Dupuytren's contracture


Dupuytren's contracture
Classification and external resources

Dupuytren's contracture of the ring finger
ICD-10 M72.0
ICD-9 728.6
OMIM 126900
DiseasesDB 4011
MedlinePlus 001233
eMedicine med/592 orthoped/81 plastic/299 pmr/42 derm/774
MeSH D004387


Dupuytren's contracture (not to be confused with Dupuytren fracture), (also known as morbus Dupuytren, Dupuytren's disease, or palmar fibromatosis,[1]) is a fixed flexion contracture of the hand where the fingers bend towards the palm and cannot be fully extended (straightened). It is an inherited proliferative connective tissue disorder which involves the palmar fascia of the hand. [2] It is named after Baron Guillaume Dupuytren, the surgeon who described an operation to correct the affliction in the Lancet in 1831.

The ring finger and little finger are the fingers most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are nearly always spared.[citation needed] 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.

Contents

Symptoms

Dupuytren's contracture of the right little finger. Arrow marks the area of scarring

In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick which can cause the fingers to curl and can result in impaired function of the fingers. The small and ring fingers are especially 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’s disease often starts with nodules in the palm of the hand and it can extend to a cord in the finger. The palmar fascia becomes abnormally thick due to the fact that there is a change of collagen type. Normally the palmar fascia exists of collagen type I, but if a patient has Dupuytren’s disease the collagen type I changes to collagen type III, which is a lot thicker than collagen type I. The contracture sets in slowly and treatment is indicated when the so called table top test is positive. With this test the patient places his hand on a table. If the hand lies completely flat on the table, the test is considered negative. When the hand can not be placed completely flat on the table, but there is a space between the table and a part of the hand as big as the diameter of a ball pen, the test is considered positive and surgery may be indicated. Additionally, finger joints may become fixed and rigid.

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 contracture is a 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]
  • 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 contracture, although there is some speculation that Dupuytren's may be caused by, 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’s 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 and indicates an aggressive course of disease. [7]

The initial description of Dupuytren’s disease diathesis included 4 factors:

  • the patient is below the age of 50 years old
  • positive family history
  • both of the hands are affected
  • ectopic lesions (Peyronie’s disease, Knuckle pads 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 Dupuytren’s disease, and ectopic lesions now include 2 additional factors: male gender and age at onset of younger than 50 years. The presence of all new Dupuytren’s disease diathesis factors in a patient increases the risk of recurrent Dupuytren’s disease by 71% compared with a baseline risk of 23% in those Dupuytren’s disease patients with none of the earlier-described factors. [7] In another study the prognostic value of diathesis was evaluated. They concluded that presence of diathesis can predict recurrence and extension. [8] A scoring system was made to evaluate the risk of recurrence and extension containing the following values: bilateral hand involvement, little finger surgery, early onset of disease, plantar fibrosis, knuckle pads and radial side involvement. [8]

Minimal invasive techniques may show higher recurrence rates. However there is no consensus on what recurrence is, different definitions are used. Furthermore different standards for recurrence are used and different ways to measure these.

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 high. The fasciectomy is seen as the golden standard treatment for Dupuytren’s disease.

The patient burden after open surgery is high, therefore less invasive techniques may be preferred. New studies have been conducted for percutaneous release, extensive percutaneous aponeurotomy with lipografting, and collagenase. These treatments show promise. [9] [10] [11] [2] Several alternate therapies, including radiation and vitamin E treatment, have been tried in the past, although those studies generally lacked control groups and most contemporary doctors do not place much value on those treatments.[12] None of these treatments have proved to be a way to stop or cure the condition permanently. In extreme cases, amputation of fingers may be needed for severe or recurrent disease, or after complications in surgery.[13]


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 commonly used procedure around the world. [14] [15]

During the treatment the patient is under regional or general anesthesia. Surgeons use a surgical tourniquet to prevent blood flow going to the limb. [16] The skin is opened with a Zig-Zag incision. After the incision is made, all diseased cords and fascia are excised. [14] [15] [16] The excision of the cords and fascia has to be very precise to spare the neurovascular bundles. [16] Because you can not see all the diseased tissue macroscopically, there is always a chance that not all the tissue has been removed. [15] A 20-year review of surgical complications associated with open surgery (fasciectomy) for Dupuytren's contracture showed that major complications occurred in 15.7% of cases, 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% of cases and wound healing complications in 22.9% of cases.[17] 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. [16]

