Postaxial polydactyly
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was changed:
Post-axial polydactyly refers to polydactyly where the additional digit is on the ulnar margin of the hand, or lateral to the 5th toe.
Epidemiology
Post-axial polydactyly is more common than pre-axial polydactyly, with an estimated incidence of 1 in 30005.
Pathology
The majority of cases are idiopathic. Associations with mutations to the GLI3 gene on chromosome 7 as well as a second loci mapped to chromosome 13 have been implicated in post-axial polydactyly 5.
Classification
Post-axial polydactyly has been defined by Temtamy and McKusick as:
- type A: additional digit at the MCP joint (or more proximally at the CMC joint)
- type B: small nubbin of non-functioning tissue or additional soft tissue on a pedicle
Or, you can use a separate three-part classification system:
- type I: nubbin or floating digit
- type II: duplications at the MCPJ
- type III: duplication of the entire ray
Associations
Post-axial polydactyly can be associated with:
- trisomy 13
- Bardet-Biedl syndrome
- Meckel Gruber syndrome
- McKusick-Kaufman syndrome
- following oral-facial-digital syndromes (OFDS)
- certain skeletal dysplasias
- Smith Lemli Opitz syndrome
- certain short rib polydactyly syndromes: e.g types I and III
-<p><strong>Post-axial polydactyly</strong> refers to <a href="/articles/polydactyly">polydactyly</a> where the additional digit is on the ulnar margin of the hand, or lateral to the 5<sup>th </sup>toe.</p><h4>Epidemiology</h4><p>Post-axial polydactyly is more common than <a href="/articles/pre-axial-polydactyly">pre-axial polydactyly</a>, with an estimated incidence of 1 in 3000.</p><h4>Pathology</h4><h5>Classification</h5><p><a href="/articles/pre-axial-and-post-axial-borders">Post-axial</a> polydactyly has been defined by Temtamy and McKusick as:</p><ul>- +<p><strong>Post-axial polydactyly</strong> refers to <a href="/articles/polydactyly">polydactyly</a> where the additional digit is on the ulnar margin of the hand, or lateral to the 5<sup>th </sup>toe.</p><h4>Epidemiology</h4><p>Post-axial polydactyly is more common than <a href="/articles/pre-axial-polydactyly">pre-axial polydactyly</a>, with an estimated incidence of 1 in 3000 <sup>5</sup>.</p><h4>Pathology</h4><p>The majority of cases are idiopathic. Associations with mutations to the <em>GLI3 </em>gene on chromosome 7 as well as a second loci mapped to chromosome 13 have been implicated in post-axial polydactyly <sup>5</sup>.</p><h5>Classification</h5><p><a href="/articles/pre-axial-and-post-axial-borders">Post-axial</a> polydactyly has been defined by Temtamy and McKusick as:</p><ul>
-<li>following <a href="/articles/oro-facial-digital-syndromes-1">oral-facial-digital syndromes (OFDS)</a><ul>- +<li>following <a href="/articles/oral-facial-digital-syndromes-1">oral-facial-digital syndromes (OFDS)</a><ul>
References changed:
- 5. Zhao H, Tian Y, Breedveld G et al. Postaxial Polydactyly Type A/B (PAP-A/B) is Linked to Chromosome 19p13.1-13.2 in a Chinese Kindred. Eur J Hum Genet. 2002;10(3):162-6. <a href="https://doi.org/10.1038/sj.ejhg.5200790">doi:10.1038/sj.ejhg.5200790</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/11973619">Pubmed</a>
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