6.7 Dupuytren’s disease

The first observation that contracture of a finger resulted from thickening and shortening of the palmar fascia was recorded in the notebook of Henry Cline Sr of St. Thomas’ Hospital, London in 1777. Guillaume Dupuytren was born in the same year and published a detailed description of the condition that bears his name in 1831. Both Cline and Dupuytren performed palmar fasciotomy. It is remarkable that all of the operations in routine use today, except dermofasciectomy, were described before 1850.

Dupuytren’s disease shows a striking geographic variation in population prevalence, with high prevalence in Norway (30% of men over 60 years) and England (approximately 15% of men over 60 years), and much lower prevalence in the Mediterranean basin. It is very uncommon in Africa and rare in much of Asia.

The prevalence of Dupuytren’s disease is strongly related to age. The onset is earlier in men and the disease is more common in men at every age (Figure 6.7.1).

A genetic influence in Dupuytren’s disease is supported by the geographic variation in prevalence and by clustering of cases in families. Many pedigrees show a pattern consistent with an autosomal dominant model of inheritance with variable penetrance but other cases appear to be sporadic. Investigation of the genetic influence is complicated by the high prevalence, late age of onset, incomplete penetrance, and the possibility that some cases have non-genetic causes. The search for a causative gene has not yet yielded conclusive results.

Smoking and alcohol independently increase the risk of Dupuytren’s contracture.

An association with epilepsy has been suggested by several studies, but a large case–control study has not confirmed it.

Dupuytren’s disease has an increased prevalence in both type I and type II diabetes. The disease is more evenly distributed across the palm and the predilection for the ring and little fingers that is seen in idiopathic cases is less marked.

In rare cases, there seems to be a clear link between a single episode of trauma and the subsequent appearance of Dupuytren’s disease at the same site. However, causal relationships with repeated trauma or types of manual work have not been established, and there is no association with exposure to vibration.

The pathogenesis of Dupuytren’s disease remains elusive. Theories have centred on traumatic, neoplastic, and inflammatory processes. Histologically, the disease has many of the features of wound healing, including proliferation of fibroblasts, presence of myofibroblasts, and synthesis of collagen. The normal palmar and digital fascia is replaced by cords of collagen that are thicker and, in cases of contracture, shorter than the original bands. The collagen has a higher ratio of type III to type I collagen than normal fascia.

Myofibroblasts are a characteristic feature of Dupuytren’s disease. They share the morphological characteristics of both fibroblasts and smooth muscles cells but are probably derived from fibroblasts. Myofibroblasts contain arrays of cytoplasmic actin filaments (stress fibres) that attach to specialized adhesion sites at the cell membrane, through which force could be transmitted to the extracellular matrix. The mechanical environment may influence the differentiation of myofibroblasts. The role of transforming growth factor (TGF)-β1 and other growth factors in this proliferative disorder remains unclear at present.

The concept that Dupuytren’s disease affects normal palmar and digital fascial structures and does not arise in a haphazard manner is fundamental to its safe and effective treatment and confirms the importance of thorough knowledge of the normal fascial anatomy.

The palmar and digital fascias form a three-dimensional array of ligaments that have transverse, longitudinal, and vertical components. The longitudinal fibres anchor the skin and resist shearing forces during gripping. They are connected to the skin and skeleton by vertical fibres and to each other by transverse fibres. The palmar fascial structures are called bands or ligaments in the normal state and cords when affected by Dupuytren’s disease.

Each ray has a pretendinous band of fascia that runs longitudinally from the proximal palm to the base of the digit (Figure 6.7.2) and terminates in three ways: into the skin between distal palmar and proximal digital crease; into the spiral band; and deeply towards the metacarpal neck (the band of Legueu and Juvara) (Figure 6.7.3). The lateral digital sheet of fascia is continuous with transversely orientated layers termed Grayson’s and Cleland’s ligaments (respectively palmar and dorsal to the neurovascular bundle) (Figure 6.7.4).

