Dupuytrens disease
Dupuytrens disease
- Details
- Written by: Dr Stuart Myers
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Dupuytren's contracture is a condition of unknown cause in which the palmar fascia or protective tissue lining underneath the skin in the palm and fingers becomes thickened and contracts. As a result, this tissue shortens and may cause puckering of the skin or limit the ability to straighten the finger.
The condition in seldom painful, but if it is, it should he checked. A similar condition may be present on the sole of the foot in some people, and rarely may involve other parts of the body. The condition is:
1. Not cancer
2. Not the finger tendons
3. It is a Progressive condition
4. It is an unpredictable condition in both its natural history & also its response to treatment.
Cause:
1. Predisposition:
- The condition may run in families.
- It is most common in people of Anglo-Saxon origin ( "Viking disease")
- It is very common in Australia affecting 7% of the population over the age of 70.
2. Other factors which can hasten the disease in a predisposed individual
- Diabetes
- Alcohol
- Smoking
- Epilepsy
- Drugs
- Work is not generally regarded as a factor in its development.
The disease is more difficult to treat the further it extends down the finger. Hence palmar and MCP joint involvement is relatively straight forward but PIP & DIP joint involvement is associated with greater risks with surgery. The results of intervention of any kind are worst in the PIP joint of the little finger.( The Devil joint)
Disease Staging:
0 = No deformity
1 = 0 – 45 *
2 = 45 – 90*
3 = 90 – 135*
4 = > 135*
Indications for Treatment:
Treatment is not indicated unless the contracture has caused limitation of finger joint movement. This can easily be tested by what is called the "table top test".
If the hand can be placed completely flat, palm and fingers down on the table, then the test is considered negative and no surgery is indicated. If the fingers or palm cannot be placed completely flat on the tabletop, then the 'tabletop test" in considered positive and some form of treatment may be indicated for the affected finger.
If you have the condition and your "table top test" is currently negative, you should check this yourself from time to time because the condition can change over the months or years. If the "table top test" changes from negative to positive, then you should be re-examined by your doctor. The rate of progression of the condition is very unpredictable.
Occasionally Dupuytren’s Nodules may be painful. These may respond to 3 Cortisone injections into the nodules over a period of 2 - 3 months.
Risk factors that predict aggressive disease and higher recurrence rates include; a
- Early age of onset – < 40 years,
- White race,
- Strong family history,
- Bilateral involvement - ie both hands
- Diabetes
- Other body parts involved:
-Soles feet
-Penis “Peyronie’s disease” More information
-PIP joint knuckles "Garrods Pads”
Management Options:
There is No cure!!!
1. Observation
Approximately 10% of patients get a poor result from Dupuytren’s surgery. Surgery is therefore not entered into lightly.
2. Cortisone injection ( Painful nodules)
(3 injections over 2 months)
3. Percutaneous fasciotomy
- Performed in Hospital
- 5mm incisions ( Fingers or palm)
- Release cords only
- Rapid recovery – 1 - 2 sutures and able to get hand wet in 48hrs
- It results in scar tissue formation so that later treatment may become more difficult
- Progression / Recurrence 50 % in 3 yrs but single dose radiotherapy following this treatment may improve this problem.
- More information on Percutaneous fasciotomy
4. Fasciectomy “Surgery”
- Fasciectomy involves surgically removing the Dupuytren’s tissue.
- Performed in a hospital.
- Extensive incisions & prolonged recovery time.
- Best for 1. Correction of deformity
2. Duration of deformity correction
- Causes extensive scar tissue formation so Subsequent surgery for recurrence of the disease much more difficult
- Progression / Recurrence 50 % in 5 yrs but this rate improves greatly if skin grafts are used.
More information on Fasciectomy.
6. Skin grafting - Prevents recurrence under skin graft but the disease can recur either side of a skin graft
7. Collagenase Injection ( Palm) "Xiaflex"
XIAFLEX IS NO LONGER AVAILABLE IN AUSTRALIA
8. Radiotherapy
- For early & Active disease. Stage 0 disease preferably present for less than 6 - 12 months.
- Long-term results not available yet.
- Delayed progression to surgery from 31% to 7% at 5 yrs
- Potential concerns include:
• Dry skin 25%,
• ???Risk of late cancer
• ???Potential for nerve pain
• ? Potential problems for future surgery although this has not been a problem yet
More information - Radiotherapy for Dupuytrens
Int J Radiat Oncol Biol Phys. 2001 Mar 1;49(3):785-98.
Radiotherapy optimization in early-stage Dupuytren's contracture: first results of a randomized clinical study.
Seegenschmiedt MH et al
More Information - See Section on Radiotherapy treatment for Dupuytren's disease
Complications:
1. Nerve damage:
Sometimes the nerves are surrounded by the Dupuytren’s tissue and they must be very carefully dissected from the abnormal tissue. Occasionally there may be a loss of feeling in the fingers as a result of surgery so close to the nerve. This is usually temporary. Some skin flaps ( Z plasties) have permanent change in sensation.
