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Rehabilitation of Congenital Limb Anomalies

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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Rehabilitation of Congenital Limb Anomalies

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  1. Rehabilitation of Congenital Limb Anomalies WasuwatKitisomprayoonkul, MD Department of Rehabilitation Medicine Chulalongkorn University

  2. 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.

  3. Congenital Limb Anomalies • Deficiency • Transverse • Longitudinal: radial def. • Hypoplastic thumb • Syndactyly • Camptodactyly • Arthrogryposis

  4. 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

  5. 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)

  6. 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

  7. 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.

  8. ISO/ISPO classification: transverse

  9. ISO/ISPO classification: longitudinal

  10. Transverse deficiency • Goals • Promote independent function • Maintain integrity of distal residual limb

  11. Transverse deficiency • Rehabilitation • Education • Psychological support • ROME, strengthening, balance • Prosthetic fitting • Prosthetic training • Activities & developmental training • Or alternative function with feet

  12. Transverse deficiency • Prosthetic fitting • Passive prosthesis: sitting ~6 months • Active body–powered prosthesis: 15 months–2 years old • Myoelectric prosthesis: 3-5 years old

  13. Transverse deficiency • Prosthetic management of unilateral congenital BE (Davis JR, et al., JBJS (Am); 2006)

  14. 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)

  15. 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

  16. 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

  17. Radial deficiency • Findings • Radial deviation  perpendicular with forearm • Stiffness of wrist, MCP, IP, forearm, elbow and shoulder joints • Thumb hypoplasia

  18. 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.

  19. 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

  20. 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

  21. Radial deficiency • Rehabilitation • ROME & stretching • Splinting • Hand function training

  22. 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

  23. 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

  24. Hypoplastic thumb II IIIA • Management • Type I • Non-surgical • Type II–IIIA • Thumb reconstruction • Type IIIB–V • Pollicization IV V

  25. 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

  26. 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

  27. 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

  28. Syndactyly • An abnormal interconnection between adjacent digits

  29. 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

  30. Camptodactyly • Painless flexion contracture of the PIPjoint that usually is gradually progressive

  31. Camptodactyly • Cathegory • Congenital • Apparent during infancy, 5th digit • Preadolescence • 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

  32. 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

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

  34. 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)

  35. 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

  36. Arthrogryposis

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

  38. Thank you for your attention

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