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Dupuytren's disease
ORTHOPAEDICS III: UPPER LIMB
Dupuytren’s disease
careful interpretation is necessary. The causative link to the associations is unknown.
Simon Craxford
Classifications
Peter G Russell
Several classification systems exist for use in Dupuytren’s, the best known is that devised by Tubiana. However, it is not commonly used in routine practice. Most practitioners rely on the measurement of angles using a goniometer. Functional scores and patient-reported outcome measures are also useful in determining the success of interventions.3,4
Abstract Dupuytren’s contracture is a common disease that principally affects those of northern European descent. It causes a progressive flexion contracture in the digits especially affecting the ulna side of the hand. The prevalence of the disease increases with age and is commoner in men than women. The current treatment is an operative correction of the contracture that generally results in good if not complete correction. However operative release is associated with a high complication rate and although they are mainly minor in nature, recovery following surgery can take many weeks. Dupuytren’s disease has a genetic predisposition and as a result recurrence is common following surgery. There is extensive research into the disease to understand the biological basis and to ultimately develop improved treatments that are less invasive and provide a greater disease-free interval.
Anatomy Dupuytren’s is a disease that affects elements of the palmar fascia of the hand. Whether the disease develops from normal palmar fascia or develops down similar tissue planes is unknown. The disease seems to affect certain longitudinal components of the palmar fascia but can also affect some areas where the palmar fascia is not well developed, such as the volar aspect at the base of the finger. The palmar fascia acts as a scaffold to provide soft tissue attachment and support. The palmar skin is intimately related to the palmar fascia, which secures it to provide a stable platform for gripping and dexterity of the hand. It is a complex threedimensional structure in which only some components of the longitudinal and vertical bands seem to be affected by the disease. The term band is given to a normal anatomical structure, whereas the term cord is given to bands that have been pathologically altered by Dupuytren’s. The digital nerves traverse through these fascial structures and are therefore at risk of being displaced from their normal anatomical position by Dupuytren’s disease. The awareness of this displacement is critical to the operating surgeon as damage to the nerves is a risk during treatment.
Keywords Dermofasciectomy; Duputyren’s contracture; fasciectomy; fibromatosis; needle aponeurotomy; palmar fascia
Aetiology and classification The key feature of Dupuytren’s contracture is that there is a genetic predisposition to the disease. There is epidemiological evidence that the disease is very common in northern Europe and populations descended from northern Europeans. The disease has a very high prevalence in Scandinavian, Scottish and Icelandic inhabitants with up to 40% of men aged over 60 having some clinical evidence of Dupuytren’s.1 This has led to speculation of a Viking origin for the disease in the UK; a topic that frequently entertains the patient as to their possible ancestry. In populations without any direct northern European ancestry the disease is rare. The pattern of genetic inheritance is not clear but is certainly not a classical Mendelian picture. Many genetic polymorphisms have been studied including those for TGF-B, ZF9 and even mitochondrial DNA, but no single gene polymorphism can yet explain Dupuytren’s. Dupuytren’s is a complex disease process that has a multifactorial aetiology and there may be other triggers required to develop the disease. Diabetes, smoking, alcohol, epilepsy and liver disease have all been reported as having an association. These historical associations made by early studies have not necessarily been supported by more modern large-scale investigations.2 Many studies are small and have inbuilt bias, so
Diagnosis The diagnosis of Dupuytren’s is primarily clinical, based on the history and examination. The classical history is that of a gradual but progressive pattern of disease. The first manifestation of the disease occurs with the appearance of nodules. These nodules then coalesce, to form cords, which often spread up the finger. These cords then contract producing a fixed flexion deformity typically affecting the ulna aspect of the hand, (Figure 1). The disease may affect all the joints of the finger or just produce a contracture of a single joint. A contracture of the Distal interphalangeal joint can occur, but this is less common. The patient may present at any point in the progression of the disease as each individual has unique requirements for the use of their hands. Those with high functional demands such as a keyboard operator who is having difficultly in extending the finger to type characters on the top row, or the pianist unable to span a full octave may present with very mild contracture. In the early stages the presence of a nodule in the palm may prompt consultation due to concern that the nodule is a malignancy. As a contracture develops, functional problems such as poking the eye while washing their face or problems with the digit becoming hooked on a door handle or the lining of a pocket are encountered. In an image-conscious society cosmesis is becoming an
Simon Craxford BM BS MRCS is a Speciality Trainee in Trauma and Orthopaedics at Royal Derby Hospital, Derby, UK. Conflicts of interest: none declared. Peter G Russell MSc FRCS(Plast) is a Consultant Hand and Plastic Surgeon at the Pulvertaft Hand Centre, Royal Derby Hospital, Derby, UK. Conflicts of interest: none declared.