The pretendinous cord may continue directly into the finger to run into the lateral digital sheet and/or Grayson’s ligament. Alternatively, it may connect only with the spiral cord and thence into Grayson’s ligament, to insert into the base of the middle phalanx or the overlying flexor tendon sheath. The spiral band passes deep to the neurovascular bundle; as it becomes contracted, the spiral cord pulls the bundle in a superficial direction so that it comes to lie immediately under the skin of the distal palm (Figure 6.7.5). The bundle is easily divided if the incision is carried directly down to the cord (Figure 6.7.6). A useful clue is that the skin is not adherent to the cord (since the cord lies deep to the bundle and its surrounding fat); a quantity of fat can be palpated between skin and spiral cord, whereas a pretendinous cord is more centrally placed in the ray and more likely to be adherent to the overlying skin.

Dupuytren’s contracture is much more common in the ring and little fingers than in other digits (Figure 6.7.7).

Knuckle pads are nodules of fibromatosis in the subcutaneous tissues over the dorsal surface of the proximal interphalangeal joints (PIPJs), described by Garrod in 1893 (Figure 6.7.8). Plantar fibromatosis (Lederhose disease) presents as nodules in the plantar fascia that may cause discomfort or interfere with footwear (Figure 6.7.9). Penile fibromatosis (Peyronie’s disease) is associated with Dupuytren’s disease. Ectopic fibromatosis may be associated with severe or aggressive Dupuytren’s disease.

There is no good evidence that splintage prevents or retards the progression of contracture.

Steroid injection of palmar nodules may reduce bulk and local tenderness. It has no reliable effect on contracture.

A randomized controlled trial of enzymatic fasciotomy by injection of collagenase has shown good correction of deformity but complications, recurrence rate and the cost–benefit relationship has not been defined.

The primary aim of operative treatment is to straighten bent digits and the secondary aim is to minimize the risk of recurrence. Surgery cannot cure the disease, which probably extends in a subclinical fashion throughout the hand. The choice of procedure remains as controversial as in Dupuytren’s day and good evidence to support one method of treatment over another is lacking.

Metacarpophalangeal joint (MPJ) contracture is correctable almost regardless of its severity and secondary joint contracture is rare. The indication for surgery is a contracture that is interfering with function of the hand. Hueston’s table-top test is a good guide; if the hand cannot be placed flat on the table and the patient reports limitation of hand function, operation may be considered.

Excision of contracted fascia often fails to restore full extension of the PIPJ because of secondary shortening of other structures that cross the palmar aspect of the joint, especially the check-rein ligaments of the palmar plate and the accessory collateral ligaments. Shortness of skin, involvement of the flexor tendon sheath, and shortening of the flexor muscles also limit extension in some cases.

The secondary contracture is variable and unpredictable, but generally the greater the severity and duration of contracture, the greater the risk of secondary joint contracture.

It might seem, therefore, that early operative treatment is appropriate. But the average residual PIPJ contracture after fasciectomy of the little finger is approximately 25 degrees and the patient is unlikely to feel that the operation was beneficial if the preoperative contracture was less than 30 degrees.

Serial measurements of the contracture with a goniometer at intervals of a few months can be most helpful. Progressive contracture is a good indication for surgery, as both patient and surgeon can see that deterioration is otherwise inevitable and a residual contracture after surgery will be more acceptable.

Involvement of the distal interphalangeal joint (DIPJ) is uncommon but often associated with a contracture of the PIPJ of the little finger by a band that spans both joints.

The risks of surgery should be explained and recorded. Risks specific to fasciectomy include delay in wound healing, incomplete PIPJ correction, temporary or permanent digital nerve impairment, limitation of flexion, recurrence and complex regional pain syndrome (CRPS) type 1.

Brachial plexus block by the axillary route gives excellent anaesthesia and postoperative analgesia. It is rare for patients to require any more than simple oral analgesia thereafter. Pain that requires strong analgesics usually signifies a complication such as tight bandage, haematoma, or infection, and requires inspection of the wound. General anaesthesia is sometimes needed for tourniquet discomfort or restlessness during a lengthy procedure. Intravenous regional anaesthesia gives a less satisfactory operative field and does not provide postoperative analgesia.

Understanding of the operative treatment of Dupuytren’s disease is simplified by considering separately the incision, the management of the diseased fascia, and the mode of closure.

A good incision should provide well-vascularized skin flaps, extensile exposure, and access for identification and preservation of the digital nerves and arteries. In the digit, a midline longitudinal incision gives excellent exposure and generally leaves the most heavily involved skin at the edge of the wound. Conversion to one or more Z-plasties breaks up the linear scar and provides some lengthening. Transfer of Z-plasty flaps is difficult in the stiff flat palmar skin; the author prefers a longitudinal incision in the digit that is extended in zigzag fashion in the palm, closing the digital component with a single large Z-plasty in the proximal segment (Figure 6.7.10).

