1. RADIAL LONGITUDINAL DEFICIENCY: Treatment and Emerging Techniques
Neil Tarabadkar
Department of Pediatric Surgery
April 18, 2013

2. Radial Longitudinal Deficiencies
Encompass a spectrum of hypoplasias and dysplasias involving the thumb, wrist, and forearm.
Radial Club Hand
Cleft Hand Deformity
Ulnar Club Hand
Radial Hypoplasia
Absence of the Radius

3. Radial deviation, ulnar bowing, instability

4. Anatomic Manifestations
NOT an isolated deformity but a spectrum of dysplasia
Bony abnormality of thumb/radius most pronounced
Associated with muscle, nerve, joint and vessel deficiency
Hand is usually radially deviated with a short ulna

6. Etiology
UNKNOWN
Upper Limb Development occurs during the first 4-7 weeks of life, along with cardiac, renal, and hematopoeitic systems
Several factors have been proposed
Intra-uterine compression
Vascular Insufficiency
Environmental Insults
Maternal Drug Exposure
Genetic Mutations

7. Incidence Between 1 in 30,000 and 1 in 100,000 live births
Male to Female ratio of 3:2
Bilateral involvement from 38%-58%
When unilateral, right upper extremity is involved twice as much as the left

8. Syndromes with Radial Defects
Holt-Oram
VACTERL
Fanconi’s anemia
Thrombocytopenia and Absent Radius
-Severe deformity can often direct attention way from underlying defects
-All pediatrics with RLD require a renal ultrasound, echo, and CBC to evaluate for genetic syndromes
-Holt-Oram: Mutation in the TBX5 gene, AD, Absent radium, ASD, 1st degree heart block
-Fanconi’s anemia: Increased risk of cancer, mylodysplastic syndrome, AML,

10. Classification of Radial Longitudinal Deficiency
Class I: Short and mildly dysplastic radius, arising from delayed growth from epiphysis
Class II: Growth noticeably diminished secondary to decreased elongation and proximal and distal epiphysis
Class III: Partial absence of the radius
Class IV: Complete absence of the radius
Types II and IV are operative

11. Pre-Operative Management
Plays an essential role in functional outcome
Serial splinting and stretching to lengthen taut radial soft tissue
Passive correction (stretching) of wrist deformity
Definitive treatment in pediatrics with mild deformity and
Patients too sick to undergo surgery
-Stretching of wrist deformity: longitudinal traction, ulnar deviation, and extension with stabilization of ulno-carpal joint

13. Hand-Forearm angle
-defined by the intersection of a line drawn down the longitudinal axis of the third metacarpal (b) with a line drawn perpendicular to the distal ulnar physis
-Normal is around 30, the larger the angle, the worse the deformity.

14. Classical Treatment: Wrist Centralization
First performed by Sayer in 1893
Initial surgery performed between 6-12 months
Realign/stabilize hand/carpus on distal ulna
Distal ulna aligned with long finger metacarpal and stabilized with K-wire, ECU transferred distally, FCU dorsally
Goal: maintain deformity correction while optimizing growth, improving digit/wrist motion, enhancing function
Hand-Forearm angle is most critical factor
-Thumb reconstruction occurs six months after wrist realignment
-All operations should be completed by age 18 months in order to allow achievement of developmental milestones
-Must watch out for median nerve and preserve distal ulnar physis
-Must detach radial extensors as well as BR and FCR
-Goal: improves hand function by eliminating radial deviation and putting the hand in more outstretched position

16. Disadvantages High recurrence rate
Increased risk of injury to ulnar physis
Often need fusion later in life

17. Changing Paradigm: Soft Tissue Distraction and Joint transfer
First decribed by Kessler
Stretching of the soft tissue via application of a distraction device
Goal: controlled and gradual stretching of radial soft tissues
Distraction at 1mm/day until passive correction achieved
2nd MTP and Proximal Phalanx can be transferred with a viable physis
-Performed at years of life in order to allow MCP time to grow
-1st and 2nd dorsal metatarsa; artery provides the blood supply to the graft

19. Outcomes Vilkki et al and de Jong et all
Average final hand-forearm angle post op 28 degrees
Average wrist motion 83 degrees
Average length of the ulna was 66% of the contralateral side

20. Complications Pin tract infection Vascular complications
Inadequate growth of the MTP physis

21. References
Jong, J. “Changing Paradigms in the Treatment of Radial Club Hand”. Clinical Orthopaedic Surgery. 4:1: Mashcke, S. “Radial Longitudinal Deficiency”. JAAOS. 15:1: Buffart, L. “Hand function and Activity Performance of Children with Radial Longitudinal Deficiency”. JBJS. 14:90: Bora, William. The Pediatric Upper Extremity. W.B Saunders Publishing. 1986