Dupuytren's contracture
Dupuytren's contracture | |
---|---|
Other names | Dupuytren's disease, Morbus Dupuytren, palmar fibromatosis, Viking disease, and Celtic hand, |
Frequency | ~5% (US)[2] |
Dupuytren's contracture (also called Dupuytren's disease, Morbus Dupuytren, Viking disease, palmar fibromatosis and Celtic hand) is a condition in which one or more fingers become permanently bent in a flexed position.[2] It is named after Guillaume Dupuytren, who first described the underlying mechanism of action, followed by the first successful operation in 1831 and publication of the results in The Lancet in 1834.[6] It usually begins as small, hard nodules just under the skin of the palm,[2] then worsens over time until the fingers can no longer be fully straightened. While typically not painful, some aching or itching may be present.[2] The ring finger followed by the little and middle fingers are most commonly affected.[2] It can affect one or both hands.[7] The condition can interfere with activities such as preparing food, writing, putting the hand in a tight pocket, putting on gloves, or shaking hands.[2]
The cause is unknown but might have a genetic component.
Initial treatment is typically with a
It was once believed that Dupuytren's most often occurs in white males over the age of 50[2] and is rare among Asians and Africans.[6] It sometimes was called "Viking disease," since it was often recorded among those of Nordic descent.[6] In Norway, about 30% of men over 60 years old have the condition, while in the United States about 5% of people are affected at some point in time.[2] In the United Kingdom, about 20% of people over 65 have some form of the disease.[6]
More recent and wider studies show the highest prevalence in Africa (17 percent), Asia (15 percent).[9]
Signs and symptoms
Typically, Dupuytren's contracture first presents as a thickening or
Generally, the cords or
In Dupuytren's contracture, the
Related conditions
People with severe involvement often show lumps on the back of their finger joints (called "
In one study those with stage 2 of the disease were found to have a slightly increased risk of mortality, especially from cancer.[18]
Risk factors
Many risk factors have been suggested or identified:
Non-modifiable
- People of Scandinavian or Northern European ancestry;[19] it has been called the "Viking disease",[6] though it is also widespread in some Mediterranean countries, e.g., Spain[20] and Bosnia.[21][22] Dupuytren's is unusual among groups such as Chinese and Africans.[23][clarification needed]
- Men rather than women; men are more likely to develop the condition (80%)[12][19][24]
- People over the age of 50 (5% to 15% of men in that group in the US); the likelihood of getting Dupuytren's disease increases with age[12][23][24]
- People with a family history (60% to 70% of those affected have a genetic predisposition to Dupuytren's contracture)[12][25]
Modifiable
- Smokers, especially those who smoke 25 cigarettes or more a day[23][26]
- Thinner people, i.e., those with a lower-than-average body mass index.[23]
- Manual work[23][27]
In January 2023, a research paper "Dupuytren's disease is a work-related disorder: results of a population-based cohort study" showed the clear link between manual work and the condition. The study was by researchers at the
Other conditions
- People with a higher-than-average fasting blood glucose level[23]
- People with previous hand injury[12]
- People with Ledderhose disease (plantar fibromatosis)[12]
- People with anti-convulsive medication)[28]
- People with
- People with HIV[6]
- Previous myocardial infarction[23][24]
Diagnosis
Types
According to the American Dupuytren's specialist Dr. Charles Eaton, there may be three types of Dupuytren's disease:[29]
- Type 1: A very 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
- 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. According to Eaton, this type may be made more severe by other factors such as diabetes or heavy manual labor.[29]
- 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.[29]
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.[citation needed]
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. As of 2016[update] 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.[31]
Needle aponeurotomy is most effective for Stages I and II, covering 6–90 degrees of deformation of the finger. However, it is also used at other stages. Collagenase injection is likewise most effective for Stages I and II. However, it is also used at other stages.[citation needed]
Hand surgery is effective at stage I to stage IV.[32]
Surgery
On 12 June 1831,
Because of high recurrence rates,[
In extreme cases, amputation of fingers may be needed for severe or recurrent cases or after surgical complications.[35]
Limited fasciectomy
Limited/selective
During the procedure, the person is under regional or
A 20-year review of surgical complications associated with fasciectomy 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.[41] 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.[39]
After surgery, the hand is wrapped in a light compressive bandage for one week. Flexion and extension of the fingers can start as soon as the anaesthesia has resolved. It is common to experience tingling within the first week after surgery.[38] Hand therapy is often recommended.[39] Approximately 6 weeks after surgery the patient is able completely to use the hand.[42]
The average recurrence rate is 39% after a fasciectomy after a median interval of about 4 years.[43]
Wide-awake fasciectomy
Limited/selective fasciectomy under
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[46])
- Recurrent disease.[37] Similar to a limited fasciectomy, the dermofasciectomy removes diseased cords, fascia, and the overlying skin.[47]
Typically, the excised skin is replaced with a
The graft is sutured to the skin surrounding the wound. For one week the hand is protected with a dressing. The hand and arm are elevated with a sling. The dressing is then removed and careful mobilization can be started, gradually increasing in intensity.[47] After this procedure the risk of recurrence is minimised,[37][47][48] but Dupuytren's can recur in the skin graft[49] and complications from surgery may occur.[vague][50]
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.[51]
The person is placed under regional anesthesia and a
The same procedure is used in the segmental fasciectomy with cellulose implant. After the excision and a careful hemostasis, the cellulose implant is placed in a single layer in between the remaining parts of the cord.[52]
After surgery people wear a light
Less invasive treatments
Studies have been conducted for percutaneous release, extensive percutaneous aponeurotomy with lipografting and collagenase. These treatments show promise.[53][54][55][56]
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.
