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|Classification and external resources|
Dupuytren's contracture of the ring finger
|eMedicine||med/592 orthoped/81 plastic/299 pmr/42 derm/774|
|Classification and external resources|
Dupuytren's contracture of the ring finger
|eMedicine||med/592 orthoped/81 plastic/299 pmr/42 derm/774|
Dupuytren's contracture (also known as morbus Dupuytren, or Dupuytren's disease), is a fixed flexion contracture of the hand due to a palmar fibromatosis, 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. 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. 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.
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 usually not associated with this condition. However, substance P nerve fibers positively correlate to the 12-fold increase in mast cells in patients with Dupuytren's contractions, and those patients report pain along with the contractions. Some studies have shown that the substance P cells can impact the proliferation of fibroblasts, and is related to Interstitial Cystitis.
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 consists of collagen type I, but if a patient has Dupuytren’s disease, the collagen type I changes to collagen type III, which is significantly 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. If the hand cannot 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 ballpoint pen, the test is considered positive and surgery or other treatment may be indicated. Additionally, finger joints may become fixed and rigid.
Dupuytren's contracture is a non-specific affliction, but primarily affects:
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.
Dupuytren’s disease has a high recurrence rate, 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.
The initial description of Dupuytren’s disease diathesis included 4 factors:
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. In another study the prognostic value of diathesis was evaluated. They concluded that presence of diathesis can predict recurrence and extension. 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.
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 for Dupuytren's disease might involve one or more different types of treatments with some hands needing repeated treatments.
The main categories listed by the International Dupuytren Society in order of stage of disease are Radiation Therapy, Needle Aponeurotomy(NA), Collagenase Injection (Xiaflex) and Hand Surgery.
Radiation Therapy is effective at the early nodules and cords stage "Stage N" and also used at the N/I stage of 10 degrees or less of deformation.
Needle Aponeurotomy is most effective at "Stage I" of 6-45 degrees of deformation. It is also used at less than 6 degrees and more than 45 degrees of deformation.
Collagenase Injection (Xiapex) is most effective at "Stage I". It is also used at "Stage II" of 46-90 degrees of deformation.
Hand Surgery is effective at Stages I- Stage IV.
Dupuytren’s may be treated at the nodules, cords and early finger deformation stages with Radiation therapy.
The effects of radiation treatment for Dupuytren's contracture were first reported in the United Kingdom by R Finney in the British Journal of Radiology in 1955.
In Germany and parts of the U.S., radiotherapy is one of the main treatments given for Dupuytren's. The Worldwide list of clinics offering radiation treatment for Dupuytren's and Ledderhose is maintained by the International Dupuytren Society.
A study published in 2012, Seegenschmiedt et al. showed comparisons with a control group.
Treatment with radiation is applied to prevent disease progression. Radiotherapy has been reported to be effective for prevention of disease progression in early stages with only mild acute or late side effects.
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. 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 according to the authors of the article on use of radiation to treat Dupuytren's contracture.
In the UK NICE published guidelines and approval in November 2010. The guidance which proposes only one phase of 15Gy (grays) of treatment as standard for non severe cases, would be ineffective and would provide only half the treatment required to stop Dupuytren's progression.
Prof HM Seegenschmiedt from the Strahlenzentrum Hamburg, whose experience treating Dupuytrens with radiotherapy dates back to 1987, presented his findings at the 2010 International Symposium on Dupuytren's Disease in Miami, USA.
Seegenschmiedt stated at the conference that Radiotherapy is an early stage treatment in which ideally the deformation of a finger should be 10 degrees or less. The most preferable state of the hand would be with no deformation, but the hand diagnosed as being in an "active" state, i.e. Dupuytren's nodules and cords still changing. In the process of diagnosis the feet are checked for Ledderhose disease.
The nodules and cords are irradiated for five days in a row with a dose of 3Gy fractions per day, with a total of 15Gy for the week. After the 5 days the patient has to wait for 12 weeks and then the treatment is repeated.
X-Ray and more recently E-beam radiation are used.
The objective of radiotherapy is to stop disease progression and has a documented success rate of 85%.
