Windblown Hand - observation

Historical Overview

• The first known description of windblown hand in the literature was in 1897, when Emile Boix used Brissaud’s descriptive phrase “deviation en coup de vent,” which means windblown fingers. Jacquemain used the term “windmill-vane hand” in 1966, “congenital ulnar drift of the fingers” was preferred by Zancolli and Zancolli in 1984, and a number of other descriptive names have been used throughout history, but ‘‘windblown hand’’ is generally accepted as the preferred term in English language literature.^1-3

Description

• Windblown hand, or congenital ulnar drift of fingers, is a rare congenital anomaly with characteristic hand deformities, which can be identified through a visual observation.³

Pathophysiology

• Windblown hand may be a variation of some type of arthrogryposis that includes several characteristic hand deformities, particularly flexion contracture of the fingers, ulnar drift of the fingers at the metacarpophalangeal (MP) joint, and thumb adduction with first web space contracture.³
• Children with windblown hand deformities associated with arthrogryposis have missing skin flexion creases.
• The cause of windblown hand is still not clearly understood, but there are numerous theories attempting to explain its etiology and pathogenesis. It is most commonly accepted to be a segmental arthrogryposis as described by Zancolli and Zancolli in 1984, but the “extensor failure” theory from Fisk and House in 1974 is also significant because the authors noted the anatomical absence of normal extensor tendons. Abnormalities in palmar fascia have been described as a causative factor by Jacquemain in 1967, and other intraoperative observations have included an attenuated extensor pollicis longus tendon, shortened flexor pollicis longus tendon, thumb MP joint instability, and volar skin shortening.^2,3

Instructions

1. Obtain an accurate and complete patient history that includes pregnancy and delivery information, family history, physical and neurological examinations, involvement of affected joints, intellectual development, and response to treatment.
2. Perform a careful examination of both upper extremities.
3. Look for the presence of any flexion contractures of the fingers, ulnar drift at the MP joints, or thumb adduction with first web space contracture.

Related Signs and Tests⁴

• Laboratory tests
  • Electrophysiological studies
  • Pathologic examinations (including muscle and/or nerve biopsy)
  • Gene sequencing 
• Prenatal screening
  • Ultrasound

Diagnostic Performance Characteristics

• Ulnar drift of the fingers is the most common abnormality found during the examination, but thumb and first web space abnormalities may also present significant impairments for patients.³

Diagnosis

• Windblown hand
• Arthrogryposis