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Dupuytren's contracture

Dupuytren's disease

Introduction

Dupuytren's contracture (also known as morbus Dupuytren, Dupuytren's disease, or palmar fibromatosis ) is a fixed flexion contracture of the hand where the fingers bend towards the palm and ca nnot be fully extended (straightened).It is a inherited proliferative connective tissue disorder which involves the palmar fascia (interne link) of the hand. Page text.(5) It is named after Baron Guillaume Dupuytren, the surgeon who described an operation to correct the affliction in the Lancet. The ring finger and little finger are the fingers that are most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are nearly always spared. Dupuytren's contracture progresses slowly and is usually painless. In patients with this condition, the palmar fascia thickens and shortens so that the tendons connected to the fingers cannot move freely. The palmar fascia becomes hyperplastic and contracts. Incidence increases after the age of 40; at this age, men are affected more often than women. After the age of 80, the gender distribution is about even.

Dupuytren can only be treated symptomatically, therefore sometimes patients need to be reoperated when the disease returns. There are many different options to treat Dupuytren’s disease. Mainly they can be distinguish into two groups: minimal invasive techniques en invasive techniques. Minimal invasive techniques are Percutaneous Needle Fasiciotomy, Extensive Percutaneous Aponeurotomy and Lipografting, Collagenase and adiation therapy. Invasive techniques are limited fasciectomy, dermofasciectomy and segmental fasciectomy with and without cellulose. The surgeon decides what is the best possible treatment.

Recurrence rates of the disease are very high. This can be dedicated to the fact that the disease is only treated symptomatically. When a patient has so called Dupuytren diathesis recurence rates are even higher.

Symptoms

In Dupuytren's disease, the palmar fascia within one's hand becomes abnormally thick which can cause the fingers to curl and can result in impaired function of the fingers, especially the small and ring fingers are affected. The main function of the palmar fascia is to increase grip of the hand; thus, over time, dupuytren's contracture decreases patients ability to grip objects. Pain is mostly not associated with this condition. Dupuytren often starts as a nodules in the palm of the hand and it can extend to a cord in the fingers. The reason why the palmar fascia becomes abnormally thick is dedicated to the fact that there is a change of collagen type. Normally the palmar fasicia exist of collagen type one ( interne link) but if a patient has Dupuytren the collagen type one changes in collagen type three ( interne link), which is a lot thicker than collagen type one. The contracture sets in slowly and treatment is indicated when the so called table top test is positive.

Related conditions

Peyronie's disease - curvature of the penis Ledderhose disease - callus under the foot and possible curling under of toes Garrod's knuckle - pads on the back of knuckles of fingers

Risk factors

Dupuytren's disease is a rather non-specific affliction, but primarily affects: People of Scandinavian or Northern European ancestry;[3] it has been called the "Viking disease",[4] though it is also widespread in some Mediterranean countries (e.g. Spain and Bosnia) and in Japan.[5] Men rather than women (men are ten times as likely to develop the condition)[3] Rock climbers or manual labourers, associated with constant stress and micro-traumas of the tendons when climbing or absorbing shock through heavy blows of sledgehammers, jackhammers, spud bars, etc. Many younger climbers (under 26) were mis-diagnosed as having malignant tumors. People over the age of 40 People with a family history (60 to 70% of those afflicted have a genetic predisposition to Dupuytren's contracture)[6] People with liver cirrhosis

Some suspected, but unproven causes of Dupuytren's contracture include trauma, diabetes, alcoholism, epilepsy therapy with phenytoin and liver disease. There is no proven evidence that hand injuries or specific occupational exposures lead to a higher risk of developing Dupuytren’s disease although there is some speculation that Dupuytren's may be caused or at least the onset may be triggered by physical trauma, such as manual labor or other over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness casts some doubt on this claim.[3]

Recurrence

Dupuytren disease has a high recurrence rates, especially when a patient has so called Dupuytren’s diathesis. The term diathesis relates to certain features of Dupuytren's disease (DD) and dictates an aggressive course of disease. (43)

The initial description of DD diathesis included 4 factors:

• ethnicity

• family history

• bilateral DD

• ectopic lesions (Peyronie’s disease and Ledderhose disease).

