Dupuytren’s Contracture

 

Dupuytren’s contracture is a condition in which one or more fingers become permanently bent in a flexed position. It usually begins as small hard nodules just under the skin of the palm. It then worsens over time until the fingers can no longer be straightened. While typically not painful some aching or itching may be present. The ring finger followed by the little and middle fingers are most commonly affected. It can interfere with preparing food, writing, and other activities.

The cause is unknown. Risk factors include family history, alcoholism, smoking, thyroid problems, liver disease, diabetes, previous hand trauma, and epilepsy. The underlying mechanism involves the formation of abnormal connective tissue within the palmar fascia. Diagnosis is usually based on symptoms.

Initial treatment is typically with steroid injections into the affected area and physical therapy. Among those who worsen, clostridial collagenase injections or surgery may be tried. While radiation therapy is used to treat this condition, the evidence for this use is poor. The condition may recur despite treatment.

Dupuytren’s most often occurs in males over the age of 50. It mostly affects white people and is rare among Asians and Africans. In the United States about 5% of people are affected at some point in time, while in Norway about 30% of men over 60 years old have the condition. In the United Kingdom, about 20% of people over 65 have some form of the disease. It is named after Guillaume Dupuytren, who first described the underlying mechanism in 1833.

Signs and Symptoms
Typically, Dupuytren’s contracture first presents as a thickening or nodule in the palm, which initially can be with or without pain. Later in the disease process, there is painless increasing loss of range of motion of the affected fingers. The earliest sign of a contracture is a triangular “puckering” of the skin of the palm as it passes over the flexor tendon just before the flexor crease of the finger, at the metacarpophalangeal (MCP) joint. Generally, the cords or contractures are painless, but, rarely, tenosynovitis can occur and produce pain. The most common finger to be affected is the ring finger; the thumb and index finger are much less often affected. The disease begins in the palm and moves towards the fingers, with the metacarpophalangeal (MCP) joints affected before the proximal interphalangeal (PIP) joints.

In Dupuytren’s contracture, the palmar fascia within the hand becomes abnormally thick, which can cause the fingers to curl and can impair finger function. The main function of the palmar fascia is to increase grip strength; thus, over time, Dupuytren’s contracture decreases a person’s ability to hold objects. People may report pain, aching and itching with the contractions. Normally, the palmar fascia consists of collagen type I, but in Dupuytren sufferers, the collagen changes to collagen type III, which is significantly thicker than collagen type I.

Related conditions
People with severe involvement often show lumps on the back of their finger joints (called “Garrod’s pads”, “knuckle pads”, or “dorsal Dupuytren nodules”) and lumps in the arch of the feet (plantar fibromatosis or Ledderhose disease). In severe cases, the area where the palm meets the wrist may develop lumps. Severe Dupuytren disease may also be associated with frozen shoulder (adhesive capsulitis of shoulder), Peyronie’s disease of the penis, increased risk of several types of cancer, and risk of early death, but more research is needed to clarify these relationships.

Risk factors
Dupuytren’s contracture is a non-specific affliction but primarily affects:

Non-modifiable
People of Scandinavian or Northern European ancestry, it has been called the “Viking disease”, though it is also widespread in some Mediterranean countries (e.g., Spain and Bosnia) Dupuytren’s is unusual among ethnic groups such as Chinese and Africans.
Men rather than women; men are more likely to develop the condition)
People over the age of 50; the likelihood of getting Dupuytren’s disease increases with age
People with a family history (60% to 70% of those afflicted have a genetic predisposition to Dupuytren’s contracture)
Modifiable
Smokers, especially those who smoke 25 cigarettes or more a day
Thinner people (i.e., those with a lower-than-average body mass index)
Manual workers
Alcoholics
Other conditions
People with a higher-than-average fasting blood glucose level
People with previous hand injury
People with Ledderhose disease (plantar fibromatosis)
People with epilepsy (possibly due to anti-convulsive medication)
People with diabetes mellitus
People with HIV
In one study, those with stage 2 of the disease were found to have a slightly increased risk of mortality, especially from cancer.

Treatment
Treatment is indicated when the so-called table top test is positive. With this test, the person places their hand on a table. If the hand lies completely flat on the table, the test is considered negative. If the hand cannot be placed completely flat on the table, leaving a space between the table and a part of the hand as big as the diameter of a ballpoint pen, the test is considered positive and surgery or other treatment may be indicated. Additionally, finger joints may become fixed and rigid.

Treatment involves one or more different types of treatment with some hands needing repeated treatment.

The main categories listed by the International Dupuytren Society in order of stage of disease are radiation therapy, needle aponeurotomy (NA), collagenase injection and hand surgery.

Needle aponeurotomy is most effective for Stages I and II, covering 6–90 degrees of deformation of the finger. However, it is also used at other stages.

Collagenase injection is likewise most effective for Stages I and II. However, it is also used at other stages.

Hand surgery is effective at Stage I – Stage IV.

Surgery
On June 12, 1831, Dupuytren performed a surgical procedure on a person with contracture of the 4th and 5th digits who had been previously told by other surgeons that the only remedy was cutting the flexor tendons. He described the condition and the operation in The Lancet in 1834 after presenting it in 1833 and posthumously in 1836 in a French publication by Hôtel-Dieu de Paris. The procedure he described was a minimally invasive needle procedure.

Because of high recurrence rates, new surgical techniques were introduced, such as fasciectomy and then dermofasciectomy. Most of the diseased tissue is removed with these procedures. Recurrence rates are high. For some individuals, the partial insertion of “K wires” into either the DIP or PIP joint of the affected digit for a period of a least 21 days to fuse the joint is the only way to halt the disease’s progress. After removal of the wires, the joint is fixed into flexion, which is considered preferable to fusion at extension.

In extreme cases, amputation of fingers may be needed for severe or recurrent cases or after surgical complications.

Prognosis
Dupuytren’s disease has a high recurrence rate, especially when a person has so called Dupuytren’s diathesis. The term diathesis relates to certain features of Dupuytren’s disease and indicates an aggressive course of disease.

The presence of all new Dupuytren’s diathesis factors increases the risk of recurrent Dupuytren’s disease by 71% compared with a baseline risk of 23% in people lacking the factors. In another study the prognostic value of diathesis was evaluated. They concluded that presence of diathesis can predict recurrence and extension. A scoring system was made to evaluate the risk of recurrence and extension evaluating the following values: bilateral hand involvement, little finger surgery, early onset of disease, plantar fibrosis, knuckle pads and radial side involvement.

Minimally invasive therapies may precede higher recurrence rates. Recurrence lacks a consensus definition. Furthermore, different standards and measurements follow from the various definitions.

Postoperative care
Postoperative care involves hand therapy and splinting. Hand therapy is prescribed to optimize post-surgical function and to prevent joint stiffness.

Besides hand therapy, many surgeons advise the use of static or dynamic splints after surgery to maintain finger mobility. The splint is used to provide prolonged stretch to the healing tissues and prevent flexion contractures. Although splinting is a widely used post-operative intervention, evidence of its effectiveness is limited, leading to variation in splinting approaches. Most surgeons use clinical experience to decide whether to splint. Cited advantages include maintenance of finger extension and prevention of new flexion contractures. Cited disadvantages include joint stiffness, prolonged pain, discomfort, subsequently reduced function and edema.

A third approach emphasizes early self-exercise and stretching.

Notable cases
Bill Frindall, who had a finger amputated.
Margaret Thatcher
Misha Dichter
Prince Joachim of Denmark
Ronald Reagan
Bill Nighy

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