ali dianat m.d orthopedic hand surgeon esfahan february 2013
TRANSCRIPT
Radial Club Hand Ali Dianat M.D
Orthopedic Hand SurgeonEsfahan February 2013
A longitudinal deficiency of the radius
◦ thumb usually deficient as well◦ bilateral in 50-72%◦ incidence is 1:100,000
Introduction
◦ 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)
Associated Disorders
Incidence 1/55000 – 1/100000 LB◦ 50 % is bilatral ◦ Male > Female (3:2)
Cause :1. Exposure to teratogenic agent (Talidomaide)2. Exposure to radiation
Epidemiology
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)
Bayne and Klug: Classification
Type N: Isolated thumb anomalyType 0: Deficiency of the carpal bonesType I: Short distal radiusType II: Hypoplastic radius in miniatureType III: Absent distal radiusType IV: Complete absent radiusType V: Complete absent radius and manifestations in the proximal humerus The term absent radius can refer to the
last 3 types.
James and Colleagues: Classification
Physical exam ◦ deformity of hand with perpendicular
relationship between forearm and wrist ◦ absent thumb
perform careful elbow examination
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.
Presentation
Radiographs ◦ entire radius and often thumb is absent
Imaging
Laboratory
must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
Other Work-up
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
The basic goals of treatment
Non-Surgical ◦ Splinting and stretching
Surgical◦ Centralization◦ Radialization
Treatment
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.
Contraindications for surgical intervention
◦passive stretching target tight radial-sided structures
◦observation indicated if absent elbow motion or biceps
deficiency
Non-Surgical Treatment
◦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
Surgical Treatment
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.
Centralization
Surgical Treatment
Surgical Treatment
Surgical Treatment
Surgical Treatment
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.
Radialization
Radialization
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
New Procedure
Dianat A, M.D Vaziri A, M.D
THANKS FOR ATTENTION