Congenital Ulnar Drift (Windblown Hand)
Congenital ulnar drift (CUD) of the fingers was first reported by Boix [1] who quoted Brissaud describing a congenital deviation of the digits (deviation des doigts en coup de vent), which Boix postulated was an “insufficiency of the palmar aponeurosis.”
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Congenital Ulnar Drift (Windblown Hand)
Congenital ulnar drift (CUD) of the fingers was first reported by Boix [1] who quoted Brissaud describing a congenital deviation of the digits (deviation des doigts en coup de vent), which Boix postulated was an “insufficiency of the palmar aponeurosis.” En coup de vent literally means windblown and other names for this condition have been congenital ulnar drift, windmill-vane hand, congenital cutaneous ulnar drift, windswept hand among others. The curious windblown eponym has survived as a colorful, somewhat descriptive term. The predominant features of the hand are metacarpophalangeal (MP) joint flexion, ulnar deviation of the digits, and in many children a flexed adducted thumb (clasped thumb) (. Fig. 19.1). The dynamic pathology causing these postures varies and as we shall see the clinical ulnar deviation and flexion may represent the end-stage of a number of processes. Within the first few years of life the drift often becomes evident as the joints are passively reducible. With time secondary soft tissue and skeletal changes will result in fixed contractures (. Fig. 19.2). There are a number of syndromes that contain conspicuous ulnar drift including: arthrogryposis, hemihypertrophy (hemihyperplasia), Marfan and other hyperlaxity syndromes, Escobar syndrome, Freeman-Sheldon syndrome, digito-talar dysmorphism, extensive lymphatic malformations, and what we shall call congenital ulnar drift without facial or lower extremity findings. The CUD per se is not a single diagnostic entity but does represent a clinical posture of the hand with a large number of associations (. Fig. 19.3). Acquired inflammatory conditions in adults such as rheumatoid arthritis may cause progressive ulnar deviation of the digits and secondary contractures of the thumb. The hands shown in . Fig. 19.1 represent what most pediatric hand surgeons call the classic windblown hand but even this particular example is subject to debate for the strict constructionist. The history is noteworthy as Freeman and Sheldon [2] in 1938 reported two children with several congenital malformations that had not been described previously. Their condition encompassed a triad of abnormal facial features, ulnar devia-
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tion of the digits, and talipes equinovarus, and they called the condition craniocarpotarsal dystrophy. Burian described four more patients who, in addition, had a small mouth, pursed lips, and hand deformities resembling arthrogryposis
Fig. 19.1 Hands and molds of the classic cleft hand a Hand molds accentuate the features of an adult and include a broad, spade-like palm, tight flexion tone of the extrinsic flexors to the central digits. Additionally a broad first web space, a pronated index ray, slight clinodactyly of the fifth digit and the thumb MP hyperextension, IP flexion deformity can be seen. b Loss of the transverse metacarpal arch is seen on the lateral view. c The clinical picture shows the flexion contractures, swan-neck posturing of the digits, deviation and excessive descent of the ulnar
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