Orthopaedic Surgery - Dupuytren Contracture

Basics

Dupuytren contracture is a fibroproliferative disorder of the palmar fascia characterized by the development of nodules and longitudinal cords that progressively shorten and produce flexion contractures of the fingers.

The metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints are most commonly affected, particularly in the ring and small fingers.

The condition is also known as Dupuytren disease.

Typical Age and Sex

Dupuytren disease most commonly develops in men during the fifth through seventh decades of life.

Men are affected considerably more often than women, with reported male-to-female ratios ranging from approximately 2:1 to 10:1.

Women generally develop the disease later and tend to have less severe involvement.

Early-Onset Disease

Patients who develop Dupuytren disease at a younger age often experience a more aggressive clinical course, faster progression, and higher recurrence rates following treatment.

A particularly aggressive form occurring in younger patients is referred to as Dupuytren diathesis.

Epidemiology

The prevalence in the United States has historically been estimated at approximately 2–3% of the general population.

Hand dominance does not appear to influence which hand becomes affected.

Associated Medical Conditions

The disease is more frequent or severe in patients with diabetes mellitus.

Associations have also been reported with epilepsy, chronic obstructive pulmonary disease, HIV infection, and use of certain antiseizure medications.

Links with alcohol consumption and tobacco exposure have been reported, although the strength and causal significance of these associations have been debated.

Risk Factors

Important risk factors include increasing age, male sex, Caucasian ancestry, Northern European heritage, and a positive family history.

Genetics

Dupuytren disease has a strong hereditary component.

It has traditionally been described as having an autosomal dominant pattern with variable penetrance, although the genetic basis is complex and likely involves multiple susceptibility genes.

A documented family history is present in only a minority of affected patients.

Etiology

The exact cause remains incompletely understood.

Abnormal regulation of connective-tissue formation appears to produce proliferation of fibroblasts and myofibroblasts with excessive deposition and contraction of collagen within the palmar fascia.

Associated Conditions

Diabetes Mellitus

Patients with diabetes may develop Dupuytren disease more frequently and may have more extensive involvement.

Epilepsy

An association with epilepsy has historically been reported, particularly in patients receiving long-term anticonvulsant therapy.

Alcohol Use

Alcohol misuse has been associated with Dupuytren disease in some studies, although whether alcohol itself is directly causal remains uncertain.

Chronic Pulmonary Disease and HIV

Increased prevalence has also been described in patients with chronic obstructive pulmonary disease and HIV infection.

Diagnosis

Early Disease

The disease usually begins with one or more firm nodules within the palmar fascia.

These nodules may be painless or mildly tender.

Skin Changes

Skin dimpling, puckering, or adherence may develop over or around the palmar nodules as the underlying fascia contracts.

Bilateral Disease

Approximately 45% of patients may have involvement of both hands.

However, disease severity is often asymmetric.

Progression

As Dupuytren disease progresses, the abnormal fascia extends into the fingers and forms palpable cords.

Flexion contracture typically develops first at the MCP joint, followed by involvement of the PIP joint.

Web-Space Contracture

Contracture may also involve the web spaces, limiting finger abduction and interfering with hand opening.

Knuckle Pads

Approximately 20% of patients may develop firm fibrous nodules over the dorsal aspect of the PIP joints.

These are known as Garrod pads or Garrod disease.

They are often asymptomatic but may become painful when prominent or repeatedly traumatized.

Dupuytren Diathesis

Patients with a strong Dupuytren diathesis may have fibromatosis at other sites in addition to the hand.

These manifestations include plantar fibromatosis and penile fibromatosis.

Ledderhose Disease

Plantar fibromatosis involving the plantar fascia is known as Ledderhose disease.

Peyronie Disease

Fibromatosis involving the penis is known as Peyronie disease.

The coexistence of these disorders may indicate a more aggressive fibromatosis tendency.

Physical Examination

Palmar Nodules

The palm should be inspected and palpated for firm nodules.

They may be tender during the early proliferative phase but often become painless later.

