DUPUYTRENS CONTRACTURE

DUPUYTRENS CONTRACTURE

Dupuytren contracture results from contracture of the palmar fascia within the hand, possibly resulting in a fixed flexion deformity of the metacarpophalangeal (MCP) joints and the proximal interphalangeal (PIP) joints.

This condition usually affects the fourth and fifth digits (the ring and small fingers).

PATHOPHYSIOLOGY

The palmar fascia within the hand may develop nodular, hypertrophic degeneration of uncertain etiology. The dense nodular fascia may become adherent to the overlying skin as the hypertrophic degeneration progresses, sometimes extending distally into the involved digit.

This anatomic change may result in contracture of the involved fascia, causing fixed flexion of the MCP joint and sometimes of the PIP joint as well. The most commonly affected area is the ulnar aspect of the hand (digits 4 and 5).

Myofibroblasts are the primary cell type in Dupuytren disease.

FREQUENCY

This disorder is relatively common, particularly in older adults.

Some studies have suggested a higher prevalence of Dupuytren contracture among people with diabetes, or among individuals who smoke or abuse alcohol.

RACE

Dupuytren contracture is seen more commonly in patients of northern European descent (hence the term Viking disease).

SEX

This condition affects men more often than women.

AGE

This condition is most often seen in individuals older than 50 years.

CLINICAL

HISTORY

Taking a thorough history from the patient often helps to exclude many other diagnostic considerations.

A patient typically presents with a history of progressive loss of range of motion (ROM) of the affected finger(s), although fibrous contractures may appear elsewhere in the body, such as in the plantar fascia, knuckle pads, and shaft of the penis.

The fourth digit most commonly is involved. The fifth, third, and second fingers are involved in decreasing order of frequency. Specifically, there is a decreased ability to fully extend the MCP joint(s); a decreased ability to fully extend the PIP joint(s) is sometimes noted.

The history may refer to an isolated nodule in this area, initially somewhat tender, which may have hardened and then disappeared. Asking about functional disabilities may elicit a history of certain tasks that the individual can no longer perform, such as grasping objects and typing.

No sensory deficits are reported unless there is a concomitant pathology. The condition is painless in its later stages.

PHYSICAL

A careful physical examination often confirms the diagnosis without the need for further tests.

• Examination reveals a palmar skin nodule, generally within the distal aspect of the palm.

  • The nodularity generally is not tender to palpation.

  • Puckering of the skin above the nodularity may be noted. Overlying skin may be adherent to the fascia, and a fibrous cord can extend into the finger.

• Flexion of the digit is normal for passive and active ROM.

  • Conversely, extension is limited at the MCP and sometimes the PIP joints of the affected digits. This limitation in finger extension occurs when testing passive and active ROM.

• The ring finger (digit 4) is the most commonly involved site, followed by the small finger (digit 5). Other digits may be involved, although less commonly.

• Loss of progressive flexion of the fingers in the resting position from the radial to ulnar side may be noted.

• Although the patient may, because of the contractures, have difficulty grasping objects, strength is normal within the available ROM.

• Sensation is typically normal.

CAUSES

• Etiology includes a dominant genetic inheritance pattern that often involves individuals of northern European descent.

• In some cases, trauma may accelerate or initiate the process.

• There is conflicting literature regarding whether Dupuytren contracture can be seen with increased frequency in patients with a variety of other conditions. One study indicates no significant increase in the occurrence of Dupuytren contracture in patients with diabetes or alcoholism or who engage in smoking.

• Another report, however, notes that Dupuytren contracture is associated with several conditions (eg, alcoholism, diabetes, epilepsy, pulmonary disease) and with smoking.Nonetheless, even if such associations exist, no clear causal relationship has been established in the literature.

DIAGNOSIS

LABORATORY STUDIES

• Generally, no laboratory studies are needed in the diagnosis of Dupuytren contracture.

IMAGING STUDIES

• As a rule, radiography or other imaging studies are unnecessary.

PROCEDURES

• Nonsurgical procedures do not appear to have a role in the diagnosis of this condition.

HISTOLOGIC FINDINGS

Nodular, hypertrophic degeneration of the palmar fascia with adherence to the overlying skin is noted. Pathologically, the contracture consists of proliferating, vascular, fibrous tissue that later develops into mature collagen.

Dermis typically is involved, resulting in fixation to the fascia.

TREATMENT

PHYSICAL THERAPY

Stretching with the application of heat and ultrasonographic waves may be helpful in the early stages of Dupuytren contracture.

The physical therapist also may recommend that the patient wear a custom splint or brace to stretch the fingers further.

ROM exercises should be performed several times a day. If the patient undergoes surgical correction of the contracture, physical therapy often is involved following the procedure.

The postsurgical program consists of wound care, massage, passive stretching, active ROM exercises, and splinting.

OCCUPATIONAL THERAPY

Through a course of occupational therapy, the patient may learn adaptive techniques and begin to use assistive devices that enhance functional abilities.

Example: Adaptive equipment can help a patient to open jars, despite contractures.

SURGICAL INTERVENTION

• A patient with mild disease can be tracked clinically for progression before considering surgical intervention.

• Consider surgical intervention for a patient with the following features:

  • Significant functional disability secondary to this disease

  • Significant MCP contracture (>30 º)

  • Any contracture of the PIP joint

• Surgical interventions consist mostly of fasciectomy. Complete extension of affected joints may be possible with earlier intervention.

• Surgical fasciectomy is the accepted treatment for Dupuytren disease; nonsurgical treatment options are either in their infancy or show little clinical efficacy. However, because surgery does not cure the disease and recurrence rates range from 26-80%, nonsurgical treatment options continue to be explored.

OTHER TREATMENT

• Intralesional corticosteroids injection may be helpful in the early stages of the disease.

• In a clinical trial in which nodules of Dupuytren disease were injected with triamcinolone acetonide, researchers concluded that this compound might modify the progression of the disease.

• Investigation of enzymatic fasciotomy using clostridial collagenase injection has shown potentially encouraging results. A prospective, randomized, double-blind, placebo-controlled trial by Badalamente and Hurst showed collagenase injections to be safe and effective, with a low recurrence rate; the authors proposed this as a viable, nonsurgical treatment option for Dupuytren contracture. Clostridial collagenase is thought to lyse and rupture excessive collagen deposition, which decreases the tensile strength of Dupuytren cords.

• Some researchers have started looking into nonsurgical treatment options for Dupuytren disease, investigating how reported cellular mediators affecting the disease's pathogenesis might be harnessed.

  • Myofibroblasts are the primary cell type in Dupuytren disease; 5-fluorouracil (5-FU) inhibits proliferation and myofibroblast differentiation in Dupuytren cell culture. Thus, 5-FU has a potential use as an adjuvant to reduce the rate of recurrence and contracture.

  • Hyperbaric oxygen is another theoretical therapeutic option. Fibroblast and myofibroblast production may cease if hypoxic conditions are reversed by high tissue oxygenation.

• An in-office percutaneous needle aponeurotomy can be considered as an alternative to surgery.

• A percutaneous needle fasciotomy has been developed by a team of French rheumatologists, with results comparable to traditional surgery.

• It is hoped that further research will elucidate or more firmly establish the role of nonsurgical interventions (eg, injections and other previously mentioned therapies) in the treatment of Dupuytren contracture.

MEDICATION

Because this condition is generally painless, analgesic medications usually are not required. No medications are known to decrease the progression of the contractures.