|Last updated: Wednesday, December 30 2009
Dupuytren's disease is a slowly progressive condition that causes flexion contractures of the fingers and thumb. This means that the affected fingers (most often the small and ring) get pulled down toward the palm and the ability to straighten the fingers is lost. Surgery is the only widely accepted treatment for this condition. Dupuytren's disease is not dangerous in terms of the patient's general health but can interfere with the patient's ability to use their hands for work or recreation. In general surgery is useful once hand function becomes significantly compromised.
The nature of this condition the role of surgery and other interventions potential complications and recovery after surgery will be discussed below.
|Characteristics of dupuytren's disease
Dupuytren's disease or Dupuytren's contracture is a condition of the hands that causes both a loss of mobility and an abnormal position of the fingers and/or thumb. Patients with Dupuytren's disease develop abnormal lumps and "cords" of tissue in the palm and fingers. These lumps and cords represent an abnormal growth of the palmar fascia — the "gristle" like tissue normally present in the hand that holds the skin of the palm in place.
As the Dupuytren's cords tighten over time the joints of the fingers are pulled into flexion causing the fingers to be pulled down into the palm and impairing the ability to straighten the fingers. The thumb may also be affected though this is less common. The rate at which symptoms progress is highly variable among patients. In many patients the disease may be stable over a long period of time and require observation only. In others it may progress more rapidly and require earlier surgical intervention.
Patients with a strong family history and the presence of related conditions such as Peyronie's disease (abnormal fascial growth in the penis) Ledderhosen disease (a condition similar to Dupuytren's disease affecting the soles of the feet) and knuckle pads (lumps of abnormal tissue over the knuckles) are more likely to have more severe and rapid progression. As the condition progresses the flexion deformity can worsen and the patient can lose the ability to straighten out the affected fingers. The lumps and cords can also be painful. Figure 1 shows the typical appearance of a hand affected by Dupuytrens contracture.
TypesDupuytren's disease has not been classified into specific subtypes. There is however a wide variation in the degree to which patients may be affected. This ranges from very mild contractures of a single digit to severe contractures of several digits. Early on in the disease process affected individuals may only have a small lump of abnormal tissue in the palm (a Dupuytren's nodule). Some patient's are said to have a strong "Dupuytren's diathesis". This refers to patients with a strong family history and who may have associated conditions such as Peyronie's disease Ledderhosen disease and knuckle pads (described above). In these patients Dupuytren's disease is often more aggressive in nature.
Similar conditionsThere are few conditions that can be confused with Dupuytren's disease. Other conditions that may cause in inability to fully straighten the fingers include trigger finger (which occurs more commonly in patients with Dupuytren's disease) extensor tendon rupture (due to injury or arthritis) joint stiffness following injury and/or immobilization of the hand in a splint or cast joint stiffness due to arthritis injury to the ulnar nerve resulting in a ¡§claw hand¡¨ deformity or subluxation (slipping) of the extensor tendons between the knuckles in patients with arthtritis. However none of these conditions result in the palmar nodules and cords seen in Dupuytren's disease making the clinical diagnosis of Dupuytren's disease relatively straight forward. Confusion may occur when Dupuytren's disease and one or more of these other conditions are present in the same patient.
Incidence and risk factors
Dupuytren's disease is most common in Caucasian males over 50 years of age though it also occurs in women and in younger patients. It has also been shown to be more common in diabetics patients with seizure disorders HIV positive patients patients with hypothyroidism and in those who smoke and consume alcohol. Having blood relatives with the disease also increases a person's chance of being affected especially in those with northern European ancestory. However not all children of an affected individual will develop the disease and not all patients with the disease will have an affected family member.
When several factors such as these are known to be linked to a disease but none can be shown to be directly responsible the cause is said to be "multifactorial". This means that there are several things that can increase a chance that a person will be affected but a single cause is not known.
DiagnosisUsually affected patients notice an abnormal lump in their palm early in the disease process and present to their family doctor. The condition is usually recognized at this point prompting referral to a hand surgeon who can assess and follow the patient with regard to the need for surgical intervention. The diagnosis is based on the patients symptoms and the physical exam. There are no special tests such as x-rays other imaging studies or blood tests required to make the diagnosis.