Palmar Cords

Longitudinal cords may extend from the palm into one or more fingers.

These cords become increasingly prominent as the disease progresses and are responsible for the development of joint contractures.

MCP Contracture

The MCP joint is the most commonly affected joint.

Progressive shortening of the palmar fascia prevents full finger extension.

PIP Contracture

PIP involvement is particularly important because established PIP contractures are generally more difficult to correct and more likely to recur.

Hueston Table-Top Test

The Hueston table-top test is used to assess functional contracture.

The patient attempts to place the palm and all fingers flat against a table.

The test is positive when the hand cannot be placed completely flat because one or more contracted fingers remain elevated.

Pathological Findings

Myofibroblasts

The characteristic proliferating cell is the myofibroblast.

These cells have contractile properties and are believed to play a major role in progressive shortening of the diseased fascia.

Collagen Abnormality

Dupuytren tissue demonstrates an increased proportion of type III collagen relative to type I collagen compared with normal palmar fascia.

The altered collagen organization contributes to formation of thickened cords.

Differential Diagnosis

Arthritis

Degenerative or inflammatory arthritis can produce finger stiffness and contracture but does not typically create characteristic palmar nodules and fascial cords.

Post-Traumatic Contracture

Previous fractures, tendon injuries, or prolonged immobilization may produce fixed finger contractures.

A history of trauma and absence of Dupuytren cords help distinguish these conditions.

Burn Contracture

Deep burns involving the palm may lead to scar contracture and limited extension.

The scar pattern and history generally establish the diagnosis.

Ulnar Nerve Palsy

Ulnar neuropathy can cause clawing of the ring and small fingers.

Unlike Dupuytren disease, clawing results from intrinsic muscle weakness rather than fascial shortening and is accompanied by neurologic findings.

Treatment

General Principles

Treatment depends primarily on the degree of contracture, rate of progression, symptoms, and functional impairment.

The presence of nodules alone does not necessarily require intervention.

Observation

Patients with painless nodules and no significant contracture may be observed.

Slowly progressive disease that does not interfere with function can also be followed with serial examinations.

Monitoring Progression

The degree of MCP and PIP contracture should be documented over time.

Changes in the table-top test, finger extension, and functional use of the hand help determine whether treatment is becoming necessary.

Hand Therapy

Role of Therapy

Hand therapy is used primarily as an adjunct after procedural or surgical treatment rather than as a means of reversing established fascial disease.

Goals

Therapy aims to maintain the extension obtained during treatment, restore flexion, minimize edema and scar formation, and recover functional hand use.

Splinting

A comfortable and appropriately fitted extension splint may be used after intervention.

The duration of splinting varies depending on disease severity, procedure, and postoperative motion.

Independent Exercises

Patients should be instructed in regular active and passive range-of-motion exercises.

Independent home exercises are an important part of recovery.

Medication and Nonoperative Procedures

Vitamin E and Splinting Alone

Vitamin E and long-term splinting alone have not been shown to reverse established Dupuytren contracture.

Corticosteroid Injection

Corticosteroid injection into painful early nodules has been used to reduce tenderness and possibly soften or temporarily suppress nodule progression.

It may also be used selectively for symptomatic knuckle pads.

Collagenase Injection

Collagenase clostridium histolyticum has been used as a minimally invasive treatment for palpable Dupuytren cords.

The enzyme is injected directly into the abnormal cord to weaken its collagen structure.

Manipulation After Collagenase

Approximately 24–48 hours after injection, the finger is manipulated under local anesthesia to rupture the weakened cord and improve extension.

Response by Joint

Collagenase treatment has generally been more predictable for MCP contractures than for PIP contractures.

PIP disease is more difficult to correct because of secondary joint and soft-tissue changes.

Surgery

Indications

Surgery is not indicated for stable, painless palmar nodules without meaningful contracture.

Knuckle pads rarely require operative treatment.

PIP Involvement

PIP contracture deserves particular attention because it can become increasingly resistant to correction.

Progressive contracture, functional loss, or clinically important inability to extend the finger may justify procedural or surgical intervention.

