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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.