Ali Dianat M.D Orthopedic Hand Surgeon Esfahan February 2013
A longitudinal deficiency of the radius ◦ thumb usually deficient as well ◦ bilateral in 50-72% ◦ incidence is 1:100,000
◦ 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)
Incidence 1/55000 – 1/100000 LB ◦ 50 % is bilatral ◦ Male > Female (3:2) Cause : 1.Exposure to teratogenic agent (Talidomaide) 2.Exposure to radiation
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)
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.
Physical exam ◦ deformity of hand with perpendicular relationship between forearm and wrist ◦ absent thumb perform careful elbow examination
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.
Radiographs ◦ entire radius and often thumb is absent
Laboratory must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
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
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.
◦ passive stretching target tight radial-sided structures ◦ observation indicated if absent elbow motion or biceps deficiency
◦ hand centralization indications 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
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.
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.
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