SURGERY 34:3
139
Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
occasionally these nodules may be painful and injection of steroid5 or excision can be helpful in carefully selected patients. It is difficult to predict from those who present with a nodule how the disease will progress. One study followed up patients who presented only with nodules showed that 8% required surgery after 6 years of follow-up. As the disease progresses there are no hard and fast rules as to when to intervene. The Table Top Test is often quoted as an indication for surgery.6 Once the patient is unable to lay their hand flat on a tabletop then the test is positive. Another benchmark often quoted is a metacarpophalangeal joint (MCPJ) contracture of greater than 30. There is no evidence to support these figures, however there is an association between greater preoperative contracture and a poorer correction of contracture postoperatively.7 The MCPJ is more forgiving in terms of ability to correct a contracture but any fixed contracture of the proximal interphalangeal joint (PIPJ) should be considered for treatment. The patient should certainly not be made to wait until these figures are reached if there is a functional loss. As in all hand surgery the postoperative hand therapy is vital and the patient must be able to take part and invest the time necessary to achieve the optimum result. Many of the treatments for Dupuytren’s result in considerable ‘down time’ from normal activities. Preoperative discussions must highlight this. The average return to work time is directly related to their job and the type of operation.8 This ranges from 3.8 weeks for a clerical worker following a fasciectomy to nearly 12 weeks for a heavy manual worker following a dermofasciectomy. Most patients do not consider themselves back to normal for at least 8e12 weeks.
Figure 1 Preoperative view. Note flexion deformity at the metacarpophalangeal joint and the proximal phalangeal joint. The cord of Dupuytren’s can be seen in the palm.
important issue. Traditionally hand surgery has always been aimed at functional goals but increasingly patients are concerned at the social embarrassment of a contracted hand and problems such as difficulty in shaking hands with Dupuytren’s. As the ability to grip is lost relatively late in the disease process and pinch grip between index and thumb often unaffected, a small but significant stoical group may present very late with several fingers contracted into the palm. Examination findings will be specifically looking for evidence of cords and nodules alongside the presence of any fixed flexion deformity. It is also important to examine the dorsum of the hand for the presence of Garrod’s pads. These are found on the dorsal aspect of the proximal interphalangeal joint. They are subcutaneously nodules but frequently the skin overlying them are hyperkeratotic and the patient may think they are calluses or warts. The presence of Garrod’s pads are said to be a sign of more aggressive Dupuytren’s. Males with radial affected digits at a young age, a strong family history and associated fibromatous conditions such as Ledderhose’s disease and Peyronie’s disease are also at risk of a more aggressive disease. Differential diagnosis of Dupuytren’s includes any other pathology that causes a fixed flexion deformity including: locked trigger finger, previous joint or tendon damage leading to fibrosis, scar contracture of the skin, loose body in the joint, Volkmann’s ischaemic contracture, camptodactyly and an established ulna nerve clawing. In most cases the diagnosis is straightforward, the main challenge in Dupuytren’s disease is choosing the best treatment plan for each patient.
Treatment Currently the mainstay of treatment for Dupuytren’s is surgery. There have been many operations described to correct the flexion contracture. The reason for the many different types of operation described is due to the recurrent nature of the disease. None of the operations will result in a ‘cure’. Choosing the method of treatment for each patient depends on many factors including the surgeon’s familiarity with individual techniques, their personal ethos for treating the disease and the requirements of the patient and their disease. The more radical the operation the more likely it is for postoperative complications and a poor functional result but potentially the benefit of a greater disease-free interval. The trend towards minimally invasive surgery with shorter ‘down time’ has influenced the practice of Dupuytren’s surgery with the advent of needle aponeurotomy and collagenase.
Non-operative treatment for Dupuytren’s
Indications for surgery
Many non-operative techniques and pharmaceuticals have been tried for the treatment of Dupuytren’s. None has really stood up to scrutiny and entered mainstream use.
