Ulnar hemimelia is a rare congenital upper limb anomaly characterized by complete or partial absence of the ulna bone.
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Epidemiology
Incidence is estimated at 1/100,000-150,000 live births, with a male to female ratio of 3:2.
Associations
Ulnar hemimelia may be associated with other skeletal anomalies like syndactyly or it may be part of a syndrome like Poland syndrome, Klippel-Feil syndrome, Goltz-Gorlin syndrome etc.
Presentation
Oligodactyly and shortening of forearm is noted with ulnar tilt of the wrist joint. The position of the hand tends to drift to the ulnar-side of the wrist. Cubital webbing is also seen in most cases which may cause fixed flexion deformity. Patients with ulnar hemimelia have motion and functional limitations as well.
Radiographic features
- absent ulna
- severely shortened ulna
- oligodactyly involving the ulnar side of carpals, metacarpals and phalanges
- fixed flexion deformity of the elbow joint
- radiohumeral synostosis may sometimes be present
Management
The management of such cases depend on the age of the patient, bilaterality, and the scope of the handicap. In unilateral cases, non surgical interventions like passive stretching exercises, prosthesis, corrective casting and splinting are performed to reduce the cosmetic deformity and improve functionality of the limb.
Surgery is indicated in bilateral involvement with severe handicap. Surgical management includes Z-plasty in the cubital fossa to decrease the cubital web, elbow disarticulation and humeral derotation osteotomy to increase range of motion.
Differential diagnosis
General imaging differential considerations include
- Madelung deformity
- short ulna
- dyschondrogenesis
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