In a study of Hindocha et al. they reevaluated these 4 factors and modified them. The original factors of family history, bilateral DD, and ectopic lesions now include 2 additional factors: male gender and age at onset of younger than 50 years. Family history and ectopic disease have now been modified to specify family history with one or more affected siblings/parents and ectopic lesions in the knuckles (Garrod’s pads) alone. The presence of all new DD diathesis factors in a patient increases the risk of recurrent DD by 71% compared with a baseline risk of 23% in those DD patients with none of the earlier-described factors. (43)

Not only diathesis shows a influence on recurrence of Dupuytren but also the treatment has an impact on the recurrence. Mainly the minimal invasive techniques shows a higher recurrence rates than the invasive techniques. Also there is no consensus on what is so called recurrence. In the literature there are different standards used for recurrence and how they are measured.

Treatment

In 1831 Baron Guillaume Dupuytren was the first to describe Dupuytren’s disease and a surgical procedure in the Lancet. The procedure he described was a minimal invasive needle procedure. Because of the high recurrence rates of the disease, new surgical techniques were introduced, such as the fasiectomy and later on also the dermofasciectomy. Although most of the diseased tissue is removed with these procedures, the recurrence rates remain the same compared to the minimal invasive procedure that Baron Dupuytren introduced. The fasciectomy is seen as the golden standard treatment for Dupuytren’s disease.

The knowledge about the pathogenesis of Dupuytren’s disease is increasing.There are recently reported results of non-surgical techniques that are promising.[30] Therefore, in the last decades there is a movement back to the minimal invasive treatments such as the Percutaneous Needle Fasciotomy and the Extensive Percutaneous Aponeurotomy and Lipografting. The most recently introduced treatment is injection of the collagen-eroding enzyme collagenase into the affected collagen cord.

Also available are radiation therapy and alternative therapies, into which there has been little evidence-based research and which have little support.[7] None of these treatments have proved to be a way to stop or cure the condition permanently.[8] In extreme cases, amputation of fingers may be needed for severe or recurrent disease, or after complications in surgery.[9]

Surgical

Limited Fasciectomy

The limited or selective fasciectomy is widely been seen as the golden standard treatment for Dupuytren’s disease. Therefore, the limited fasciectomy is a very common used procedure around the world.[28,30]

During the treatment the patient is under regional or general anesthesia. Surgeons use a surgical tourniquet (interne link) to prevent the bloodflow from going to the limb.[32] The skin is opened with a Zig-Zag incision. After the incision is made, all diseased cords and fascia are excised. [28,30,32] The excision of the cords and fascia has to be very precise to spare the neurovascular bundles.[32] Because you can’t see all the diseased tissue macroscopically (interne link), there is always a chance that not all the tissue has been removed. Nevertheless, this technique shows good results, low complication rates and a low recurrence rate.[30] When all the tissue has been removed, the surgeon closes the skin. In the case of a shortage of skin, the transverse part of the Zig-Zag incision is left open. Stitches are removed 10 days after surgery.[32]

After surgery the hand is wrapped in a light compressive bandage for one week. Patients should start practicing bending and extending their fingers as soon as the anesthesia has resolved. Only when needed hand therapy is recommended.[32] Approximately 6 weeks after surgery patients are able to completely use their hand. [33]

A 20-year review of surgical complications associated with open surgery (fasciectomy) for Dupuytren's showed that major complications occurred in 15.7%, including digital nerve injury 3.4%, digital artery injury 2%, infection 2.4%, hematoma 2.1%, and complex regional pain syndrome 5.5% in addition to minor complications including painful flare reactions in 9.9%, and wound healing complications in 22.9%.[16]

Dermofasciectomy and free vascular flaps

Dermofasciectomy is a surgical procedure that is mainly used in recurrent Dupuytren’s disease. It is also used in patients with a high chance of recurrence of the disease.[30] Just like the limited fasciectomy, with the dermofasciectomy al the diseased cords and fascia are excised. With the cords and the fascia, the overlying skin is taken out as well.[37]

After the skin and the subcutaneous tissue (interne link) has been removed, the skin needs to be closed with a skin graft. In almost all dermofasciectomies the surgeon chooses for a full-thickness skin graft. [3,4] A full-thickness skin graft consists of the epidermis (interne link) and the entire thickness of the dermis (interne link). In most cases the skin graft is taken from the elbow flexion crease or the proximal (interne link) inner side of the arm. [31,37] This place is chosen, because the color of the skin matches best with the color of the skin in the palm of the hand; the skin on the proximal inner side of the arm is thin and it is a place where there is enough skin to take some for a full-thickness skin graft. Therefore, the donor site can be easily closed with a direct suture. [37]

