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Dr Sumer Yadav
Mch – Plastic and Reconstructive surgery
sumeryadav2004@gmail.com
Embryology of upper limb
 A bulge or limb bud on the ventrolateral wall
of the embryo on day 26 (4-mm crown-to-
rump leng...
 A thickening develops on the ventromedial border of the
limb bud: the apical ectodermal ridge (AER) and is vital in
axis...
 The limb bud continues to grow outward from the ventral wall, and
at day 32 it develops a somewhat flattened, paddle-sha...
 In the seventh week, the upper extremity continues outgrowth and
rotates 90° so that the elbows project posteriorly and ...
 Anatomically, the fetal period is signified by
vascular penetration of the humerus
 Most of the morphologic differentia...
a brief timetable of hand
development (modified from
Tickle)
 Onset of development of arm bud - 27 days
 Well-developed ...
sumeryadav2004@gmail.com
Few out of so many…………..
 Camptodactyly, syndactyly, and polydactyly are
the most common abnormalities.
 Assoc with 40, ...
Something about anomalies
 When 3 or more minor anomalies exist in a
single patient, the chance of the presence of
a majo...
 Only 5% of congenital hand anomalies occur as part of a
recognized syndrome. Examples include the following:
 Holt-Oram...
Classification: ASSH
 Type I – Failure of formation:
 Transverse arrest
 Longitudinal arrest
 Type II - Failure of dif...
 Type IV – Overgrowth: Includes conditions such
as hemihypertrophy and macrodactyly
 Type V – Undergrowth: Most commonly...
sumeryadav2004@gmail.com
Syndactyly : disorder of
separation
 Defn : variable fusion of soft tissue/or skeletal
elements of adjacent fingers
 Due...
 Syndactyly is classified according to
 completeness (complete, incomplete)
 presence of bony union (simple, complex,
c...
Presentation
 As
 Isolated
 Syndromic
 Associated syndromes:
 poland (brachiocephalic -subclav disruption)
 apert (F...
Management
 Surgery
 Indications
 Contraindications
 Principles of separation: from incision to
splintage
sumeryadav20...
 Timing
 Order of release
 Commissure reconstruction
 Separation of digit
 Resurfacing of digit
 Paronychial fold
su...
Complications
 Early
 Delayed
sumeryadav2004@gmail.com
 Early surgery is defined as that performed within
the first 2 years of life
 Advantages
 Disadvantages
sumeryadav2004@...
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
Polydactyly : disorder of
duplication
 They are
 Radial
 Ulnar
 Central
 Mirror hand, etc
 Old classification
 Prea...
Radial polydactyly
 Difference in black and other populations
 Classification :
 Iowa : 6+1, along a longitudinal level...
classification of Stelling
(1963) and Turek (1967)
 Type I - Soft tissue attachment
 Type II - Bony articulation
 Type ...
Presentation
 No two thumbs are alike
 Examine as per
 Radial or ulnar partner
 Each digit : nail, soft tissue, joint,...
Other types
 Central
 Ulnar
 Mirror hand
 Triphalangeal thumb
 Are less common than radial polydactyly.
sumeryadav200...
Treatment
 Primarily surgical
 The principle of surgical treatment is to ablate the most
useless digit and to reconstruc...
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
Bent finger in Greek
sumeryadav2004@gmail.com
Failure of differentiation
 Types
 Congenital / Non-Congenital
 Sporadic / AD familial
 Flexion extension imbalance is...
Presentation & Diagnosis
 Age
 Deformity
 Order of frequency of occurrence
 Range of contractures
 Diagnosis-clinical...
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
CRS
 Type of transverse deficiency
 Digit absent and proximal support str are
present to some degree
 Various eponyms u...
 Associated malformations in 40 to 80%
 2 types:
 Am Def Adh Mut complex : wuth craniofacial
 Am band disrup complex: ...
 Classification
 Patterson’s
 Presentation :
 Time
 As rings, grooves,multiple extr
 As congenital amputation / acro...
Treatment
 Timing :
 In utero/Neonatal/When child walks/Complex
procedures/Before School age
 Principles of correction:...
 Specific management of
 Acrosyndactyly
 Balloon digits
 Sinuses
 Short thumb
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
 Classification depends on
 Deviation of hand
 Thumb , phalanges and metacarpal size
 Radial carpals
 Ist web space
...
