Download TreatMENT OF RADIAL LONGITUDINAL DEFICIENCY:

Neil Tarabadkar
Department of Pediatric Surgery
April 18, 2013
 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
 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
 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
 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
 Holt-Oram
 VACTERL
 Fanconi’s
anemia
 Thrombocytopenia and Absent Radius
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
 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
 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
 High
recurrence rate
 Increased risk of injury to ulnar physis
 Often need fusion later in life
 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
 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
 Pin
tract infection
 Vascular complications
 Inadequate growth of the MTP physis
Jong, J. “Changing Paradigms in the Treatment of Radial
Club Hand”. Clinical Orthopaedic Surgery. 4:1:36-45.
2011
Mashcke, S. “Radial Longitudinal Deficiency”. JAAOS.
15:1:41-52. 2007
Buffart, L. “Hand function and Activity Performance of
Children with Radial Longitudinal Deficiency”. JBJS.
14:90:2408-15. 2008
Bora, William. The Pediatric Upper Extremity.W.B
Saunders Publishing. 1986