Wednesday, October 10, 2007

Dupuytren’s Disease

You will have noticed a lump or band in the palm of your hand developing over the past months to years. Some people will have noticed nothing else but some patients will notice that the band extends into one or more fingers and the fingers will not straighten fully. The lump or band is the Dupuytren’s tissue and the bending of the fingers is due to this band getting shorter. (photo credit)

We do not fully understand the nature of this tissue but we do know it is similar to scar tissue. The condition can occur in other parts of the body as well when it is known by other names:

Who gets it?
We know that the commonest reason for getting Dupuytren’s is
  • Genetic – you are born with the tendency to develop the problem. The greater your tendency then the earlier you will develop it and the more severe it will be. However, other things can increase your risk or make the disease worse:
  • Alcohol abuse
  • Smoking
  • Taking medication for epilepsy
  • In other diseases such as Diabetes people develop a type of Dupuytren’s but it tends to be less severe.
How does the disease develop?

The first thing most people notice is the lump (or sometimes a little hole or pit) in the skin of the palm near the base of the ring finger. This lump is usually painless but can be tender to direct pressure.

Months or even years later the lump can develop into a band passing into the finger or fingers. Again months or years later this can start to pull the finger down to the hand so the finger will not fully straighten. This is called a Dupuytren’s contracture. People often notice this when they cannot put their hand down flat or have problems putting their hand in a pocket or putting on gloves.

The disease usually affects both hands but one hand may be much worse. Eventually in the worst cases all the fingers and even the thumb can be affected.

The ability to grip is not affected until the very severe stages when the fingers are pulled so far down that it is impossible to open them to hold things.

Many people find that the condition is a nuisance but does not preclude most normal activities. Some activities you may have problems with include:

  • Holding some tools
  • Holding a ball eg for bowling
  • Getting your hand into small places.
Treatment


Many people with Dupuytren’s do not require surgery. Surgery is offered for three reasons:

  1. There is a significant contracture progressing rapidly which will require major surgery if left for longer.
  2. There is a contracture causing significant problems with your hand function
  3. The lump in the palm is very painful
There are various types of treatment available which your surgeon will discuss with you.

Conservative: This means treatment without operation. There is some evidence that splints will help Dupuytrens and so some people can be given a splint to wear at night time to prevent progression of their problem. However most surgeons believe that the success rate of this treatment is very low and will not suggest it for rapidly advancing disease.

Injections: Patients with painful lumps in the palm can be treated with a steroid injection into the lump. This injection is quite painful and probably has a 50% success rate in the short term. There is no evidence that this prevents progression of the disease into a band.


Surgery:

This is the mainstay of treatment and can be of several types varying in complexity and complications.

Needle Aponeurotomy is a technique that dates back to Dupuytren and his earlier English colleagues Cline and Astley Cooper. The needle technique uses small hypodermic needles to poke into the skin and then transect the fibrous cords of Dupuytren's contracture with a back and forth cutting motion. It is an office technique using local anesthesia and without a need for a surgicenter or anesthesia costs. This technique does not address joint and tendon releases which may be necessary to fully release the contracted proximal interphalangeal joint (PIP or middle joint). (photo credit)

Segmental fasciectomy is the simplest and easiest operation which is usually reserved for disease confined to the palm although it can be used for the fingers. A series of small cuts are made in the palm and the Dupuytren's tissue removed. You will usually have a few stitches and a dressing with a splint. The operation will usually be done as a day case.

Fasciectomy is the standard operation for Dupuytren's Contracture. This operation will usually be done under a general anaesthetic although alternatives are often available. The operation involves making a cut from the palm into the finger to remove as much of the tissue as possible. We will then make the cut into a series of zig-zags to try and reduce recurrence. There will be a lot of stitches in the finger. We will usually do all the affected fingers at the same time. You will have a lot of dressings and a plaster on your hand. (photo credit) Dermofasciectomy is the operation we use for people with very severe or advanced disease and involves removing the skin on the front of the finger and replacing it with a skin graft. We usually take the skin graft from the inner arm or groin region. This is a more major operation and you will normally be in hospital for a couple of days.