Classification
 Type I : mild
 Type II : moderate
 Type III : severe, A,B,C
 Type IV : floating thumb(pouce flottant)
...
sumeryadav2004@gmail.com
Treatment
 Timing : early vs late
 Principles
 CMC jt
 MP jt stability
 2/3 jt mobility
 Web space
 Motors for MP &...
 Management as per severity of situation
 Consists of Z-plasties, local and distant flaps ,
tendon transfers, reconstruc...
Pollicisation
 Principles
 Racquet shaped incision
 Dissection of intrinsics & A1 pulley
decompression
 Skeletal short...
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
sumeryadav2004@gmail.com
Some terms to know better….
 Symbrachydactyly or atypical cleft hand
 Symphalangism
 Brachysymphalangism
 Polysyndacty...
sumeryadav2004@gmail.com
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common congenital deformities of hand

  1. 1. Dr Sumer Yadav Mch – Plastic and Reconstructive surgery sumeryadav2004@gmail.com
  2. 2. Embryology of upper limb  A bulge or limb bud on the ventrolateral wall of the embryo on day 26 (4-mm crown-to- rump length).  Also known as Wolff crest and lies opposite the 5 lower cervical and 2 upper thoracic segments sumeryadav2004@gmail.com
  3. 3.  A thickening develops on the ventromedial border of the limb bud: the apical ectodermal ridge (AER) and is vital in axis orientation, outgrowth, and digitation  It covers a layer of undifferentiated, proliferating mesenchymal cells 5-15 cells thick, known as the progress zone (PZ), essential in limb outgrowth.  The AER and PZ work as a functional unit responsible for the outgrowth of the limb along the proximodistal axis sumeryadav2004@gmail.com
  4. 4.  The limb bud continues to grow outward from the ventral wall, and at day 32 it develops a somewhat flattened, paddle-shaped hand plate (8-mm crown-to-rump length).  During the fifth week, differentiating mesenchymal cells condense in a proximal-to-distal fashion to form blastemas, which eventually develop into cartilaginous models of, ultimately, the bones of the upper extremity.  At this time, nerve ingrowth from the rami of the spinal cord also occurs proximally. During the sixth week, hyaline cartilage models of the proximal bones of the extremity are formed.  Vessels : 3+1, br-med-uln-int-rad sumeryadav2004@gmail.com
  5. 5.  In the seventh week, the upper extremity continues outgrowth and rotates 90° so that the elbows project posteriorly and the developing hands lie on the anterior thorax.  Cartilaginous models of the proximal bones are undergoing ossification  Mesenchymal cells derived from the dermomyotome condense within connective tissue (mesenchymal) scaffolds to form 2 common muscle masses. They are immediately penetrated by the spinal nerves. These dorsal and ventral common muscle masses split later to form the muscles of the extensor and flexor compartments, respectively. sumeryadav2004@gmail.com
  6. 6.  Anatomically, the fetal period is signified by vascular penetration of the humerus  Most of the morphologic differentiation of the limb occurs during the embryonic period; the most critical period for the development of anomalies is from 24-36 days. sumeryadav2004@gmail.com
  7. 7. a brief timetable of hand development (modified from Tickle)  Onset of development of arm bud - 27 days  Well-developed arm bud - 28-30 days  Elongation of arm bud - 34-36 days  Formation of hand paddle - 34-38 days  Onset of finger separation - 38-40 days  Full separation of fingers - 50-52 days sumeryadav2004@gmail.com
  8. 8. sumeryadav2004@gmail.com
  9. 9. Few out of so many…………..  Camptodactyly, syndactyly, and polydactyly are the most common abnormalities.  Assoc with 40, 38 and unkn no. of sydromes too. sumeryadav2004@gmail.com
  10. 10. Something about anomalies  When 3 or more minor anomalies exist in a single patient, the chance of the presence of a major anomaly is 90%  Developmentalists have designated the following 3 types of sequences:  Malformation sequence  Deformation sequence  Disruption sequence sumeryadav2004@gmail.com
