Radial hemimelia is a congenital absence of the radius bone of the forearm characterized by partial or total absence of the radius.
It occurs in 1:30,000-100,000 live births, and is slightly more common in males than in females (sex ratio of 3:2).
This deformity is believed to develop at about 28th to 56th day of gestation. Exact etiology has not been proved though uterine compression, vascular injury and certain maternal drug consumption have been postulated.
This deformity can be detected in the anomaly scan done in the second trimester.
- type I: short distal radius
- type II: hypoplastic radius
- type III: partial absence of radius
- type IV: complete absence of radius 3
- Holt-Oram syndrome
- Roberts syndrome
- Rothmund-Thomson syndrome
- thrombocytopenia-absent radius (TAR) syndrome (in which characteristically the thumb is present)
- Townes-Brocks syndrome
- VACTERL association
- Fanconi anemia
Treatment and prognosis
Straightening of the hand and wrist stabilization through splinting are both techniques utilized after birth 4. Physiotherapy input is required in order to improve passive extension of the elbow and wrist. Surgical correction (e.g. osteotomy, arthrodesis, fibular transplants, centralisation) is used for severe cases, generally performed at age 1 5,6. Pollicisation is used if an absent thumb is present.
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- 2. Corbera JA, Pulido M, Morales M et-al. Radiological findings in three cases of paraxial radial hemimelia in goats. J. Vet. Med. Sci. 01;64 (64): 843-5. doi:10.1292/jvms.64.843 - Pubmed citation
- 3. Heikel HV. Aplasia and hypoplasa of the radius: studies on 64 cases and on epiphyseal transplantation in rabbits with the imitated defect. (1959) Acta orthopaedica Scandinavica. Supplementum. 39: 1-155. Pubmed
- 4. Bora FW, Nicholson JT, Cheema HM. Radial meromelia. The deformity and its treatment. (1970) The Journal of bone and joint surgery. American volume. 52 (5): 966-79. Pubmed
- 5. Watson HK, Beebe RD, Cruz NI. A centralization procedure for radial clubhand. (1984) The Journal of hand surgery. 9 (4): 541-7. Pubmed
- 6. Nanchahal J, Tonkin MA. Pre-operative distraction lengthening for radial longitudinal deficiency. (1996) Journal of hand surgery (Edinburgh, Scotland). 21 (1): 103-7. Pubmed