Currently there are no medications used to manage Dupuytren's disease. However clinical trials evaluating the effectiveness of collagenase injections are underway. Collagenase is an enzyme that breaks down collagen the molecule that makes up the cords of Dupuytren's tissue in the hand. Initial studies have suggested that the majority of patients may derive benefit in terms of improved finger motion when treated with collagenase instead of surgery. The results of the current trials will shed more light on this new technique. It will also be important to assess the results over time in order to determine the rate of contracture recurrence.
Other non-surgical treatments that have been reported include radiotherapy ultrasound preoperative splinting topical and injected steroids dimethyl sulfoxide (DMSO) colchicine and gamma-interferon. None of these have shown sufficient benefit or become established treatment options for Dupuytren's disease.
ExercisesExercises have not been found to be an effective treatment for the contractures associated with Dupuytren's disease. However some surgeons may refer patients to a hand therapist for stretching of a severely involved finger prior to surgery though this is not common.
Possible benefits of palmar fasciectomy
In Dupuytren's disease the flexion contractures that cause disability are the result of an abnormal growth of the palmar fascia. This abnormal fascia arranges itself as "cords" which tighten over time pulling the affected fingers into an abnormal position. Surgery for Dupuytren's disease involves the removal or excision of this abnormal tissue. By removing the abnormal fascia the surgeon can often restore motion to the affected fingers. Sometimes the finger cannot be fully restored to normal and some flexion contracture persists however most patients experience an improvement in hand function after surgery.
|Types of surgery recommended
Surgery is the mainstay of treatment for Dupuytren's disease. Surgery is indicated once the patient and surgeon agree that the condition is causing significant problems for the patient and that these problems are likely to be improved by surgery. Sometimes only the most affected fingers will be operated on. If several fingers are severely affected then all of these may be operated on.
Surgery for Dupuytren's contracture is called "fasciectomy" because it involves the removal of the abnormal palmar fascia that causes the flexion deformity. The abnormal fascia is sometimes called "Dupuytren's tissue". Surgery is usually done on an outpatient basis which means that the patient will go home the same day of surgery. It can be done with the patient asleep (general anesthetic) or awake with the arm "frozen" (local or regional anesthetic). The decision as to which type of anesthetic to use is usually made by the patient and their surgeon or anesthetist. During the operation the surgeon makes incisions in the palm and the affected fingers. Several different types of incisions can be used (Figure 2). The surgeon removes the abnormal Dupuytren's tissue in order to correct the flexion deformity and allow the finger to extend. It is not always possible to achieve full correction depending on which joint is affected the severity of the deformity and the amount of time that the deformity has been present. Figures 3 and 4 show the appearance of hands after fasciectomy.
|Who should consider palmar fasciectomy?
Initially Dupuytren's disease is often observed for a period of time. This is because it often does not significantly interfere with hand function. The disease is not curable with surgical intervention and often recurs after surgery (about 50% of the time). Therefore it is not preferable to undergo surgery unless the condition is causing a significant problem with hand function or is causing persistent discomfort. Some guidelines for when surgery is warranted include flexion contracture >30 degrees at the metacarpal phalangeal joint (the first knuckle closest to the wrist) progressive flexion contracture at the proximal interphalangeal joint (the second knuckle) and persistent pain. These guidelines are not followed rigidly and generally surgery is warranted when the patient and surgeon feel that the condition is causing significant problems for the patient that are likely to be improved by surgery.
What happens without surgery?Often it is preferable to postpone surgery if the condition is not severe enough to interfere with the patients day to day activities. The condition may remain stable for long periods in which case no surgery is needed. In other cases the disease progresses more rapidly. It is difficult to predict how quickly the disease will progress in each affected patient.
Several different operations have been described for Dupuytren's disease. The most widely accepted is called partial palmar fasciectomy in which the surgeon removes the abnormal Dpuytren's tissue from the palm and the affected fingers. This can be done through a number of different incisions depending on the surgeon's preference. At the end of the operation all of the incisions may be sewn closed or some may be left open to allow drainage. Both are accepted methods.
Other approaches include: the removal of all of the fascia whether it is involved or not (total palmar fasciectomy); simply cutting the Dupuytren's cords in the palm (palmar fasciotomy) — a method associated with higher recurrence but often useful in patients severely debilitated by other conditions; collagenase injections — an experimental approach described above that is currently in clinical trials. Sometimes (though not commonly) skin is excised as well especially in very severe cases and replaced by skin grafts (thin pieces of skin taken from another area of the body and placed onto the open areas of the palm).
