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

27. THANKS FOR ATTENTION
Dianat A, M.D Vaziri A, M.D