Dupuytren's Contracture

Dupuytren's contracture is a progressive disorder that affects the palmar fascia causing the fibrous tissue to shorten and thicken.¹ This contracture results in flexion deformity of the fingers and loss of hand function.

• Most commonly affects the ring finger, followed by the little finger and then the middle finger. Can affect any digit.
• Commonly bilateral (45%).³ Unilateral cases more often affect the right hand. Hand dominance is not a factor.
• Starts with pitting and thickening of the palmar skin and underlying subcutaneous tissue with loss of mobility of the overlying skin.
• Next a nodule forms which is firm and painless and fixed to the skin and deeper fascia. The nodule is palpable and later becomes visible.
• A cord (a linear thickening that can resemble a tendon) then develops which begins to contract over months to years.
• The contraction of the cord pulls on the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints and leads to a progressive flexion deformity in the fingers.
• Deformity can affect activities of daily living and occupation. The stage at which the patient presents will depend upon tolerance of this and is likely to depend upon the degree to which work or hobbies may be affected.
• A more aggressive form can occur in which there is early age of onset, a strong family history, bilateral hand involvement and associated ectopic disease (see below).

• Typically affects Northern European men (especially from Scotland, Iceland and Norway).⁴
• Also common in Australia.⁴
• Onset usually in mid 50s.
• Women can also be affected but onset tends to be in mid 60s.
• Autosomal dominant inheritance of varied penetrance suggested.
• It may affect as many as 2 million people in the UK but only a small number will require surgery.²

• The aetiology is uncertain.
• There is initial fibroblast proliferation as the nodule forms. This then slows down and leads to connective tissue assembling to form the cord. There is also collagen deposition.
• Dupuytren's and wound healing seem to have many similar features.⁵ The initial triggers in Dupuytren's are unclear.

Risk factors

• Smoking
• Alcohol excess (but most patients are not alcoholics)⁴
• Diabetes, especially if insulin-dependent⁶
• Hypercholesterolaemia
• Anticonvulsant drugs/epilepsy (controversial)⁷
• Hand trauma and manual labour (also controversial, studies show no consensus; history of manual labour has worse prognosis)⁸

• MCP joint contracture should be measured whilst applying passive extension to the PIP joint.
• PIP joint contracture is measured while the MCP joint is held in flexion.
• When there is 30 degrees of flexion deformity at the MCP joint, the patient is unable to place their palm flat against a hard surface, for example a table. This is known as the Hueston tabletop test.¹
• If there are knuckle pads on the dorsal PIP surfaces, there is more aggressive disease (see below).
• Swan-neck deformities and boutonnière deformities may rarely occur.

Other areas of the body can be affected (also known as ectopic disease):

• The knuckles: Garrod's knuckle pads (44-54%) are thickened knuckle pads over the dorsal surface of the PIP joints
• The soles of the feet: Plantar fibromatosis, or Ledderhose disease (6-31%)
• The penis: Penile fibromatosis, or Peyronie's disease (2-8%)

• Ganglion
• Trigger finger
• Post-traumatic joint contracture
• Epithelioid sarcoma

• Usually a clinical diagnosis.
• Due to the association with excess alcohol intake it may be judicious to take a drinking history and check liver function tests.
• If there is any suggestion of undiagnosed diabetes then fasting blood glucose should be checked.

Many patients with Dupuytren's contracture do not develop significant disability and will not need treatment, but:

• Treatment is usually considered when (or ideally before) functional disability occurs. It aims to restore hand function and prevent progression.
• A positive Hueston tabletop test is a good indication for referral.¹
• If the patient is amenable to surgery, early referral is recommended (i.e. ≥30° of contracture at the MCP joint and any degree at the PIP joint).^1,2 This is because contracture and disability can become irreversible due to ligament remodelling, and early referral means surgeons are then best placed to decide on the timing of any interventions.

Surgical treatment

The exact timing of surgery varies. It is usually performed when the MCP joint contracture is >40° or when PIP joint contracture is >20°.¹ Procedures include:

• Fasciotomy
  • This can be done as either an open or closed procedure.
  • Closed fasciotomy (or percutaneous needle fasciotomy) may be done in the outpatient clinic and has received a lot of press recently.
  • The contracted cord is divided in the palm, the finger, or both, using a blade or the bevel of a needle. Once partial section has been achieved the finger is extended causing the fibrous band to snap. A dry bandage is then secured by elastic tape and placed over the injection site for at least 48 hours.¹⁰
  • With closed fasciotomy, there may be short-term benefit but by 3 to 5 years the recurrence rate can be around 50%.¹⁰
  • It is suggested that closed fasciotomy is not suitable for everyone and that it is unlikely to be of benefit in the long term if there is severe deformity.¹¹
  • It may be more suitable for those with a prominent cord and predominant metacarpophalangeal contracture¹² or for those who want minimally invasive surgery.
  • It may have a place in delaying fasciectomy.¹³
  • It has been reviewed and approved by NICE who consider that it may be of benefit for older patients unsuitable for more major surgery.¹⁰
  • Some people are concerned about the risk of nerve damage and tendon injury with percutaneous needle fasciotomy.^10,13
• Fasciectomy
  • This can be a segmental fasciectomy (short segments of the cord are removed), a regional fasciectomy (the entire cord is removed) or a dermofasciectomy (the cord and overlying skin is removed followed by skin grafting).
  • The procedure is usually done under regional block or general anaesthetic as a day case.
  • Regional fasciectomy is the most common procedure undertaken for Dupuytren's contracture.¹⁴
• Finger amputation
  • This is rarely done.
  • Used if severe (usually because presentation has been very delayed).

Splinting and hand physiotherapy are needed after surgery. A night splint is normally worn for 3 months.

Medical treatment

A large number of medical treatments has been tried with variable effects including:

• Radiotherapy
• Splinting
• Steroids
• Topical vitamin A

More promising newer treatments include:

• Intralesional gamma-interferon¹⁵
• Collagenase enzymatic fasciotomy (involves injecting collagenase into the lesion)¹⁶
• Intralesional triamcinolone acetonide¹⁷
• 5-fluorouracil¹⁸

During surgery there is a risk of nerve, tendon or blood vessel injury. Possible post-operative complications include:¹⁹

• Infection
• Haematoma and flap necrosis
• Skin slough and scar contraction
• Carpal tunnel syndrome
• Reflex sympathetic dystrophy

• The course of the disease is variable in terms of how fast and how far it will progress.
• 10% of cases can regress without treatment.²⁰
• Most patients do not require surgery.
• Surgery, when performed, improves function²¹ but postoperative recurrence may be up to 50%.

Dupuytren's contracture is noted in European medical literature as early as the 17th century. Medical historians have associated it with the Vikings, the Scottish bagpipe-playing MacCrimmon clan and the Papal Sign of Benediction.²²

Baron Dupuytren performed his first palmar fasciotomy in 1831 and his name has become associated with the condition ever since. Dupuytren was born in 1777 and was later appointed surgeon at the Hotel-Dieu in Paris. He was created a baron by Louis XVIII. He died in 1835 at the age of 58, having suffered a stroke.