DD is a genetic disorder which gives rise to the development of lumps of scar like tissue in the palm and fingers, causing contracture. The normal hand has a layer of fibrous tissue (fascia) deep to the skin and fat, which has the specialist function of binding the skin to the skeleton in order to allow a stable grip. This fascia can be likened to a 3 dimensional scaffolding system, which is attached to bone, tendon sheath and skin. In DD, the normal architecture of this scaffold is disrupted, and nodules nad cords form along the lines of the fascia.
The disease progresses at a viariable rate in each patient, ranging from minor lumps in the palm, to very severe contractures of the fingers, and inability to open the hand. DD can also be associated with lumps on the knuckles (knuckle pads), disease on the sole of the foot, which also contains the specialist layer of fascia, and disease in the penis in men.
In mild cases of DD which does not impact on function, no treatment is recommended. There are a number of procedures available for established contracture ranging from injection to open surgery, all of which have pros and cons.
This is a recently approved drug which is designed to break down the DD, and in injected directly into the nodule or cord. The finger is manipulated between 1 and 5 days afterwards, to straighten the finger. Advantages – no open surgery is needed, and time off work is minimal. Disadvantages – this procedure is recommended only in certain types of contracture, and has some rare but very serious complications.
This is a surgical procedure performed under local anaethetic, in which the cords are divided under the skin using a needle, allowing the finger to straighten. Advantages – very little time off work, with no significant hand swelling. Disadvantages – has a higher recurrence rate than open operation, with a small risk to the nerve and artery supplying the finger.
Under local or general anaesthesia, the DD cords are divided through skin incisions, usually allowing better correction than with a needle alone. Although this is similar to needle fasciotomy, the wounds from this operation are left to heal without sutures, and take 2-4 weeks to close. Most people can function fairly normally within 10 days of the surgery, with a light dressing in place. Heavy manual work, however, is impracticle with these wounds.
Fascietomy / Dermofasciectomy
These operations are usually done under a general anaesthetic, and involve the surgeon removing all visible diseased tissue plus or minus the overlying skin. In dermofasciectomy, a skin graft is taken from futher up the arm and used to cover the finger. Both operations require significant work postoperatively to mobilise the hand.