Ulnar Longitudinal Deficiency

Ulnar longitudinal deficiency represents a rare spectrum of malformations that involve the digits, partial to complete absence of the ulna, carpals, and hypoplasia of related joints, muscles, tendons, nerves, and blood vessels. Ulnar longitudinal deficiency appears to be non-inherited, but does have a close association with other musculoskeletal anomalies, such as proximal femoral focal deficiency, fibular and tibial ray deficiency, phocomelia, scoliosis, clubfeet, absent patellae, and hip abnormalities. Clinically, the entire upper limb is hypoplastic, with the elbow abnormal or fused. Partial absence of the ulna is most common; usually with a stable wrist. The digits and thumb are commonly involved, and most of the surgical treatment is focused on the hand, including syndactyly release, web space deepening, and thumb metacarpal rotational osteotomy.