Transcript Rehabilitation of Congenital Limb Anomalies Wasuwat Kitisomprayoonkul, MD Department of Rehabilitation Medicine

Rehabilitation of
Congenital Limb Anomalies
Wasuwat Kitisomprayoonkul, MD
Department of Rehabilitation Medicine
Chulalongkorn University
Congenital Committee Report
IFSSH Congress - Seoul, 2010
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Care of the child with a congenital
anomaly is complex and rewarding, and
must be long term and ongoing.
Enabling a child to interface with the
environment and become more
independent must be the goal of any
treatment.
Congenital Limb Anomalies
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Deficiency
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Transverse
Longitudinal: radial def.
Hypoplastic thumb
Syndactyly
Camptodactyly
Arthrogryposis
General guideline for evaluation
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History taking
Physical examination
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Functional assessment
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ROM, strength, prehensile patterns, sensory
Developmental milestones
Observe of upper limb position during
activities
Adaptive technique/equipment
Functional scale such as FIM
Client/family goals
Congenital limb deficiency classification
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1:9400 live births
Classification
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Traditional Classification
Frantz O’Reilly Classification
ISO/ISPO Classification System (International
Standards Organization/International Society
for Prosthetics and Orthotics)
Traditional classification
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Amelia—absence of a limb
Meromelia—partial absence of a limb
Hemimelia—absence of half a limb
Phocomelia—flipper-like appendage
attached to the trunk
Acheiria—missing hand or foot
Adactyly—absent metacarpal
Aphalangia—absent finger
Frantz O’Reilly classification
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Terminal: the complete loss of the distal
extremity
Intercalary: the absence of intermediate parts
with preserved proximal and distal parts of
the limb.
ISO/ISPO classification: transverse
ISO/ISPO classification: longitudinal
Transverse deficiency
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Goals
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Promote independent function
Maintain integrity of distal residual limb
Transverse deficiency
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Rehabilitation
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Education
Psychological support
ROME, strengthening, balance
Prosthetic fitting
Prosthetic training
Activities & developmental training
Or alternative function with feet
Transverse deficiency
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Prosthetic fitting
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Passive prosthesis: sitting ~ 6 months
Active body–powered prosthesis:
15 months–2 years old
 Myoelectric prosthesis: 3-5 years old
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Transverse deficiency
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Prosthetic management of unilateral congenital BE
(Davis JR, et al., JBJS (Am); 2006)
Transverse deficiency
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Prescription of the first prosthesis and later use in children with
congenital unilateral upper limb deficiency: A systematic review.
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The search yielded 285 publications, of which four studies met
the selection criteria.
Lower rejection rates in children who were provided with their
first prosthesis at less than two years of age.
Higher rejection rate in children who were fitted over two years
of age (pooled OR = 3.6, 95% CI 1.6 - 8.0).
No scientific evidence was found concerning the relation
between the age at which a prosthesis was prescribed for the
first time and functional outcomes.
(Meurs M, et al., Prosthet Orthot Int 2006 Aug;30:165-73)
Transverse deficiency
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Time to get new prosthetic
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Age 0-5 years old: every year
Age 5-12 years old: every 1.5 years
Age 12-21 years old: every 2 years
Transverse deficiency
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Postoperative management
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Excision of the bone spicule/removal of the
rudimentary nubbins  scar management +
desensitization  prosthetic fitting & training
Toe to thumb transfer  functional training
Radial deficiency
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Findings
 Radial deviation 
perpendicular with
forearm
 Stiffness of wrist,
MCP, IP, forearm,
elbow and shoulder
joints
 Thumb hypoplasia
Radial deficiency
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Syndromes associated with radial def.
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Holt–Oram: heart defects e.g. septal defect
TAR: thrombocytopenia absent radius
syndrome
VACTERL: vertebral abnormality, anal atresia,
cardiac abnormality, tracheoesophageal fistula,
esophageal atresia, renal defects, radial
dysplasia, lower limb abnormality
Fanconi’s anemia: aplastic anemia, radial def.
