Dupuytren's Disease: Pathology and Management

Dupuytren's disease is a benign fibromatosis affecting the palmar and digital fascia of the hand, leading to debilitating flexion contractures. This comprehensive overview delves into the intricate pathology and management of Dupuytren's disease, exploring its origins, progression, and various treatment approaches. Understanding this condition is crucial for students studying hand anatomy, pathology, and surgical interventions.

Understanding the Pathology and Management of Dupuytren's Disease

Dupuytren's disease starts with a palpable mass, known as a Dupuytren’s nodule, usually at the distal palmar crease. These nodules can be transient or mildly painful. As they enlarge, they form pathologic cords that extend distally and proximally, causing flexion contractures in the joints.

The fourth and fifth digits are most commonly involved, though the thumb, index, and long fingers can also be affected. The disease on the radial side of the hand is more prevalent in diabetic patients. Diagnosis involves a careful patient history and physical examination, including the 'table top test' (Figure 5.1B), where the hand and fingers cannot be simultaneously placed flat on a surface.

Epidemiology and Associated Risk Factors

Dupuytren's disease is the most common heritable connective tissue disease in Caucasians. Studies show an incidence of 9% in men and 3% in women, increasing with age, with global prevalence estimated at 3% to 6% in white populations. It is very rare in people of African descent and common in Japan but rare in other Asian countries.

Genetic factors play a significant role, with an autosomal dominant inheritance pattern and variable penetrance. Associated conditions include diabetes, alcoholism, epilepsy, smoking, AIDS, and vascular disorders, though these do not imply a direct cause-and-effect relationship. While heavy work was once suggested, occupation is not a clear cause; however, a single injury can initiate the disease in genetically predisposed individuals.

The Histology and Biochemistry Behind Dupuytren's

Microscopic studies classify Dupuytren’s disease into three stages: proliferative, involutional, and residual. The nodular myofibroblast is responsible for contracture, containing actin, myosin, and ATPase, enabling contraction. Myofibroblast density correlates with disease recurrence.

Biochemical changes include an increased ratio of type III to type I collagen, with type III collagen being significantly elevated in nodules and cords compared to normal fascia. Oxygen free radicals stimulate abnormal fibroblast proliferation. Fibronectin anchoring strands connect myofibroblasts to the matrix, allowing transmission of contractile forces to collagen. Growth factors like prostaglandins, basic fibroblast growth factor (bFGF), platelet-derived growth factor (PDGF), and transforming growth factor-β (TGF-β) are all implicated in myofibroblast activity and proliferation.

Normal Anatomy vs. Pathologic Cords in the Hand

Normal connective tissues of the hand, such as fascia, ligaments, and bands, form a fibrous continuum. These include the digital fascia, palmar digital junctional fascia, and palmar fascia. Key structures are the lateral digital sheath, superficial fibrofatty palmar and dorsal fascia, Cleland’s ligaments (dorsal to neurovascular bundle), and Grayson’s ligaments (volar to neurovascular bundle) (Figure 5.4).

In Dupuytren's disease, normal fascial structures transform into pathologic cords, which are the building blocks of contractures. Understanding the pathoanatomy of these predictable cords is crucial for safe and effective treatment. Common cords include:

• Pretendinous Cord: Originates from pretendinous band.
• Central Cord: Arises from pretendinous band and superficial palmar fibrofatty fascia, often causing combined MP and PIP joint contractures (Figure 5.5).
• Spiral Cord: A complex cord formed from the pretendinous band, spiral band of Gosset, lateral digital sheath, vertical band, and Grayson’s ligament. It displaces the neurovascular bundle, making it vulnerable during surgery (Figure 5.6).
• Natatory Cord: Involves natatory ligaments.
• Abductor Digiti Minimi Cord: Forms from the abductor digiti minimi tendon, particularly in fifth finger contractures (Figure 5.7).
• Lateral Cord: Results from contracture of the lateral digital sheath.
• Retrovascular Cord: Arises from digital fascia dorsal to the neurovascular bundle.
• Commissural Cord: Develops from natatory ligaments in the first web space, affecting thumb function.
• Radial Thumb Cord: Rarely causes contractures but may require fasciectomy if function is impaired.

Cords often act in combination, such as central cord–lateral cord or central cord–spiral cord combinations, which can encase the neurovascular bundle. Central cord–natatory cord or spiral cord–natatory cord combinations can cause simultaneous MP joint contractures in adjacent fingers (Figure 5.8).

Diagnostic Considerations and Severity Assessment

Diagnosis relies primarily on patient history and physical examination, revealing palpable nodules and cords with a positive table top test. The severity of fixed joint contractures is measured with a digital goniometer. Dynamic contractures can occur, where a central cord crossing MP and PIP joints may show varying flexion contracture depending on MP joint position (Figure 5.2).

Radiographs or MRI are generally not needed for diagnosis, except in rare cases with associated osteoarthritis, rheumatoid arthritis, or to evaluate a solid knuckle pad or isolated palmar wrist nodule.

Management of Dupuytren's Disease: Therapeutic Options

The goal of treatment is to release joint contractures and improve hand function, as surgery cannot cure the disease. Treatment is indicated when an MP joint contracture is 30 degrees or greater, or a progressing PIP joint contracture is 20 degrees or greater, causing functional disability. Patients with Dupuytren's diathesis (positive family history, bilateral disease, ectopic lesions, male gender, age <50) are more prone to multiple surgeries.

