Dupuytren's Disease

Dupuytren's Disease

INVITATIONAL LECTURE: AMERICAN SOCIETY OF HAND THERAPISTS NINETEENTH ANNUAL MEETING Dupuytren's Disease Robert McFarlane, MD Professor Emeritus, De...

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INVITATIONAL LECTURE: AMERICAN SOCIETY OF HAND THERAPISTS NINETEENTH ANNUAL MEETING

Dupuytren's Disease

Robert McFarlane, MD

Professor Emeritus, Department of Surgery, Hand and Upper Limb Centre, St. Joseph's Health Ctmtre, London, Ontario, Cartada

ou might recall that a recent issue of the y Journal of Hand Therapy UHT) devoted to

the wrist included a paper by Ken Flowers entitled "The Hand Therapist's Role in Diagnosing Wrist Pain." He discussed the ethical dilemma presented to the therapist who receives a patient with wrist pain but without a diagnosis and has to treat the patient. It occurred to me that this is a rather common situation in other problems of the upper extremity and perhaps also in Dupuytren's disease, in which you might well receive a patient with a lump in the palm of the hand or a flexed finger and be asked to treat this patient without a diagnosis. So I thought it might be appropriate to give you an overview of Dupuytren's disease. Dupuytren's disease is a familial disease. It is supposedly due to a single dominant gene of variable penetrance. This may be so, but today, with the development of molecular genetics, it's not enough to say this; rather, for familial diseases, one should identify the involved gene or genes. Once you do that you can identify the susceptible persons within a family. This technology is available today; in the very near future, the geneticists will be able to treat some of the familial diseases by genetic engineering. Perhaps in your time, not in mine, it will be possible by genetic manipulation to prevent these familial diseases, some of which are Correspondence and reprint requests to Robert M. McFariane, MD, 268 Grosvenor Street, London, Ontario N6A 4L6, Canada.

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fatal, from occurring. This is encouraging, and it is simply a matter of time and money. Another indirect suggestion of a genetic influence of Dupuytren's disease is the fact that the prevalence is much greater in people of northern European extraction. It does occur in most countries probably on the basis of a genetic admixture over the centuries, but it is most common in northern Europe. It may well be that this is on the basis of the development of the Celtic civilization in the area that we now refer to as Europe, where the prevalence of Dupuytren's is very high. During the period from about 1500 B.C. until 50 B.c., several Celtic tribes created a very sophisticated civilization in this area, but eventually they were annihilated by the Romans or by other tribes. Further evidence of the genetic aspect is the fact that in areas of Scotland, Ireland, Wales, and Brittany, where the Celtic culture and language have survived, the prevalence of Dupuytren's is even higher than in Europe overall. Now a few comments about Dupuytren. He was a renowned French surgeon, but beyond that he was considered to be the outstanding surgeon of his time in Europe, which was in the early part of the last century. He died in 1835. He had many surgical achievements beyond describing the contracture that bears his name. For instance, he differentiated congenital dislocation of the hip from traumatic dislocation, described Madelung's deformity before Madelung, and he even described a

FIGURE 1.

Early lesion in Dupuytren's disease (DD).

FIGURE 3.

Two types of contracture.

fracture of the lower end of the tibia-all of this before the advent of x-rays. Dupuytren was not the first to describe the disease, but the eponym is appropriate simply because he was the first to definitely attribute the pathology of the disease to the palmar aponeurosis and its digital extensions rather than involving the underlying flexor tendons. He also described, what was appropriate for the time, a method of treatment. Figure 1 shows the early lesion of Dupuytren's disease in this patient's right hand: i.e., nodules near the distal crease of the palm. You will notice that there is some thickening of the fascia proximal to the nodule, but, distally in the early stages, there is no palpable fascia. When the patient extends the finger there is blanching, indicative of tension in the fascia in this area early on. This is a progressive disease. It is most likely to be bilateral and most likely to be more advanced on one side than the other but not necessarily in the dominant hand. In Figure 2 you see it has progressed to the point where there is palpable fascia distal to the nodule, and now we see some early metacarpophalangeal (MP) joint contracture. We see in Figure 3 two types of contracture; at the proximal interphalangeal (PIP) joint in the small and ring fingers, and also a side to side contracture. This patient is unable to separate the ring and long fingers because of the disease in the natatory ligament, which is a band

FIGURE 2.

FIGURE 4.

Progression of lesion.