The indications for surgery are tailored to the individual and the degree of functional impairment the Dupuytren’s is causing. Factors that also need to be considered include: the patient’s age, rate of onset of the disease, the degree of contracture and whether previous surgery has been performed. Dupuytren’s is an incurable condition and the treatment should be aimed at palliation of the symptoms. In the early stages of the disease there is no place for preventative treatment. Reassurance and education is all that is required when a patient presents with nodules. Very
SURGERY 34:3
Collagenase A recent development in the treatment of Dupuytren’s is the use of collagenase.9 Initial results are encouraging and the first commercially approved product is available. The enzyme is produced from Clostridium histolyticum. The enzyme is injected
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Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
into the cord and left to digest it for 24 hours. The digit is then manipulated and the cord is often felt to snap or give way. The advantages of such technique are that it is an outpatient procedure and the patient benefits from rapid recovery. Reported side effects include: injection site pain, swelling, pruritus, skin tears and regional lymph gland enlargement. Perhaps the most concerning complication is that there have been cases of flexor tendon rupture during the development trials, as these too are made of collagen and are at risk of being degraded by the collagenase. As experience in this technique grows and more information on long-term success rates become available it will need to find its place in the treatment rationale of Dupuytren’s.
typically 5/0 interrupted monofilament. Following a straight-line incision the scar vector must be broken up with Z-plasties to prevent a contracture of the skin scar leading to a new cause of a flexor contracture. Once the finger is straightened the volar skin is often taut as the skin has contracted due to the chronicity of the contracture. A Z-plasty will lengthen the scar and recruit a small amount of tissue to help address tightness in the skin. If the initial incision was a zig-zag pattern then Y-V-plasty is a good way of achieving recruitment of extra skin to relieve tension on the finger. The hand is initially bandaged in a bulky dressing for a few days to allow any wound discharge to dry up and the postoperative swelling to have passed its peak. The dressing is then taken down and a light dressing applied to prevent any restriction in movement. Hand therapy is then commenced. It is important for the hand to be mobilized even if the wound has delayed healing. A resting night splint may be used to keep the fingers stretched at night to prevent a scar contracture. The sutures are then removed after between 10 and 14 days.
Percutaneous needle aponeurotomy Percutaneous needle aponeurotomy is the use of a hypodermic needle to section the Dupuytren’s cord. The technique uses a 23-gauge needle mounted on a 2.5-ml syringe filled with 1% lidocaine. The cord is palpated and then marked out to select the best place to target with portals. The needle is inserted through the skin over the cord and a very small bleb of local anaesthetic injected to obtain skin anaesthesia. Only the very tip of the needle has a sharp edge so the cord is cut with multiple sweeps of the needle sectioning just a few strands each pass moving from superficial to deep. It is frequently necessary to use several needle portals to break the cord in multiple places to improve the contracture. Once this is done the needle holes are dressed with simple Elastoplast dressings. The clear advantage of needle aponeurotomy is the minimal postoperative care required and the speed in return to normal activity. The patient also sees the hand therapist to be educated in a simple exercise regime and provided with a static thermoplastic night splint. Most patients are driving within 48 hours of the operation and back to sports such as swimming and golf within a week or two.
Dermofasciectomy Dermofasciectomy is the use of a skin graft to close the wound. It typically involves the removal of a whole block of skin and soft tissue from one mid-axial line to the other in the so-called preaxial amputation of the digit. As in the fasciectomy it is vital to preserve the neurovascular bundles but it is also important to make sure the bed of the wound is suitable for grafting. In particular the flexor sheath needs to remain intact, as exposed tendons do not allow a graft to take. A full-thickness graft is then used to resurface the defect (Figure 4). Skin grafts need to be immobilized to allow them to ‘take’. Most surgeons leave the graft undisturbed for 7e10 days. Once the graft has begun to consolidate then it is possible to go on to mobilize the hand. Loss of small parts of the graft is not uncommon and this can be managed conservatively by dressings until the wound has healed by secondary intention. Complete
Fasciectomy As the name suggests this procedure involves the excision of the fascial cord that is causing the contracture. How much of the diseased cord is removed depends on the preference of the surgeon. A segmental fasciectomy involves the removal of small sections of the Dupuytren’s cord approximately 1 cm areas. It is not as extensive operation as a regional fasciectomy but more invasive than a needle aponeurotomy. A regional or selective fasciectomy remains the commonest operation for Dupuytren’s. It involves the removal of as much of the macroscopically obvious cord as possible. The patient is anaesthetized either with a general anaesthetic or a regional block. The limb is exsanguinated and a tourniquet is inflated to produce a bloodless field. Multiple different skin incisions have been described but commonly a zig-zag incision or a straight-line incision is used. Care should be taken raising the skin flaps; a common complication with fasciectomy is wound-healing problems. Every attention should be paid to careful tissue handling and preservation of viable skin flaps (Figures 2 and 3). Once the flaps are raised and there is good exposure, the position of the neurovascular bundles must be established before further dissection of the cord. The cord is then excised and the contracture is corrected. Closure is performed using fine sutures,
SURGERY 34:3
Figure 2 Intraoperative dissection of cord.