1. Observational Management:

• Primary treatment for Dupuytren's nodules remains observational. Tenderness usually resolves, even if the nodule persists.
• Symptomatic nodules may be treated with triamcinolone injections, showing regression but frequent recurrence.
• Surgical excision of a nodule is reserved for specific situations, such as superficial to a trigger finger or for unrelenting pain.
• Knuckle pads are also primarily observational, with surgical excision considered for pain, excessive size, or interference with activities.

2. Surgical Interventions (Fasciectomies and Fasciotomies):

Many surgical skin incisions exist (Figure 5.9), all following basic principles: avoiding right-angle creases, preserving flap thickness, and dissecting proximally to distally.

• Limited Fasciectomy: The most popular method, involving excision of pathologic cords while preserving uninvolved fascia. It offers excellent exposure and correction, but recurrence remains possible (Figure 5.13).
• Segmental Open Fasciectomy: Excision of a portion of the cord through short incisions, minimizing secondary scarring (Figure 5.10).
• Radical Fasciectomy: Less popular, involves excising all normal-appearing fascia in addition to pathologic cords. It causes more morbidity without significantly reducing recurrence rates.
• Dermofasciectomy: Excision of contracted fascia with overlying skin, followed by skin grafting. The graft acts as a 'fire break' to reduce recurrence, particularly useful in young patients with aggressive or recurrent disease.
• McCash Technique (Open Palm): Transverse incisions are left open, allowing immediate active range of motion. This avoids skin grafts, especially beneficial for older patients with arthritic joints.
• Open Fasciotomy: Cutting but not removing pathologic cords, used in selected cases for significant functional improvement.
• Closed Needle Fasciotomy (Percutaneous Needle Fasciotomy): A 25-gauge needle pierces the skin and repeatedly cuts the cord, followed by passive extension. It has a high recurrence rate (58% at 3 years) and risks nerve injury or tendon rupture.

3. Enzymatic Fasciotomy (Collagenase Injection):

Approved by the FDA, collagenase derived from Clostridium histolyticum is injected directly into the cord (Figure 5.12). The enzyme disrupts collagen bonds, weakening the cord. The following day, the contracted joint is passively extended, rupturing the cord. This method has shown excellent results for MP and PIP joint contractures (Table 5.3, Table 5.4), with minimal adverse events like injection site pain, ecchymosis, and edema, but rare tendon ruptures have occurred. Recurrence rates are approximately 10% for MP joints and 20% for PIP joints at 5 years.

Postoperative Management and Expectations

After surgery, the hand is splinted in extension with a bulky dressing and elevated for 48 hours. Gentle active motion is encouraged. Drains are removed within 1-2 days, and hand therapy begins, including range of motion exercises, soaks, and a removable nighttime extension splint for 3-4 months.

Patients must understand that Dupuytren's surgery improves function but does not cure the disease. Recurrence and disease extension are common. Potential complications include failure to correct contracture, loss of flexion, digital nerve laceration (1.5%), arterial laceration, skin flap loss, digit loss, tendon damage, hematoma, infection, and complex regional pain syndrome. Prompt repair of nerve and artery lacerations is critical, and prophylactic measures like tourniquet deflation before closure and careful hemostasis are essential. Digit loss is a rare but possible complication in severe cases.

Frequently Asked Questions about Dupuytren's Disease

What is Dupuytren's diathesis?

Dupuytren's diathesis refers to a subgroup of patients, typically Caucasians, with a positive family history, bilateral disease, ectopic lesions (like plantar fibromatosis or knuckle pads), male gender, and an age of onset younger than 50. These individuals often experience more aggressive disease and a higher likelihood of recurrence, requiring multiple surgeries.

How does a central cord cause contractures in Dupuytren's disease?

The central cord originates from the pretendinous band and palmar superficial fibrofatty fascia. It inserts into the skin over the proximal phalanx, the tendon sheath distal to the PIP joint, periosteum at the base of the middle phalanx, and the lateral digital sheath. By shortening and thickening, this cord directly pulls on these attachment points, leading to flexion contractures, particularly affecting both the metacarpophalangeal (MP) and proximal interphalangeal (PIP) joints.

Why is the spiral cord considered risky during surgery?

The spiral cord is particularly hazardous during surgery because of its unique pathoanatomy that displaces the neurovascular bundle. In the pathologic state, this cord pulls the radial digital neurovascular bundle proximally, centrally, and superficially. As a result, when an incision is made, especially at the first digital flexion crease, the neurovascular bundle might be the first structure encountered, increasing the risk of surgical injury compared to other Dupuytren's cords.

What are the main differences between fasciotomy and fasciectomy?

Fasciotomy involves simply cutting the pathologic cord to release the contracture, but the tissue is not removed. This can be done openly or percutaneously (needle fasciotomy). Fasciectomy, on the other hand, involves excising (removing) the diseased fascial tissue. While a fasciotomy is less invasive and has a faster recovery, it often has higher recurrence rates. Fasciectomy aims to remove the source of the contracture and is the more common surgical approach, offering a potentially more durable correction, especially in limited or partial fasciectomies.

Can Dupuytren's disease recur after treatment?

Yes, recurrence and extension of Dupuytren's disease are common after both surgical and non-surgical treatments. After surgery, recurrence rates range from 8% to 54%, with PIP joint contractures recurring more often than MP joint contractures. After collagenase injection, recurrence is approximately 10% for MP joints and 20% for PIP joints at 5-year follow-up. Patients should be educated that treatment aims to improve function, not to cure the disease definitively.