental ankylosis can affect both primary and permanent teeth, may occur at any time during eruption and can lead to submergence. Permanent molars are less frequently affected than deciduous molars. Varying numbers of teeth may be affected. After eruption, it halts any adaptive changes. In a growing child, an ankylosed tooth appears to 'submerge' as adjacent unaffected teeth and alveolar bone continue their normal pattern of growth occlusally. The disorder may result in loss of the retained molar and neighboring teeth due to caries and periodontal disease, and in deformation of the facial skeleton (reduction in the height of the lower face, relative mandibular prognathism, posterior open bite). The major characteristic of a secondarily retained molar is infraocclusion that may result in malocclusion. Occasionally, dental ankylosis may be associated with fifth finger clinodactyly.

Etiology
Etiology remains uncertain but a genetic predisposition to ankylosis with autosomal dominant inheritance has been suggested. Familial occurrence has been shown in several families. Trauma, inflammation or infection may play a causative role.

Diagnostic methods
Clinical examination and X-ray are the main diagnostic methods for detecting ankylosis.

Management and treatment
The recommended management includes removing the ankylosed tooth to ensure development and eruption of the permanent teeth, and surgery to expose, protect, or reposition the emerging tooth.

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Reduced number of teeth
Decreased tooth count
0009804
30%-79% of people have these symptoms
Abnormality of dental enamel
Abnormal tooth enamel
Enamel abnormalities
Enamel abnormality

[ more ]

0000682
5%-29% of people have these symptoms
Clinodactyly of the 5th finger
Permanent curving of the pinkie finger
0004209
Mandibular prognathia
Big lower jaw
Increased projection of lower jaw
Increased size of lower jaw
Large lower jaw
Prominent chin
Prominent lower jaw

[ more ]

0000303
Percent of people who have these symptoms is not available through HPO
Abnormality of the dentition
Abnormal dentition
Abnormal teeth
Dental abnormality

[ more ]

0000164
Autosomal dominant inheritance
0000006

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Ankylosis of teeth. Click on the link to view a sample search on this topic.