Dupuytren’s contracture

Baron Dupuytren put his name to this condition in the early part of the 19th century, but it had been recorded long before that. The condition most often starts with a firm knot (nodule) in the skin of the palm. This may stay the same for months or years, or it may progress to the next stage in which cords of fibrous tissue form in the palm and may run into the fingers or thumb, pulling them into a flexed (bent) position. In some patients the finger contracture develops without the condition in the palm. The initial nodule can be painful, since it presses on, or surrounds, the nerves, but this initial pain generally settles and should not be a reason for early surgery.

If the nodules and cords contract (shorten) they may seriously interfere with function, limiting the range of movement of the fingers. There is great variation in the rate of progress, but it is usually possible to distinguish the more aggressive form of the disease early on. The fingers furthest from the thumb are most frequently affected, but any part of the hand including the wrist, can be involved. Sometimes the cords develop from palm to finger, sometimes across the joints within the fingers, and sometimes both, when it produces the most troublesome contracture. In severe cases it can affect other parts of the body, most often the feet. This produces an uncomfortable lump on the sole but only rarely leads to contracture of the toes.

The cause of Dupuytren’s contracture is not fully understood. There is a genetic predisposition, which has been identified, so there may be a family history, and in some cases it appears to come to the patient’s attention after an injury or operation, but it is debatable whether these can be regarded as a sole cause. It may be that such incidents precipitate earlier contracture that was going to occur anyway. Patients with certain other conditions have been found to be more likely to develop Dupuytren’s contracture, but this does not mean that they cause it, nor that people with Dupuytren’s are likely to develop other illnesses. The conditions where there has been found to be an association include diabetes, epilepsy (possibly due to the drugs that are used), and liver disease, possible associated with high alcohol intake. Some people have heard of this association and worry that Dupuytren’s will be taken as an indicator of high alcohol intake, but there is no truth in this. It is accepted that Dupuytren’s disease is inherited, but that other conditions can bring it on earlier, or can be associated with it, but are not the primary cause.

The abnormal tissue develops in the sheet of naturally occurring fibrous tissue that lies beneath the skin of the palm, with extensions into the fingers and thumb. This is called the “palmar fascia”, and has the function of stabilizing the skin of the palm during grasping and gripping, so that it does not slide around like the skin on the back of the hand. Fibres of palmar fascia run in all directions, but the fibres that form cords are longitudinal (in the line of the fingers), and as they have the capacity to shorten, they pull the affected finger(s) into a flexed position. It is important to appreciate that the abnormal tissue does not involve the tendons that bend the fingers, and they can function normally once the contracting bands are removed as long as the joints are still mobile.

Treatment of the early “nodule” phase (without contraction) has not proved very helpful. Some have used steroid injections into the nodule, but without any dramatic effect. Others have advocated Radiotherapy (Xrays) to limit the extent or to slow down progress. Once a contracture has developed to an extent that interferes with function, surgery is accepted as the best option. This stage is reached when the hand cannot be laid flat on a surface and make full contact through palm and fingers (Table Top Test). It is important to seek advice at this time, since neglecting this may lead to secondary fixed contracture of the joints which cannot then be improved by surgery. Surgery cannot cure the condition, but can restore the range of movement. (It folllows that, if the range of movement is not restricted, surgery is not indicated).

Surgery can take many forms depending on the distribution of the cords, and the stage of disease or recurrence. In some patients less invasive methods may be indicated. Needle fasciotomy has gained some popularity because of its simplicity and the lack of a wound to heal, with rapid recovery. However it is important to appreciate its limitations – not all patients are suitable for this treatment. The technique involves nicking one or more tight cords of Dupuytren’s tissue with a needle passed through the skin of the palm. This only works if there is an isolated cord without deep attachments, and this can be identified because the suitable cord lifts up the skin when placed under tension. When such a cord is released, the ends spring apart, and with the correct approach the skin remains intact. Rarely it can be helpful to treat a cord entering the finger in the same way, but the risk of nerve damage is higher, since the nerve is often stuck to, or involved in, the cord. Needle fasciotomy in the palm is most often indicated in the elderly, but can buy some time for younger patients with appropriate distribution of the disease. A few days splintage is advisable to discourage early recurrent contracture.

Open surgical treatment is known as fasciectomy. The aim of this surgery is to remove all abnormal tissue and to restore range of movement. This can usually be done with the patient awake, as a day case with anaesthesia of the arm (the arm is numbed by injection in the armpit). The abnormal tissue is removed taking care to avoid damage to nerves and arteries running into the fingers. This can be very difficult especially when there has been a previous operation, but every effort is made to protect them. Division of a nerve results in loss of feeling on one side of a digit.

