Congenital absence of radius is also called Radial Club Hand
Varying severity depends upon the amount of radial anlage present congenitally
It represents a longitudinal failure of formation along the preaxial or radial border during the growth period.
See also: Congenital anomalies of the hand
Congenital absence of radius is associated with
- Fanconi syndrome
- Holt-Oram syndrome
- TAR ( Thrombocytopenic Absent Radius)
- VACTREL (vertebral anomaly, anal atresia, cardiac defect, trachea-esophageal fistula, Esophageal atresia, Radial and Renal anomalies, and limb defects)
Bilateral radial dysplasia with present thumbs of relatively normal size is seen in association with TAR syndrome
Etiology
Commonly seen in trisomy (13 and 18)
Due to the insult during the intrauterine period
Primary insult to the apical ectodermal ridge occurs early in pregnancy (between 3-6 weeks)
Radial Club Hand
Pathoanatomy
Reduction in the size of the bones of the whole upper limb
The scapula and humerus are smaller in size
The ulna is characteristically short, thick, and invariably curved in the middle third
The wrist has deviated medially until it lies at a right angle to the long axis of the forearm
Radial carpal bones are absent and fused
Contracture exists in the radial side of the forearm in form of muscle So they are shortened
Fibrotic, fused to each other or absent
The radial structures are hypoplastic and the ulnar soft tissues are not affected and the median artery can be persistent.
Classification of Congenital absence of radius
| Types | Features |
|---|---|
| Type I | Short distal radius |
| Type II | Hypoplastic radius |
| Type III | Partial absence of the radius |
| Type IV | A total absence of the radius |
Treatment
Bayne outlined five categories of patients in which treatment is contraindicated.
- Patients with minimal anomalies
- Patients with severe associated anomalies, who have severe retardation, poor prognosis, and short predicted lifespan
- Adult or older, who have adjusted to the disability and have acquired dexterity in performing activities of daily living
- Patient with a stiff elbow in which the straightened hand will not be able to reach the mouth or the perineum
- Patient with severe soft tissue contracture involving neurovascular status.
Options
Contracture is released and stretching/splinting is done
In severe cases: Surgical release is done
Soft tissue release with centralization of carpus: Thump reconstruction and tricep transfer
In radial dysplasia radicalization involves tendon transfers of radial tendons to create an ulnar balancing force
The complication of surgery
- Recurrence of deformity
- Growth arrest of the distal ulna
- Ankylosing or recurrent instability of the wrist
- Ulna fracture, Vascular insult, damage to neural structure