Less severe DD in 55-year-old man.

of fascia that extends across the distal palm from one flexor tendon sheath to the next. Occasionally we see a distal interphalangeal (DIP) joint contracture due to Dupuytren's disease. Involvement of the thumb and thumb web is common but it does not usually cause enough contraction to justify treatment. Like other diseases, there is a spectrum of severity in this condition, ranging from those patients who have very severe disease to those who have less severe disease. You might consider this patient (Fig. 4) to be typical in the sense that he is about 55 years of age with a single MP joint contracture. This is very amenable to treatment and likely he will have no further trouble. He would also n;present the less severe patient who has unilateral disease; he has probably reached the age of 60 and has no discernible disease in the other hand. This is usually mild disease and may not require an operation. The prevalence of Dupuytren's in the female is a bit less than in the male, but you and I see far fewer females simply because they have mild disease that very often does not require treatment. When it does require treatment others say that females do badly following operations for Dupuytren's. I do not think that is true. Granted, they have a bit higher prevalence of reflex sympathetic dystrophy (RSD) but it is not high enough to conJanuary-March 1997

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FIGURE 5. Diabetic patient with DD.

demn the female patient to a worse result than the male patient. The typical female patient is over 60 years of age. She has a single PIP joint contracture, which is very often bilateral. These patients do well after operations. Bear in mind that occasionally you will see very severe disease in a female patient as you do in the male. Figure 5 shows the typical appearance of the diabetic patient with Dupuytren's. There are nodules in the palm without contracture, but, what is more important, you will notice that these nodules are in line with the long and ring fingers rather than in line with the ring and the small fingers, which is the common distribution. This is a marker of diabetes. We see these patients not so much for the Dupuytren's as for the fact that many of them have an associated trigger finger. There is another type of contracture associated with the juvenile diabetic. I refer to limited joint mobility or juvenile diabetic cheiroarthropathy that has been drawn to our attention mainly by Rosenbloom, a pediatrician specializing in diabetes. These patients have fixed flexion contractures, mainly at the distal but also at the proximal interphalangeal joints. To my knowledge it is not possible to correct these contractures by therapy or by surgery. As they get older, these patients can develop Dupuytren's in the palm, with contractures, as do other diabetic patients. This is a condition that you should recognize because it is not correctable and you may well have patients sent to you for treatment (Fig. 6). Another condition that may be mistaken for Dupuytren's is camptodactyly. You usually see these patients in their early teens. Occasionally, they will have a tense fascial band on the ulnar side of the small finger which could be misinterpreted as Dupuytren's contracture. It should not be a difficult diagnostic problem for you. The other end of the spectrum has to do with those patients who are said to have an increased diathesis to the disease, or, in genetic terms, an in:' crease in genetic penetrance. I think it is worth your while to identify these people because they do so badly. They have the onset of their Dupuytren's disease well before the age of 40, often in the late 10

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FIGURE 6. Contracture with juvenile diabetes.

FIGURE 7. Severe bilateral disease.

teens or early 20's, certainly before the age of 40. And they have a clear family history. Sometimes it is difficult to obtain a family history because families move around so much, but in these patients close relatives have severe disease that is well known to the patient. These people usually have knuckle pads and very often plantar fibromatosis. In fact the knuckle pads and plantar involvement may occur before any evidence of palmar involvement. These patients have very severe bilateral disease (Fig. 7). Usually more than two digits are involved, and typically the patients have radial side disease. They invariably have recurrence of their disease after operation as well as involvement of other parts of the hand. Now a few comments about non-operative treatment. To my mind there is not much value in the various modalities of therapy. Perhaps a laser of some sort may become effective. Messina in Italy has used skeletal traction to straighten very severely contracted digits, but, once he removes the skeletal traction, the flexion contracture recurs. As far as medication is concerned, nothing to date has been satisfactory, although Ketchum in Kansas City has been injecting nodules with steroids for many years; he feels that this not only slows the progression of the disease but sometimes halts it. Figure 8 shows the hand of a patient with reflex sympathetic dystrophy (RSD). It is quite com-

FIGURE 8.

Nodules in the distal palm.

mon for patients with RSD to develop thickening in the palmar skin with nodular formation. Many people have referred to this as Dupuytren's contracture. You'll see this in patients with fractures of the lower end of the radius as well. It's not Dupuytren's contracture but simply fibrosis. I have had the opportunity to biopsy three patients with RSD. The nodules are scar tissue compared with the histologic appearance of Dupuytren's disease, which is well-organized structure, fibromatosis. As far as surgical treatment is concerned, sometimes people complain of pain in the nodules in their palm, and usually the pain goes away if they are reassured. For some reason knuckle pads can be painful. They respond well to steroid injections. If a patient has about 30 degrees of joint contracture, either at the MP or PIP joint, usually it is a nuisance for them and they will accept some form of surgical intervention. There is a great difference between the results of correcting an MP joint contracture and a PIP joint contracture. At the MP joint, the surgeon should be able to completely correct the contracture, and it should not recur. At the PIP joint, the contrary is true. The surgeon might not be able to completely correct the contracture and recurrent contracture is, unfortunately, likely. So bear that in mind, and don't blame yourself for some of the problems that are inherent at the present time in the treatment of this disease. I do not want to talk about the various modalities of post-operative therapy because I don't know much about them compared with your knowledge, but I draw your attention to the socalled flare reaction. Some people say this is a type of RSD, or it is the beginning of RSD, but I do not believe that is the case. If you look at this hand, you will see that the flare is localized to the area of operation involving the small and the ring fingers (Fig. 9). The patient has no undue swelling of the hand. He has no undue pain. There is no hyperhidrosis or evidence of trophic change. You should not ignore the flare reaction; the patient should be observed and receive appropriate therapy. Under those circumstances, the flare reaction disappears within a very few weeks. I would like to make some comments about splinting. Ideally, every patient who has been op-

FIGURE 9.

