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

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

Anatomy

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

Pretendinous Bands

  • 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

Natatory Ligament

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

Spiral Bands

  • Termination of pretendinous band
  • Pass deep then lateral to neurovascular bundle

Lateral Digital Sheet

  • Lateral condensation superficial fascia

Grayson’s Ligament

  • Volar to neurovascular bundle
  • Arises tendon sheath
  • Inserts to lateral digital sheet

Cleland’s ligament

  • Dorsal to nv structures & same origins as Grayson’s ligaments
  • Not involved

Transverse fibres of the palmar aponeurosis

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

Cords

  • 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 Cord

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

Pathology

Myofibroblasts

  • 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 Collagen

  • Increased secondary to ↑ cell density
  • Controversial role

Abundant Fibroblasts

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

Classification

History

  • 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 Diathesis

  • 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

Investigations

Differential Diagnosis

Treatment

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

Hueston’s “Table 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

  • Incision
  • 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

  • Revision
    • Allen’s Test Finger
    • 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%
  • Classic triad
    • Haematoma
    • 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