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Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
Figure 3 Dupuytren’s cord excised with correction of the contracture.
loss of a graft is less common and creates a significant reconstructive challenge if bare tendons lie at the bottom of the wound. The advantage of using such a radical operation is that it will import fresh skin for patients with poor-quality skin and also gives a longer disease-free interval.
Salvage procedures A range of salvage procedures is available when the finger has a severe, chronic contracture especially if it has been operated on numerous times. Arthrodesis of the PIPJ is a useful way of regaining some function in a severely contracted finger. The natural shortening that occurs as a result of removing the joint allows the finger in a less flexed position. The final option for salvage, especially for the little finger is amputation. The surgeon often sees this as a failure of treatment but the patient often reports a much more useful hand postoperatively. This is especially the case if the finger suffers from cold intolerance or previous iatrogenic nerve damage. Figure 4 (a) Recurrent Dupuytren’s with surgical scars and dermal involvement. (b) Dermofasciectomy at first dressing change. Initially the graft is delicate. (c) Two months postoperatively. Graft consolidated well and the contracture has been corrected. Note contour defect has filled out.
Complications Complications following Dupuytren’s surgery are known to be high. Studies into complications range widely with figures quoted between 3 and 50%.10 A figure order of 20% will develop some form of complication is not unreasonable. It must be stressed that the majority of these are minor. Complications can be divided into those that occur at the time of the operation, those that appear in the early postoperative recovery phase and those that occur later in the rehabilitation of the patient. Immediate complications include: nerve damage, vascular damage and tendon injury. Early complications include: wound healing problems, haematoma, infection and chronic regional pain syndrome (CRPS). Late problems include: stiffness, decreased range of movement, weak grip, scar sensitivity, cold intolerance, scar contracture and recurrence.
SURGERY 34:3
Recurrence of Dupuytren’s following previous treatment is common. Not all recurrence will reach a stage that requires further treatment and the vast majority will need only one intervention. Recurrent cases are technically more demanding and time consuming. Counselling the patient preoperatively is even more important as their expectations need to be carefully managed.
Summary The treatment of Dupuytren’s disease remains a challenge. Treatment options are varied and evolving. It is important to
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Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
tailor the treatment to each patient and their individual requirements and expectations. Patients must be aware of the limitations of an intervention and in particular the risk of recurrence requiring further procedures. Scientific progress is beginning to reveal the cellular processes involved and will be key to driving development of new treatments. A
4 Zyluk A, Jagielski W. The effect of the severity of the Dupuytren’s contracture on the function of the hand before and after surgery. J Hand Surg Eur Vol 2007; 32: 326e9. 5 Ketchum LD, Donahue TK. The injection of nodules of Dupuytren’s disease with triamcinolone acetonide. J Hand Surg Am 2000; 25: 1157e62. 6 Hueston JT. The table top test. Hand 1982; 14: 100e3. 7 Dias JJ, Braybrooke J. Dupuytren’s contracture: an audit of the outcomes of surgery. J Hand Surg Br 2006; 31: 514e21. 8 Tonkin MA, Burke FD, Varian JP. Dupuytren’s contracture: a comparative study of fasciectomy and dermofasciectomy in one hundred patients. J Hand Surg Br 1984; 9: 156e62. 9 Hurst LC, Badalamente MA, Hentz VR, et al. Injectable collagenase Clostridium histolyticum for Dupuytren’s contracture. N Engl J Med 2009; 361: 968e79. 10 Bulstrode NW, Jemec B, Smith PJ. The complications of Dupuytren’s contracture surgery. J Hand Surg Am 2005; 30: 1021e5.
REFERENCES 1 Lennox IA, Murali SR, Porter R. A study of the repeatability of the diagnosis of Dupuytren’s contracture and its prevalence in the grampian region. J Hand Surg Br 1993; 18: 258e61. 2 Geoghegan JM, Forbes J, Clark DI, Smith C, Hubbard R. Dupuytren’s disease risk factors. J Hand Surg Br 2004; 29: 423e6. 3 Sinha R, Cresswell TR, Mason R, Chakrabarti I. Functional benefit of Dupuytren’s surgery. J Hand Surg Br 2002; 27: 378e81.
