Ulnar Club hand deformity Ali Dianat Hand orthopedic surgoen Esfahan February 2013

2. Ulnar Club hand deformityAli DianatHand orthopedic surgoenEsfahan February 2013

3. History : • Goller : first described this deformity in 1693 • Priestly in 1856 presented a case: this rare deficiency occurs during week 4-5 of fetal development in the earliest stage of upper limb formation

4. Other terms for this deformity • Ulnar dysmelia • Ulnar dysplasia • Paraxial ulnar hemimelia • Congenital absence of the ulnar

5. Incidence : • 1/100.000 L.B • 1/10 redial deficiency • 4-10 less than redial deficiency • Always unilateral 70% • Sporadically without systemic condition

6. Clinical findings : • Hypoplasia of the entire upper extremity • The elbow is malformed or fused • The ulna may be partially or completely absent (ulnar shorting ) • Radial bowing • Abnormal digits • Carpal bones are absent or deformed • Synostosis with humerus • Radial head is dislocated 50%

7. Associated deformities: • Ulnar deviation of the hand • Absent ulnar digits • Syndactly • Elbow stiffness • Forearm limitation of movement • Deficient carpal bone

8. Classification : Type 1 – ulnar shortening (distally ) with minor radial bow Type 2 – significant ulnar shortening with significant radial bowing Type 3 – complete absence of the ulna Type 4 – complete absence of the ulna with radio humeral synostosis at the elbow

9. Ulnar club hand treatment & management • Non surgical treatment : gradual stretching of the tight ulnar structures and long – arm cast to ( 6 mos)

10. Surgical treatment • Excise the(50%) of anlage before the occurrence of radial bowing or radial head dislocation (6 mos) age • Excise the entire anlage , Z-plasty of the skin and selective tenotomies • ulnohumeral or radiohumeralartherodesis in unstable elbow

11. New treatment strategies :(paley and Herzenberg classification) Type 1 hypoplastic ulna with the distal epiphysis intact . This type is the most common with congenital dislocation of the radial head Type 2 – absence of the distal third of the ulna Type 3 – absence of the distal two thirds of the ulna Type 4 – complete absence of the ulna Type 5 – radiohumeralsynostosis

12. Treatment algorithms : Type 1 – without radial head dislocation : ulnar lengthening radius shortening both Type 1 – with radial head dislocation : radial head reduction by ulnar corrective osteotomy and radial shortening

13. Treatment algorithms : • Type 2 and 3 with radial dislocation if only the distal third of the ulna is absent , the ulna can be transported distally support the carpus If the distal two thirds of the ulna is absent , creation one bone forearm

14. Treatment algorithms : Type 4 – ulnar hand deviation and elbow instability should be correct by osteotomy of forearm

15. Treatment algorithms : Type 5 • an elbow – level osteotomy to correct the elbow position • A forearm osteotomy • Lengthen the forearm