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

 

3.  Management of hypoplastic thumb