SURGERY 34:3
143
Ó 2016 Published by Elsevier Ltd.
Dupuytren’s disease
careful interpretation is necessary. The causative link to the associations is unknown.
Simon Craxford
Classifications
Peter G Russell
Several classification systems exist for use in Dupuytren’s, the best known is that devised by Tubiana. However, it is not commonly used in routine practice. Most practitioners rely on the measurement of angles using a goniometer. Functional scores and patient-reported outcome measures are also useful in determining the success of interventions.3,4
Abstract Dupuytren’s contracture is a common disease that principally affects those of northern European descent. It causes a progressive flexion contracture in the digits especially affecting the ulna side of the hand. The prevalence of the disease increases with age and is commoner in men than women. The current treatment is an operative correction of the contracture that generally results in good if not complete correction. However operative release is associated with a high complication rate and although they are mainly minor in nature, recovery following surgery can take many weeks. Dupuytren’s disease has a genetic predisposition and as a result recurrence is common following surgery. There is extensive research into the disease to understand the biological basis and to ultimately develop improved treatments that are less invasive and provide a greater disease-free interval.
Anatomy Dupuytren’s is a disease that affects elements of the palmar fascia of the hand. Whether the disease develops from normal palmar fascia or develops down similar tissue planes is unknown. The disease seems to affect certain longitudinal components of the palmar fascia but can also affect some areas where the palmar fascia is not well developed, such as the volar aspect at the base of the finger. The palmar fascia acts as a scaffold to provide soft tissue attachment and support. The palmar skin is intimately related to the palmar fascia, which secures it to provide a stable platform for gripping and dexterity of the hand. It is a complex threedimensional structure in which only some components of the longitudinal and vertical bands seem to be affected by the disease. The term band is given to a normal anatomical structure, whereas the term cord is given to bands that have been pathologically altered by Dupuytren’s. The digital nerves traverse through these fascial structures and are therefore at risk of being displaced from their normal anatomical position by Dupuytren’s disease. The awareness of this displacement is critical to the operating surgeon as damage to the nerves is a risk during treatment.
Keywords Dermofasciectomy; Duputyren’s contracture; fasciectomy; fibromatosis; needle aponeurotomy; palmar fascia
Aetiology and classification The key feature of Dupuytren’s contracture is that there is a genetic predisposition to the disease. There is epidemiological evidence that the disease is very common in northern Europe and populations descended from northern Europeans. The disease has a very high prevalence in Scandinavian, Scottish and Icelandic inhabitants with up to 40% of men aged over 60 having some clinical evidence of Dupuytren’s.1 This has led to speculation of a Viking origin for the disease in the UK; a topic that frequently entertains the patient as to their possible ancestry. In populations without any direct northern European ancestry the disease is rare. The pattern of genetic inheritance is not clear but is certainly not a classical Mendelian picture. Many genetic polymorphisms have been studied including those for TGF-B, ZF9 and even mitochondrial DNA, but no single gene polymorphism can yet explain Dupuytren’s. Dupuytren’s is a complex disease process that has a multifactorial aetiology and there may be other triggers required to develop the disease. Diabetes, smoking, alcohol, epilepsy and liver disease have all been reported as having an association. These historical associations made by early studies have not necessarily been supported by more modern large-scale investigations.2 Many studies are small and have inbuilt bias, so
Diagnosis The diagnosis of Dupuytren’s is primarily clinical, based on the history and examination. The classical history is that of a gradual but progressive pattern of disease. The first manifestation of the disease occurs with the appearance of nodules. These nodules then coalesce, to form cords, which often spread up the finger. These cords then contract producing a fixed flexion deformity typically affecting the ulna aspect of the hand, (Figure 1). The disease may affect all the joints of the finger or just produce a contracture of a single joint. A contracture of the Distal interphalangeal joint can occur, but this is less common. The patient may present at any point in the progression of the disease as each individual has unique requirements for the use of their hands. Those with high functional demands such as a keyboard operator who is having difficultly in extending the finger to type characters on the top row, or the pianist unable to span a full octave may present with very mild contracture. In the early stages the presence of a nodule in the palm may prompt consultation due to concern that the nodule is a malignancy. As a contracture develops, functional problems such as poking the eye while washing their face or problems with the digit becoming hooked on a door handle or the lining of a pocket are encountered. In an image-conscious society cosmesis is becoming an
Simon Craxford BM BS MRCS is a Speciality Trainee in Trauma and Orthopaedics at Royal Derby Hospital, Derby, UK. Conflicts of interest: none declared. Peter G Russell MSc FRCS(Plast) is a Consultant Hand and Plastic Surgeon at the Pulvertaft Hand Centre, Royal Derby Hospital, Derby, UK. Conflicts of interest: none declared.