Operative Options

Procedures used to treat Dupuytren contracture include percutaneous needle aponeurotomy, open fasciotomy, limited or selective fasciectomy, more extensive fasciectomy, dermofasciectomy with skin grafting, and, rarely, amputation.

The choice depends on disease severity, recurrence, anatomy, and patient factors.

Percutaneous Needle Aponeurotomy

Needle aponeurotomy divides the pathological cord percutaneously.

It offers rapid recovery and minimal surgical trauma but generally has a higher recurrence rate than more extensive fasciectomy.

Open Fasciotomy

Open fasciotomy releases the cord through an incision without removing large amounts of diseased fascia.

Limited Fasciectomy

Limited or selective fasciectomy removes the pathological fascial cords responsible for the contracture while preserving uninvolved tissue.

This is one of the most commonly used operative approaches.

Extensive Fasciectomy

More extensive fasciectomy may be considered for severe or recurrent disease but carries increased risks of wound complications, nerve injury, stiffness, and vascular compromise.

Dermofasciectomy

In aggressive or recurrent disease, involved skin and underlying diseased fascia may be removed together.

A skin graft is then used to cover the defect.

Amputation

Amputation is rarely required and is reserved for severe, recurrent, painful, or nonfunctional digits in which repeated reconstructive procedures are unlikely to provide useful function.

Factors Affecting Procedure Selection

Treatment planning should consider the degree and location of contracture, patient age, occupation, general health, condition of the palmar skin, presence of arthritis, previous procedures, and likelihood of recurrence.

Postoperative Splinting

The frequency and duration of postoperative splinting vary according to the procedure and severity of disease.

Historically, many patients have used extension splints for several months, including nighttime splinting during later recovery.

Return to Activity

Return to normal use depends on the treatment performed and wound healing.

After open surgery, substantial recovery of hand function is often expected within approximately 2–3 months, although more extensive disease may require longer rehabilitation.

Follow-Up

Prognosis

Approximately 80% of patients undergoing primary surgery may regain near-full flexion and extension, particularly when severe fixed PIP deformity is absent.

Aggressive Disease

Young male patients with a strong family history and other features of Dupuytren diathesis are more likely to experience rapid progression and recurrent contracture.

Associated Conditions and Severity

Patients with diabetes, epilepsy, or heavy alcohol exposure have historically been reported to develop more severe disease.

Recurrence

Recurrence rates vary widely according to disease biology, procedure, duration of follow-up, and definition of recurrence.

Published long-term recurrence rates have ranged from approximately 26–80%.

Need for Repeat Procedures

Many patients with recurrent disease do not require another operation.

Repeated procedures are most often necessary in younger patients with an aggressive Dupuytren diathesis.

Complications

Joint Stiffness

Finger stiffness is an important postoperative complication.

Early controlled motion, hand therapy, and patient education can reduce this risk.

Nerve Injury

Digital nerve injury may occur during surgery because the neurovascular bundles can be displaced or wrapped by diseased cords.

Historical rates of nerve injury have been approximately 1–3%, with greater risk during revision surgery.

Recurrence

Recurrence remains the major long-term problem.

Substantial recurrent disease may develop within 5–10 years, particularly in patients with aggressive disease.

Wound Problems

Skin necrosis, delayed wound healing, hematoma, infection, and scar sensitivity may occur following open fasciectomy, especially in severe or recurrent disease.

Vascular Injury

Digital vessels may be injured during difficult dissection, particularly when longstanding contracture has distorted normal anatomy.

Complex Regional Pain Syndrome

A small proportion of patients may develop disproportionate pain, swelling, stiffness, and autonomic changes consistent with complex regional pain syndrome.

Patient Monitoring

Patients undergoing surgery should initially be followed closely, often weekly during the first postoperative month, to monitor wound healing and identify stiffness, infection, or neurovascular problems.

After healing is established, follow-up may be performed as clinically needed.

Long-term reassessment should document finger extension, MCP and PIP contracture, recurrence of cords, hand function, and the need for additional treatment.