The Bruner zigzag incision also provides good exposure. However, raising large flaps over a midline longitudinal cord of Dupuytren’s disease may damage the subdermal plexus at the base of the flap, leaving its tip ischaemic. The modified Bruner incision, with shorter flaps, is less prone to this problem (Figure 6.7.10).

Multiple ray disease in the palm is more easily exposed through a transverse distal palmar crease incision that can be extended as necessary into each digit and proximally in the palm. The palmar skin in each web is supplied by perforating vessels and will survive provided that these vessels are preserved.

The contracted fascia may be divided or removed; removal may be segmental, regional, or total.

Percutaneous fasciotomy can be employed for well-defined bowstringing cords that cause contracture at the MPJ but recurrence is common. Under local anaesthesia, a knife is slid between skin and cord and used to separate skin from cord. The knife is then turned through 90 degrees and used to cut the cord from superficial to deep as the cord is held taut by pulling the finger firmly into extension. The wound is dressed and an extension splint is worn at night for a few weeks. Fasciotomy is relatively safe if performed at or proximal to the distal palmar crease, as the digital nerves lie beneath the superficial transverse ligament at that level. In the distal palm and finger, fasciotomy carries a risk of nerve injury because of the variability of the position of the neurovascular bundle.

French rheumatologists developed needle fasciotomy as an alternative to fasciectomy. As with percutaneous fasciotomy, the technique is more effective at the MPJ than at the PIPJ, and recurrences are more common than after fasciectomy. In 100 cases followed for 3.2 years the recurrence rate was 58%. Damage to digital nerves and flexor tendons appears to be less frequent than one might imagine.

Though several short C-shaped incisions, 1-cm segments of contracted fascia are excised. There is no attempt to remove all affected fascia from a digit. Postoperative extension splintage is continued for several months. The concept is that breaking up the longitudinal transmission of force in the fascia encourages the remaining fascia to remodel. Recurrence rates are reported to be similar to those of regional fasciectomy.

Regional fasciectomy is the most widely performed procedure for Dupuytren’s contracture and has a low rate of recurrent MPJ contracture. The digital neurovascular bundles are identified proximally and traced distally (or vice versa), removing diseased fascia that is causing contracture from one or more rays. Safety dictates that the nerves are identified at points where their location is predictable—at the distal palmar crease, over the middle phalanx, and (usually) at the base of the finger. Some surgeons emphasize thorough clearance of diseased fascia from the fingers while others limit the excision to the cord causing contracture. It is not known if the former approach reduces the risk of recurrence. But the difficulty of any subsequent procedure is increased by extensive dissection. The more tissue is left undisturbed around the neurovascular bundle, the easier its dissection will be at the next operation.

The options for handling the wound after dealing with the fascia are:

Zigzag incisions are closed directly. A longitudinal midline incision is closed with one or more Z-plasties, which are cut after fasciectomy is completed as the orientation will depend on the location of thin skin. The author leaves the proximal limb of the palmar wound open, to minimize the risk of haematoma.

In primary Dupuytren’s disease, no skin is missing. The transverse distal palmar can be left open and allowed to heal spontaneously. Skin that has undergone shortening over the contracted fascia will return to its original length as the open wound heals. The open palm technique is not appropriate when there is a true shortage of skin; in this case, a graft is required.

The open palm method is associated with a low incidence of complications. Haematoma cannot occur and the risk of skin flap necrosis due to tension is avoided. The chief disadvantages are the need to wear a dressing for up to 4 weeks and a slight risk of infection of the open wound. The open method is less applicable in the fingers, where the wound interferes with rehabilitation.

Hueston’s concept that recurrence of Dupuytren’s disease is reduced substantially if the overlying skin is excised and replaced with a full-thickness skin graft is supported by many studies. An absolute indication for grafting is lack of viable skin flaps at the completion of fasciectomy. Relative indications include skin shortage, recurrent disease, aggressive primary disease, and primary disease in a young patient. The proximal digital segment is the area most often needing replacement (Figure 6.7.11). As grafting requires an intact flexor tendon sheath, a longitudinal or zigzag incision is used for exposure and the skin is excised only if the tendon sheath has remained intact after completion of the fasciectomy. The graft should extend to the midaxial line on each side, to avoid the risk of contracture of the scar along its edges. The graft may be obtained from the medial surface of the arm, from the antecubital fossa or from the groin crease. Graft take is very reliable if attention is given to precise operative technique and haemostasis.

