Postaxial polydactyly
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Post-axial polydactyly refers to polydactyly where the additional digit is on the ulnar margin of the hand, or lateral to the 5th (little) toe.
Epidemiology
Post-axial polydactyly is more common than pre-axialpreaxial polydactyly, with an estimated incidence of 1 in 3000 5.
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
Pathology
The majority of cases are idiopathic. Associations with mutations to the GLI3 gene on chromosome 7 as well as a second locilocus 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 13Bardet-Biedl syndromeMeckel Gruber syndromeMcKusick-Kaufman syndrome-
followingoral-facial-digital syndromes (OFDS) -
certainskeletal dysplasias Smith Lemli Opitz syndromecertain 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 <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>type A: additional digit at the MCP joint (or more proximally at the CMC joint)</li>-<li>type B: small nubbin of non-functioning tissue or additional soft tissue on a pedicle</li>-</ul><p>Or, you can use a separate three-part classification system:</p><ul>-<li>type I: nubbin or floating digit</li>-<li>type II: duplications at the MCPJ</li>-<li>type III: duplication of the entire ray</li>-</ul><h5>Associations</h5><p>Post-axial polydactyly can be associated with:</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>(little) toe.</p><h4>Epidemiology</h4><p>Post-axial polydactyly is more common than <a href="/articles/pre-axial-polydactyly">preaxial polydactyly</a>, with an estimated incidence of 1 in 3000 <sup>5</sup>.</p><h5>Associations</h5><p>Post-axial polydactyly can be associated with:</p><ul>
-<a href="/articles/oral-facial-digital-syndrome-ofds-type-ii">oral-facial-digital syndrome (OFDS) type II </a>- <a href="/articles/mohr-syndrome">Mohr syndrome </a>- +<a href="/articles/oral-facial-digital-syndrome-ofds-type-ii">oral-facial-digital syndrome (OFDS) type II </a>- <a href="/articles/mohr-syndrome">Mohr syndrome</a>
-<li><a href="/articles/oral-facial-digital-syndrome-ofds-type-vi">oral-facial-digital syndrome (OFDS) type VI </a></li>- +<li><a href="/articles/oral-facial-digital-syndrome-ofds-type-vi">oral-facial-digital syndrome (OFDS) type VI</a></li>
-<a href="/articles/chondroectodermal-dysplasia">chondroectodermal dysplasia </a>- <a href="/articles/chondroectodermal-dysplasia">Ellis-van Creveld syndrome </a>- +<a href="/articles/chondroectodermal-dysplasia">chondroectodermal dysplasia </a>- <a href="/articles/chondroectodermal-dysplasia">Ellis-van Creveld syndrome</a>
-<a href="/articles/asphyxiating-thoracic-dysplasia">asphyxiating thoracic dysplasia </a>- <a href="/articles/asphyxiating-thoracic-dysplasia">Jeune syndrome</a>- +<a href="/articles/asphyxiating-thoracic-dysplasia">asphyxiating thoracic dysplasia </a>- <a href="/articles/asphyxiating-thoracic-dysplasia">Jeune syndrome</a>
-<li><a href="/articles/smith-lemli-opitz-syndrome-2">Smith Lemli Opitz syndrome </a></li>-<li>certain short rib polydactyly syndromes: e.g types I and III</li>- +<li><a href="/articles/smith-lemli-opitz-syndrome-2">Smith Lemli Opitz syndrome</a></li>
- +<li>certain <a title="short rib polydactyly syndrome" href="/articles/short-rib-polydactyly-syndrome">short rib polydactyly syndromes</a>: e.g types I and III</li>
- +</ul><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 locus 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>type A: additional digit at the MCP joint (or more proximally at the CMC joint)</li>
- +<li>type B: small nubbin of non-functioning tissue or additional soft tissue on a pedicle</li>
- +</ul><p>Or, you can use a separate three-part classification system:</p><ul>
- +<li>type I: nubbin or floating digit</li>
- +<li>type II: duplications at the MCPJ</li>
- +<li>type III: duplication of the entire ray</li>
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