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Congenital Upper Limb Anomalies

Congenital Upper Limb Anomalies. Dr O O Were. Congenital deformities are attributable to faulty development and are present at birth, though they may not be recognised till later

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Congenital Upper Limb Anomalies

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  1. Congenital Upper Limb Anomalies Dr O O Were

  2. Congenital deformities are attributable to faulty development and are present at birth, though they may not be recognised till later • Vary from severe malformations incompartible with life to minor structural abnormalities with no practical significance

  3. Congenital anomalies affect 1-2% newborns with ≈ 10% with upper limb anomalies • Accurate diagnosis and communication to family required

  4. Embryogenesis of upper limb between 4-8 week gestation guided by signalling centres within developing limb bud • Anomalies may be associated with systemic disorders, in isolation or in combination with other musculoskeletal problems

  5. Signalling centres • Apical ectodermal ridge • Zone of polarizing activity • Wingless-type( Wnt) signalling centre • Abnormalities within 1 centre indirectly prohibit functioning of other 2 and affect limb formation

  6. Apical ectodermal ridge • Ectoderm layer guiding underlying mesoderm to differentiate to appropriate structures • Limb development in proximal to distal direction • Interdigital necrosis

  7. Zone of polarizing activity • Within posterior margin of limb bud • Signalling centre for anterior to posterior(radioulnar) limb development • Signalling molecule for limb orietation sonic hedgehog protein

  8. Wnt signalling centre • Dorsal ectoderm • Secrete factors that induce mesoderm to adopt dorsal characteristics • Mediates development of dorsal-to-ventral axis configuration and alignment of the limb with dorsal orientation( dorsalization)

  9. Causes • Genetic • Environmental • Combined genetic and environmental: most congenital malformations in man

  10. Classification • I: Failure of formation of parts • II: Failure of differentiation of parts • III: Duplication • IV: Overgrowth • V: Undergrowth • VI: Congenital constriction band syndrome • VII: Generalized skeletal abnormalities International Federation of Societies for Surgery of the hand- 1976

  11. Failure of longitudinal formation- radial club hand

  12. Failure of longitudinal formation- Cleft hand

  13. Failure of differentiation- Syndactily

  14. Congenital constriction band syndrome

  15. Constriction bands • Type 1: mild transverse/oblique digital groove • Type 2: deeper groove with abnormal distal part • Type 3: incomplete or complete syndactyly of distal part( acrosyndactyly) • Type 4: Complete amputation distal to constriction

  16. Incomplete type 1 treated by observation • If can cause vascular impairment, surgical release by multiple Z-plasty closure • Entire release if shallow • If deep, only 1 side of digit released at each procedure to avoid vascular compromise • Acrosyndactyly separation initiated at 6/12

  17. Madelung’s deformity • Abnormal growth of volar and ulnar distal radial epiphysis • Ulnar and volar tilt of distal radial articular surface • Premature fusion of ulnar half of radial physis • Short, bowed radius • Short ulna • Wedging of carpus between distal radius and ulna

  18. Ulnar head enlarged and dorsally subluxated • Apparent in late childhood/ early adolescence • Degrees of motion limitation and pain due to ulnocarpal impingement

  19. Rx • Initial splinting( Can provide complete and permanent relief) • Surgery if persistent pain • Radial osteotomy plus ulna shortening osteotomy • Radial osteotomy with distractionlengthening of the radius

  20. Functional development of child’s hand • Well coordinated control of the grasp and pinch between thumb and fingers at end of 1st year • 1-3, refined coordination with bimanual dexterity and ↑ strength of grasp and pinch

  21. Maximal functional gain before child conforms to fixed pattern of activities • Most reconstruction should be completed before school

  22. Initial assessment • Definition and classification of abnormality • Assessment of severity of deformity with provision for deterioration with growth • If necessary, conservative treatment by splintage, stretching exercises • Inform parents if later surgery needed • Clinical geneticist consult if necessary

  23. Surgery • Achievement of function and cosmesis( In order of priority) • 2-handed pattern superior to single hand • Unsightly abnormality may lead to psychological strain thus affecting function • No operative intervention should compromise existing function

  24. Timing • Depends on specific anomaly • Usually 6-18 months • Earlier Rx for conditions that threaten limb viability e.g. constriction bands

  25. Goals of reconstructive surgery • Functional position of components of the hand • Provide good skin cover with adequate sensation • Satisfactory power grip and precision pinch

  26. Pitfalls in reconstructive surgery • Diversity of anomalies leads to difficulty in surgery( Every pt should be individualised) • Unpredictable growth potential e.g. recurrence of webbing in syndactily • Lack of cooperation from child complicates post operative rehabilitation

  27. Rehabilitation • Team approach • Functional capability assessment by P.T./O.T. • Parental care and encouragement • Counselling by clinical psychologist

  28. Child prosthesis • Only realistic option in some anomalies e.g. transverse arrest at or above forearm • Training as early as 18/12 • ↑ complexity in accordance with functional need

  29. Chronological management plan

  30. Thumb anomalies

  31. Isaac Newton • “ In the absence of any other proof, the thumb alone would convince me of God’s existence”

  32. Thumb hypoplasia • Short thumb: Doesn’t reach PIPJ of index finger • Adducted thumb • Abducted thumb • Pouceflottant: Short, unstable thumbmore distal and radial than normal • Absent thumb( Index pollicization) Blauth classification

  33. Other anomalies • Congenital trigger thumb • Congenital clasped thumb • All neonates hold thumb in that position till 3/12 age

  34. THUMB DUPLICATION • Both radial and ulnar duplicates display some hypoplasia, but radial usually affected more • Intrinsic muscles innervated by ulnar nerve insert on ulnar duplicate while those by median nerve on radial duplicate • Digits may be angulated and joints may be stiff • M˃ F

  35. Many forms ranging from degrees of splitting to complete duplication • Occasionally only fleshy nubbins on radial border • Duplication alone usually unilateral and sporadic • Duplication associated/w triphalangeal thumb usually AD

  36. Treatment • Surgical treatment recommended • Recommended time • Combination of elements of both digits • For unequal digits, excision of smaller digit with collateral ligament reconstruction • Care to ensure normal articular contour of distal phalanx

  37. Individualised for more complex deformities • For type 4, least developed digit( usually radial duplicate) amputated • Intrinsic and extrinsic tendon transfers, with radial collateral ligament reconstruction • Occasionally, osteotomy or growth plate arrest to improve alignment

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