  11. 11.  Only 5% of congenital hand anomalies occur as part of a recognized syndrome. Examples include the following:  Holt-Oram syndrome - Cardiac defects (atrial septal defect or ventriculoseptal defect) plus upper limb defects  VATER syndrome - Vertebral (defects), (imperforate) anus, tracheoesophageal (fistula), radial and renal (dysplasia)  Fanconi syndrome - Pancytopenia, preaxial limb defects, renal abnormalities, hyperpigmentation, short stature, microcephaly, or mental retardation  Nager syndrome - Preaxial aplasia associated with mandibulofacial dysostosis (Treacher Collins syndrome)  Roberts syndrome - Four deficient limbs plus cleft lip and palate sumeryadav2004@gmail.com
  12. 12. Classification: ASSH  Type I – Failure of formation:  Transverse arrest  Longitudinal arrest  Type II - Failure of differentiation  Soft tissue - Syndactyly, trigger thumb, Poland syndrome, camptodactyly  Skeletal - Various synostoses and carpal coalitions  Tumorous conditions - Include all vascular and neurologic malformations  Type III – Duplication: May apply to whole limb, mirror hand, polydactyly sumeryadav2004@gmail.com
  13. 13.  Type IV – Overgrowth: Includes conditions such as hemihypertrophy and macrodactyly  Type V – Undergrowth: Most commonly, radial hypoplasia, brachysyndactyly, or brachydactyly  Type VI - Constriction band syndromes: Streeter hypoplasia - Occurs with or without lymphedema; invs amputation at any level  Type VII - Generalized anomalies and syndromes  Unclassified category sumeryadav2004@gmail.com
  14. 14. sumeryadav2004@gmail.com
  15. 15. Syndactyly : disorder of separation  Defn : variable fusion of soft tissue/or skeletal elements of adjacent fingers  Due to : failure of normal processes of digital separation and web space formation sumeryadav2004@gmail.com
  16. 16.  Syndactyly is classified according to  completeness (complete, incomplete)  presence of bony union (simple, complex, complex-complicated). sumeryadav2004@gmail.com
  17. 17. Presentation  As  Isolated  Syndromic  Associated syndromes:  poland (brachiocephalic -subclav disruption)  apert (FGFR2 acrocephalosyndactyly)  Acrosyn(CRS)  Considerations for examination sumeryadav2004@gmail.com
  18. 18. Management  Surgery  Indications  Contraindications  Principles of separation: from incision to splintage sumeryadav2004@gmail.com
  19. 19.  Timing  Order of release  Commissure reconstruction  Separation of digit  Resurfacing of digit  Paronychial fold sumeryadav2004@gmail.com
  20. 20. Complications  Early  Delayed sumeryadav2004@gmail.com
  21. 21.  Early surgery is defined as that performed within the first 2 years of life  Advantages  Disadvantages sumeryadav2004@gmail.com
  22. 22. sumeryadav2004@gmail.com
  23. 23. sumeryadav2004@gmail.com
  24. 24. sumeryadav2004@gmail.com
  25. 25. sumeryadav2004@gmail.com
  26. 26. Polydactyly : disorder of duplication  They are  Radial  Ulnar  Central  Mirror hand, etc  Old classification  Preaxial/ postaxial  Balanced/ unbalanced sumeryadav2004@gmail.com
  27. 27. Radial polydactyly  Difference in black and other populations  Classification :  Iowa : 6+1, along a longitudinal level  German : 10 levels of longitudinal axis and also transverse axis  Wassel :7 types  Buck –Gramcko universal  Etiology  Imbalance between AER and mesoderm  Cell division and apoptosis sumeryadav2004@gmail.com
  28. 28. classification of Stelling (1963) and Turek (1967)  Type I - Soft tissue attachment  Type II - Bony articulation  Type III - Ray duplication  When it occurs in isolation, postaxial polydactyly has a strong inheritance pattern (AD)  Preaxial polydactyly most often presents as triphalangeal thumb. sumeryadav2004@gmail.com
  29. 29. Presentation  No two thumbs are alike  Examine as per  Radial or ulnar partner  Each digit : nail, soft tissue, joint, MC head, its collateral ligaments, web space configuration, CMC joint, carpals sumeryadav2004@gmail.com
  30. 30. Other types  Central  Ulnar  Mirror hand  Triphalangeal thumb  Are less common than radial polydactyly. sumeryadav2004@gmail.com
