Ask the Expert: When Is the Right Time to Treat Dupuytren's Contracture?
The genetic disorder affects the palmar fascia, the thick layer of tissue found in the palm of the hand. Treatment is dependent on an individual's symptoms and quality of life.
Dupuytren's disease is a disorder that can lead to Dupuytren's contracture over time. The exact cause of Dupuytren's disease, as well as the contracture, is unknown. It is a genetic disorder, however, that is often inherited. There are many risk factors that can contribute to developing Dupuytren's disease and ultimately Dupuytren's contracture.
Dupuytren's disease is a disorder of the palmar fascia.
The palmar fascia is a thick tissue layer in the palm of the hand that protects the muscles, tendons, nerves, arteries, and veins in the hand. This tissue begins to undergo changes that lead to contractures.
The process starts with overstimulation of a pathway that regulates cell growth. Cells called fibroblasts that make up the fascia are responsible for producing proteins like collagen, which give tissues their strength and flexibility.
These cells are activated and change into myofibroblasts. These myofibroblasts produce excess collagen as they are typically activated as a stress response for tissue healing.
In Dupuytren's, however, this excessive collagen formation leads to nodules. Growing nodules form cords, which are thickened bands that can extend into the fingers. These cords pull the fingers into a fixed flexed position most commonly at the knuckles and at the middle joint (proximal interphalangeal joint).
This happens most frequently in the ring and little finger. This 'flexion contracture' can lead to difficulty gripping objects, placing the hand flat, or shaking hands.
What causes Dupuytren's contracture?
The exact cause of Dupuytren's contracture is unknown. It is a genetic disorder that is often inherited. Certain risk factors can contribute to the development of Dupuytren's disease, and, ultimately, Dupuytren's contracture.
Are there increased risk factors?
Research published in 2001 indicates Dupuytren's disease mainly affects people from Scandinavia, Great Britain, Ireland, parts of France, Germany, and the Netherlands.
It also appears to be an inheritable condition. Canadian Robert McFarlane published a preliminary report in 1985 that evaluated the family history of 812 people with DD. Findings showed that 68% were of Northern European ancestry.
Research indicates an association with Ledderhose disease which is a similar phenomenon that occurs in the foot with the plantar fascia and Peyronie's disease which affects a connective tissue layer in the penis.
There is also an association with the use of vibratory tools. For workers exposed to repetitive handling tasks or vibration, the risk of contracting Dupuytren's disease is three times higher. To date there have been no studies to prove that these tasks caused the disease — but there is an association.
Some other risk factors include:
having diabetes
smoking
alcoholism
HIV
vascular disease
How is Dupuytren's contracture diagnosed?
The diagnosis can be made by physical examination. No imaging is needed. The history of a typically painless loss of extension of the fingers is sufficient to make the diagnosis.
The presence of nodules, skin puckering, or cords can also aid in the diagnosis.
Does Dupuytren's contracture need treatment?
Without treatment, disease progression occurs in about 50% of patients. Spontaneous improvement is rare. About 10% to 15% of patients will have no progression. Roughly 70% will have gradual progression over the years leading to contractures.
Rapid progression is rare but can occur when patients present at younger ages. Therapy is palliative as there is no cure for the condition.
When does Dupuytren's contracture need treatment?
Indications for treatment are based on the effects of the disease and on the patient's quality of life. Patients who cannot fully flatten their hand against a table (positive tabletop test) and those with a flexion of about 30° at the knuckle or 15° to 20° at the middle finger joint typically opt for treatment.
What is the importance of early treatment?
Currently, there are no completed studies that prove that early treatment can slow disease progression. However, there is promising research on the effects of anti-tumor necrosis factor therapy. Tumor necrosis factor (TNF) plays a role in increased collagen formation.
Radiotherapy has also been proposed to slow disease progression, though there remains a concern for the potential long-term adverse effects of radiation exposure.
What are the treatment options?
Treatments for Dupuytren's disease include conservative management, collagen injection, and surgery. In fact, there are a number of nonsurgical treatment options to consider.
Mild disease
Observation is appropriate for individuals with painless, stable disease with no impairment in function. Physical therapy and occupational therapy can help maintain range of motion during early stages of the disease.
Those who have mild symptoms from nodules early on in the disease may benefit from modifying the tools that they are using — if applicable. Using gloves with padding across the palm or using pipe insulation around handles might be helpful.
Patients with persistent or progressive symptoms might benefit from glucocorticoid (cortisone) injections if tenderness is present. This can occur in nodules or if the protective layer around the tendon becomes inflamed (tenosynovitis).
For patients with flexion contracture, options include collagenase injection, percutaneous needle aponeurotomy, and open fasciectomy.
Collagenase injection
Collagenase comes from a bacterium called Clostridium histolyticum, which produces an enzyme that breaks down collagen. Collagenase is injected directly into a cord, and the affected digit is manipulated under local anesthesia 24 to 48 hours after the injection.
Night splints are recommended for 6 months after the procedure. The likelihood of full or nearly full correction is higher for patients with less severe contracture (less than 50°) or with early stage disease.
Research indicates collagenase injections have resulted in a 75% contracture reduction. Research shows mixed results regarding recurrence, including a 9% recurrence at 2 years and a 47% recurrence in 5 years.
Percutaneous needle aponeurotomy
Percutaneous needle aponeurotomy (aponeurosis is the palmar fascia, and aponeurotomy involves cutting the palmar aponeurosis) is a procedure where a small needle is inserted through the skin to cut the Dupuytren's cord at multiple points.
The finger is then extended to rupture the weakened cord. This is done in the office like the collagenase injection. A splint is also recommended to maintain correct finger position. There is substantial improvement immediately but up to 65% recurrence within 3 to 5 years.
Older research shows that when the procedure is combined with triamcinolone, patients experienced a significantly greater maintenance of correction of flexion deformity at 6 months compared to aponeurotomy alone.
This procedure is typically reserved for milder contractures.
External beam radiation therapy
External beam radiation therapy can prevent progression and provide symptomatic benefit in patients with mild to moderate flexion deformities.
While no controlled studies have been performed, in one small study, contractures regressed in over 50% of patients at 1 year and stabilized in 37% of patients.
In a long-term follow-up of an average of 13 years of early stage contractures, more than 70% of patients remained stable. Roughly 60% of those in more advanced stages progressed.
Surgical fasciectomy
Surgical fasciectomy is mostly performed for advanced stages of disease. Surgery should be considered only with functional impairment. This procedure can be partial or total.
Partial palmar fasciectomy is the removal of diseased tissue within a finger. This is indicated if there is a flexion deformity of 30° at the knuckle or 20° at the middle joint. The recurrence rate is about 20% to 40% at 5 years.
Total fasciectomy is infrequently performed because it requires resection of all palmar and digital fascia, including nondiseased tissue. This is indicated if cords have formed in the digits or recurrence after a partial surgical procedure. Recurrence risk is lowest for total fasciectomy at 10% to 20% over 5-plus years.
Postoperatively, patients are entered into hand therapy to help maintain range of motion. Therapy and splinting should occur for at least 3 months to prevent contractures.
Benefits are seen after 6 to 8 weeks postoperatively. The postoperative management is thought to account for the majority of the positive surgical outcomes.
What are the most common side effects of treatment?
Steroid injection side effects include:
skin atrophy at the injection site
pain
swelling
tendon rupture
Radiotherapy side effects include:
skin dryness/peeling
redness or irritation
stiffness
hyperpigmentation
There is an extremely low risk of cancer at the site of radiation.
Collagenase injection complications include:
swelling
skin tearing
tendon rupture
bruising
Percutaneous needle aponeurotomy side effects include:
mild local pain
swelling
skin tears
bruising
Surgical fasciectomy complications include:
pain
nerve injury
damage of vessels leading to significant tissue death
infection
swelling
scarring
postoperative flare (pain, swelling, redness, stiffness)
Can Dupuytren's contracture lead to other conditions, such as anxiety and depression?
Dupuytren's contracture can affect quality of life and emotional well-being. Patients with contractures that affect both hands or those with significant hand disability may experience depressive symptoms.
Recommended treatments
Cognitive behavioral therapy is the first-line treatment for depression and anxiety related to chronic disease. It helps to reframe thoughts around the illness and to develop coping strategies.
If depression or anxiety becomes significant enough to also impact daily living, there are medications that can also be recommended for treatment of these symptoms.
Does Dupuytren's contracture treatment address the pain?
Pain is not the most common symptom of Dupuytren's. Discomfort resulting from the stretching of the skin can be relieved by some of the treatment options.
Steroid injections to alleviate pain can be prescribed for patients who have tenosynovitis or painful nodules.
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