Ulnar Club hand deformityAli Dianat

Hand orthopedic surgoen Esfahan February 2013

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

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

Incidence :1/100.000 L.B1/10 redial deficiency4-10 less than redial deficiency Always unilateral 70% Sporadically without systemic condition

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%

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

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

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

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 radiohumeral artherodesis in unstable elbow

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 ulnaType 4 – complete absence of the ulna Type 5 – radiohumeral synostosis

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

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

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

Treatment algorithms :Type 5 o an elbow – level osteotomy to correct the

elbow position o A forearm osteotomy o Lengthen the forearm