  31. 31. Treatment  Primarily surgical  The principle of surgical treatment is to ablate the most useless digit and to reconstruct it.  Correction of type III deformities involves a ray resection.  Complex central reduplications require individualized treatment.  Conversion to a 3-finger hand may be the best option in some patients. sumeryadav2004@gmail.com
  32. 32. sumeryadav2004@gmail.com
  33. 33. sumeryadav2004@gmail.com
  34. 34. sumeryadav2004@gmail.com
  35. 35. sumeryadav2004@gmail.com
  36. 36. sumeryadav2004@gmail.com
  37. 37. Bent finger in Greek sumeryadav2004@gmail.com
  38. 38. Failure of differentiation  Types  Congenital / Non-Congenital  Sporadic / AD familial  Flexion extension imbalance is caused by  Abnormal intrinsic muscle anatomy:lumbrical insertion  Other contributing factors sumeryadav2004@gmail.com
  39. 39. Presentation & Diagnosis  Age  Deformity  Order of frequency of occurrence  Range of contractures  Diagnosis-clinical/radiological  D/D: DC, absent ext tendon,trigger sumeryadav2004@gmail.com
  40. 40. sumeryadav2004@gmail.com
  41. 41. sumeryadav2004@gmail.com
  42. 42. sumeryadav2004@gmail.com
  43. 43. sumeryadav2004@gmail.com
  44. 44. CRS  Type of transverse deficiency  Digit absent and proximal support str are present to some degree  Various eponyms used  Incidence: 1:3000 to 1:15000  Depends on environ / genetic factors sumeryadav2004@gmail.com
  45. 45.  Associated malformations in 40 to 80%  2 types:  Am Def Adh Mut complex : wuth craniofacial  Am band disrup complex: only limbs  Caused due to  Defective germ plasm  Early rupture of amnion  Intrauterine trauma sumeryadav2004@gmail.com
  46. 46.  Classification  Patterson’s  Presentation :  Time  As rings, grooves,multiple extr  As congenital amputation / acrosyndactyly  D/D: cong amput , symbrachy, ADAM sumeryadav2004@gmail.com
  47. 47. Treatment  Timing :  In utero/Neonatal/When child walks/Complex procedures/Before School age  Principles of correction:  Scar, digital length and jnt mobility, growth, stump, web-space sumeryadav2004@gmail.com
  48. 48.  Specific management of  Acrosyndactyly  Balloon digits  Sinuses  Short thumb sumeryadav2004@gmail.com
  49. 49. sumeryadav2004@gmail.com
  50. 50. sumeryadav2004@gmail.com
  51. 51. sumeryadav2004@gmail.com
  52. 52.  Classification depends on  Deviation of hand  Thumb , phalanges and metacarpal size  Radial carpals  Ist web space  Ulnar and median innervated muscles  Pollex abductus sumeryadav2004@gmail.com
  53. 53. Classification  Type I : mild  Type II : moderate  Type III : severe, A,B,C  Type IV : floating thumb(pouce flottant)  Type V : absent(with and without radius)  Others :  Central defic  CRS  Five fingered hand  Radial polydactyly  Sydromic short skeletal thumb ray sumeryadav2004@gmail.com
  54. 54. sumeryadav2004@gmail.com
  55. 55. Treatment  Timing : early vs late  Principles  CMC jt  MP jt stability  2/3 jt mobility  Web space  Motors for MP & IP jt  Opposition sumeryadav2004@gmail.com
  56. 56.  Management as per severity of situation  Consists of Z-plasties, local and distant flaps , tendon transfers, reconstruction of pulleys and eventually pollicisation sumeryadav2004@gmail.com
  57. 57. Pollicisation  Principles  Racquet shaped incision  Dissection of intrinsics & A1 pulley decompression  Skeletal shortening with metacarpal osteotomy  Tendon and intrinsic muscle rebalancing & final positioning of thumb  Broad web reconstruction and closure  Diligent post-op regime sumeryadav2004@gmail.com
  58. 58. sumeryadav2004@gmail.com
  59. 59. sumeryadav2004@gmail.com
  60. 60. sumeryadav2004@gmail.com
  61. 61. sumeryadav2004@gmail.com
  62. 62. sumeryadav2004@gmail.com
  63. 63. Some terms to know better….  Symbrachydactyly or atypical cleft hand  Symphalangism  Brachysymphalangism  Polysyndactyly or central polydactyly  Wind blown hand  Macrodactyly or gigantism sumeryadav2004@gmail.com
  64. 64. sumeryadav2004@gmail.com
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