Dupuytren's Disease

What is Dupuytren's disease?

Dupuytren’s disease is an abnormal thickening of the tissue just beneath the skin known as fascia. This thickening occurs in the palm and can extend into the fingers (see Figure 1). Firm cords and lumps may develop that can cause the fingers to bend into the palm (see Figure 2), in which case it is described as Dupuytren’s contracture. Although the skin may become involved in the process, the deeper structures—such as the tendons—are not directly involved. Occasionally, the disease will cause thickening on top of the finger knuckles (knuckle pads), or nodules or cords within the soles of the feet (plantar fibromatosis).  Dupuytren's Disease is named after a French anatomist and military surgeon Baron Guillaume Dupuytren(1777-1835).

What causes Dupuytren's disease?

The cause of Dupuytren’s disease is unknown but may be associated with certain biochemical factors within the involved fascia. The problem is more common in men over age 40 and in people of northern European descent. There is no proven evidence that hand injuries or specific occupational exposures lead to a higher risk of developing Dupuytren’s disease.

What are the symptoms and signs of Dupuytren's disease?

Symptoms of Dupuytren’s disease usually include lumps and pits within the palm. The lumps are generally firm and adherent to the skin. Thick cords may develop, extending from the palm into one or more fingers, with the ring and little fingers most commonly affected. These cords may be mistaken for tendons, but they actually lie between the skin and the tendons. These cords cause bending or contractures of the fingers. In many cases, both hands are affected, although the degree of involvement may vary.

The initial nodules may produce discomfort that usually resolves, but Dupuytren’s disease is not typically painful. The disease may first be noticed because of difficulty placing the hand flat on an even surface, such as a tabletop (see Figure 3). As the fingers are drawn into the palm, one may notice increasing difficulty with activities such as washing, wearing gloves, shaking hands, and putting hands into pockets.  Progression is unpredictable. Some individuals will have only small lumps or cords while others will develop severely bent fingers. More severe disease often occurs with an earlier age of onset.

What are the treatment options for Dupuytren's disease?

In mild cases especially if hand function is not affected, only observation is needed.

For more severe cases various  techniques are available in order to straighten the finger(s). Your treating surgeon will discuss the method most appropriate for your condition based upon the stage of the disease and the joints involved. The goal of any treatment is to improve finger position and thereby hand function. Despite  treatment the disease process may recur. Before treatment, your doctor should discuss realistic goals and results.

Types of treatment may include Needle Aponeurotomy, Partial or Complete Fasciectomy, and limited release as well as on Collagenase injections or cortisone injection in a nodule.  The rationale behind each treatment depends upon the treating physician and the patient.

Surgical Fasciectomy (Partial or Complete) uses open incisions and the cords and nodules are removed

Incisonal Aponeurotomy or Fasciotomy uses small incisions  or portals and the cords are released or perforated

Needle Aponeurotomy  (NA) or Percutaneous Aponeurotomy (PA) or Percutanoeous Needle Fasciotomy (PNF)

Needle aponeurotomy uses a small gauge needle or a microblade as a cutting device to sever the abnormal cords of tissue in the palm and digits which cause the fingers to flex down.  The tissue is not removed it is essentially perforated or cut in multiple places along the palm  to release the contracture (see figure 3) Incisional aponeurotomy fasciotomy is done in some instances.

Collagenase Injection (Xiaflex)

Collagenase is an enzyme that digest collagen a structural protein in tissues.  Xiafllex is a collagenase derived  from the bacteria Clostridium Histolyticum.  Xiaflex is a mixture of several types of collagenase, titrated to achieve digestion of  tissue or cords that are present in the hands  of those who have Dupuytren's disease. (Figure 4)

Corticosteroid Injection (cortisone shot)

When a steroid or cortisone injection  is given to the palm, in a nodule or small cord  it will often soften the cord. There are studies that state that this may limit progression of the disease While there have been no large scale prospective double blinded studies or dose dependent studies many surgeons now will attempt to inject a nodule or soft cord that is not ready for surgery in an attempt to treat it.

IMPORTANT considerations:

  1. The presence of a lump in the palm does not mean that surgery  or treatment is required or that the disease will progress.
  2. Correction of finger position is best accomplished with milder contractures and contractures that affect the base of the finger. Complete correction sometimes can not be attained, especially of the middle and end joints in the finger. no matter what method is used.
  3. Skin grafts are sometimes required to cover open areas in the fingers if the skin is deficient during open fasciectomy or open removal of cord tissue..
  4. The nerves that provide feeling to the fingertips are often intertwined with the cords and may be affected by any treatment
  5. Splinting and hand therapy are often required after surgery  or other treatment procedures in order to maximize and maintain the improvement in finger position and function.
  6. All treatments for Dupuytren's may involve the risk of tendon, nerve, joint, skin: injury, infection, and stiffness. as well other conditions that may negatively affect the result.

Figure 1: Dupuytrens disease may present as a small lump, pit, or thickened cord in the palm of the hand

Figure 2: In advanced cases, a cord may extend into the finger and bend it into the palm

Figure 3: In Needle Aponeurotomy, a cord may be released or perforated without the need for standard incisions.

Figure 4: Xiaflex is a collagenase, a drug that is injected into a cord  to dissolve a small segment of that cord, to treat the contracture.

These pictures are before and one day after injection  (just after manipulation). Xiaflex  treatment requires that a manipulation take place the next day

*Based on Phase I clinical trials, collagenase injections work better for metacarpophalangeal (MP) joint contractures than for proximal interphalangeal (PIP) joint contractures, and for lower severity contractures than for higher severity contractures.

*Ideally, patients for collagenase injection should have a well-defined, palpable cord, ideally one that is strung away from the flexor tendon system. The worst patient is probably someone who has a small finger IP contracture that’s more than 50 degrees and has been there for 5 or 10 years. Collagenase can only affect the cord itself; it won’t be able to act on the secondary tissues that have changed. *(source; http://www.aaos.org/news/aaosnow/oct10/clinical2.asp)

portions © 2009 American Society for Surgery of the Hand. Developed by the ASSH Public Education Committee

taken modified from ASSH and other sources including AAOS by www.handctr.com

XIAFLEX® is a registered trademark of Auxilium Pharmaceuticals, Inc. 0510-013.c

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