Rehabilitation of Congenital Limb Anomalies

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Rehabilitation of Congenital Limb Anomalies

• Wasuwat Kitisomprayoonkul, MD
• Department of Rehabilitation Medicine
• Chulalongkorn University

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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.

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Congenital Limb Anomalies

• Deficiency
• Transverse
• Longitudinal radial def.
• Hypoplastic thumb
• Syndactyly
• Camptodactyly
• Arthrogryposis

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General guideline for evaluation

• History taking
• Physical examination
• ROM, strength, prehensile patterns, sensory
• Functional assessment
• Developmental milestones
• Observe of upper limb position during activities
• Adaptive technique/equipment
• Functional scale such as FIM
• Client/family goals

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Congenital limb deficiency classification

• 19400 live births
• Classification
• Traditional Classification
• Frantz OReilly Classification
• ISO/ISPO Classification System (International
  Standards Organization/International Society for
  Prosthetics and Orthotics)

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Traditional classification

• Ameliaabsence of a limb
• Meromeliapartial absence of a limb
• Hemimeliaabsence of half a limb
• Phocomeliaflipper-like appendage
• attached to the trunk
• Acheiriamissing hand or foot
• Adactylyabsent metacarpal
• Aphalangiaabsent finger

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Frantz OReilly classification

• Terminal the complete loss of the distal
  extremity
• Intercalary the absence of intermediate parts
  with preserved proximal and distal parts of the
  limb.

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ISO/ISPO classification transverse
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ISO/ISPO classification longitudinal
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Transverse deficiency

• Goals
• Promote independent function
• Maintain integrity of distal residual limb

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Transverse deficiency

• Rehabilitation
• Education
• Psychological support
• ROME, strengthening, balance
• Prosthetic fitting
• Prosthetic training
• Activities developmental training
• Or alternative function with feet

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Transverse deficiency

• Prosthetic fitting
• Passive prosthesis sitting 6 months
• Active bodypowered prosthesis
• 15 months2 years old
• Myoelectric prosthesis 3-5 years old

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Transverse deficiency

• Prosthetic management of unilateral congenital
  BE

(Davis JR, et al., JBJS (Am) 2006)
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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
Aug30165-73)
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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

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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

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Radial deficiency

• Findings
• Radial deviation ? perpendicular with forearm
• Stiffness of wrist, MCP, IP, forearm, elbow and
  shoulder joints
• Thumb hypoplasia

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Radial deficiency

• Syndromes associated with radial def.
• HoltOram 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
• Fanconis anemia aplastic anemia, radial def.

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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

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Radial deficiency

• Classification
• Type I Short radius ? rehab
• Type II Hypoplastic ? surgery rehab
• Type III Partial absence ? surgery rehab
• Type IV Total absence ? surgery rehab

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Radial deficiency

• Rehabilitation
• ROME stretching
• Splinting
• Hand function training

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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

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Radial deficiency

• Rehabilitation post-Ilizarov
• 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

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Hypoplastic thumb
IIIA
II

• Management
• Type I
• Non-surgical
• Type IIIIIA
• Thumb reconstruction
• Type IIIBV
• Pollicization

IV
V
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Hypoplastic thumb

• Rehabilitation
• 1st web spreader
• ROME maintain ROM of radial digit in type IIIBV
  
• Strengthening of potential donor muscles for
  future tendon transfer
• Function training promote thumb pinch in type
  IIIIA

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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

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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

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Syndactyly

• An abnormal interconnection between adjacent
  digits

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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

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Camptodactyly

• Painless flexion contracture of the PIP joint
  that usually is gradually progressive

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Camptodactyly

• Cathegory
• Congenital
• Apparent during infancy, 5th digit
• Preadolescence
• Develops between age of 711 years, may progress
  to severe flexion deformity
• Syndromic
• Multiple digits of both extremities, with
  craniofacial disorders/short stature/chromosomal
  abnormality

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Camptodactyly

• Goals
• Prevent progression of contracture
• Improve PIP joint contracture
• Surgical correction in severe cases with
  disability

Non-operative case if - contracture lt 30-40
degrees - no activities of daily living
interfere - no functional handicap
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Camptodactyly

• Rehabilitation
• Splinting
• Static progressive splint
• Forearm-based
• Hand-based
• Serial casting
• Night time vs. full time
• Continue until skeletal mature
• ROME stretching

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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)
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Camptodactyly

• Rehabilitation post-FDS tendon transfer
• Cast wk 1-3
• Forearm-based splint for fulltime AROM
  place-hold 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

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Arthrogryposis
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Arthrogryposis

• Rehabilitation
• ROME stretching
• Splinting
• Increase function such as hand grip
• Increase/maintain ROM
• Adaptive activity training
• Post-operative rehabilitation

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Thank you for your attention