SURGERY 34:3
139
Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
occasionally these nodules may be painful and injection of steroid5 or excision can be helpful in carefully selected patients. It is difficult to predict from those who present with a nodule how the disease will progress. One study followed up patients who presented only with nodules showed that 8% required surgery after 6 years of follow-up. As the disease progresses there are no hard and fast rules as to when to intervene. The Table Top Test is often quoted as an indication for surgery.6 Once the patient is unable to lay their hand flat on a tabletop then the test is positive. Another benchmark often quoted is a metacarpophalangeal joint (MCPJ) contracture of greater than 30. There is no evidence to support these figures, however there is an association between greater preoperative contracture and a poorer correction of contracture postoperatively.7 The MCPJ is more forgiving in terms of ability to correct a contracture but any fixed contracture of the proximal interphalangeal joint (PIPJ) should be considered for treatment. The patient should certainly not be made to wait until these figures are reached if there is a functional loss. As in all hand surgery the postoperative hand therapy is vital and the patient must be able to take part and invest the time necessary to achieve the optimum result. Many of the treatments for Dupuytren’s result in considerable ‘down time’ from normal activities. Preoperative discussions must highlight this. The average return to work time is directly related to their job and the type of operation.8 This ranges from 3.8 weeks for a clerical worker following a fasciectomy to nearly 12 weeks for a heavy manual worker following a dermofasciectomy. Most patients do not consider themselves back to normal for at least 8e12 weeks.
Figure 1 Preoperative view. Note flexion deformity at the metacarpophalangeal joint and the proximal phalangeal joint. The cord of Dupuytren’s can be seen in the palm.
important issue. Traditionally hand surgery has always been aimed at functional goals but increasingly patients are concerned at the social embarrassment of a contracted hand and problems such as difficulty in shaking hands with Dupuytren’s. As the ability to grip is lost relatively late in the disease process and pinch grip between index and thumb often unaffected, a small but significant stoical group may present very late with several fingers contracted into the palm. Examination findings will be specifically looking for evidence of cords and nodules alongside the presence of any fixed flexion deformity. It is also important to examine the dorsum of the hand for the presence of Garrod’s pads. These are found on the dorsal aspect of the proximal interphalangeal joint. They are subcutaneously nodules but frequently the skin overlying them are hyperkeratotic and the patient may think they are calluses or warts. The presence of Garrod’s pads are said to be a sign of more aggressive Dupuytren’s. Males with radial affected digits at a young age, a strong family history and associated fibromatous conditions such as Ledderhose’s disease and Peyronie’s disease are also at risk of a more aggressive disease. Differential diagnosis of Dupuytren’s includes any other pathology that causes a fixed flexion deformity including: locked trigger finger, previous joint or tendon damage leading to fibrosis, scar contracture of the skin, loose body in the joint, Volkmann’s ischaemic contracture, camptodactyly and an established ulna nerve clawing. In most cases the diagnosis is straightforward, the main challenge in Dupuytren’s disease is choosing the best treatment plan for each patient.
Treatment Currently the mainstay of treatment for Dupuytren’s is surgery. There have been many operations described to correct the flexion contracture. The reason for the many different types of operation described is due to the recurrent nature of the disease. None of the operations will result in a ‘cure’. Choosing the method of treatment for each patient depends on many factors including the surgeon’s familiarity with individual techniques, their personal ethos for treating the disease and the requirements of the patient and their disease. The more radical the operation the more likely it is for postoperative complications and a poor functional result but potentially the benefit of a greater disease-free interval. The trend towards minimally invasive surgery with shorter ‘down time’ has influenced the practice of Dupuytren’s surgery with the advent of needle aponeurotomy and collagenase.
Non-operative treatment for Dupuytren’s
Indications for surgery
Many non-operative techniques and pharmaceuticals have been tried for the treatment of Dupuytren’s. None has really stood up to scrutiny and entered mainstream use.
The indications for surgery are tailored to the individual and the degree of functional impairment the Dupuytren’s is causing. Factors that also need to be considered include: the patient’s age, rate of onset of the disease, the degree of contracture and whether previous surgery has been performed. Dupuytren’s is an incurable condition and the treatment should be aimed at palliation of the symptoms. In the early stages of the disease there is no place for preventative treatment. Reassurance and education is all that is required when a patient presents with nodules. Very
SURGERY 34:3
Collagenase A recent development in the treatment of Dupuytren’s is the use of collagenase.9 Initial results are encouraging and the first commercially approved product is available. The enzyme is produced from Clostridium histolyticum. The enzyme is injected
140
Ó 2016 Published by Elsevier Ltd.
