Kirner deformity

Last revised by Daniel J Bell on 6 Jan 2020

A Kirner deformity, also known as dystelephalangy, is characterized by a curvature of the distal phalanx of the 5th digit in both a palmar and radial direction.

The deformity typically presents in late childhood to early adolescence, although a mild deformity may be present at birth. Both sexes are affected, although there is a predominance of cases in women reported, perhaps indicating a gender predilection 2.

Inheritance has been described as autosomal dominant with variable penetrance, however many cases appear sporadic 1,2

Associations may include:

Although the presentation is usually with deformity, in some patients swelling and erythema may be present 4.

Radiographic changes affect the terminal phalanx of the 5th digit, in most cases bilaterally, and are characterized as:

  • palmar and radial deviation
  • radiolucent nidus (1-2 mm) within the terminal tuft 2
  • shortened diaphysis
  • sclerosis of the diaphysis
  • metadiaphyseal defect

As this is purely a cosmetic problem (unless fine motor skills are required) medical treatment is optional. It is unclear whether splints alter the degree of deformity 4.  Osteotomy is an option if treatment is deemed necessary.

It was first described by J Kirner in 1927 3.

Consider

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