Repeat operations are often a mixture of the above operations but may require more surgery to the actual joints in the fingers which adds to the recovery time.

Joint fusion is a last stage operation as is amputation when there is no other way of improving the function of the hand. They are very rare operations.

Postoperative care

Dressings: You will have a big bandage and Plaster of Paris on your hand which will be changed somewhere between 24 hours and 10 days from the time of surgery. You will have to come back to the hospital for the dressings to be changed for up to six weeks. The stitches will usually be removed after approximately 2 weeks. Some wounds and skin grafts need dressings for even longer or require special pressure garments to help the scar to soften.
Splints: Almost every patient will be made a special splint which must be worn at night time for six months.
Physiotherapy: Every patient will be referred to physiotherapy for advice on mobilising the hand. This can be quite painful and may need to be continued for up to six months

Complications

  • Incomplete correction: In some patients it is not possible to fully straighten the finger. This is especially common if the finger has been bent for many years or if the finger is bent at the small joints as well as at the knuckle.
  • Nerve damage: The Dupuytren's tissue is always wrapped around the small nerves in the finger. In a small proportion of patients (5-10%) the nerve is damaged and you will be left with some permanent or temporary loss of feeling at the tip of the finger. In addition a number of patients will find that the finger is painful in cold weather (10%).
  • Recurrence: Dupuytren's is a disease that can come back. However the majority of patients will not require further surgery to an operated finger. Approximately 15% of patients requiring surgery to the little finger will develop a recurrent contracture of the same or worse severity in the first six months after surgery.
  • Infection: A small proportion of patients will develop infection (more common in patients with diabetes) and require further surgery or even amputation.
  • Skin graft failure: 10% of skin grafts can be expected to fail partially or completely and require either prolonged dressings or a further skin graft.
  • Reflex Sympathetic Dystrophy: This is a condition that affects 1 in 2000 patients having hand surgery. The patients develop severe pain, swelling and stiffness in the hand. Despite treatment no patient will have a normal hand after this problem.

Despite this list of complications please remember that the vast majority of patients have an uncomplicated routine operation with a very satisfactory recovery.
Experimental--"Clinical trials are in progress to investigate non-operative therapy for Dupuytren's disease using BioSpecifics injectable Collagenase (Cordase tm). The FDA has accorded "Orphan Drug Status" to this usage and a U.S. patent has been granted. A research grant for continued clinical investigation in this area at Stony Brook Medical Center was awarded to BioSpecifics by the New York State Center for Advanced Technology in Medical Biotechnology. The FDA Office of Orphan Product Development has provided some of the funding for the trials in Stony Brook. A second double blind controlled trial is now underway to further define collagenase's usefulness for this condition."


References

4 comments:

Carolyn said...

Could you send me to the source of the connection between treatment for epilepsy and Dupuytren's Disease? I have developed a lump in the palm of my hand and have been taking Topamax for almost a year for migraine prevention. I'll discuss it with my neurologist at our next appointment, but I'd like to see the research, too.

Thanks.

rlbates said...

Caroly, you may need to go to a medical library to have access to these.


Ross, D. C. Epidemiology of Dupuytren's disease. Hand Clin. 15: 53, 1999.

Hueston, J. T. Overview of etiology and pathology. In J. T. Hueston and R. Tubiana (Eds.), Dupuytren's Disease. Edinburgh: Churchill Livingstone, 1985.

Critchley, E. M., Vakil, S. D., Hayward, H. W., et al. Dupuytren's disease in epilepsy: Result of prolonged administration of anticonvulsants. J. Neurol. Neurosurg. Psychiatry 39: 498, 1976.

Anonymous said...

I believe I have this occuring in my left hand. The fingers are not curling in yet, but I have a lump between bones of little finger and ring finger 2/3 way out the palm. I also have tingly feeling in those two fingers for 1+1/2 wks now. Sound right?

Don

Anonymous said...

I have a severe case of dupuytren's and believe me my hands hurt. It effects nearly all fingers. I am hoping that there will be new treatment soon. I had surgery 10 years or more ago on my right hand and recently I had a flare up and now it is in my left hand. It is distressing. Please continue to post new treatments.

Patti