The full-thickness skin graft is placed on the defect in the palm of the hand and sutured to the skin surrounding. For one week the hand must be protected with a dressing. Also the hand and arm need to be elevated with a sling. After this week, when the skin graft successfully adherenced, the dressing can be removed and careful mobilization can be started. Two weeks after the skin graft has stabilised, the mobilization can be more intensive. [37] After this procedure the recurrence of the disease remains low. [30,31,37]

Segmental Fasciectomy with/without Cellulose

The segmental fasciectomy is less invasive than the limited fasciectomy, because not all the diseased tissue is excised and the skin incisions are smaller. The principle of this procedure is that the contracted cord will disappear or cease to act as a contracture, because parts of the contracted cord are excised, creating a discontinuity. [29]. This technique is not as widely used as the limited fasciectomy.

During the treatment the patient is under regional anasthesia. With this procedure, a surgical tourniquet (interne link) is also used. The skin is opened with small curved incisions over the diseased tissue in the palm of the hand. If nescessary, incisions are also made in the fingers. [29] The diseased tissue is kept under tension, while small pieces of cord and fascia of approximately one centimetre are excised. At the base of the proximal phalanx it is important to work very careful because of the neurovascular bundles. Some precautions are taken to minimize the risk of cutting one of those bundles. In the first place the cords need to be under maximum tension while cutting them. Second, only a scalpel is used to seperate the tissues. [29] The surgeon keeps removing small parts untill the finger can fully extend and then closes the skin. [29,34] After surgery the patients start with active mobilization the next day. They also need to wear an extension splint for two to three weeks, except during the physical therapy. [29]

The same procedure as described above, is used in the segmental fasciectomy with cellulose implant. After the excision of the pieces of the cord and a careful haemostasis (interne link), the cellulose implant is placed in a single layer in between the parts of the remaining cord. [34]

After surgery patients need to wear a light pressure dressing for four days, followed by an extension splint. the splint must be worn two-hours-on, two-hours-off for four weeks during day time. When the splint is off, patients need to exercise their fingers. During night time the splint has to be worn continuously for eight weeks. [34]

Minimal invasive surgery

Percutaneous Needle Fasciotomy

Needle aponeurotomy is a minimally invasive technique where the cords are weakened through the insertion and manipulation of a small needle. The cord is sectioned at as many levels as possible in the palm and fingers, depending on the location and extent of the disease, using a 25 Gauge needle mounted on a 10 ml syringe (41). Once weakened, the offending cords may be snapped by simply pulling the finger(s) straight. After the treatment a small dressing is applied for 24 hours. After these 24 hours patient are able to use their hands normally. No splint are used or physiotherapy is given.(41)

The advantage claimed for needle aponeurotomy is the minimal intervention without incision (done in the office under local anesthesia) and the very rapid return to normal activities without need for rehabilitation, but the nodules are not removed and might start growing again. [17]

A study reported that the postoperative gain is greater at the metacarpophalangeal joint level than at the interphalangeal level and found a reoperation rate of 24%; complications are scarce.[18] Needle aponeurotomy may be performed on fingers that are severely bent, stage IV, and not just on early stage Dupuytren's contracture.

Extensive Percutaneous Aponeurotomy and Lipografting

A minimal invasive technique to treat Dupuytren disease is extensive percutaneous aponeurotomy with lipografting.( 38) The procedure consists of an extensive percutaneous aponeurotomy that completely disintegrates the cord and separates it from the dermis. Subsequently, the resultant loosened structure is grafted with autologous lipoaspirate from the abdomen and ipsilateral flank. (38) This is a very new technique and the benefits are that it shortens the recovery time, adds to the deficient subcutaneous fat, and leads to scar less supple skin.(38)

