Dupuytren’s Disease

Video

Definition

Fibroproliferative disorder of palmar aponeurosis

Aetiology

Murrell’s Theory of Pathogenesis

  • Initiating event is microvascular ischaemia
  • Ischaemia itself leads to conversion of
    • ATP toHypoxanthine
    • Endothelial Xanthine Hydrogenase to Xanthine Oxidase
  • Xanthine Oxidase catalyses reaction
    • Hypoxanthine to Uric Acid
    • Which gives off OH- (hydroxyl free radicals)
  • OH- stimulate fibroblast proliferation
    • Increasing Type III Collagen
  • Fibroblasts strangle microvessels
    • Vicious Cycle
  • ATP : Hypoxanthine : Uric acid + OH- : fibroblast proliferation : microvascular strangulation

Epidemiology

  • 5% Caucasians
  • Especially Vikings
  • Rare Blacks & Asians
  • M:F – 5:1
  • 20% Male > 65 years

R>Risk factors

  • Local ischaemia
    • DM
    • Smoking
    • Trauma (including fractures of the wrist)
    • Occupation? (weight of evidence against it)
    • Alcoholic cirrhosis
    • Anti-epileptic medication (phenobarbitone)
    • HIV
  • Genetic
    • Vikings – Anglo-Saxon/ Celtic origin
    • AD with variable penetrance

Ana>Anatomy

  • Key to dissection
  • Normal structures (bands) become cords & nodules
  • Pneumonic “bland bands become crazy cords”

Pretend>Pretendinous Bands

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  • Volar & midline to neurovascular bundle
  • Form from palmar aponeurosis as it travels to digits
  • Splits at MCPJ
  • Some into skin
  • Rest deep to neurovascular bundle as spiral band
  • Natator>Natatory Ligament

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  • Transverse & superficial to neurovascular bundle at web space
  • Attached to flexor sheath/ skin
  • Almost always involved
  • Spiral >Spiral Bands

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  • Termination of pretendinous band
  • Pass deep then lateral to neurovascular bundle
  • Lateral>Lateral Digital Sheet

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  • Lateral condensation superficial fascia
  • Grayson>Grayson’s Ligament

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  • Volar to neurovascular bundle
  • Arises tendon sheath
  • Inserts to lateral digital sheet
  • Cleland>Cleland’s ligament

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  • Dorsal to nv structures & same origins as Grayson’s ligaments
  • Not involved
  • Transvers>Transverse fibres of the palmar aponeurosis

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  • “Skoogs fibres”
  • Superficial to nv bundles at level MC heads
  • Never involved
  • Where you should look to trace nv bundles
  • Cords

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  • Central, Spiral, Lateral, Natatory
  • Form along normal fascial pathways
  • Tighten up
  • Follows three dimensional anatomy
  • Neurovascular bundle spirals around spiral cord
  • Between distal palm crease & proximal crease finger
  • Coalesce into one
  • Spiral CordSpiral Cord

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  • Formed by
    • Pretendinous band (medial to nv bundle)
    • Spiral Band (deep)
    • Lateral Digital Sheet (lateral)
    • Grayson’s ligament (superficial)
  • Pathology

    Pathology"wp-block-heading">Myofibroblast>Myofibroblasts"wp-block-list">
  • Contractile cell
  • Occur elsewhere (GUT)
  • But not normally in palmar fascia
    • Originate as Perivascular Fibroblasts
  • Convert secondary to local hypoxia
    • Mainly in nodules
  • None in cords
  • Responds to cell growth factors
    • PDGF/ FGF/ TGF B
  • Type III Coll>Type III Collagen

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  • Increased secondary to ↑ cell density
  • Controversial role
  • Abundant Fibrob>Abundant Fibroblasts

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  • Cords only
  • Peri-arterial
  • Organized along stress lines
  • Occluded microvessels
  • Classification<>Classification

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  • M>F – 7:1
  • Right > Left
  • RF > LF > MF > thumb > IF
  • Tender nodules beyond distal palmar crease
  • Painless fixed flexion deformity with disability
  • Nodules, cords, pits
  • Dupuytren’s Diath>Dupuytren’s Diathesis

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  • Aggressive Disease : rapid progression & marked contractures
    • Young males
    • Significant family history
    • Multiple digits
    • Bilateral
    • Garrod’s Nodes (dorsal thickening of knuckle pads at PIPJ vs carpet layers at MCPJ)
    • Lederhose Disease
    • Peyronie’s Disease
  • Examination

