Windblown hand

Congenital ulnar drift or windblown hand describes a congenital upper limb anomaly. There is ulnar deviation of the metacarpophalangeal joints of the hand with intrinsic tightness causing a flexion contracture. It is associated with congenital clasped thumb, arthrogryposis and Freeman-Sheldon Syndrome. Mostly sporadic, there can be an autosomal dominant inheritance.

The aetiology is a subluxation of the extensor tendons into the spaces between the metacarpal heads.

Clinically, the deformity is present and birth and progresses with growth. Function can be surprisingly good.

Treatment is unsatisfactory. Non-operative measures include extension splinting of the MCPJ's and the first web space.

• metacarpophalangeal digital deformities:
  • soft tissue releases including release of the intrinsic muscles
  • extensor tendon centralization over the dorsal aspect of the MCPJ
  • rarely, shortening of the metacarpals to permit adequate extension
• thumb deformities:
  • widening of first web with a dorsal transposition flap or Z-plasty flap
  • flexor pollicis longus lengthening