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 may resume growing.
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 3 years.
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.[60]
Extensive percutaneous aponeurotomy and lipografting
A technique introduced in 2011 is extensive percutaneous aponeurotomy with lipografting.
Before the aponeurotomy, a
After the treatment the person wears an extension splint for 5 to 7 days. Thereafter the person returns to normal activities and is advised to use a night splint for up to 20 weeks.[54]
Collagenase
The cords are weakened through the injection of small amounts of the enzyme collagenase, which breaks peptide bonds in collagen.[55][61][62][63][56][excessive citations]
In February 2010 the US
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.
Radiation therapy
Radiation therapy has been used mostly for early-stage disease, but is unproven.[8] Evidence to support its use as of 2017[update], however, was scarce —efforts to gather evidence are complicated due to a poor understanding of how the condition develops over time.[8][31] It has only been looked at in early disease.[8] The Royal College of Radiologists concluded that radiotherapy is effective in early stage disease which has progressed within the last 6 to 12 months.[68]
Alternative medicine
Several alternate therapies such as vitamin E treatment have been studied, though without control groups. Most doctors do not value those treatments.[69] 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."[70][71]
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.[73]
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,[74] leading to variation in splinting approaches. Most surgeons use clinical experience to decide whether to splint.[75] Cited advantages include maintenance of finger extension and prevention of new flexion contractures. Cited disadvantages include joint stiffness, prolonged pain, discomfort,[75] subsequently reduced function and edema.
A third approach emphasizes early self-exercise and stretching.[45]
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.[30]
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.
Minimally invasive therapies may precede higher recurrence rates. Recurrence lacks a consensus definition. Furthermore, different standards and measurements follow from the various definitions.[citation needed]
Notable cases
- Chelsea Handler (born 1975), American comedian, actress and writer[77][78]
- Tim Herron (born 1970), American golfer[79]
- Prince Joachim of Denmark (born 1969)[80]
- Joanne Harris (born 1964), British author[81]
- Jonathan Agnew (born 1960), English cricketer[82]
- John Elway (born 1960), American football player[83]
- Nanci Griffith (1953–2021), American singer, guitarist, and songwriter[84][85]
- Bill Murray (born 1950), American actor and comedian[86]
- Bill Nighy (born 1949), English actor[87]
- Mitt Romney (born 1947), American politician[77]
- Misha Dichter (born 1945), American pianist[88]
- José Feliciano (born 1945), Puerto Rican musician, singer and composer[89]
- Bill Frindall (1939–2009), English cricket player and statistician, who had a finger amputated.[90]
- David McCallum (1933–2023), Scottish/British actor and musician[91]
- Paul Newman (1925–2008), American actor and film director[77]
- Margaret Thatcher (1925–2013), Prime Minister of the United Kingdom[92]
- Ronald Reagan (1911–2004), American President and actor[92]
- Andrew Wyeth (1917–2009), American visual artist[77]
- Frank Sinatra (1915–1998), American singer, actor, and producer[93]
- Samuel Beckett (1906–1989), Irish novelist, poet and playwright[77]
- Max Planck (1858–1947), German theoretical physicist and Nobel Prize laureate[77]
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