Dupuytren's contracture

Typically, Dupuytren's contracture first presents as a thickening or nodule in the palm, which initially can be with or without pain. Later in the disease process, which can be years later, there is increasing loss of range of motion of the affected finger(s). The earliest sign of a contracture is a triangular "puckering" of the skin of the palm as it passes over the flexor tendon just before the flexor crease of the finger, at the metacarpophalangeal (MCP) joint. Dupuytren disease is generally considered painless, but can be painful if nerve tissue is involved, although this is not usually discussed in the literature. The most common finger to be affected is the ring finger; the thumb and index finger are much less often affected. The disease begins in the palm and moves towards the fingers, with the metacarpophalangeal (MCP) joints affected before the proximal interphalangeal (PIP) joints. The MCP joints at the base of the finger responds much better to treatment and are usually able to fully extend after treatment. Due to anatomic differences in the ligaments and extensor tendons at the PIP joints, they may have some residual flexion. Proper patient education is necessary to set realistic treatment expectation. In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick, which can cause the fingers to curl and can impair finger function. The main function of the palmar fascia is to increase grip strength; thus, over time, Dupuytren's contracture decreases a person's ability to hold objects and use the hand in many different activities. Dupuytren's contracture can also be experienced as embarrassing in social situations and can affect quality of life. People may report pain, aching, and itching with the contractions. Normally, the palmar fascia consists of collagen type I, but in Dupuytren patients, the collagen changes to collagen type III, which is significantly thicker than collagen type I. Related conditions People with severe involvement often show lumps on the back of their finger joints (called "Garrod's pads", "knuckle pads", or "dorsal Dupuytren nodules"), and lumps in the arch of the feet (plantar fibromatosis or Ledderhose disease). In one study those with stage 2 of the disease were found to have a slightly increased risk of mortality, especially from cancer. ==Risk factors==

Many risk factors have been suggested or identified: Non-modifiable • Scandinavian or Northern European ancestry; Dupuytren's has been called the "Viking disease", and Bosnia. Dupuytren's is uncommon among groups including Chinese and Africans. • In June 2023 a study found that gene variants that were inherited from Neanderthals dramatically increased the odds of developing the condition • Male sex; men are 80% more likely to develop the condition • Age of 50 or over (5% to 15% of men in that group in the US); the likelihood of getting Dupuytren's disease increases with age Modifiable • Smoking, especially 25-plus cigarettes per day • Lower-than-average body mass index (thinness). a 2023 paper by researchers at the University of Groningen Medical Centre and Oxford University, "Dupuytren's disease is a work-related disorder: results of a population-based cohort study", found that people whose jobs involved significant manual work were 1.29 times more likely to develop Dupuytren's disease than others, with a linear dose–response relationship with cumulative manual labour over 30 years. • Higher-than-average fasting blood glucose level • Diabetes mellitus • HIV • Macrophallism • Previous myocardial infarction == Pathophysiology ==

Dupuytren’s contracture is a fibroproliferative disorder of the palmar fascia in which abnormal activation of fibroblasts and myofibroblasts, driven by mediators such as transforming growth factor-beta, platelet-derived growth factor, epidermal growth factor, interleukin-1 beta, and connective tissue growth factor, leads to excess deposition of type III collagen and progressive remodeling of fascial tissue. Studies have suggested that intracellular signaling, as opposed to paracrine or endocrine signaling, may be the strongest driver of abnormal fibroblast activity in most cases of Dupuytren's contracture. ==Diagnosis==

Types There may be three types of Dupuytren's disease: • Type 1: An aggressive form of the disease found in only 3% of people with Dupuytren's, which can affect men under 50 with a family history of Dupuytren's. It is often associated with other symptoms such as knuckle pads and Ledderhose disease. This type is sometimes known as Dupuytren's diathesis. • Type 2: The more normal type of Dupuytren's disease, usually found in the palm only, and which generally begins above the age of 50. This type may be made more severe by other factors such as diabetes or heavy manual labor. • Type 3: A mild form of Dupuytren's which is common among diabetics or which may also be caused by certain medications, such as the anti-convulsants taken by people with epilepsy. This type does not lead to full contracture of the fingers, and is probably not inherited. ==Treatment==