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. Hand therapy is recommended. [16] Approximately 6 weeks after surgery patients are able to completely use their hand. [18]

Dermofasciectomy

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. [15] Just like the limited fasciectomy, with the dermofasciectomy all the diseased cords and fascia are excised. With the cords and the fascia, the overlying skin is taken out as well. [19]

After the skin and the subcutaneous tissue 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. [15] [20] A full-thickness skin graft consists of the epidermis and the entire thickness of the dermis. In most cases the skin graft is taken from the elbow flexion crease or the proximal inner side of the arm.[20] [19] 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. [19]

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, the dressing can be removed and careful mobilization can be started. Two weeks after the skin graft has stabilized, the mobilization can be more intensive. [19] After this procedure the recurrence of the disease can be low. [15] [20] [19]

Free vascular flaps

In severe cases of Dupuytren’s disease a free vascular flap may be preferred to treat the disease . Not many studies are conducted, but the idea is that there will be little recurrent disease after this kind of treatment. Recently a one-year follow-up of one patient has been described. This patient did not suffer of recurrent disease. [21]


Minimal invasive surgery

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.[22] 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 is also used. The skin is opened with small curved incisions over the diseased tissue in the palm of the hand. If necessary, incisions are also made in the fingers. [22] The diseased tissue is kept under tension, while small pieces of cord and fascia of approximately one centimeter 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 separate the tissues. [22] The surgeon keeps removing small parts until the finger can fully extend and then closes the skin. [22] [23] 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. [22]

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, the cellulose implant is placed in a single layer in between the parts of the remaining cord. [23]

After surgery patients need to wear a light pressure dressing for four days, followed by an extension splint. The splint must be worn continuously during night-time for eight weeks. The first weeks after surgery the splint may also be worn during day time. [23]

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. [24] Once weakened, the offending cords can be snapped by putting tension on the finger(s) and 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. [24]

The advantage of 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. [25] A study reported postoperative gain is greater at the MCP-joint level than at the level of the IP-joint and found a reoperation rate of 24%; complications are scarce.[26] Needle aponeurotomy may be performed on fingers that are severely bent (stage IV), and not just on early-stage Dupuytren's contracture.

However a recent studie showed 85% recurrence rate after 5 years. [27]

Extensive Percutaneous Aponeurotomy and Lipografting

A recently introduced technique to treat Dupuytren disease is extensive percutaneous aponeurotomy with lipografting. [9] This procedure also uses a needle to cut the cords. The difference with the percutaneous needle fasciotomy is, that with this procedure the cord is cut at many places. The cord is also being separated form the skin to make place for the lipograft that is taken from the abdomen or ipsilateral flank. [9] This technique is very promising, because it shortens the recovery time, also the fatgraft results in supple skin. [9]

Before the hand is operated, a liposuction is done to the abdomen and ipsilateral flank to collect the lipograft. [9] 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, because 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 is completely cut and separated from the skin the lipograft is injected under the skin. A total of about 5 to 10 ml is injected per ray. [9]

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. [9]

At this moment this treatment is only performed in Miami or in Rotterdam. Prospective randomized studies with other techniques are in process to fully determine its role in the treatment of Dupuytren’s disease. [9]


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.[28] The treatment is marketed by Auxilium Pharmaceuticals as Xiaflex.[29] 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.[30][31][32][33]

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. [10]

The treatment with collagenase is different for the MCP joint and the PIP joint. In a MCP joint contracture the needle must be placed at the point of maximum bowstringing of the palpable cord. [10] The treatment consist of one injection with 0.58 mg 0.25 ml. collagenase clostridium histolyticum. [11]

The needle must be placed vertical on the bowstring and there is a 3-point distribution of each total injection volume. [10] For the PIP joint the needle must be placed not more than 4 mm distal to palmar digital crease to 2-3 mm depth. [10] The injection for PIP also consist of one injection but filled with 0.58 mg collagenase clostridium histolyticum/ 0.20 ml. [11] The needle must be placed horizontal to the cord and there is a 3-point distribution of each total injection volume. [10] 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. [10]

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. [10]

A study where patients were treated with these collagenase injections showed a recurrence rate of 67% in the MCP joint and 100% in the PIP joint. Although these recurrent rates are high, the recurrence was not that severe as the primary disease.[34] Collagenase injection is a nonsurgical option to treat Dupuytren’s disease en it provides the benefits of avoiding the potential surgical complications such as nerve injury, hematoma and skin necrosis. Primary surgery reports a 5% incidence of nerve injury and 12% in second surgery.

