Congenital anamolies upper limb - Dr.KK

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Congenital Anamolies of Upper Limb

Apert syndrome

Club hand

Cleft hand

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  • 1. CONGENITAL ANAMOLIES OF UPPER LIMB DR. KK
  • 2. DEFINITION • Deformities since birth called congenital anamolies • Anamolies of hand, forearm, arm, shoulder…
  • 3. Incidence each year about 4 out of every 10,000 babies will have upper limb anamolies
  • 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
  • 5. EMBRYOLOGY
  • 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. 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. 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. 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. CLUB HAND • Club hand deformites are classified into two main categories radial and ulnar. • Radial club hand • Ulnar club hand
  • 11. RADIAL CLUB HAND
  • 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. 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)
  • 14. RADIAL CLUB HAND
  • 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.
  • 16. RADIAL CLUB HAND
  • 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
  • 18. RADIAL CLUB HAND
  • 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. 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. TREATMENT Exercise limb lengthening Osteotomy Radialization Splint Wrist centralization
  • 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. Madelung deformity
  • 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. Madelung deformity
  • 26. Apert's syndrome craniofacial anomalies with mitten hands.
  • 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. Apert's syndrome
  • 29. Apert's syndrome
  • 30. CLEFT HAND
  • 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. CLEFT HAND
  • 33. Syndromes Cornelia de Lange’s syndrome Oculodigital complex Orodigital complex Otodigital complex (Wildervank syndrome) Silver-Russell syndrome Electrodactyly-ectodermal dysplasia-clefting syndrome
  • 34. RING CONSTRICTIONS
  • 35. RING CONSTRICTIONS These are released by cutting large Z-plasties, which often have to be repeated as the finger grows.
  • 36. Polydactyly
  • 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. THANKZ "The only disability in life is a bad attitude.“ Scott Hamilton
  • 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.