2. Incomplete correction:
The more bent the finger before treatment, the more difficult it will be to get the finger completely straight with treatment. This is especially true for the middle (P.I.P.) joint of the finger. At the completion of treatment it is far more important to be able to make a full fist than to get your finger fully straight. Sometimes a small amount of residual bend is accepted at surgery rather than risking more scarring in an attempt to get the finger completely straight. If the contracture has been present for a long period of time, then the joint itself may have become stiff and surgery may be necessary within the joint. Sometimes metal pins may be used to hold the joint straight for a short period of time after the operation.
If a full correction has not been achieved at surgery then splints may be required at night to improve the final correction. These splints are made by a hand therapist.
3. Healing problems:
In order to remove as much tissue as possible the skin must be left somewhat thin. Occasionally a flap of skin may die. If this happens, then the wound will heel gradually with dressings or a skin graft might be necessary. It may not be possible to straighten out the finger all the way because the blood vessels have shortened and when the finger is straightened out completely, circulation to the fingertip is cut off. In this case, the finger must be left slightly bent and the additional straightening obtained gradually after surgery, with physiotherapy. Failure of a skin graft to “take” is very rare.
The dark areas around the wound edges seen at 10 days after the surgery are not uncommon. This skin often peals to leave normal skin underneath.
Sometimes the skin of the palm may not be sewn closed, in order to reduce tension which might interfere with blood circulation in the skin. In this situation, the skin in allowed to heal gradually on its own over a period of weeks. The “open palm technique” does not interfere with the therapy after surgery and the scar which results after healing is usually no different from one left by a wound that is sutured.
The purple thickened areas along the line of the scar seen at 4 weeks will flatten with a silicone pad provided by the hand therapist. A skin graft was applied in this case.
4. Stiffness:
After surgery, hand therapy is sometimes necessary to regain the flexibility of the fingers. In approximately 10% of patients the hand will become stiff and swollen and it may take several months to regain the bending ability of the fingers. A night splint is worn for 6 weeks.
5. Progression / Recurrence of Dupuytren’s disease:
Although the surgical removal of diseased palmar fascia tissue for Dupuytren's contracture is usually successful in improving the ability to straighten the fingers, in some people the condition may reoccur in the same area at a later time.
Percutaneous fasciotomy 50% in 3 years.
Open Fasciectomy 50% in 5 years.
This is especially true for people who have had a rapid progression of the condition initially. If the condition reoccurs, it may be possible to remove it again by a similar operation, or it may be necessary to remove the overlying skin as well and replace it with grafted skin. Occasionally surgery in one part of the hand can trigger the disease in another part of the hand.
7. Complex Regional Pain Syndrome ( More information)
For these reasons Dr Myers only recommends surgery when he feels that the benefits of surgery outweigh the risks.
8. Skin shortage after deformity correction
If the finger has been bent for a long time the skin contracts on the front of the finger. The relative skin shortage once the finger deformity has been corrected can be overcome with either local skin flaps ( "Z" Plasty or “V-Y” plasty) or for larger defects with skin grafting.
The advantage of skin grafting is that the Dupuytren’s tissue does NOT reform under a skin graft and so this tissue can act as a “fire break” to the disease. Grafting does slow the rehabilitation process and it is absolutely critical to keep the hand elevated for 2 weeks after the surgery to allow the skin graft to “take”. An ellipse of skin is removed from the inner aspect of the arm. Because the skin is lax here it can be easily closed leaving a straight line scar ( a buried suture is used to close the wound)
LAST UPDATED ON 29 / 5 / 2018
Skin Grafting
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- Written by: Dr Stuart Myers
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Skin Grafting for Dupuytrens
When Dupuytrens cords have been present for some time the skin on the volar ( Palm) side of the finger contracts.
- When the deformity is corrected there is not enough skin to allow the finger to straighten. The solution is either:
- Local skin flaps Z plasties or V-Y plasties.
- Skin grafts
- Leaving the wound open to allow it to heal from underneath
There is plenty of loose / available skin on the inner aspect of the arm. This can be used as a skin graft.
The skin can then be applied to the palm or finger to:
1. Provide skin cover to the hand
2. The skin graft PREVENTS Dupuytrens cords from reforming.
The donor skin is elliptical in shape but the donor site can be closed and a straight line scar results.
In the palm a straight line incision becomes and elliptical defect after the finger deformity is corrected.
This is a case of recurrent Dupuytrens disease.
Excess tissue from the deep surface of the skin graft is removed before the graft is applied to the hand.
After the skin is sutured in the palm “Tie –Over” dressings are sutured over the top. They force the skin onto the underlying tissue. The graft has no blood supply when it is transferred so there is a race going on between the skin graft dying and the blood vessels growing up into the overlying skin. Thankfully the race is usually won and the graft survives.
If a blood clot or infection develops under the graft then this process is delayed and the graft may die. It is therefore absolutely critical for the hand to be kept ELEVATED to prevent bleeding. Drains are also inserted at the end of the procedure to remove any ongoing bleeding. The drains are usually removed within 24 hours.
The procedure is documented on a diagram so that the Hand therapist is informed of the degree of correction obtained at Surgery. Here there was a residual 10 * deformity at the little finger PIP joint that was accepted.
By 6 months after the surgery the scars have lightened and softened. Early on it is very common for the scars to go through a purple ( reactive) phase.