The cell responsible for development of the disease is the myofibroblast. Researchers (Liaquat S. Verjee et al, 2013) excised myofibroblasts from patients with the disease to try and define the signaling pathways for their formation. They found significant numbers of immune cells, including classically activated macrophages and high levels of proinflammatory cytokines. They compared the effects of these cytokines on contraction and profibrotic signaling pathways in fibroblasts from the palmar and nonpalmar dermis of Dupuytren’s patients and palmar fibroblasts from non-Dupuytren’s patients. Addition of TNF (Tumor necrosis factors), but not other cytokines, promoted differentiation of fibroblasts into myofibroblasts in Dupuytren's patients. Neutralizing antibodies to TNF inhibited the contractile activity of myofibroblasts derived from Dupuytren’s patients, reduced their expression of α-smooth muscle actin, and mediated disassembly of the contractile apparatus.
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. For some individuals, the partial insertion of "K wires" into either the Dip or Pip joints of the effected digit for a period of a least 21 days in order to fuse the joint is the only way to halt the progress of the disease. After removal of the wires, the joint will be fixed into flexion, the idea being that it is better to have a joint fused at flexion than fused at extension.
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. Several alternate therapies such as 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. 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.
During the treatment the patient is under regional or general anesthesia. Surgeons use a surgical tourniquet to prevent blood flow to the limb. The skin is often opened with a zig-zag incision but straight incisions with or without Z-plasty are also described- and may reduce damage to the neurovascular bundles (see below). After the incision is made, all diseased cords and fascia are excised. The excision of the cords and fascia has to be very precise to spare the neurovascular bundles. Because you can not see all the diseased tissue macroscopically, there is always a chance that not all the tissue has been removed. 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. 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.
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 often recommended. Approximately 6 weeks after surgery patients are able to completely use their hand.
The average recurrence rate is 39% after a fasciectomy at a median time of about 4 years.
Three centres worldwide have published the results of limited/selective fasciectomy under local anesthesia (LA) with epinephrine but no tourniquet. The original description of the technique was in 2005 by Dr Denkler, a Plastic Surgeon, from San Francisco; his 60 case series clearly refuting several decades of surgical dogma that adrenaline cannot be used in digits, and indeed that Dupuytren's fasciectomy cannot be done under LA without a tourniquet. Then in 2009, a Canadian group under Dr Don Lalonde, Plastic Surgery Professor from Dalhousie University, described a multicentre comparative study of 111 cases either having surgery under general or local anesthesia: with equivalent results. In 2012, the Orthopaedic Surgeons MSK & QMK Bismil, based in London, described in a publication of the Royal Society of Medicine the first high volume totally wide awake Dupuytren's service- with 270 cases treated. Through their One Stop Wide Awake surgery (OSWA) approach to Dupuytren's fasciectomy: all patients are seen and treated within one thirty to forty-five minute management slot; and hence the benefits of the traditional surgical approach (in removing the diseased tissue) are combined with the efficiency, cost-effectiveness and lower risk of outpatient, same day LA surgery. With the wide awake approach patients can be taught range-of-motion exercises during the procedure and the surgeon can use dynamic information to optimize the surgery. Splinting is not mandatory if accelerated rehabilitation is employed and a modified boxing-glove bandage has been shown to eradicate significant post-operative haematoma.
Overall results of wide awake fasciectomy are equivalent to surgery under general or regional anesthesia, with less risk; but the technique is more challenging. Hand surgeons have to become comfortable operating without a tourniquet, which indeed is the only (comfortable) option for a wide awake patient- this is contrary to most hand surgeons' training. Hence, at present whilst most hand surgeons are now starting to learn wide awake techniques and can offer some of their surgery wide awake- the technique is only routinely available for all Dupuytren's cases irrespective of severity under the care of Robbins in Australia, Denkler in the US, Lalonde in Canada or Bismil in the UK at present. The largest series of wide awake fasciectomy utilizes entirely the skin incisions described by Robbins, a Plastic Surgeon based in Melbourne but published by the Royal College of Surgeons of England, over 30 years ago- demonstrating that straight incisions with or without deferred Z-plasty are ideal for the wide awake technique, with increased safety for the patient (wide awake) and the neurovascular bundle (straight incisions).
