Dupuytren's Disease Flashcards Preview

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Flashcards in Dupuytren's Disease Deck (22):
1

what is Dupuytren's disease?

  • A Benign Proliferative disorder CHARACTERISED by FASCIAL NODULES and CONTRACTURES of the hands

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2

Can you describe the epidemiology of Dupuytren's?

  • AUTOSOMAL Dominant
  • 5-7th decade of life
  • 2:1 M:F
  • Highest incidence in  Caucasian males European descent
    • Linked to Diabetes Mellitis
    • Alcholism
    • Epilepsy
    • HIV

3

What is its pathology?

  • MYOFIBROBLASTS are the dominant cell type found histologically



  • Cytokine mediated Transformation of normal fibroblasts into mylofibroblasts 
     





  • Increase in type 3 collagen to type 1 







  • The disease orginates in longitudinally orientated fascial structures 









  • Early proliferative phase characterised by high no of immature fibroblasts & myofibroblasts in a whorled pattern- hostological nodule
     

4

What 3 stages of disease have been described?

By Luck ( himself)!!!!- NB LUCKy Pir

1)PROLIFERATIVE- hypercellular, large myofibroblasts, minimal extracellular matrix

2)INVOLUTIONAL -dense myofibroblast network increase collagen 3 cf 1

3)RESIDUAL - myofibroblasts DISAPPEAR leave FIBROCYTES as predom cell

5

What does it using present with?

  • A nodule in the palmar fascia maybe painful and progress insidiously -> diseased cords and finally digitally flexion contractions beginning at MCP and progressing distally
  • Reduced rom MCPJ/PIPJ/DIPJ

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6

what has duptyren's been associated with?

  • ETOH xs
  • DM
  • epilepsy
  • chronic pulmonary disease
  • TB
  • HIV

7

Can you describe/draw the normal fascial structures that become involved?

  • PRETENDINOUS BAND- palm

  • SPIRAL BAND- palmodigital transition( underneath NV bundle)
     
  • NATATORY BAND- palmodigital transition

IN DIGIT 

  • GRAYSON LIGAMENT (palmar to NV bundle, passes flexor sheath to the skin, maintain digitial skin position)
  • LATERAL DIGITAL SHEET- formed from fibres form natatory & spiral band

Cleland ligaments relatively not involved in D.

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8

What does the disease change the bands into?

Can you name them all?

Diseased CORDS

  • Central
  • Spiral
  • Natatory
  • Lateral 
  • Retrovascular

9

What is not involved in the disease process?

CLELAND ligaments (nb Clevland)

10

Can you name the palm cords?

Pretendinous cords

11

What is the SPIRAL CORD made up from?

  • SPIRAL BAND
  • PRETENDINOUS BAND
  • LATERAL DIGITAL BAND
  • NATATORY BAND/LIGAMENT
  • GRAYSON LIGAMENT

(NB Simon Phillips Never Loves Grapes)

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12

What does the spiral cord lead to ?

  • PIPJ contraction
  • NV bundle = As travels under NV bundle it displaces it MIDLINE and SUPERFICIAL( VOLAR) increased risk during surgical resection
  • Best predictors of displacement = PIPJ flexion contracture ( 77% PPV), Interdigital soft tissue mass (71% PPV)
  • Clinically the most important contracture as pipj flexion warrants surgery

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13

What makes up the natatotory cord?

What is its function?

  • Develops from fibres of natatory ligament, just under commissure skin
  • causes Web space contracture

14

What is the central cord in the phalanx?

What does it cause?

  • The distal  extent of the pretendinous cord
  • Fibres fro cord extend and insert into flexor sheath around PIPJ -> MCPJ contraction
  • Not involved with neurovascular bundle

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15

What makes up the retrovascular cord?

What does it cause?

  • Can arise dorsal to the NV bundle taking origin fro the proximal phalanx and inserting onto the distal phalanx
  • DIPJ flexion

16

What are the best predicts of displacement of nv bundle ?

PIPJ flexion contraction -77% PPV

interdigital Soft tissue mass 71% PPV

17

What are the signs of D?

  • Nodule in pretendinous band of palmar fascia
  • involves commonly small or ring finger
  • Postive Heuston Table top test- look for mcpj/pipj contracture
  • look for bilateral involvement and ectopic
  • Ledderhose disease- plantar fascia
  • Peyronie's disease- dartos fascia of penis
  • Garrod's disease ( kuckle pads)

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18

What are the tx options?

Non op

  • Range of motion exercises
  • largely unsuccessful
  • Emerging COLLAGNASE injections- derived from clostridium hystolyticum are promising.
  • Causing lysis and rupture of cords but swelling, bruising, flexor tendon rupture early results better for MCPJ and contractors less than 50 degrees

19

What are the indications for surgery?

  • MCP contracture >30 degrees
  • Any PIPJ contracture
  • Painful nodules ARE NOT INDICATIONS for surgery

20

What are the surgical options?

  • Needle cordotomy- for frail patients- risk of nerve injury
  • Regional palmar fasciectomy - Brunner incision, V to y or sequential z plasties can be used to lengthen the skin- removed disease tissue only in digits with preservation of skin- iatrogenic nerve injury 7%. early rom day 5-7, night tiemextension brace/splint 
  • Dermofasciectomy with full thickness skin graft or z plasty, v-y advancements or healing by secondary intention- recurrence is less with this procedure as remove skin
  • Open palm technique- McCash- approach transverse skin incision at level of distal palm crease ( see pic)- may be left open with/ wout delayed wound healing- low risk of haematoma formation- useful older pts at risk of stiffnes/low risk of complx due to lack of haematoma formation
  • Recurrent disease- dermofascectomy+FTSG / arthrodesis/ amputation maybe required

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21

What are the complications ?

  • Haematoma - most common -> FLAP Necrosis
  • Wound complications
  • Digital ischaemia
  • Post op swelling
  • Recurrence- long term 50% less with dermofasciectomy
  • Flare reaction -pain w diffuse swelling. hyperthesia, stiffness
  • NV injury- due to displaced by spiral cord- central adn superficial. identify prior to excising cord 
  • CRPS
  • skin loss
  • amputation

22

is physio important?

Yes with active rom and static splinting to maintain extension