Before the hand is operated, the abdomen and ipsilateral flank are prepared and draped. (38) The collected lipoaspirate is settle on the side table while the extensive percutaneous aponeurotomy is performed. (38) The treatment can be performed under regional or general anesthesia. The digits are placed under maximal extension tension using a firm lead hand retractor. Then the surgeon makes multiple palmar puncture wounds with small nicks. The tension on the cords is crucial, tight constricting bands are most susceptible to be cut and torn by the small nicks, whereas the relatively looser neurovascular structures are spared. After the cord completely is disintegrates and separates from his dermis the lipoaspirate is injected. A total of about 5 to 10 ml is injected per ray.(38)

After the treatment the patient has to wear an extension splint for 5 tot 7 days. After this 1 week of postoperative splinting the patient is allowed to return to his normal activities and he is advised to use a night splint for up to 20 weeks. (38)

At this moment this treatment is only performed in Miami or in Rotterdam. Different comparative prospective randomized studies with other techniques are in process to fully determine its role in the treatment of Dupuytren. (38)

Non-Surgical

Collagenase

In February 2010 the US Food and Drug Administration (FDA) approved injectable collagenase extracted from Clostridium histolyticum for the treatment of Dupuytren's contracture.[10] It is marketed by Auxilium Pharmaceuticals as Xiaflex.[11] In February 2011, the European Commission's Committee for Medicinal Products for Human Use approved the preparation for use in Europe, where it is marketed by Pfizer as Xiapex. The cords are weakened through the injection of small amounts of the enzyme collagenase, which breaks peptide bonds in collagen.[12][13][14][15]

Clostridial collagenase is a new nonsurgical treatment option of considerable potential in the management of Dupuytren disease but there is a need for further data on long-term results, complications and rate of recurrence with the use of this emerging treatment option. (39)

The treatment with collagenase is different for the MCP joint (interne link) and the PIP joint (interne link). In a MCP joint contracture the needle must be placed at the point of maximum bowstringing of the palpable cord. (39) The treatment consist of one injection with 0.58 mg 0.25 ml. collagenase clostridium histolyticum. (40)

The needle must be placed vertical on the bowstring and there is a 3-point distribution of each total injection volume. (39) For the PIP joint the needle must be placed not more than 4 mm distal to palmar digital crease to 2-3 mm depth. (39) The injection for PIP also consist of one injection but filled with 0.58 mg collagenase clostridium histolyticum/ 0.20 ml.(40)The needle must be placed horizontal to the cord and there is a 3-point distribution of each total injection volume. (39) After the injections the patient’s hand is wrapped in bulky gauze dressing and must be elevated for the rest of the day. After 24 hours the patient returns for passive digital extension to rupture the cord. Moderating pressure for 10-20 seconds ruptures the cord. (39)

After the treatment with collagenase the patient should use a night splint and perform digital flexion/extension exercises several times per day for 4 months. (39)

Radiation therapy

Radiation therapy treatment of Dupuytren's contracture with low energy x-rays, has shown some positive results in trials lacking a control group.[19][20] Treatment with radiation is applied to prevent disease progression. (42) Radiotherapy has been reported to be effective for prevention of disease progression in early stages with only mild acute or late side effects. (42) The nodules and cords are irradiated for five days in a row with a efficient dose. After these 5 days the patient has to wait for 6 weeks and then the treatment is repeated.

The effects of radiation therapy on a long-term outcome was evaluated by Betz et al.They had a 13 years follow up for patients who received radiation therapy. Late treatment toxicity and objective reduction of symptoms as change in stage and numbers of nodules and cords were evaluated and used as evidence to assess treatment response. (42) They concluded that after a mean follow-up of 13 years radiotherapy is effective in prevention of disease progression and improves patients' symptoms in early-stage Dupuytren's contracture (stage N, N/I). In case of disease progression after radiotherapy, a "salvage" operation is still possible.

Alternative therapies

A number of compounds have been claimed to provide benefit, but there is little or no experimental evidence to support these claims.[21]

• Quercetin
• Bromelain
• DMSO
• MSM
• Acetylcarnitine Hcl
• PABA
• Nattokinase
• Vitamin E[22] This was investigated in the 1940s[23]
• Copper
• Vitamin C
• Massage

Postoperative care

The after-treatment almost always consists of hand therapy and splinting. Hand therapy is prescribed to optimize the hand function after surgery and prevent the patient from joint stiffness. Besides the hand therapy, many surgeons advise the use of static or dynamic splints after surgery to maintain the extension of the finger achieved through surgery. The splint is used to provide prolonged stretch to the healing tissues and prevent flexion contractures. Although splinting is a widely used post-operative intervention, the evidence on the effectiveness remains scarce. [35] Due to this lack of high quality evidence, there is lots of variation in the way of splinting. Most of the surgeons decide on clinical experience and personal preference whether to use a splint or not. [36]