FIGURE 10.

Localized flare.

Severe PIP joint contracture.

erated on for Dupuytren's contracture should receive the benefit of a therapist and should have a splint applied, but this is not the real world, as you well know. For practical purposes, a patient with an MP joint contracture does not require splinting. The patient requires observation, but it is not essential to splint MP joints that have been fully corrected. On the contrary, regardless of the degree of correction at the PIP joint, all of these joints should be splinted and of course have appropriate therapy as well. Figure 10 shows a patient with a severe PIP joint contracture corrected at operation and splinted. We like to use a splint that is static and dorsally forearm based. We suggest that the patient wear this splint all of the time for 6 weeks (Fig. 11). During that time the patient can remove the splint for appropriate exercises. After the first 6 weeks, if all is well, the patient will gradually decrease the use of the splint during the day but should wear the splint at night for the next 6 weeks. A patient should be splinted for a good long time-as much as 6 months in some cases. As you know, this is the ideal situation but not the one always followed by the patient. Figure 12 shows the result that we obtained in this patient. It was not very satisfactory, but it is shown because not all of our patients do well. Some of this has to do with compliance. As you well January-March 1997

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FIGURE 11.

FIGURE 13.

Postsurgical splinting.

Open palm procedure and skin grafts.

know, people in this older age group are not the most compliant patients. It is important to discuss the specifics of therapy for the open-palm technique and when skin grafts are used. This patient (Fig. 13) has had an open palm procedure and skin grafts applied at the base of the small and ring fingers. It is not fair to a young therapist or a therapist who is not seeing this type of patient regularly to send this patient without some fore-warning. I have made this mistake, and the therapist tells the patient to come back when the wound is healed! With the open-palm technique you should splint the MP joints in extension at least until the palmar wound heals (Fig. 14). That makes good sense because the wound will heal by contraction, and, if the MP joints are not splinted, they will flex to make it easier for the wound to heal. If the patient also has had a PIP joint corrected, splinting of that joint should continue for a longer period of time, as already mentioned. Evelyn Mackin told me many years ago to use the whirlpool bath for these patients, and this is a blessing for the patient because it's much more comfortable to remove the dressing with the aid of the whirlpool. It's so much more comfortable to debride the wound by the action of the current rather than by picking away at it. The same applies to skin grafts. You need not be frightened about skin grafts. They heal in the 12

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FIGURE 12.

Less-than-satisfactory postsurgical result.

FIGURE 14. MP joints splinted in extension.

FIGURE 15.

Results of whirlpool therapy.

same way as an incised wound. Within 4 to 7 days, if the graft has taken, it is secure enough to be managed in the same way as you would manage a sutured wound. Figure 15 shows an open palm that has been subjected to whirlpool treatment. You can see how neat and clean the hand looks and how clean the wound is. It is granulating nicely with no evidence of infection. A skin graft will be unstable for 5 to 7 days but thereafter can tolerate any type of therapy and splint that an incised wound can tolerate. I perspnally prefer a splint to have a bit of wrist flexion

FIGURE 16.

Splinting after skin graft.

which encourages MP and PIP extension. It does not cause any problem of stiffness of the wrist thereafter (Fig. 16). I should comment on one other surgical procedure because you will be confronted with it: the patient who has some type of soft tissue arthro-

plasty to further release the PIP joint. The two names that are associated with these procedures are Curtis and Watson. There is a recent paper from Boston on this very problem. They report some exceptionally good results. I personally refrain from doing this procedure except in the young patient in the 40's or early 50's with a severe PIP joint contracture. In this patient we were able to get the PIP joint out to about 50 degrees of extension and, with a soft tissue release, gained another 10 degrees. I am content with this degree of flexion contracture as long as it is maintained in the postoperative period. This is not an easy problem to deal with. So in summary I would reiterate my thoughts on splinting. First, the MP joint is not nearly as important to splint as the PIP joint. The reason that these joints contract again is not because of the Dupuytren's fascia but simply due to postoperative scar contracture. You can overcome this to some extent by splinting. The other point is that splinting and therapy are designed to maintain the correction accomplished by the surgeon. The surgeon should not expect the therapist to improve upon this, although very occasionally this does occur.

January - March 1997

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