    <>Examinationk-heading">Investigations >Investigationsk-heading">Differential Diagnosi>Differential Diagnosisk-heading">Treatment

    Treatment

    k-heading">Don’t operate if negative Table Top Test
    • Don’t operate if nodule is painful – can trigger surrounding tissue & ↑ proliferation
    • On occasion may need to operate if deep skin folds & pits cause repeated maceration & infection

    Nonoperative

    • No proven benefit
      • Corticosteroids (to nodules & Garrod’s pads – Ketchum 1991)
      • Occupational Therapy
      • Allopurinol
      • 5-FU
      • Vitamin E
    • Patient Education

    Operative

    Hueston̵>Operativee Top” Test

    • Palm unable to touch table when trying to place hand flat on table
    • If FFD of PIPJ & MCPJ able to hyperextend & allows palm to touch table then this is a –ve test
    • Positive when MCPJ fixed flexion deformity > 40° ?

    MCPJ fixed flexion deformity

    • easily correctable (see below)
    • Often becomes troublesome with FFD > 30°
    • Generally correctable at any degree of FFD

    PIPJ fixed flexion deformity

    • difficult to correct
    • 30° fixed flexion deformity usual end result
    • Hence McFarlane (1990) advocates
      • Release of PIPJ only if fixed flexion deformity > 30°
    • Preoperative splint?

    Three technical considerations

    >Three technical considerationson
  • Fasciotomy/ fasciectomy
    • Fasciotomy
    • Segmental fasciectomy
    • Selective fasciectomy
    • Radical fasciectomy
  • Wound closure
  • “Operation is dissection of the neurovascular bundles”

    Skin Options

    • Direct Closure ± Flaps
      • Longitudinal with Z-plasty
        • Midline longitudinal
        • Points at creases in midlateral line passing to midline
        • Angle 60° to longitudinal axis
        • Allows dissection from palm to finger
        • Does not allow wide dissection in palm : ↓ postoperative haematoma
      • Transverse
        • Used in palm when 2-3 rays involved
        • Can be combined with longitudinal incisions
        • Simple, good exposure & rapid healing
        • Skin necrosis & ↑ postop haematoma
      • Brunner ± VY plasty
        • Popular
        • Can do lazy Brunner (lazy “S”)
        • Zig-zag prevents scar contracture
        • However difficult to design flaps with finger contracted & flap difficult to construct with intimate proximity of diseased fascia
    • Dermofasciectomy
      • Skin excision
      • Full thickness flaps
      • Less recurrence
      • Graft failure
    • McCash Open Technique
      • Transverse mid palm wound at distal palmar crease
      • Leave open but review regularly
      • Allows haematoma to drain
      • Closes by contraction (takes 3-5/52)

    Fascia Options

    • Fasciotomy/ Segmental Fasciectomy
      • Limited procedure with cord divided or short portion of fascia excised
      • Partial or complete correction
      • Elderly or mild disease
      • Can be performed as outpatient procedure
    • Selective Fasciectomy
      • Only Dupuytren’s tissue excised – leave normal looking tissue
      • Most popular technique
      • Mainstay
      • Subclinical disease may progress but often does not warrant further surgery
    • Radical Fasciectomy
      • Excision of normal & diseased fascia
      • Less popular now
      • Still get recurrence
      • For those with Dupuytren’s diasthesis?

    Wound closure

    • Options
      • Suture
      • Graft
      • Leave open
    • Principle is closure without tension as this predisposes haematoma & wound breakdown

    Release of PIPJ FFD

    • Volar capsule/ volar plate released proximally at pars flaccida
    • Checkrein Ligament (at proximal end of volar plate) released
    • Accessory Collateral Ligaments (“sides of the wheelbarrow”) released
    • Then pass along volar portion of collateral ligaments (ie “remove the arms off the wheelbarrow”)
    • Then can release one collateral ligament if still tight

    Management of Recurrence

    • Revis>Management of Recurrence
    • Test Sensation
    • High risk neurovascular bundle injury
    • Selective fasciectomy & graft
    • PIPJ fusion if severe
  • Amputation of finger if
    • PIPJ fixed flexion deformity > 90°
    • Insensate
    • Pain
    • Dysvascular
  • Complications

    • 20%
    • Class>Complicationsmatoma
    • Infection
    • Skin loss
  • Neurovascular bundle injury
    • Start dissection of nerve proximally
  • Recurrence 50%
    • Dupuytren’s in a surgically treated field
  • 15% need repeat operation
  • RSD
  • Prognosis

    References

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