There are two groups of people regarding to splinting. One group of people believes in the use of splints, because the splints will maintain the extension of the finger achieved through surgery and prevent the finger from a flexion contracture. On the other hand there is a group of people that don’t believe in post-operative splinting, because it can result in joint stiffness, prolonged pain, subsequently reduced function and oedema. In this way, the splinting works completely opposite to the hand therapy. [36]

At this moment there is, due to the lack of evidence, no valid indication that post-operative splinting has a positive outcome in Dupuytren’s disease.

Notable sufferers

See Category:People with Dupuytren's contracture

Actors David McCallum and Bill Nighy,[24] politicians Bob Dole, Ronald Reagan,[25] and Margaret Thatcher,[25] playwright Samuel Beckett,Singer/Songwriter Rod Lunn, pianist Misha Dichter,[26] 16th-century slave trader John Hawkins, cricketers Jonathan Agnew, David Gower, and Graham Gooch, who opted to have a finger amputated.[27]

References

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http://www.nytimes.com/2010/03/16/business/16hand.html?scp=1&sq=dupuytren&st=cse

28. The Surgical Treatment of Dupuytren’s Contracture: A Synthesis of Techniques. Hillel D. Skoff, M.D., 2004

29. Segmental aponeurectomy in Dupuytren’s disease. J.P. Moermans, 1991

30. Dupuytren's Disease: Review of the Current Literature. Morsi Khashan*,1, Peter J. Smitham2, Wasim S. Khan3 and Nicholas J. Goddard2, 2011

31. Does a ‘firebreak’ full-thickness skin graft prevent recurrence after surgery for Dupuytren’s contracture? A PROSPECTIVE, RANDOMISED TRIAL. A. S. Ullah, J. J. Dias, B. Bhowal, 2009

32. A comparison of the direct outcomes of Percutaneous Needle Fasciectomy and Limited Fasciectomy for Dupuytren’s disease: A 6 week Follow-Up Study. A.L. van Rijssen, F.S.J. Gerbrandy, H. ter Linden, H. Klip, P.M.N. Werker, 2006

33. Ned Tijdschr Geneeskd. 2009;153;A129

34. Improved postoperative outcome of segmental fasciectomy in Dupuytren’s disease by insertion of an absorbable cellulose implant. I. Degreef, S. Tejpar, L. de Smet, 2011.

35. Splinting after contracture release for Dupuytren’s contracture (SCoRD): protocol of a pragmatic, multi-centre, randomized controlled trial. C. Jerosh - Herold, L. Shepstone, A.J. Chojnowski and D. Larson. 2008

36. Clinical effectiveness of post-operative splinting after surgical release of Dupuytren’s contracture: a systematic review. D. Larson, C. Jerosch-Herold

37. Dermofasciectomy in the management of Dupuytren’s disease. J.R. Armstrong, J.S. Hurren, A.M. Logan. 2000

38. Extensive percutaneous aponeurotomy and lipografting: a new treatment for dupuytren disease. Extensive percutaneous aponeurotomy and lipografting: a new treatment for Dupuytren disease. Hovius SE, Kan HJ, Smit X, Selles RW, Cardoso E, Khouri RK.

39. The emerging role of Clostridium histolyticum collagenase in the treatment of Dupuytren disease. Thomas A, Bayat A.

40. Injectable collagenase clostridium histolyticum for Dupuytren's contracture. Hurst LC, Badalamente MA, Hentz VR, Hotchkiss RN, Kaplan FT, Meals RA, Smith TM, Rodzvilla J; CORD I Study Group.

41. Percutaneous needle fasciotomy in dupuytren's disease. van Rijssen AL, Werker PM.

42. Radiotherapy in early-stage Dupuytren's contracture. Long-term results after 13 years. Betz N, Ott OJ, Adamietz B, Sauer R, Fietkau R, Keilholz L.

43. Dupuytren's diathesis revisited: Evaluation of prognostic indicators for risk of disease recurrence.Hindocha S, Stanley JK, Watson S, Bayat A.