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Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013

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Presentation on theme: "Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013"— Presentation transcript:


2 Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013
Radial Club Hand Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013

3 Introduction A longitudinal deficiency of the radius
thumb usually deficient as well bilateral in 50-72% incidence is 1:100,000

4 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) 

5 Epidemiology Incidence 1/55000 – 1/100000 LB Cause : 50 % is bilatral
Male > Female (3:2) Cause : Exposure to teratogenic agent (Talidomaide) Exposure to radiation

6 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)

7 James 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 humerus The term absent radius can refer to the last 3 types.

8 Presentation Physical exam perform careful elbow examination
deformity of hand with perpendicular relationship between forearm and wrist  absent thumb perform careful elbow examination

9 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.

10 Imaging Radiographs entire radius and often thumb is absent 

11 Other Work-up Laboratory
must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions

12 The basic goals of treatment
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

13 Treatment Non-Surgical Surgical Splinting and stretching
Centralization Radialization

14 Contraindications 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.

15 Non-Surgical Treatment
passive stretching target tight radial-sided structures observation indicated if absent elbow motion or biceps deficiency

16 Surgical Treatment hand centralization indications contraindications
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

17 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.

18 Surgical Treatment

19 Surgical Treatment

20 Surgical Treatment

21 Surgical Treatment

22 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.

23 Radialization

24 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




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