Treatment is indicated when the so-called table-top test is positive. With this test, the person places their 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, leaving 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. There are several types of treatment, with some hands needing repeated treatment. The main categories listed by the International Dupuytren Society in order of stage of disease are radiation therapy, needle aponeurotomy (NA), collagenase injection, and hand surgery. the evidence on the efficacy of radiation therapy was considered inadequate in quantity and quality, and difficult to interpret because of uncertainty about the natural history of Dupuytren's disease. Use of a splint to keep treated fingers straight following various forms of treatment, typically at all times for some days, then at nighttime for some weeks, is usual. However, a 2015 Cochrane review concluded: "low-quality evidence suggests that postoperative splinting may not improve outcomes and may impair outcomes by reducing active flexion. Further trials on this topic are urgently required". after presenting it in 1833, and posthumously in 1836 in a French publication by Hôtel-Dieu de Paris. The procedure he described was a minimally invasive needle procedure. Because of high recurrence rates, new surgical techniques were introduced, such as fasciectomy and then dermofasciectomy. Most of the diseased tissue is removed with these procedures. For some individuals, the partial insertion of "K-wires" into either the DIP or PIP joint of the affected digit for a period of a least 21 days to fuse the joint is the only way to halt the disease's progress. After removal of the wires, the joint is fixed into flexion, which is considered preferable to fusion at extension. Research using large datasets in the UK has shown surgery to be safe and effective. When surgery needs to be repeated, however, the research suggests there are higher risks of serious complications such as finger amputation. Amputation of fingers may be needed for severe or recurrent cases or after surgical complications. Limited fasciectomy Limited/selective fasciectomy removes the pathological tissue, and is a common approach. A 2015 Cochrane review reported that low-quality evidence suggested that fasciectomy may be more effective for people with advanced Dupuytren's contractures. During the procedure, the person is under regional or general anesthesia. A surgical tourniquet prevents 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 neurovascular bundles. All diseased cords and fascia are excised. After the tissue is removed the incision is closed. 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. The average recurrence rate is 39% after a fasciectomy after a median interval of about four years. Wide-awake fasciectomy Limited/selective fasciectomy under local anesthesia (LA) with epinephrine but no tourniquet is possible. In 2005, Denkler described the technique. Dermofasciectomy Dermofasciectomy is a surgical procedure that may be used when: • The skin is clinically involved (pits, tethering, deficiency, etc.) • The risk of recurrence is high and the skin appears uninvolved (subclinical skin involvement occurs in ~50% of cases) • Recurrent disease. Typically, the excised skin is replaced with a skin graft, usually full thickness, This place is chosen because the skin color best matches the palm's skin color. The skin on the inner side of the upper arm is thin and has enough skin to supply a full-thickness graft. The donor site can be closed with a direct suture. and complications from surgery may occur. Segmental fasciectomy with/without cellulose Segmental fasciectomy involves excising part(s) of the contracted cord so that it disappears or no longer contracts the finger. It is less invasive than the limited fasciectomy, because not all the diseased tissue is excised and the skin incisions are smaller. The person is placed under regional anesthesia and a surgical tourniquet is used. The skin is opened with small curved incisions over the diseased tissue. If necessary, incisions are made in the fingers. The patient is encouraged to start moving their hand the day after 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. It is applicable only if the contracture is clearly visible. The hand is first numbed by injection with local anaesthetic. The cord is then sectioned at as many levels as possible in the palm and fingers, depending on the location and extent of the disease, using perhaps 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 which people are able to use their hands normally. No splints or physiotherapy are given. 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 in early stages. A 2003 study showed 85% recurrence rate after five years. A comprehensive review of the results of needle aponeurotomy in 1,013 fingers was performed by Gary M. Pess, MD, Rebecca Pess, DPT, and Rachel Pess, PsyD, and published in The Journal of Hand Surgery April 2012. Minimal follow-up was three years. Metacarpophalangeal joint (MP) contractures were corrected at an average of 99% and proximal interphalangeal joint (PIP) contractures at an average of 89% immediately post procedure. At final follow-up, 72% of the correction was maintained for MP joints and 31% for PIP joints. The difference between the final corrections for MP versus PIP joints was statistically significant. When comparing people aged below and above 55 years of age there was a statistically significant difference at both MP and PIP joints, with greater correction maintained in the older group. Gender differences were not statistically significant. Needle aponeurotomy provided successful correction to 5° or less contracture immediately post procedure in 98% (791) of MP joints and 67% (350) of PIP joints. There was recurrence of 20° or less over the original post-procedure corrected level in 80% (646) of MP joints and 35% (183) of PIP joints. Complications were rare except for skin tears, which occurred in 3.4% (34) of digits. This study showed that NA is a safe procedure that can be performed in an outpatient setting. The complication rate was low, but recurrences were frequent in younger people and for PIP contractures. Extensive percutaneous aponeurotomy and lipografting A technique introduced in 2011 is extensive percutaneous aponeurotomy with lipografting. including the UK, Australia, and Asia in March 2020. (It is also used in the US as a dermatological treatment for cellulite aka "cottage cheese thighs"). 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. It has been studied in early disease. Alternative medicine Several alternate therapies such as vitamin E treatment have been studied, though without control groups. Most doctors do not value those treatments. None of these treatments stops or cures the condition permanently. A 1949 study of vitamin E therapy found that "In twelve of the thirteen patients there was no evidence whatever of any alteration. ... The treatment has been abandoned." "Cold" laser treatment or "photomodulation" (using red and infrared at low power) was informally discussed in 2013 at an International Dupuytren Society forum, as of which time little or no formal evaluation of the techniques had been completed. The term "photobiomodulation" has expanded to include other low-power light sources such as blue LEDs, making it a form of light therapy rather than specifically a laser-based treatment. No human trials have been performed yet. In 2021 improvement of Dupuytren's disease in a single patient by ablative laser surgery with a fractionated 10.6 μm carbon-dioxide laser was reported. This is different from a cold laser: in laser surgery, tissue is physically removed by heat by photoablation (also known as "laser blasting"). Postoperative care Postoperative care involves hand therapy and splinting. Hand therapy is prescribed to optimize post-surgical function and to prevent joint stiffness. The extent of hand therapy is depending on the patient and the corrective procedure. Besides hand therapy, many surgeons advise the use of static or dynamic splints after surgery to maintain finger mobility. 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, evidence of its effectiveness is limited, leading to variation in splinting approaches. Most surgeons use clinical experience to decide whether to splint. Cited advantages include maintenance of finger extension and prevention of new flexion contractures. Cited disadvantages include joint stiffness, prolonged pain, discomfort, subsequently reduced function and edema. A third approach emphasizes early self-exercise and stretching. ==Prognosis==

Dupuytren's disease has a high recurrence rate, especially when a person 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 presence of all new Dupuytren's diathesis factors increases the risk of recurrent Dupuytren's disease by 71%, compared with a baseline risk of 23% in people lacking the factors. A scoring system was made to evaluate the risk of recurrence and extension, based on the following values: bilateral hand involvement, little-finger surgery, early onset of disease, plantar fibrosis, knuckle pads, and radial side involvement. Minimally invasive therapies may precede higher recurrence rates. Recurrence lacks a consensus definition. Furthermore, different standards and measurements follow from the various definitions. ==Notable cases==