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Radial Club Hand Ali DianatM.D Orthopedic Hand Surgeon Esfahan February 2013
Introduction • A longitudinal deficiency of the radius • thumb usually deficient as well • bilateral in 50-72% • incidence is 1:100,000
Associated Disorders • TAR • autosomal recessive condition with thrombocytopenia and absent radius • different in that thumb is typically present • Fanconi's anemia • autosomal recessive condition with aplastic anemia • Fanconi screen and chromosomal breakage test to screen • treatment is bone marrow transplant • Holt-Oram syndrome • autosomal dominant condition characterized by cardiac defects • VACTERL Syndrome • vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis, and limb defects) • VATER Syndrome • vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis)
Epidemiology • Incidence 1/55000 – 1/100000 LB • 50 % is bilatral • Male > Female (3:2) • Cause : • Exposure to teratogenic agent (Talidomaide) • Exposure to radiation
Bayne and Klug: Classification • Type I: deficient distal radial epiphysis • Type II: deficient distal and proximal radial epiphyses • Type III: present proximally (partial aplasia) • Type IV: completely absent (total aplasia - most common)
James and Colleagues: Classification Type N: Isolated thumb anomalyType 0: Deficiency of the carpal bonesType I: Short distal radiusType II: Hypoplastic radius in miniatureType III: Absent distal radiusType IV: Complete absent radiusType V: Complete absent radius and manifestations in the proximal humerus • The term absent radius can refer to the last 3 types.
Presentation • Physical exam • deformity of hand with perpendicular relationship between forearm and wrist • absent thumb • perform careful elbow examination
Presentation • Perpendicular relationship between wrist and forearm in radial clubhand. The right-angled position further shortens the limb and limits the ability to reach into space.
Imaging • Radiographs • entire radius and often thumb is absent
OtherWork-up • Laboratory • must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
Thebasicgoalsoftreatment • Correct radial deviation of the wrist • Balance the wrist on the forearm • Maintain wrist and finger motion • Promote growth of the forearm • Improve function of the extremity • Enhance limb appearance for social and emotional benefit
Treatment • Non-Surgical • Splinting and stretching • Surgical • Centralization • Radialization
Contraindicationsforsurgicalintervention • Mild (type I) deformity in children and elbow extension contractures that prevent the hand from reaching the mouth if the deformity at the wrist is corrected. • Surgery is also contraindicated for adults who have adjusted to their deformity.
Non-Surgical Treatment • passive stretching • target tight radial-sided structures • observation • indicated if absent elbow motion or biceps deficiency
Surgical Treatment • hand centralization • indications • good elbow motion and biceps function intact • done at 6-12 months of age • followed by tendon transfers • contraindications • older patient with good function • patients with elbow extension contracture who rely on radial deviation • proximate terminal condition
Centralization • Centralization is indicated in radial clubhand types II, III, and IV, in which there is severe radial wrist deviation and insufficient support of the carpus.
Radialization • A new technique for operative treatment of the radial club hand, It is named “Radialization" because after all fibrotic tissues are excised, the hand and radial carpal bones are placed over the distal end of the ulna; the hand is fixed with a Kirschner wire in a position of moderate ulnar deviation. Usually, no carpal bones need to be removed. The improved mechanical forces are further stabilized by transposition of the radial wrist extensor and flexor to the ulnar side; this favors a better muscle balance. The optimal age for surgery is between 6 and 12 months.
New Procedure • Villki reported (2008) a different approach in During this procedure a vascularised MTP-joint of the second toe is transferred to the radial side of ulna, creating a platform that provides radial support for the wrist. The graft is vascularised and therefore maintains its ability to join the growth of the supporting ulna
THANKS FOR ATTENTION Dianat A, M.D Vaziri A, M.D