Pterygium Cubitale

Ulnar Deficiency The Forearm
Figure 77—3. Clinical photograph of a 7-year-old boy with type III ulnar clubhand with absence of the ulna, pterygium cubitale, and severe hand anomalies.

Radial deficiency is more common and associated with visceral abnormalities (e.g., cardiac or genitourinary), whereas ulnar deficiency is linked with musculoskeletal anomalies that may involve the contralateral limb, spine, or lower extremities (e.g., proximal focal femoral deficiencies, congenital amputations, or scoliosis). In radial deficiency, the radius is often absent and the wrist unstable in severe radial deviation, whereas in ulnar deficiency the ulna is frequently partially absent and the wrist stable in slight ulnar deviation. Arthrogryposis multiplex congenita is a syndrome of multiple joint contractures that are present at birth and may be diffuse in presentation or isolated to the upper or lower extremity. The skin is often waxy and devoid of skin creases, and considerable muscle wasting is evident. The typical upper extremity posture is shoulder adduction and internal rotation, elbow extension, forearm pronation, wrist flexion, and ulnar deviation. Radioulnar synostosis is a transverse fusion of the proximal radius and ulna that prevents forearm rotation, not flexion/extension. The condition may be bilateral, but is not associated with hand deformities. Pterygium cubitale is a syndrome of congenital webbing of the neck, axilla, knees, and digits. In type III ulnar deficiency there may be pterygium cubitale, but it is an isolated finding (Fig. 77-3).


Ulnar Deficiency

Ulnar deficiency is a failure of formation or longitudinal deficiency of the postaxial border of the upper extremity. Most cases are sporadic, nonhereditary, and without

Table 77-1 Classification of Ulnar Clubhand




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