Release of the secondary capsular contracture of the PIPJ is controversial and data on its effect are limited. Although release of the check-rein ligaments and accessory collateral ligaments can improve a contracture persisting after fasciectomy, the decision to proceed to joint release should balance the likely gain against the significant risk of losing flexion, which is more disabling than loss of extension. Tenotomy of the terminal tendon of the dorsal aponeurosis over the middle phalanx can improve flexion of the distal joint when Dupuytren’s disease results in a boutonnière deformity.

The thumb web is affected in at least 25% of cases undergoing surgery on the ulnar side of the hand but in most older patients the contracture is minimal and does not require operative treatment. However, thumb web contracture may be severe and disabling, especially in individuals showing risk factors for aggressive disease (young age at onset, family history, and ectopic disease). A cord affecting the thenar fascia along the radial border of the thumb may cause contracture at the interphalangeal joint. Contracture of the thumb web may be due to fibres passing from the radial end of superficial transverse ligament of the palm to the thenar fascia, or to involvement of the fascia running in the crest of the web between the thenar fascia and the base of the index finger (Figure 6.7.12), with contracture of the index. Moderate contractures can be treated by regional fasciectomy but in severe cases restoration of the width of the thumb web requires dermofasciectomy and skin grafting.

Non-adherent dressings of paraffin gauze, dry gauze, and wool are held with a non-elastic bandage. Many surgeons feels that a palmar plaster slab from forearm to fingertips helps to control wrist position for the duration of the regional anaesthetic block, relieve pain, and prevent reactionary bleeding, which can occur if the hand is moved excessively in the first day or two. The fingers sit in midflexion; no attempt is made to hold them in extension, so as to avoid tension on skin flaps. Some surgeons inspect the wound the next day. The author leaves the dressings in place for 2–4 days unless a skin graft has been used, in which case the hand is immobilized for 7 days.

Supervision by a hand therapist and diligent exercise by the patient are crucial to achieving consistent results after fasciectomy. The first priority is restoration of flexion by active exercise, with the objective of flexion of the fingertip to the palm within 2 weeks of surgery. A palmar thermoplastic extension splint is worn at night until the scar is mature. Silicone inserts attached to the inner surface of the splint may assist scar remodelling.

A new contracture after operative management can be due to extension (appearance of disease in previously unoperated areas), recurrence (new disease in the previous operation field), scar contracture, or some combination of these. Recurrence is common and exceeds 50% at 10 years, though not all recurrences require further surgery. The usual presentation of recurrence is a contracture of the PIPJ, most frequently in the little finger.

Assessment of recurrent disease should include evaluation of the digital nerves (by two-point discrimination), digital arteries (by digital Allen’s test), severity of contracture, and quality of skin (Box 6.7.5). Digits that lack a patent digital artery are surviving on collateral vessels and are at risk of ischaemia during extensive procedures; they are poor candidates for surgery. Digital nerves may be densely adherent to scarring from previous operations and their preservation may be difficult.

Surgery for recurrent Dupuytren’s disease is more difficult and more risky than primary operations. The indication for operation is a recurrent contracture that is interfering with function of the hand, but the decision is influenced by the condition of the finger. The state of the digital nerves, digital arteries, and skin will give an estimate of the risks of surgery and these can be balanced against the severity of contracture and degree of functional impairment. An additional factor is the pattern of recurrent disease. A well-defined cord lying beneath good skin is favourable. Diffuse thickening with adherence to skin is not.

Recurrent palmar disease is amenable to fasciectomy and is seldom problematic. The difficulties lie in the finger, where the mixture of scar tissue and recurrent disease may greatly hamper the safe dissection of nerves and arteries. It is also important to preserve the tendon sheath, as most cases of recurrence will require skin grafting. If the tendon sheath is deficient, closure with the original skin flaps or by a local flap will be needed.

Dermofasciectomy and full-thickness skin grafting is the procedure used most frequently for recurrent disease (see Figure 6.7.11). The rarity of recurrence beneath a skin graft, demonstrated by Hueston, probably reflects the importance of residual disease in skin flaps as a source of recurrence; when this skin is excised, recurrence is rare. Recurrence can occur, however, at the graft margins.

