ali dianat m.d orthopedic hand surgeon esfahan february 2013

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