In this condition there is a slowly progressive thickening and contracture of the fascia of the palm and hand. The fascia is a layer of tissue below the skin but above the tendons in the hand. The condition is entirely benign (i.e. not cancerous) but can make many day-to-day activities difficult.
The cause for this condition is unknown but there is sometimes a family history and it is more common in more Northern countries, such as Scandinavia. Heavy smoking, heavy alcohol consumption, injury or physically demanding work and diabetes can also increase the chances of developing this condition. It is also more more common in men. Often no cause is identified.
The thickening of the tissue causes a firm, lumpy texture in the hand and the contracture pulls the fingers toward the palm so limiting their ability to straighten out. Patients with condition do not have difficulty making a full fist, ie bending the fingers is not limited.
There can be a little discomfort in the palm in the early stages of the disease but largely this is a painless condition. The ring and little fingers are most commonly affected but all the digits and even the soles of the feet can exhibit the condition.
Patients with this condition manage without surgery but the general trend is for the contracture of the fingers to gradually worsen over several years.
Often the condition is obvious and requires no further investigation. Occasionally other causes for the swelling are suspected and further imaging is sought, such as an ultrasound or MR (magnetic resonance) scan.
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Stretches and splints make no difference to this condition but the rate of progress varies from person-to-person. Often the deformity is accommodated and surgery is not required.
The most common treatment is with an operation called a fasciectomy. This involves lifting up the skin over the lumps and carefully removing all the abnormal or thickened tissue from the nearby tendons, nerves and blood vessels.
This needs to be a slow and careful process and is usually performed under local anaesthetic around the main nerves at the top of the arm (regional block). A general anaesthetic may also be used.
On average approximately 50% of patients will have some sort of recurrence of their disease within 8 years of the surgery, but only about half of those patients will require further surgery.
A dermofasciectomy is similar to a fasciectomy but the skin over the affected finger is also removed and replaced with a skin graft taken from elsewhere in the arm. This is thought to decrease the risk of the disease returning in certain situations and can be necessary if several operations have already been carried out and the skin is too damaged or thin to heal properly.
In some cases significant improvement in the position and function of the hands can be achieved by just dividing the thick cord in the palm, and sometimes the fingers, in one or two places only. This is called a fasciotomy. This can be done under local anaesthetic in the clinic. Either a sharp needle or an injection with an enzyme to dissolve the cord can be used to do this. There is a higher rate of recurrence of the contracture with this approach but the recovery time is much quicker, with fewer complications.
The recovery from a fasciotomy is much quicker than for a fasciectomy or dermofasciectomy. For a fasciotomy you will have a small dressing and bandage and be encouraged to start stretches immediately. A hand therapy appointment will be made for further advice and a night splint to be made.
Following a fasciectomy or dermofasciectomy the hand is dressed in a bandage containing a plaster to keep the affected fingers straight. The unaffected fingers are kept free to be used. You will need to keep the arm elevated for most of the first two weeks to reduce swelling.
Between one and two weeks after the operation, a hand therapist will check the wounds and make a custom made, removable splint to support the affected fingers in a straight position. This is to be worn at night with exercises to start in the day to regain movement of the fingers which have become stiff after the surgery.
Once the wounds are healed, you will be encouraged to massage the scar tissue to soften it. The stitches are removed 2 weeks after the operation. It usually takes about six weeks to feel like the hand is getting back to normal but light activities are encouraged from a very early stage. The night splint is worn for 6 months after the surgery to keep the scar tissue stretched as it matures.