Skin grafting also corrects the skin shortage that is often evident at the base of the finger in recurrent disease and it allows removal of skin flaps of uncertain viability.

Local skin flaps have limited application in Dupuytren’s disease and are used mainly in surgery for recurrent disease when the flexor tendons are exposed.

Arthrodesis of the PIPJ in 30–40 degrees of flexion can overcome severe recurrent contractures that are not amenable to fasciectomy. Some skeletal shortening is necessary to overcome the flexion deformity. Arthrodesis is most applicable in the little finger, especially when some hyperextension at the MPJ compensates for the fixed PIPJ.

Silicone rubber implant arthroplasty of the PIPJ has been used in combination with fasciectomy and dermofasciectomy. A good arc of motion cannot be expected and the possibility of recurrent deformity remains.

Liberal use of skin grafts in recurrent disease has reduced the need for amputation. Patients who request amputation of the little finger should be warned of the adverse effect on power grip, reminded that a flexion contracture of 60 degrees at the PIPJ is compatible with good function if the MPJ will extend fully and advised about the risk of a painful neuroma. But amputation may be appropriate for a digit that is interfering with function because of persistent contracture after multiple operations. Its dorsal skin may be useful in resurfacing the adjacent digit or palm.

Dissection of the digital neurovascular bundle is the essence of fasciectomy—once the bundle has been protected, excision of the diseased fascia is straightforward. Manipulation of the nerve may impair its function temporarily. Division of a digital nerve should be a very rare event during a primary fasciectomy if appropriate care and magnification are employed. The nerves are at much greater risk at second or subsequent operations because they may be heavily embedded in scar or recurrent disease. A divided digital nerve should probably be repaired, though most patients are at an age where useful recovery is unlikely.

The plentiful cross-connections between the two digital arteries will ensure survival of the finger if one artery is damaged. However, every effort should be made to preserve both arteries because of the likelihood that further operations will be needed. Loss of one artery will restrict the surgeon’s room for manoeuvre at any subsequent procedure and will increase the risk of digital ischaemia. Injury of a single digital artery is not an indication for repair. Repair may be required if the other artery is also damaged. Spasm of the arteries may be caused by extensive dissection or by forcible stretching of the finger into extension. Positioning in flexion, patience, and application of warm saline will usually restore perfusion.

Dupuytren’s contracture causes moderate functional impairment that is proportional to the loss of extension. Complications of surgery can lead to much more severe functional loss, particularly if the range of flexion is compromised. Prevention of complications is crucial to achieving consistently good results.

Small areas of skin loss (<5mm) create a risk of sepsis but will heal spontaneously. Larger areas may need excision and skin grafting. Spontaneous healing of larger areas is slow and likely to result in scar contracture, especially at the base of the finger.

Haematoma is a potentially disastrous complication that may lead to permanent loss of flexion, skin necrosis, or infection. The risk can be minimized by careful attention to haemostasis and by appropriate drainage, either by leaving a portion of the palmar wound open or by inserting a drain.

Maceration of skin in the folds and pits of a severely contracted digit increases the risks of infection. Prophylactic antibiotic therapy should probably be given if any pit or crease is macerated, but antibiotics are not needed routinely. A preliminary palmar fasciotomy under local anaesthesia may allow the skin to be brought into good condition for fasciectomy a short time later. Infection may develop in wounds that have been left open, in which case the response to appropriate antibiotic therapy is usually good.

The combination of diffuse swelling, stiffness and shiny redness of the hand has been termed ‘flare’. Its cause is unknown but it may represent a minor variant of CRPS type 1. Treatment comprises elevation, control of pain and swelling, active exercise, and extension splintage at night. Early recognition and treatment are essential in preventing permanent loss of finger flexion.

This serious complication is rare but can have devastating consequences for function of the hand. The cardinal features are excessive pain, swelling, stiffness and vasomotor instability. Early recognition and treatment are essential (see Chapter 1.11).

Dupuytren’s disease affects at least 10% of older men of Northern European origin. Its cause and predilection for the ulnar side of the hand are unexplained. Operative treatment aims to straighten bent digits, not to cure the disease. Deeper understanding of the nature of the disease will be required to devise means of modifying its behaviour and, in particular, reducing the likelihood of recurrence after surgery.