The progress made with wide awake Dupuytren's surgery by Robbins, Denkler, Lalonde and Bismil has resulted in the phenomenon of wide awake complex hand surgery steadily sweeping the globe. These surgeons have pushed the boundaries of local anesthetic hand surgery to include Dupuytren's fasciectomy which was traditionally seen as complex, GA-only surgery: by refuting surgical dogma such as “no epinephrine in fingers, nose, gonads or toes”; and demonstrating that general anesthesia, tourniquets and zig-zag incisions are by no means mandatory for Dupuytren's surgery. Wide awake fasciectomy removes the diseased tissue through a low risk procedure which can be performed in the outpatient setting and in just one management stop. It has the advantages of the traditional open surgical approach of limited/selective fasciectomy (lower recurrence rates and visualisation of the pathology and the neurovascular bundle and tendon) with the convenience of a clinic procedure.
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. 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.
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. 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. 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.
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. After this procedure the recurrence of the disease can be low but the re-operation and complication rate may be high.
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.
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. 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. 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. The surgeon keeps removing small parts until the finger can fully extend and then closes the skin. 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.
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.
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.
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. 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.
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. 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. 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 study showed 85% recurrence rate after 5 years.
A recently introduced technique to treat Dupuytren disease is extensive percutaneous aponeurotomy with lipografting. 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 from the skin to make place for the lipograft that is taken from the abdomen or ipsilateral flank. This technique is very promising, because it shortens the recovery time, also the fatgraft results in supple skin.
Before the hand is operated, a liposuction is done to the abdomen and ipsilateral flank to collect the lipograft. 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.
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.
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.
Clostridial collagenase is a pharmaceutical treatment option. The cords are weakened through the injection of small amounts of the enzyme collagenase, which breaks peptide bonds in collagen.
This treatment has 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.
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. The treatment consist of one injection with 0.58 mg 0.25 ml. collagenase clostridium histolyticum.
The needle must be placed vertical on the bowstring and there is a 3-point distribution of each total injection volume. For the PIP joint the needle must be placed not more than 4 mm distal to palmar digital crease to 2–3 mm depth. The injection for PIP also consist of one injection but filled with 0.58 mg collagenase clostridium histolyticum/ 0.20 ml. The needle must be placed horizontal to the cord and there is a 3-point distribution of each total injection volume. 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.
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.
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 as severe as the primary disease. Collagenase injection is a nonsurgical option to treat Dupuytren’s disease and 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.
In February 2010 the US Food and Drug Administration (FDA) approved injectable collagenase extracted from Clostridium histolyticum for the treatment of Dupuytren's contracture. The treatment is marketed by Auxilium Pharmaceuticals as Xiaflex. 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.
Laser treatment (using red and infrared at low power) has been informally discussed as of 2013[update] at an International Dupuytren Society forum, though there has been little or no formal evaluation of the experimental techniques. It would be well to remember that tendons require far longer than bones to heal, so alternative therapies might take 6 months and require continuing exercise.
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.
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. 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.
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 and hence some authors advocate an approach which avoids either, but instead focuses on early self-exercise and stretching. Custom-made splints are also very expensive and can be very uncomfortable for patients.
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.
The International Dupuytren Society was founded in Germany in 2003. It states that it is a non-profit organization based in Germany where patients and medical experts cooperate, without promoting any specific treatment. Stated goals are informing about Dupuytren's Disease and therapy options, and supporting research, patients and organizations. Reliable medical results are published, and there is a discussion forum for practitioners and sufferers.
The society was founded by Wolfgang Wach, a doctor in chemistry who suffers from Dupytrens Disease. In a presentation at the International Symposium on Dupuytrens Disease in Miami in 2010 he discussed receiving radiation therapy 30 years before, and having surgery.
As of 2013[update] the chairmen of the Society were Wach, Charles Eaton MD, and Prof Heinrich Seegenschmiedt.
Actors David McCallum, Bill Nighy, and Michael Parks, politicians Bob Dole, Ronald Reagan, and Margaret Thatcher, playwright Samuel Beckett, pianist Misha Dichter, 16th-century slave trader John Hawkins, Prince Joachim of Denmark, cricketers Jonathan Agnew, David Gower, Graham Gooch, wrongly reported in the Daily Mail as having had a finger amputated,  and Bill Frindall, who did have a finger amputated.