ORTHOPAEDICS III: UPPER LIMB
into the cord and left to digest it for 24 hours. The digit is then manipulated and the cord is often felt to snap or give way. The advantages of such technique are that it is an outpatient procedure and the patient benefits from rapid recovery. Reported side effects include: injection site pain, swelling, pruritus, skin tears and regional lymph gland enlargement. Perhaps the most concerning complication is that there have been cases of flexor tendon rupture during the development trials, as these too are made of collagen and are at risk of being degraded by the collagenase. As experience in this technique grows and more information on long-term success rates become available it will need to find its place in the treatment rationale of Dupuytren’s.
typically 5/0 interrupted monofilament. Following a straight-line incision the scar vector must be broken up with Z-plasties to prevent a contracture of the skin scar leading to a new cause of a flexor contracture. Once the finger is straightened the volar skin is often taut as the skin has contracted due to the chronicity of the contracture. A Z-plasty will lengthen the scar and recruit a small amount of tissue to help address tightness in the skin. If the initial incision was a zig-zag pattern then Y-V-plasty is a good way of achieving recruitment of extra skin to relieve tension on the finger. The hand is initially bandaged in a bulky dressing for a few days to allow any wound discharge to dry up and the postoperative swelling to have passed its peak. The dressing is then taken down and a light dressing applied to prevent any restriction in movement. Hand therapy is then commenced. It is important for the hand to be mobilized even if the wound has delayed healing. A resting night splint may be used to keep the fingers stretched at night to prevent a scar contracture. The sutures are then removed after between 10 and 14 days.
Percutaneous needle aponeurotomy Percutaneous needle aponeurotomy is the use of a hypodermic needle to section the Dupuytren’s cord. The technique uses a 23-gauge needle mounted on a 2.5-ml syringe filled with 1% lidocaine. The cord is palpated and then marked out to select the best place to target with portals. The needle is inserted through the skin over the cord and a very small bleb of local anaesthetic injected to obtain skin anaesthesia. Only the very tip of the needle has a sharp edge so the cord is cut with multiple sweeps of the needle sectioning just a few strands each pass moving from superficial to deep. It is frequently necessary to use several needle portals to break the cord in multiple places to improve the contracture. Once this is done the needle holes are dressed with simple Elastoplast dressings. The clear advantage of needle aponeurotomy is the minimal postoperative care required and the speed in return to normal activity. The patient also sees the hand therapist to be educated in a simple exercise regime and provided with a static thermoplastic night splint. Most patients are driving within 48 hours of the operation and back to sports such as swimming and golf within a week or two.
Dermofasciectomy Dermofasciectomy is the use of a skin graft to close the wound. It typically involves the removal of a whole block of skin and soft tissue from one mid-axial line to the other in the so-called preaxial amputation of the digit. As in the fasciectomy it is vital to preserve the neurovascular bundles but it is also important to make sure the bed of the wound is suitable for grafting. In particular the flexor sheath needs to remain intact, as exposed tendons do not allow a graft to take. A full-thickness graft is then used to resurface the defect (Figure 4). Skin grafts need to be immobilized to allow them to ‘take’. Most surgeons leave the graft undisturbed for 7e10 days. Once the graft has begun to consolidate then it is possible to go on to mobilize the hand. Loss of small parts of the graft is not uncommon and this can be managed conservatively by dressings until the wound has healed by secondary intention. Complete
Fasciectomy As the name suggests this procedure involves the excision of the fascial cord that is causing the contracture. How much of the diseased cord is removed depends on the preference of the surgeon. A segmental fasciectomy involves the removal of small sections of the Dupuytren’s cord approximately 1 cm areas. It is not as extensive operation as a regional fasciectomy but more invasive than a needle aponeurotomy. A regional or selective fasciectomy remains the commonest operation for Dupuytren’s. It involves the removal of as much of the macroscopically obvious cord as possible. The patient is anaesthetized either with a general anaesthetic or a regional block. The limb is exsanguinated and a tourniquet is inflated to produce a bloodless field. Multiple different skin incisions have been described but commonly a zig-zag incision or a straight-line incision is used. Care should be taken raising the skin flaps; a common complication with fasciectomy is wound-healing problems. Every attention should be paid to careful tissue handling and preservation of viable skin flaps (Figures 2 and 3). Once the flaps are raised and there is good exposure, the position of the neurovascular bundles must be established before further dissection of the cord. The cord is then excised and the contracture is corrected. Closure is performed using fine sutures,
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Figure 2 Intraoperative dissection of cord.