EffectivenessMost patients have an improvement in hand function after surgery for Dupuytren's disease. This is related to an improved ability to straighten or extend the fingers and decreased flexion contracture. The amount of benefit that a patient experiences is difficult to predict preoperatively but is related to the severity of the contracture being corrected and the joint involved. In general contractures of the proximal interphalangeal joint (PIP) are more difficult to correct. Also long standing contractures are more difficult to correct. The improved motion experienced by the patient usually persists for several years though approximately 50% of patients will have some degree of recurrence over a 5-10 year period after surgery.
UrgencySurgery is not urgent and it is not dangerous to the patient's general health to postpone surgery. In fact surgery should not be undertaken unless the contractures are causing significant impairment of the patient's ability to perform activities related to work or recreation. Occasionally surgery is undertaken to relieve the pain that is sometimes associated with Dupuytren's nodules or too facilitate personal hygiene in debilitated patients with severe contactures.
RisksThere are potential complications with any operation. In surgery for Dupuytrens disease these include: scarring infection hematoma (a collection of blood under the skin) skin necrosis (loss of skin due to poor blood supply) recurrence of disease finger stiffness requiring physiotherapy and injury to nerves and vessels. Nerve injury can cause finger numbness which is usually temporary but can be permanent. Vessel injury due to the accidental cutting of a vessel or stretching of vessels when the affected finger is straightened can cause loss of the entire finger. This has been reported in the medical literature but is rare.
Managing riskIf complications such as nerve or vessel injury occur during surgery and are recognized they are repaired at that time. It is uncommon for a nerve or vessel to be cut though this complication can occur. Vessels can also be damaged by straightening the finger during surgery causing stretching of the vessels which have become shortened due to the contracture. This can cause poor blood flow to the finger which usually improves over several minutes of relaxing the finger and applying warm sponges. When vessels are damaged the worst case scenario is loss of the finger though this is very rare. Post operatively if infection occurs this is usually managed with antibiotics either by mouth or intravenously depending on the severity of the infection. Occasionally an infection may require return to the OR for drainage. If skin necrosis occurs (death of an area of skin due to poor blood supply) this is usually managed initially with observation and later removal of the dead tissue. Often the resulting wound will heal in on its own with dressing changes or a skin graft may be required to achieve wound closure. Hematoma (a collection of blood under the skin) is managed with observation if the hematoma is small enough to absorb in a reasonable amount of time. Larger hematomas require drainage to prevent skin necrosis and infection. Recurrence of contracture is prevented with physical therapy and splinting though despite the best efforts some degree of recurrence is fairly common.
PreparationUsually patients are instructed not to eat or drink anything after midnight prior to the day of surgery. This is to ensure that the stomach is empty. If patients eat on the day of surgery and have a general anesthetic they risk vomiting and aspirating. This means that vomit enters the lungs — a complication that can cause serious infections and death.
TimingSurgery can safely be delayed until the disease has progressed to the point that it is interfering with the patients daily activities. At this point surgery is indicated if the surgeon feels that the patient would benefit.
The surgeon's office should provide a reasonable estimate of:
Surgical teamSurgery for Dupuytren's contracture should be performed by a surgeon with additional training in hand surgery. Hand surgeons can be orthopedic surgeons plastic surgeons or general surgeons who have received training in hand surgery.
Finding an experienced surgeonMost hand surgeons are familiar with the diagnosis and treatment of Dupuytren's disease. Hand surgeons are usually orthopedic surgeons plastic surgeons or general surgeons with extra training in hand surgery. In general primary care providers can arrange a referral to a qualified surgeon. The homepage of the American Society for Surgery of the Hand (ASSH) provides contact information for qualified hand surgeons (www.assh.org).
FacilitiesBecause patients can usually be discharged home on the day of surgery surgery for Dupuytren's disease can be performed in large hospitals or smaller OR settings.
