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Congenital anamolies upper limb - Dr.KK


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Congenital Anamolies of Upper Limb

Apert syndrome

Club hand

Cleft hand

Published in: Health & Medicine
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Congenital anamolies upper limb - Dr.KK

  2. 2. DEFINITION • Deformities since birth called congenital anamolies • Anamolies of hand, forearm, arm, shoulder…
  3. 3. Incidence each year about 4 out of every 10,000 babies will have upper limb anamolies
  4. 4. EMBRYOLOGY • • • • • • Onset of development of arm bud - 27 days 4th week Well-developed arm bud - 28-30 days Elongation of arm bud - 34-36 days 5th week Formation of hand paddle - 34-38 days Onset of finger separation - 38-40 days Full separation of fingers - 50-52 days 7th week
  6. 6. International Federation of Societies for Surgery of the Hand (IFSSH - 1983) I. Failure of formation of parts (arrest of development) A. Transverse arrest (common levels are upper third of forearm, wrist, metacarpal, phalangeal) B. Longitudinal arrest (including phocomelia, radial/ulnar club hands, typical cleft hand, atypical cleft hand otherwise referred to as part of the spectrum of symbrachydactyly)
  7. 7. International Federation of Societies for Surgery of the Hand (IFSSH - 1983) II. A. B. C. Failure of differentiation of parts Soft tissue involvement Skeletal involvement Congenital tumorous conditions radio-ulnar synostosis, symphalangism (stiff PIP joints with short phalanges), camptodactyly, arthrogryposis, syndactyly.
  8. 8. International Federation of Societies for Surgery of the Hand (IFSSH - 1983) III. Duplication IV. Overgrowth V. Undergrowth (thumb hypoplasia, Madelung's deformity (abnormal distal radial growth) VI. Congenital constriction band syndrome VII. Generalised skeletal abnormalities.
  9. 9. Diagnosis before birth Antenatal diagnosis by ultrasound is increasingly possible, particularly in cases of aplasia. This will prepare the parents for the deficiency before birth.
  10. 10. CLUB HAND • Club hand deformites are classified into two main categories radial and ulnar. • Radial club hand • Ulnar club hand
  12. 12. RADIAL CLUB HAND • Radial club hand includes a wide spectrum of disorders that encompass absent thumb, thumb hypoplasia, thin first metacarpal and absent radius. • Radial club hand is frequently syndromatic • 1733
  13. 13. RADIAL CLUB HAND Well-developed arm bud - 28-30 days males : females (3:2) 1:55000 to 1:100000 live births maternal drug exposure, compression of the uterus, vascular injury, Apical Ectodermal Ridge (AER)
  15. 15. RADIAL CLUB HAND Type I: Short distal radius - mildest type, Type II: Hypoplastic radius, Type III: Partial absence of radius, Type IV: Total absence of radius - most common variant.
  17. 17. RADIAL CLUB HAND Syndromes associated Holt-Oram, TAR syndrome , thrombocytopenia pancytopenia of Fanconi's anaemia VATER syndrome vertebral Anal Tracheo-oesohageal Radial club hand DeLange, Daune, Ives Houston, Roberts, Rothmund Thompson Shokeir syndromes. Aase syndrome
  19. 19. RADIAL CLUB HAND TREATMENT : slow distraction by external fixation hand can be aligned on the forearm preserve some wrist movement index finger is pollicised.
  20. 20. Ulnar club hand • Ulnar club hand is much less frequent than radial club hand • ranges from mild deviation of hand on the ulnar side of forearm to complete absence of ulna. • ulnar club hand is usually isolated anomaly
  21. 21. TREATMENT Exercise limb lengthening Osteotomy Radialization Splint Wrist centralization
  22. 22. Madelung deformity A growth disturbance in the volar-ulnar distal radial physis volar and ulnar tilted distal radial articular surface, volar translation of the hand and wrist, a dorsally prominent distal ulna.
  23. 23. Madelung deformity
  24. 24. Madelung deformity adolescent females pain, decreased range of motion, and deformity. genetic etiology and is associated with mesomelic dwarfism and a mutation on the X chromosome. Surgery addressing the deforming bony and ligamentous lesions, correcting the abnormal position of the radial articular surface, and equalizing the longitudinal levels of the distal radius and ulna
  25. 25. Madelung deformity
  26. 26. Apert's syndrome craniofacial anomalies with mitten hands.
  27. 27. Apert's syndrome • Apert syndrome is a form of acrocephalosyndactyly, a congenital disorder characterized by malformations of the skull, face, hands and feet. • It is classified as a branchial arch syndrome, affecting the first branchial (or pharyngeal) arch, the precursor of the maxilla and mandible • Disturbances in the development of the branchial arches in fetal development create lasting and widespread effects.
  28. 28. Apert's syndrome
  29. 29. Apert's syndrome
  30. 30. CLEFT HAND
  31. 31. CLEFT HAND • Typical cleft hand is usually bilateral, often affecting the feet as well • AD • The deficiency is maximal on the ulnar size, leaving in severe cases only a hypoplastic thumb.
  32. 32. CLEFT HAND
  33. 33. Syndromes Cornelia de Lange’s syndrome Oculodigital complex Orodigital complex Otodigital complex (Wildervank syndrome) Silver-Russell syndrome Electrodactyly-ectodermal dysplasia-clefting syndrome
  35. 35. RING CONSTRICTIONS These are released by cutting large Z-plasties, which often have to be repeated as the finger grows.
  36. 36. Polydactyly
  37. 37. THUMB HYPOPLASIA AND APLASIA • no trapezium or trapeziometacarpal joint. • Hypoplastic thumbs can be stabilised and given more movement by tendon transfers. • The operation of choice is to pollicise the index finger
  38. 38. THANKZ "The only disability in life is a bad attitude.“ Scott Hamilton
  39. 39. CONGENITAL ANAMOLIES OF UPPER LIMB …….. Known is a drop…. …………………..unknown is an ocean……….. Dr. Kalaivanan Kanniyan Assistant Professor – Orthopaedics Arthroplasty and Adult Reconstruction Unit SMCH, Saveetha University, chennai, Tamil Nadu, India.