Radial deficiency
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Goals
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Correct wrist radial deviation
Balance the wrist on the forearm
Maintain ROM
Promote growth of forearm
Improve function
Enhance limb appearance for social and
emotional benefit
Radial deficiency
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Classification
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Type
Type
Type
Type
I: Short radius  rehab
II: Hypoplastic  surgery + rehab
III: Partial absence  surgery + rehab
IV: Total absence  surgery + rehab
Radial deficiency
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Rehabilitation
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ROME & stretching
Splinting
Hand function training
Radial deficiency
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Rehabilitation
post-centralization
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Splinting: cast for 6-8
weeks  full time wrist
support 4 weeks  night
splint until skeletal mature
ROME of digits
Start wrist PROM,
strengthening and weight
bearing at wk 12
Hand function training
Radial deficiency
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Rehabilitation postIlizarov
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Splinting:
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finger sling for daytime and
resting hand splint for nighttime
until soft tissue equilibrium
full time wrist support + ROME
wean from daytime splint to
night splint within 6 weeks
night splint until skeletal mature
Hand function training
Hypoplastic thumb
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II
IIIA
IV
V
Management
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Type I
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Type II–IIIA
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Non-surgical
Thumb reconstruction
Type IIIB–V
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Pollicization
Hypoplastic thumb
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Rehabilitation
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1st web spreader
ROME: maintain ROM of
radial digit in type IIIB–V
Strengthening of potential
donor muscles for future
tendon transfer
Function training:
promote thumb pinch in
type I–IIIA
Hypoplastic thumb
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Rehabilitation after reconstruction
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Splinting:
cast for 6-8 weeks
 full time wrist support 4 weeks
 night splint until skeletal mature
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ROME of digits
Start wrist PROM, strengthening
and weight bearing at wk 12
Hand function training
Hypoplastic thumb
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Rehabilitation after pollicization
Splinting:
 Long arm cast for 4-6 weeks
 Thumb spica for wk 6-7 
use only night for wk 8-12
 ROME of thumb
 PROM of CMC after wk 12
 No limit ROM of thumb MCP and IP
after wk 12
 Start strengthening at wk 12
 Hand function training to promote
thumb pinch
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Syndactyly
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An abnormal interconnection between
adjacent digits
Syndactyly
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Goals
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Separate syndactyly  promote function
Avoid separation of digits that function better
as a unit than they would as individual digits
Postoperative rehabilitation
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Scar management
Hand function training – play activities
Camptodactyly
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Painless flexion contracture of the PIP joint
that usually is gradually progressive
Camptodactyly
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Cathegory
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Congenital
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Preadolescence
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Apparent during infancy, 5th digit
Develops between age of 7–11 years, may
progress to severe flexion deformity
Syndromic
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Multiple digits of both extremities, with craniofacial
disorders/short stature/chromosomal abnormality
Camptodactyly
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Goals
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Prevent progression of contracture
Improve PIP joint contracture
Surgical correction in severe cases with
disability
Non-operative case if:
- contracture < 30-40 degrees
- no activities of daily living interfere
- no functional handicap
Camptodactyly
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Rehabilitation
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Splinting
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Static progressive splint
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Forearm-based
Hand-based
Serial casting
 Night time vs. full time
 Continue until skeletal
mature
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ROME & stretching
Camptodactyly
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Camptodactyly: classification and therapeutic
results. Apropos of a series of 50 cases.
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50 patients with camptodactyly of one/several fingers
Treatment by dynamic splint for a mean duration of
20 months gives good results in fixed or mobile
camptodactylies of small children
Goffin D, et al., Ann Chir Main Memb Super 1994; 13)
Camptodactyly
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Rehabilitation post-FDS tendon transfer
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Cast: wk 1-3
Forearm-based splint for fulltime + AROM + placehold exercise: wk 3-6
Use splint during strenuous activity and nighttime +
light resistive strengthening + funct training: wk 6-8
Nighttime only + gradual increase resistive
strengthening: wk 8-12
Forceful composite MCP and IP extension/flex: wk 12
Arthrogryposis
Arthrogryposis
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Rehabilitation
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ROME & stretching
Splinting
Increase function such as hand grip
 Increase/maintain ROM
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Adaptive activity training
Post-operative rehabilitation
Thank you for your attention