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.
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Epidemiology
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
Associations may include:
- Down syndrome
- pes cavus
- absence of the flexor digitorum superficialis tendon in the 5th digit
- myositis ossificans
- Turner syndrome
- Silver syndrome
- Cornelia de Lange syndrome
- congenital cardiac abnormalities 2
Clinical presentation
Although the presentation is usually with deformity, in some patients swelling and erythema may be present 4.
Radiographic features
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
Treatment and prognosis
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.
History and etymology
It was first described by J Kirner in 1927 3.
Differential diagnosis
Consider
- clinodactyly: radial deviation at the DIP joint
- camptodactyly: flexion deformity at the PIP joint
- frostbite
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