Radiation therapy

Radiation therapy treatment of Dupuytren's contracture with low energy x-rays, has shown some promising results in trials lacking a control group.[35][36]

Treatment with radiation is applied to prevent disease progression. [37] Radiotherapy has been reported to be effective for prevention of disease progression in early stages with only mild acute or late side effects. [37] 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. [37] 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

Anecdotal evidence cites a number of compounds as providing benefits for Dupuytren's patients, but there is little or no formal scientific evidence to support these claims.[38]

Postoperative care

Postoperative care is 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. [41] 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. [42]

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 edema. In this way, the splinting works completely opposite to the hand therapy. Custom-made splints are also very expensive and can be very uncomfortable for patients. [42]

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. Therefore, you can question whether the widespread use of splints can still be justifiable to purchasers, providers and patients. [42]


Notable sufferers

See Category:People with Dupuytren's contracture

Actors David McCallum and Bill Nighy,[43] politicians Bob Dole, Ronald Reagan,[44] and Margaret Thatcher,[44] playwright Samuel Beckett, pianist Misha Dichter,[45] 16th-century slave trader John Hawkins, cricketers Jonathan Agnew, David Gower, Graham Gooch, wrongly reported in the Daily Mail as having had a finger amputated,[46] [47] and Bill Frindall, who did have a finger amputated. [47]


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


External links


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Look at other dictionaries:

  • Dupuytren's contracture —       flexion deformity of the hands caused by thickening of the fascia, or fibrous connective tissue, of the palm. The proliferation of connective tissue causes the tendons of one or more fingers to shorten and tighten, leaving the finger… …   Universalium

  • Dupuytren's contracture — Du·puy·tren s contracture də .pwē tranz , pwē trənz n a condition marked by fibrosis with shortening and thickening of the palmar aponeurosis resulting in flexion contracture of the fingers into the palm of the hand Du·puy·tren də pwē tran… …   Medical dictionary

  • Dupuytren's contracture — a flexion deformity of the fingers (usually the ring and little fingers) caused by a nodular hypertrophy and contracture of the fascia in the palm. The characteristic sign is a nodule at the distal palmar crease or over the proximal phalanx of… …   The new mediacal dictionary

  • dupuytren's contracture — də|pwē.|traⁿz , dəˈpwē.trə̇nz noun Usage: usually capitalized D Etymology: after Guillaume Dupuytren died 1835 French surgeon and anatomist : a condition marked by fibrosis with shortening and thickening of the palmar fascia resulting in flexion… …   Useful english dictionary

  • Dupuytren's contracture — n. condition of thickening of the connective tissue causing one or more fingers to be bent permanently into the palm so that they cannot be extended (named after Baron Guillaume Dupuytren) …   English contemporary dictionary

  • Dupuytren's contracture — [djʊ pwi:trəns] noun Medicine fixed forward curvature of one or more fingers, caused by a growth of fibrous tissue. Origin C19: named after the French surgeon Baron Guillaume Dupuytren …   English new terms dictionary

  • Dupuytren&’s contracture — Fibroma like lesion of the palm of the hand that causes flexion contracture. Heritable and commoner in men. Never metastasises …   Dictionary of molecular biology

  • Контрактура Дюпюитрена (Dupuytren 'S Contracture) — сгибательная контрактура одного или нескольких пальцев (обычно третьего и/или четвертого) кисти в результате сжатия поверхностного ладонного апоневроза (ред.) и сращения сухожилия сгибателя пораженного пальца с кожей ладони. Данная контрактура… …   Медицинские термины

  • Contracture de Dupuytren — Maladie de Dupuytren Maladie de Dupuytren CIM 10: M72.0 {{{CIM10}}}.0 {{{X.0}}} …   Wikipédia en Français

  • Contracture — This article refers to orthopedic contractures; for other types of contractures see the See Also section below. Contracture Classification and external resources ICD 10 M24.5, M62.4, M67.1, M72.0, T …   Wikipedia


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