Radial Club Hand

- Discussion:
    - presents w/ differing degrees of shortening (or absence) of radius, carpal bones, and radial rays (including thumb);
          - bowing of the ulna may contribut to the deformity;
    - elbow may have fixed extension contracture or may have deficit of active motion;
    - patients may do well with no special treatment;
          - they may develop side to side pinch between adjacent digits;
          - handling of large objects may be a problem, but some children will be able to grasp objects between the hand and the arm;

- Associated Disorders:
    - VATER association;
         - vertebral anomalies, anal atresia, tracheo-esophageal fistula, radial and, renal anomalies;
    - cardiac anomalies (atrial septal defect and absent radius = Holt Oram);
    - pan-cytopenia or thrombocytopenia - absent radius (TAR Syndrome);

- Exam:
    - extremity shortening;
    - radial and volar angulation;
    - motion of wrist and elbow;
         - pts w/ a stiff elbow may prefer to keep hand in the radial deviated position, because the hand is in a better position for feeding;
    - decreased grip strength;
    - ability to grasp small and large objects;
    - hypoplasia

- Classification:
    - Type I:
           - elbow and proximal radius is normal;
           - distal radial physis is deficient and radius is shortened;
           - radial clubbing due to hypoplastic radial carpi;
           - soft tissue contractures may limit motion;
           - thumb may show variable degree of hypoplasia;
           - treatment: soft tissue release and transfer of the ECRL to the ECU;
    - Type II:
           - hypoplastic radius (more than 1-2 cm shorter than opposite side);
           - wrist is unstable and is radially deviated;
           - may be amenable to radial lengthening;
    - Type III:
           - parital absence of the radius;
    - IV:
           - most common type;
           - complete absence of the radius, and often there is absence of the radial carpi and first and second metacarpal;
           - elbow may be unstable or stiff;
           - there are severe soft tissue contractures on the radial (and volar) side of the wrist;
                  - these deformities increase during growth;

- Non Operative Treatment:
    - in infants, splints are difficult to apply, and therefore initial treatment involves wrist and elbow ROM and stretching the radial and flexion 
    - serial casts are used to stretch tight structures on radial side in order to achieve longitudinal alignment;
            - serial cast will not correct carpal malalignment;

- Surgical Treatment Options:
    - considerations:
         - historically reconstructive procedures on the wrist were avoided w/ a stiff elbow, however, in many cases, the PT can increase elbow 
                ROM (which allows surgery to procede);
    - radialization:
         - the head of the ulna is brought under the radial carpal bones, and the hand is fixed in ulnar deviation w/ a K wire;
         - procedure includes release of tight radial soft tissue structures along with possible tendon transfers to from the radial to ulnar side of the 
         - as would be expected, worse results are seen with the more severe cases;
    - centralization technique:
         - primary addresses radial angulatory deformity and may improve wrist motion;
         - this attempts to re-align the 3rd metacarpal at right angles to the plane of the distal ulnar epiphyseal plate;
         - in most cases centralization should be performed prior to one year of age;
         - centralization may be performed along w/ radial soft tissue release and as well as transfer of flexor and extensor carpi radialis tendons 
                to the ulnar side;
         - caution:
                - the procedure is contra-indicated w/ a stiff elbow (elbow needs to flex to 90 deg);
         - technique:
                - performed through longitudinal dorsal radial incision;
                - distal ulna is squared off and inserted into a rectangular notch on radial side of the carpus;
                - removal of carpal bones effects a closing wedge osteotomy;
                - resection of carpi relies on formation of fibrous pseudarthrosis to provide wrist stability;
                - carpi need not be resected if procedure is performed prior to age 12 months;
                - w/ significant ulnar bowing, ulnar osteotomy can be carried out as well;                
         - disadvantages:
                - late results show variable success;
                - resection of proximal central carpus may result in ulno-carpal fusion, with resultant wrist stiffness;
    - Ulnar Lengthening:
         - bowing of the ulna is usually not progressive;
         - may be considered following centralization if bowing is severe

Radial club hand. A continuing study of sixty-eight patients with one hundred and seventeen club hands.

Opening versus closing wedge osteotomy of the curved ulna in radial clubhand.      

Radialization as a new treatment for radial club hand.

Centralization of the ulna for congenital radial hemimelia.

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Tuesday, May 22, 2012 9:39 am