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Dupuytren’s Disease

A fibroproliferative disorder of the hand characterized by the development of new tissue in the form of nodules and cords resulting in a progressive contracture of the fingers into the palm.
• Most common in men over 60 who are Scandinavian, Irish or eastern European

History
First description credited to Felix Plater (1614, Basel Switzerland).
  • Henry Cline (1808, London)described typical palmar fibrosis and flexion contractures and recommended surgery.
  • Astley Cooper (1822) also described finger contractures in A Treatise on Dislocations and Fractures of the Joints
  • Guillaume Dupuytren (1777-1835, Paris) published a description of 2 patients with palmar fibromatosis in 1834

Demographics
  • May be autosomal dominant with variable penetrance or sporadic (approximately 10% have a positive family history)
• Mainly Scandinavia and Great Britain
• Rarer in Europe further south
• Common in Australia due to British/ Irish
• virtually unknown in Greece, Middle East and orient • though Indians living in the UK have developed disease
• therefore possible environmental component

Dupuytren’s Diathesis
Spectrum of physical findings in patients with particularly strong gene expression • Present earlier (20’s or 30’s)
• Very aggressive cord development
• Multiple digit and bilateral
• Garrod’s nodes (knuckle pads)
• Lederhose’s disease (plantar fibromatosis)
• Peyronie’s disease (penile fascia involvement)

Significance
• High risk of poor surgical outcome/ early recurrence
• Complications • Longer rehab

Pathology of nodules and cords
  • Nodules and cords are pathological (abnormal) structures
  • Distinct histological features
  • Nodules are dense cellular collections of myofibroblasts
  • The myofibroblast in the nodule accounts for active contraction
  • Nodules produce flexion contractures by pulling through cords that have extended
  • past joints

  • Recurrence after surgery may be 2 to myofibroblast populations that have migrated into adjacent palm dermis and epidermis

Anatomy
• Palmar aponeurosis is a triangular thin sheet of fascial tissue organized distally into pre- tendinous bands (normal anatomic structures)
• Forms a 3D fibrous tissue continuum which acts as a framework for longitudinally running structures
The distal longitudinal fibres can be divided into 3 layers
! 1. Superficial; insert into the skin over the distal palm
• Insertion progressively more distal from radial to ulnar
! 2. Spiral fibers either side of the flexor tendon deep to the neurovascular bundle to the lateral digital sheet
! 3. Deep longitudinal fibers pass deeply either side of flexor tendons and MCP jts

Cord Contraction
• the Central cord follows layer 1 but passes more distally • Follow longitudinal pretendinous fibers
• Attaches to deep dermis and middle phalanx
• the Lateral cord is from the natatory ligament to the lateral digital sheet • does not usually cause PIP flexion contracture
• BUT on ulnar LF attaches to abductor cord and in this case can cause severe contracture
• the Spiral cord is from the longitudinal pretendinous fibers and follows layer 2
  • Through the spiral cord of Gosset to the lateral digital sheath
  • Attached to P2 via Grayson’s ligament
  • The spiral cord contracts and displaces the neurovascular bundles towards the midline and superficially
• making them prone to injury during surgery or injections

Clinical Presentation
• Men to women >7:1
  • Less severe or atypical in women
  • Nodule forms first but may disappear as disease progresses
  • or may present when tender nodule or noticed
  • Often present late with severe joint restriction if the disease is completely painless (common)
  • Often referred as trigger finger or “joint stiffness”
  • Progression unpredictable
  • Trauma may be a stimulus for progression

Table top test of Hueston – place hand and finger flat on table
o If your hand can go flat then surgery is usually not indicated
- Correlates with MCP (knuckle) joint contracture>30 deg Functional impairment – affected finger gets in the way of:
o Washing your face/combing hair o Putting your hand in your pocket o Racquet sports or golf

Treatment
Non-operative
-Education and reassurance

  • Disease is often mild and may never require surgical treatment
  • Progression of the disease is very difficult to predict
  • Stretching, massage or exercises are not helpful and make no difference to outcome
  • A steroid injection may help a tender nodule (not useful in cord disease) or for knuckle
pads

• Collagenase (Xiaflex) injection dissolves the cord and may be useful in early disease

Operative
• Needle fasciotomy
  • performed under Local Anaesthetic (LA) to divide cords
  • simple and useful in early contractures with quick recovery but may get rapid recurrence

• Percutaneous fasciotomy
• cords are cut through small incisions (LA) • similar indications to needle fasciotomy

• Open surgery (selective fasciectomy)
aim is to excise the diseased fascia to release contractures
• preserving main nerves and vessels
• maintaining full flexion of the fingers (avoiding stiffness)
combination of longitudinal and zig-zag (Brunner) incisions used with Z plasties to lengthen skin wounds (moves skin from the side to gain length)!
+/- skin grafts
• may decrease recurrence by bringing in normal skin
• BUT potentially slows rehabilitation resulting in more stiffness AND altered sensation, poorer wearcharacteristics (to normal skin) and poorer cosmesis
I use in severe disease/ Dupuytren’s diathesis or in revision (2nd) surgery

  • PIP (proximal finger) joint release
  • controversial
  • if the joint is still bent after cord excision the joint is often released
  • BUT may result in significant stiffness
  • AND difficult to maintain initial results
  • therefore I only perform joint releases if a severe contracture persists after cord excision; it is more important to be able to bend the finger easily after surgery than have a perfectly straight finger
Surgical risks
• long term recurrence is about 50%
• skin/ flap necrosis - usually heals with dressings only • Nerve/ vessel injury - more likely with severe disease •Complex Regional Pain Syndrome (CRPS)
  • difficult to predict and approximately 5% will develop
  • results in stiffness and often a poor result
  • Vitamin C (daily for 6 weeks) may decrease the risk but using and moving the hand with the guidance of hand therapy is more important

Post-operative management/ rehabilitation
• Hand therapy is extremely important in achieving a good result
• night splint (maintaining extension) for 4- 6 months
• out of plaster/ splints and moving, using the hand during the day in the first week • swelling/ oedema control helps decrease the resistance to movement (and pain) • wound and scar management