It is not always possible to restore full straightening to finger joints, even when all abnormal tissue is removed, because of tight ligaments which cannot always be released. Part of the incision is commonly left open in a crease in the palm; this allows freer movement and avoids painful collection of blood under the skin of the palm. It heals as well as if it had been stitched over a period of some four weeks. After operation the hand is rested in a splint and bandage, and elevation in a sling at all times reduces swelling. The healing wound does require regular dressing changes in the two weeks after operation. Within this time a smaller splint is fitted and the hand mobilized. The splint should be worn at night for up to six months.

Cases of recurrence (when the contracture returns after surgery) are usually technically more challenging, require more extensive surgery sometimes including skin graft, and extended physiotherapy.

Newer treatments are always being investigated and interest is currently focussed on Collagenase, an injectable element which may be able to dissolve the hard cords, in one area. This has just been licensed (February 2011) for use by recognised, trained, Hand Surgeons in appropriate cases.

Most patients regain movement without too much difficulty, although determination and persistence are needed. Once the wound is healed, physiotherapy may be required once a week for up to two months, or sometimes more. A small proportion of patients have difficulty moving, and can have a stiff hand for some weeks or rarely longer. This is not possible to predict. A few patients develop a condition called ‘Dystrophy’ (see under Complex Regional Pain Syndrome), with sweating, stiffness or sensitivity to cold. When this occurs extended treatment including drugs and physiotherapy may be required.

Dupuytren’s contracture is not a fully curable condition. Eventually recurrence is likely in some form, elsewhere in the hand on in the same area, but the correct approach is to maintain function and mobility as far as possible, accepting that further surgery may eventually be necessary.

IN SUMMARY:

1. Dupuytren’s contracture is very variable, producing small nodules in some, or dense cords and contractures in others. It also progresses at variable speed.
2. The condition is inherited and no other factors have been shown directly to cause it (but may trigger it in predisposed individuals).
3. Treatment is indicated only when the contracture limits the range of movement (Table Top Test)
4. The form of treatment varies with each case, depending on suitability, and includes division of the cord using a needle, surgical removal of all affected tissue and, in recurrent cases, removal of skin and replacement with skin graft.
5. Surgery is the most accepted, and longlasting form of treatment though other forms have been, and continue to be, investigated. In some cases, needle aponeurotomy (division of the cord by a needle) or injection of collagenase (which dissolves the cord) may be indicated.
6. The aim of all forms of treatment is to restore range of movement.
7. Surgery must be followed by intensive and specialized physiotherapy and splintage.
8. Recurrence is always possible, and eventually occurs in most hands.

Some Information on types of treatment:

1. Needle aponeurotomy

This is indicated in those with a narrow tight cord which is virtually raising the skin of the palm or finger. It is not suitable in those where the hard tissue is in a patch or mound, spreading in the palm or round the digit. This involves inserting a needle beneath the skin of the palm of finger using a minimum of local anaesthetic, The finger is held tense and the tip of the needles is used to interrupt the cords. In suitable cases the finger extends immediately, released from the restraining cord. The main risk os of damage to the nerves which can be close, or even inside the abnormal tissue. No tissue is removed and the contracture is likely to recur (most will accept a figure of 65% recurrence of contracture at 5 years). Many surgeons will recommend the wearing of a splint for a variable time after this.

2. Collagenase injection

This is a new treatment, developed and available in the USA for some 2 years and, since February 2011 available and licensed to suitable practitioners in the UK. The injectable solution breaks down collagen, the firm, structural tissue forming the cords. Like needle aponeurotomy, a suitable case would have a clearly felt, discrete, preferably raised, cord. This is injected directly, without anaesthetic, into the cord beneath the skin and causes breakdown of the collagen over a short segment, interrupting the restraining tissue. At 48 hours, pressure is put on the finger to extend it straight. Surrounding structures, such as nerves and tendons, also consist of a collagen structure and these are at risk if collagenase is injected in or around them. The main risk is therefore of damage to nerves or tendon. The recurrence rate of contracture is yet to be established.

The indications for needle aponeurotomy and injection of collagenase would appear to be similar (ie there appears to be no significant advantage of one over the other and they are applicable to similar patients) but this is a new product and, as it is used by more practitioners, the characteristics may become clearer.

3. Surgery

This involves opening the skin under anaesthesia and removing all abnormal tissue, identifying and protecting all vital structures. The restraining tissue is removed and the contracture is relieved (the fingers straighten). Clearly this involves much more upheaval in terms of treatment and recovery. Expert physiotherapy after surgery is vital since without this, secondary scarring will give a poor result. There are several types of open operation, ranging from simple removal of segments of cord, to removal of all abnormal tissue and involved skin in cases of recurrence. these severe, recurrent, cases, usually require skin grafting. Each case is considered on its own merits depending on the distribution of fibrosis, the areas of contracture and, importantly, whether this is a new or a recurrent pathology. Surgery offers the best chance of delay in recurrence of contracture, but is clearly a major undertaking by comparison with other methods.