Radial hemimelia is a congenital abnormality characterized by partial or total absence of the radius bone of the forearm. It falls within the spectrum of radial ray anomalies.
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Epidemiology
Radial hemimelia occurs in 1:30,000-100,000 live births, and is slightly more common in males than in females (M:F = 3:2).
Associations
thrombocytopenia-absent radius (TAR) syndrome (in which characteristically the thumb is present)
Pathology
This deformity is believed to develop at around the 28th to 56th day of gestation. The exact etiology has not been proven, however uterine compression, vascular injury, and certain maternal drug consumption have been postulated.
Classification
type I: short distal radius
type II: hypoplastic radius
type III: partial absence of radius
type IV: complete absence of radius 3
Radiographic features
Antenatal ultrasound
Radial hemimelia can be detected in the anomaly scan done in the second trimester.
Treatment and prognosis
Straightening of the hand and wrist stabilization through splinting are both techniques utilized after birth 4. Physiotherapy is required to improve passive extension of the elbow and wrist. Surgical correction (e.g. osteotomy, arthrodesis, fibular transplants, and centralisation) is used in severe cases, generally performed at age 1 5,6. Pollicisation is used if an absent thumb is present.
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