June 20, 2024

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

Congenital absence of radius
Congenital absence of the radius
TypesFeatures
Type IShort distal radius
Type IIHypoplastic radius
Type IIIPartial absence of the radius
Type IVA total absence of the radius
Classification of Congenital absence of radius

Treatment

Bayne outlined five categories of patients in which treatment is contraindicated.

  1. Patients with minimal anomalies
  2. Patients with severe associated anomalies, who have severe retardation, poor prognosis, and short predicted lifespan
  3. Adult or older, who have adjusted to the disability and have acquired dexterity in performing activities of daily living
  4. Patient with a stiff elbow in which the straightened hand will not be able to reach the mouth or the perineum
  5. 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