Radial Club Hand

Definition

Radial hemimelia

- longitudinal failure of formation of parts along radial border / preaxial border

Incidence

1:100 000

4 x Ulna Club Hand

Bilateral 50%

Associations

Scaphoid & trapezium absent in 50% cases

Thumb absent in 80%

VACTERAL

Vertebral segmentation deficiencies

Anal atresia

Cardiac

Tracheoesophageal fistula

Endocrine including Fanconi's anaemia

Renal abnormalities

Radial ray deficiencies atresia

Limb - CTEV, radial club hand

Syndromes

Holt-Oram Syndrome

- ASD & RCH

Fanconi

- anaemia & RCH (pancytopenia)

TAR syndrome

- thrombocytopenia absent radius

Bayne Classification

1. Short distal radius

- distal radial epiphysis present but delayed in appearance

2. Hypoplastic radius

- delayed proximal & distal radial epiphyses appearance

- moderate shortening of the radius & thickening of the ulna

3. Partial absence of radius

- usually distal third absent

- carpus unsupported & radially deviated

4. Total absence of radius

- most common type

Pathology

All pre-axial structures may be affected

- forearm is radially bowed

- deviated in radial & volar direction

- progresses with growth

Ulna is bowed & short by 25%

Carpus attached to ulna by fibrous tissue or small synovial joint

There are also abnormalities of finger joints most marked in radial digits

Median nerve

- is thickened as carries sensation for radial nerve as well

- it is located deep to deep fascia on radial border of forearm

- at risk from surgery

Radial artery can be absent

Clinical

Radial & palmar flexed hand

- forearm is short (50-70%) & radially deviated

- thumb absent / deformed / almost normal thumb

25% have significant elbow extension contracture

- radial deviation may allow hand to mouth

- imperative this function is not disturbed

NHx

Worse than ulna club hand as tend to radially deviate

- OT may not prevent re-deformity

- if occurs wrist fusion at maturity

Management

1. Splinting & stretching from birth

- prevent excessive radial deviation

2. Centralisation procedure

Concept

- centralising carpus over ulna

- soft tissue release at 1 - 2 years

- maintain with tendon transfers

- transfer ECRL to ECRB

Prerequisites

- lack of hand to mouth function is a contraindication to surgery

- elbow mobility is prerequisite for centralisation