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Figure 3 Dupuytren’s cord excised with correction of the contracture.
loss of a graft is less common and creates a significant reconstructive challenge if bare tendons lie at the bottom of the wound. The advantage of using such a radical operation is that it will import fresh skin for patients with poor-quality skin and also gives a longer disease-free interval.
Salvage procedures A range of salvage procedures is available when the finger has a severe, chronic contracture especially if it has been operated on numerous times. Arthrodesis of the PIPJ is a useful way of regaining some function in a severely contracted finger. The natural shortening that occurs as a result of removing the joint allows the finger in a less flexed position. The final option for salvage, especially for the little finger is amputation. The surgeon often sees this as a failure of treatment but the patient often reports a much more useful hand postoperatively. This is especially the case if the finger suffers from cold intolerance or previous iatrogenic nerve damage. Figure 4 (a) Recurrent Dupuytren’s with surgical scars and dermal involvement. (b) Dermofasciectomy at first dressing change. Initially the graft is delicate. (c) Two months postoperatively. Graft consolidated well and the contracture has been corrected. Note contour defect has filled out.
Complications Complications following Dupuytren’s surgery are known to be high. Studies into complications range widely with figures quoted between 3 and 50%.10 A figure order of 20% will develop some form of complication is not unreasonable. It must be stressed that the majority of these are minor. Complications can be divided into those that occur at the time of the operation, those that appear in the early postoperative recovery phase and those that occur later in the rehabilitation of the patient. Immediate complications include: nerve damage, vascular damage and tendon injury. Early complications include: wound healing problems, haematoma, infection and chronic regional pain syndrome (CRPS). Late problems include: stiffness, decreased range of movement, weak grip, scar sensitivity, cold intolerance, scar contracture and recurrence.
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Recurrence of Dupuytren’s following previous treatment is common. Not all recurrence will reach a stage that requires further treatment and the vast majority will need only one intervention. Recurrent cases are technically more demanding and time consuming. Counselling the patient preoperatively is even more important as their expectations need to be carefully managed.
Summary The treatment of Dupuytren’s disease remains a challenge. Treatment options are varied and evolving. It is important to
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tailor the treatment to each patient and their individual requirements and expectations. Patients must be aware of the limitations of an intervention and in particular the risk of recurrence requiring further procedures. Scientific progress is beginning to reveal the cellular processes involved and will be key to driving development of new treatments. A
4 Zyluk A, Jagielski W. The effect of the severity of the Dupuytren’s contracture on the function of the hand before and after surgery. J Hand Surg Eur Vol 2007; 32: 326e9. 5 Ketchum LD, Donahue TK. The injection of nodules of Dupuytren’s disease with triamcinolone acetonide. J Hand Surg Am 2000; 25: 1157e62. 6 Hueston JT. The table top test. Hand 1982; 14: 100e3. 7 Dias JJ, Braybrooke J. Dupuytren’s contracture: an audit of the outcomes of surgery. J Hand Surg Br 2006; 31: 514e21. 8 Tonkin MA, Burke FD, Varian JP. Dupuytren’s contracture: a comparative study of fasciectomy and dermofasciectomy in one hundred patients. J Hand Surg Br 1984; 9: 156e62. 9 Hurst LC, Badalamente MA, Hentz VR, et al. Injectable collagenase Clostridium histolyticum for Dupuytren’s contracture. N Engl J Med 2009; 361: 968e79. 10 Bulstrode NW, Jemec B, Smith PJ. The complications of Dupuytren’s contracture surgery. J Hand Surg Am 2005; 30: 1021e5.
REFERENCES 1 Lennox IA, Murali SR, Porter R. A study of the repeatability of the diagnosis of Dupuytren’s contracture and its prevalence in the grampian region. J Hand Surg Br 1993; 18: 258e61. 2 Geoghegan JM, Forbes J, Clark DI, Smith C, Hubbard R. Dupuytren’s disease risk factors. J Hand Surg Br 2004; 29: 423e6. 3 Sinha R, Cresswell TR, Mason R, Chakrabarti I. Functional benefit of Dupuytren’s surgery. J Hand Surg Br 2002; 27: 378e81.
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