Rehabilitation of Congenital Limb Anomalies Wasuwat Kitisomprayoonkul, MD Department of Rehabilitation Medicine
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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
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
Deficiency
Transverse
Longitudinal: radial def.
Hypoplastic thumb
Syndactyly
Camptodactyly
Arthrogryposis
General guideline for evaluation
History taking
Physical examination
Functional assessment
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
1:9400 live births
Classification
Traditional Classification
Frantz O’Reilly Classification
ISO/ISPO Classification System (International
Standards Organization/International Society
for Prosthetics and Orthotics)
Traditional classification
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
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
Goals
Promote independent function
Maintain integrity of distal residual limb
Transverse deficiency
Rehabilitation
Education
Psychological support
ROME, strengthening, balance
Prosthetic fitting
Prosthetic training
Activities & developmental training
Or alternative function with feet
Transverse deficiency
Prosthetic fitting
Passive prosthesis: sitting ~ 6 months
Active body–powered prosthesis:
15 months–2 years old
Myoelectric prosthesis: 3-5 years old
Transverse deficiency
Prosthetic management of unilateral congenital BE
(Davis JR, et al., JBJS (Am); 2006)
Transverse deficiency
Prescription of the first prosthesis and later use in children with
congenital unilateral upper limb deficiency: A systematic review.
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
Time to get new prosthetic
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
Postoperative management
Excision of the bone spicule/removal of the
rudimentary nubbins scar management +
desensitization prosthetic fitting & training
Toe to thumb transfer functional training
Radial deficiency
Findings
Radial deviation
perpendicular with
forearm
Stiffness of wrist,
MCP, IP, forearm,
elbow and shoulder
joints
Thumb hypoplasia
Radial deficiency
Syndromes associated with radial def.
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
Goals
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
Classification
Type
Type
Type
Type
I: Short radius rehab
II: Hypoplastic surgery + rehab
III: Partial absence surgery + rehab
IV: Total absence surgery + rehab
Radial deficiency
Rehabilitation
ROME & stretching
Splinting
Hand function training
Radial deficiency
Rehabilitation
post-centralization
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
Rehabilitation postIlizarov
Splinting:
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
II
IIIA
IV
V
Management
Type I
Type II–IIIA
Non-surgical
Thumb reconstruction
Type IIIB–V
Pollicization
Hypoplastic thumb
Rehabilitation
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
Rehabilitation after reconstruction
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
Hypoplastic thumb
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
Syndactyly
An abnormal interconnection between
adjacent digits
Syndactyly
Goals
Separate syndactyly promote function
Avoid separation of digits that function better
as a unit than they would as individual digits
Postoperative rehabilitation
Scar management
Hand function training – play activities
Camptodactyly
Painless flexion contracture of the PIP joint
that usually is gradually progressive
Camptodactyly
Cathegory
Congenital
Preadolescence
Apparent during infancy, 5th digit
Develops between age of 7–11 years, may
progress to severe flexion deformity
Syndromic
Multiple digits of both extremities, with craniofacial
disorders/short stature/chromosomal abnormality
Camptodactyly
Goals
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
Rehabilitation
Splinting
Static progressive splint
Forearm-based
Hand-based
Serial casting
Night time vs. full time
Continue until skeletal
mature
ROME & stretching
Camptodactyly
Camptodactyly: classification and therapeutic
results. Apropos of a series of 50 cases.
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
Rehabilitation post-FDS tendon transfer
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
Rehabilitation
ROME & stretching
Splinting
Increase function such as hand grip
Increase/maintain ROM
Adaptive activity training
Post-operative rehabilitation
Thank you for your attention