2Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013 Radial Club HandAli Dianat M.DOrthopedic Hand SurgeonEsfahan February 2013
3Introduction A longitudinal deficiency of the radius thumb usually deficient as wellbilateral in 50-72%incidence is 1:100,000
4Associated DisordersTARautosomal recessive condition with thrombocytopenia and absent radiusdifferent in that thumb is typically present Fanconi's anemiaautosomal recessive condition with aplastic anemiaFanconi screen and chromosomal breakage test to screentreatment is bone marrow transplantHolt-Oram syndromeautosomal dominant condition characterized by cardiac defectsVACTERL Syndromevertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis, and limb defects) VATER Syndromevertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis)
5Epidemiology Incidence 1/55000 – 1/100000 LB Cause : 50 % is bilatral Male > Female (3:2)Cause :Exposure to teratogenic agent (Talidomaide)Exposure to radiation
6Bayne and Klug: Classification Type I: deficient distal radial epiphysis Type II: deficient distal and proximal radial epiphysesType III: present proximally (partial aplasia)Type IV: completely absent (total aplasia - most common)
7James and Colleagues: Classification Type N: Isolated thumb anomaly Type 0: Deficiency of the carpal bones Type I: Short distal radius Type II: Hypoplastic radius in miniature Type III: Absent distal radius Type IV: Complete absent radius Type V: Complete absent radius and manifestations in the proximal humerusThe term absent radius can refer to the last 3 types.
8Presentation Physical exam perform careful elbow examination deformity of hand with perpendicular relationship between forearm and wrist absent thumbperform careful elbowexamination
9PresentationPerpendicular relationship between wrist and forearm in radial clubhand. The right-angled position further shortens the limb and limits the ability to reach into space.
10ImagingRadiographsentire radius and often thumb is absent
11Other Work-up Laboratory must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
12The basic goals of treatment Correct radial deviation of the wristBalance the wrist on the forearmMaintain wrist and finger motionPromote growth of the forearmImprove function of the extremityEnhance limb appearance for social and emotional benefit
13Treatment Non-Surgical Surgical Splinting and stretching CentralizationRadialization
14Contraindications for surgical intervention 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.
15Non-Surgical Treatment passive stretchingtarget tight radial-sided structuresobservationindicated if absent elbow motion or biceps deficiency
16Surgical Treatment hand centralization indications contraindications good elbow motion and biceps function intactdone at 6-12 months of agefollowed by tendon transferscontraindicationsolder patient with good functionpatients with elbow extension contracture who rely on radial deviationproximate terminal condition
17CentralizationCentralization is indicated in radial clubhand types II, III, and IV, in which there is severe radial wrist deviation and insufficient support of the carpus.
22RadializationA 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.
24New ProcedureVillki 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