postaxiale Polydaktylie

Post-axial polydactyly refers to polydactyly where the additional digit is on the ulnar margin of the hand, or lateral to the 5(little) toe.

Epidemiology

Post-axial polydactyly is more common than preaxial polydactyly, with an estimated incidence of 1 in 3000 .

Associations

Post-axial polydactyly can be associated with:

Pathology

The majority of cases are idiopathic. Associations with mutations to the GLI3 gene on chromosome 7 as well as a second locus mapped to chromosome 13 have been implicated in post-axial polydactyly .

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
Siehe auch: