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Dr. Wolfe interviewed on Dupuytren’s Contracture Fall 2018

Dupuytren’s Contracture – Symptoms, Diagnosis and Treatment

Dupuytren’s contracture is a genetic disease that affects the hands and fingers. The condition causes the fascia, a layer of tissue underneath the palm of the hand, to thicken. This can cause the fingers of one or both hands to contract toward the palm, making it difficult for even daily activities. It’s one of the most common conditions that hand surgeons treat.

The earliest sign is a skin nodule in the palm that can be mistaken for a callus. It may enlarge and become painful, evoking concern for a tumor. However, the nodule caused by Dupuytren’s disease is always benign. A cancerous growth in the palm of the hand is exceedingly rare.

The disease is named after Guillaume Dupuytren, who is widely recognized as the greatest French surgeon of the 19th century. He described the disease and performed his first surgery to correct the condition in 1831, publishing his results in The Lancet medical journal.

The Cause of Dupuytren’s Disease

Although the precise cause of Dupuytren’s is unknown, those with a family history are at greater risk, although not everyone whose parent had the disease will develop it. There is no indication that it comes from performing a specific job or certain activities. However, a hand injury or even hand surgery may trigger the condition in individuals with a genetic predisposition. It affects more men than women.

Mild versus Aggressive Form of Dupuytren’s

Many cases of Dupuytren’s disease are mild, and the condition may progress very slowly. The small nodule will remain the same for years or even decades, not causing any problems, and many people will never need treatment. In general, Dupuytren’s is not painful unless aggravated by forceful activities that put pressure on the nodule.

The aggressive form of the disease can be debilitating, limiting the ability to perform everyday activities. In severe cases, bands of tissue underneath the skin develop into thick cords that contract the fingers, causing them to curl toward the palm. It most often affects the ring finger and the pinkie, but can affect all fingers, including the thumb. Over time, as the disease progresses, secondary contractures develop in the ligaments and tendons of the fingers, making the condition at least partly irreversible.

In its most severe form, known as “Dupuytren’s diathesis,” the disease affects patients at an early age, involves both hands, most digits, progresses more rapidly and is more likely to recur following treatment.

The physician usually makes the diagnosis based on the appearance of a patient’s hands and fingers and the range of movement. An MRI or other imaging studies are generally not needed.

Nonsurgical Treatment of Dupuytren’s

For patients with an early manifestation of the disease, such as a small lump in their palm but no noticeable symptoms, the best course of action is to be evaluated by a hand specialist, who can make an accurate diagnosis and record a baseline examination. Patients are generally advised to do nothing about the condition other than return to their physician every three to six months to see if the disease has progressed. Many will not develop symptoms for years, if at all.

However, if the disease progresses and starts interfering with hand function, different treatment options are available to slow the progression and improve motion in the affected fingers.

For some patients who are experiencing a painful nodule, a series of three cortisone injections may provide relief and slow the progression of a contracture. The effectiveness of a steroid injection varies among patients.

Another treatment entails the injection of an enzyme known as collagenase into the hand to break up the cord and enable the individual to regain movement in the affected fingers. This treatment is very effective, especially in the earlier stages of the disease. However, about 30 percent of patients may see a recurrence of Dupuytren’s within three years.

Surgical Treatment of Dupuytren’s

For those with progressive contractures, surgery is often the best course of action to restore hand function. Contracted fingers may make daily activities difficult or impossible, including reaching into a pocket, shaking hands, putting on gloves, or even washing one’s face. Using a keyboard is particularly troublesome, both because of the inability to straighten the involved digit and the impaired reach of the small finger. Pianists find the “extra octave” increasingly difficult as the span of their hand narrows progressively.

At HSS, we have strict criteria for recommending surgery. We do not recommend it for patients with early disease that is not interfering with their lives. We do advise patients to have their hand checked periodically, and we recommend a simple “table-top test” that can be done at home to help recognize progression. This is performed by simply placing one’s hand down flat on a surface – if one or more fingers can no longer lie flat on the table, it is time to visit the hand specialist.

Surgery is reserved for those with Dupuytren’s who are experiencing symptoms that impede hand function. The goal is to restore motion in their fingers so they can regain normal use of their hand.

The surgery entails removing excess tissue in the palm of the hand. It is an intricate outpatient procedure that should be performed by an experienced hand surgeon under magnification, as care must be taken to protect the nearby blood vessels, nerves and tendons.

When seeking a diagnosis or treatment, it’s better to see a doctor sooner rather than later. Once a contracture is severe, Dupuytren’s disease is more difficult to correct.

First heralded by a thickened nodule in the palm or a tight band tethering a finger, Dupuytren’s Disease is a common genetic disorder that can progressively impair hand mobility and function. It is more common in men, but women may have a particularly aggressive involvement.

The disease may be staged by its severity and corresponding treatment options. In general, the stages are progressive, and involve fewer individuals in the more advanced stages. Dr. Wolfe has over 26 years of experience in treating hundreds of patients in each stage of the disease using a variety of methods, and, when needed, a combination of methods.

Dr. Wolfe and his colleagues at HSS participated in the first FDA trials of a revolutionary and effective new treatment for Dupuytren’s Disease that releases the contracture without surgery. An enzyme (collagenase) is injected into the affected area in the office and the contracture or “cord’ is broken a day or two later by gentle manipulation. This short and non-surgical procedure is done under a local anesthetic to insure a minimum of discomfort. Patients begin to use their hand within minutes of the procedure. Dr. Wolfe and his colleagues at HSS have refined the technique with hundreds of patients to minimize pain and associated risks of nerve or tendon injury.

Clinical staging system and treatment alternatives

Stage I(nascent):

A solitary firm nodule or subtle thickened cord is identified in the palm. The fingers are not contracted. A nodule is most frequent at the base of the ring finger, and may be uncomfortable or occasionally painful with activities such as weight lifting, hammering, golf or tennis that require a firm grip. A cord differs from a nodule and is recognized in this stage as a firm, linear thickening within the palm that may be mistaken for a tendon. These cords generally run in the direction of the fingers, and may be tight when stretching the hand. In many affected individuals, there may be an additional thin cord crossing the thumb web space in the palm. The condition may appear spontaneously, or may arise after an episode of hand or wrist trauma, or even after hand or wrist surgery. It often progresses very slowly or not at all, but deserves to be measured and watched every 3-6 months for progression.

Treatment: Stage I rarely requires treatment, but an examination by a hand specialist is important to confirm the disease, define its extent and identify risk factors for progression (Table I). Presence of several risk factors makes it more likely that this stage will progress to contracture and may even spread to uninvolved fingers. Solitary nodules may respond to a series of corticosteroid injections performed in the office. Collagenase (Xiaflex) does not generally improve nodules in Dupuytren’s Disease, and may be ineffective at treating minor cords that do not contract the finger. Surgery is not indicated without a contracture.

Table I. Risk factors for progression

Strong family history of Dupuytren’s disease
Onset at a young age
Involvement of both hands
Rapid worsening
“Ectopic” disease (disease in other areas [soles of feet, knuckles, penis])

Stage II (simple):

The nodules have linked to become a thickened and solitary “cord” that stretches across the palm into the finger, causing a tethering or a contracture of the digit (Figure 1). The ring and small fingers are most commonly involved, but any finger can be affected. The condition is not generally painful, but progressive involvement of the MPJ (the set of knuckles closest to the palm) can cause increasing difficulties with daily activities, sports and work. There may be a “daughter cord” branching off the main cord to a neighboring, and usually less severely involved finger. The disease may appear to be contracting the PIP joint (the next-to-last joint on the finger), but in this stage, the PIP joint can always be straightened manually, provided the MP joint is bent towards the palm.

Figure 1A-B. Stage II disease, recognized by an MPJ cord contracting the middle finger but sparing the PIP joint.

Stage III (complex):

The disease has extended across the finger to involve the PIP joint (the next-to-last joint on the finger); or has arisen exclusively as a contracture of the PIP joint and spares the palm. Several digits may be involved to a variable extent. The characteristic finding of this stage is that the PIP joints cannot be straightened in any position of the fingers. Occasionally, even the DIP joint (the last joint, next to the nail) is contracted as well. This stage is complex for several reasons:

A “fixed” contracture of the PIP joint implies the ligaments around that joint have become involved in the disease, and have developed a secondary contracture of their own, and
Collagenase (Xiaflex™) has a higher reoccurrence rate at the PIP joint, may not be effective at overcoming the secondary contracture of the joint, may make any future surgery more difficult, and
The cord may be entwined around the nerves and vessels of the finger, or may lie behind the nerves and vessels, hindering access with either collagenase or surgery.

Figure 2. Stage III disease; note the contracture of the MPJ and PIP joints.

Treatment (stage III): Fixed and severe contractures of the PIP joints (figure 2) are generally treated with open surgery to ensure the most complete correction and the lowest chance of recurrence. Collagenase injections may help to gain some improvement and may provide temporary relief when open surgery is impractical or too risky because of other health conditions. Splinting and hand therapy offer little relief at this stage.

Stage IV (complicated):

The disease involves both the MPJ and PIP joints of several digits of both hands, and the patient may have most or all of the risk factors for progression (Table I). Other examples of complicated cases include recurrence after previous surgery, because it adds a potential incisional contracture, as well as surgical scar around the joints, nerves and vessels. Dupuytren’s disease can also be complicated when it is present with another injury, such as a fracture, laceration or pulley rupture. Occasionally, neglected or late Dupuytren’s disease can present with fixed contractures of the fingers so that the fingers are folded down into the palm and cannot be extended. Severe contractures involving the PIP joints can stretch or rupture the tendons responsible for straightening the finger, upsetting the very fine tendon balance and causing a paradoxical extension deformity of the DIP joint.

Figure 2. Stage III disease; note the contracture of the MPJ and PIP joints.

Figure 3. Stage IV (complicated) disease with severe (90 degree) fixed PIP contracture, previous surgical scarring, MPJ contracture and pulley disruption.

Treatment (stage IV): Customized and frequently combined treatment modalities may be necessary for complicated cases with severe contracture. In addition to open surgery, Dr. Wolfe may occasionally employ an external device that uses rubber bands to gradually stretch the diseased tissue and contracted ligaments (the Digit Widget™) (Figure 4). Skin grafts are employed when the native skin is deficient, severely involved in the disease, or compromised by previous interventions. Collagenase can also be used to initially break the palmar bands in a staged procedure, allowing Dr. Wolfe access to the more complicated PIP joints.

Figure 2. Stage III disease; note the contracture of the MPJ and PIP joints.

Figure 4. Same patient from figure 3 several weeks following release and application of Digit Widget.

Figure 2. Stage III disease; note the contracture of the MPJ and PIP joints.

Figure 5. Combined treatment of complex contractures using open surgery, skin graft and digit widget application for the ring and small fingers

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Dupuytren’s Disease
Collagease (Xiaflex) Injection

Who would think that just a two millimeter variation in the length of the two forearm bones could cause intense pain and impairment with everyday activities? When playing racquet sports, not only is the top spin of a forearm shot excruciating, but simply lifting a gallon of milk from the refrigerator becomes an almost impossible task. The disorder is called Ulnar Impaction Syndrome, and it most commonly affects young and active individuals. Repeated impaction of the longer ulnar bone against the small wrist (or carpal) bones creates cumulative damage to the bones and soft tissues about the distal radio-ulnar joint, (insert image), the triangular fibrocartilage, and the carpal bones.

Recreational and professional athletes can be temporarily sidelined by the recurrent pain and swelling of this condition. Patients may be treated for months with various splints, injections and anti-inflammatory medications. Dr. Wolfe diagnoses the condition with a thorough examination of the wrist and forearm, and confirms the diagnosis with plain radiographs and the pronated grip film of each wrist (insert image). On occasion, an MRI scan (insert image) is helpful to demonstrate involvement of other bones or to rule out other diagnostic possibilities such as DRUJ arthritis, TFCC detachment and radial-ulnar-instability.

While several surgical techniques have been utilized to treat the disorder, Wolfe and his colleagues published successful results of their technique for ulnar shortening osteotomy a decade ago. Their method has a zero incidence of nonunion and excellent success in relieving the pain of ulnar impaction syndrome. They will publish their larger, comparative experience of a technical refinement that further reduces complications and nearly eliminates the need for suture or hardware removal.

Dr. Wolfe treats a wide range of hand conditions. If you do not see yours listed, please call his office to discuss your individual needs. Dr. Wolfe has particular expertise in treating:

Surgical Videos

Scott W. Wolfe, MD performs a demonstration of the volar approach for PIP joint implant arthroplasty on a right hand.