Dupuytren's Contracture

Dupuytren's contracture, also known as Dupuytren's disease, Morbus Dupuytren, Viking disease, palmar fibromatosis, and Celtic hand, is a condition characterised by the permanent bending of one or more fingers due to a build-up of scar tissue within the palm.

This condition was first described by Guillaume Dupuytren, who performed the first successful operation in 1831.

Signs and Symptoms

Typically, Dupuytren's contracture presents as a thickening or nodule in the palm, which can be either painful or painless. Over time, this nodule causes increasing loss of range of motion in the affected fingers. The earliest sign of a contracture is a triangular "puckering" of the skin over the flexor tendon at the metacarpophalangeal (MCP) joint. The ring finger is most commonly affected, followed by the little and middle fingers. The disease begins in the palm and progresses to the fingers, affecting the MCP joints before the proximal interphalangeal (PIP) joints.

Risk Factors

Several risk factors for Dupuytren's contracture have been identified:

Non-Modifiable

• Scandinavian or Northern European ancestry
• Gene variants inherited from Neanderthals
• Male sex
• Age over 50
• Family history

Modifiable

• Smoking
• Lower-than-average body mass index
• Alcohol consumption
• Manual work involving significant physical labour

Other Conditions

• Previous hand injury
• Ledderhose disease
• Epilepsy (possibly due to anti-convulsive medication)
• Higher-than-average fasting blood glucose level
• Diabetes mellitus
• HIV
• Previous myocardial infarction

Diagnosis

Diagnosis of Dupuytren's contracture is primarily clinical, based on physical examination. The condition is classified into three types:

1. Type 1: An aggressive form affecting men under 50, often with a family history.
2. Type 2: The more common type, usually beginning above age 50.
3. Type 3: A mild form often seen in diabetics or those on certain medications.

Treatment

Treatment is indicated when the so-called table-top test is positive. The main treatment options include:

Surgical Options

Limited Fasciectomy

This involves the removal of pathological tissue and is commonly performed under regional or general anaesthesia. The skin is typically opened with a zig-zag incision, and all diseased cords and fascia are excised. Complications may include digital nerve or artery injury, infection, and complex regional pain syndrome.

Dermofasciectomy

This procedure involves excising diseased cords, fascia, and overlying skin, typically replaced with a skin graft. It is used when the skin is clinically involved or in cases of recurrent disease.

Segmental Fasciectomy

Less invasive than limited fasciectomy, this procedure involves excising parts of the contracted cord. It is performed under regional anaesthesia, and people are encouraged to start moving their hands the day after surgery.

Less Invasive Treatments

Percutaneous Needle Fasciotomy

A minimally invasive technique where the cords are weakened through the insertion of a small needle. This procedure allows for minimal intervention and a rapid return to normal activities.

Extensive Percutaneous Aponeurotomy and Lipografting

Introduced in 2011, this procedure uses a needle to cut the cords at multiple points and separate them from the skin, followed by the injection of a lipograft.

Collagenase Injection

The cords are weakened through the injection of collagenase, an enzyme that breaks peptide bonds in collagen. This treatment is effective and minimally invasive.

Radiation Therapy

Radiation therapy is used mostly for early-stage disease but is unproven. The Royal College of Radiologists concluded that it is effective in early-stage disease that has progressed within the last 6 to 12 months.

Alternative Medicine

Various alternative therapies such as vitamin E treatment and laser treatment have been studied, though without control groups and are generally not considered effective.

Postoperative Care

Postoperative care involves hand therapy and splinting to optimise post-surgical function and prevent joint stiffness. Although splinting is widely used, evidence of its effectiveness is limited and varies by surgeon preference. Early self-exercise and stretching are often recommended.

Prognosis

Dupuytren's disease has a high recurrence rate, especially in individuals with so-called Dupuytren's diathesis. Recurrence rates are higher in younger people and for proximal interphalangeal joint contractures.

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Self-assessment MCQs (single best answer)

Who first described Dupuytren's contracture and performed the first successful operation?

Which finger is most commonly affected by Dupuytren's contracture?

Which of the following is a non-modifiable risk factor for Dupuytren's contracture?

What is a common complication of limited fasciectomy for Dupuytren's contracture?

What is the primary method of diagnosing Dupuytren's contracture?

Which of the following treatments for Dupuytren's contracture involves injecting an enzyme to break down collagen?

What is a sign of early-stage Dupuytren's contracture?

Which type of Dupuytren's contracture is considered the most aggressive?

What is the purpose of postoperative care in Dupuytren's contracture?

Which treatment option for Dupuytren's contracture is primarily used for early-stage disease but is considered unproven?