Technical detailsPrior to surgery the surgeon will decide with the patient which fingers will be operated on. At the time of surgery a tourniquet (similar to a blood pressure cuff) is placed around the arm above the elbow. The arm and hand are prepared with an antimicrobial solution to decrease the risk of infection. After applying sterile drapes around the hand and arm the tourniquet is inflated to control bleeding in the hand. Next the surgeon will make incisions in the palm and in the affected fingers. The incisions are all on the palm side of the hand. Through these incisions the surgeon identifies and exposes the abnormal fascia (Dupuytren's tissue). During the dissection of the Dupuytren's tissue the arteries and nerves going to the fingers are identified and protected to prevent injury. All abnormal fascia is removed allowing improved motion of the affected finger. Sometimes it is necessary to further release structures around affected joints in order to achieve improved motion. The surgeon may make "Z" shaped incisions over areas of skin tightness to allow lengthening of the skin further improving finger motion. Next the tourniquet is deflated and bleeding controlled. The incisions are closed (though some may be left open to allow drainage) and a dressing and splint are applied.
AnestheticSurgery for Dupuytren's disease can be done with local or regional anesthetics (techniques to "freeze" the hand or limb) or general anesthesia ("going to sleep"). The decision as to what type of anesthetic to use is made by the patient anesthetist and surgeon and depends on the anticipated length of the procedure and the patients overall health.
Length of palmar fasciectomyThe length of the surgical procedure is directly related to the number of fingers involved and the severity of involvement. For this reason surgery can take anywhere from one to several hours.
Pain and pain managementPostoperative pain is usually manageable with oral (by mouth) medications. Usually patients will be given a prescription for a pain medication to take once they are discharged home. Narcotic medications are usually only required for the first few postoperative days. It is preferable to discontinue narcotic use and to take over the counter medications such as acetaminophen or ibuprofen once these medications provide sufficient pain relief. If patients are taking acetaminophen or ibuprofen as well as a narcotic medication it is important to know if the narcotic pill also contains acetaminophen or ibuprofen — otherwise patients may inadvertently take too much of one of these medications.
Important side effectsMost patients receive a prescription for a narcotic pain medication to take after surgery. There are several side effects to narcotic medications. These include but are not limited to: rash drowsiness nausea and vomiting itchiness constipation and serious allergic reactions (anaphylaxis). Patients cannot drive or operate machinery while taking narcotic medication. It is important for patients to inform their doctor if they have an allergy to narcotic medications. In general narcotics should only be used for the first few days after surgery and only when needed.
Hospital stayUsually the patient is placed in a soft dressing and a splint after the operation and referred to a hand therapist shortly after surgery. Patients are usually discharged home on the same day as their operation unless there are other complicating medical factors (eg. heart or lung disease) that require a period of observation in the hospital.
Convalescent assistancePatients can usually go home on the same day as their operation. Depending on their living situation most patients are able to function at home with the use of their non-operated hand. In the case of surgery on both hands patients may require some additional help at home.
Physical therapyAfter surgery for Dupuytren's disease hand therapy is important in order to maximize the benefits of the operation. Patients should be referred to and followed by a hand therapist who will instruct them in exercises to be done in the post-operative period. Special splints may also be used and may change as the time from surgery increases. Stitches are usually removed approximately 2 weeks after surgery. Some incisions may be left open to allow drainage. These will heal in on their own with daily dressing changes performed by the patient at home.
Rehabilitation optionsRehabilitation after surgery for Dupuytren's contracture consists of hand exercises and splinting. Generally the patient is referred to a hand therapist to guide them in this process. The exercises are designed to work on improved extension of the affected fingers through active finger motion and stretching. Splints are often used at night to hold the fingers in an extended position. These interventions are aimed at maintaining the improved finger motion obtained in surgery and lessening recurrence of contractures.
Can rehabilitation be done at home?Yes. The hand therapist is there to guide patients in their postoperative hand therapy but it is up to the patient to do most of the work! It is important to have a good relationship with the hand therapist and to ask a lot of questions so that the reasons for doing hand exercises and using splints are clearly understood.
Usual responseHand therapy after fasciectomy for Dupuytren's disease usually allows the patient to maintain most of the improved finger motion obtained at surgery. With time however approximately 50% of patients will have some amount of contracture recurrence.
RisksThere are no significant risks to hand therapy after surgery for Dupuytren's disease. Sometimes hand exercises will cause an incision in the hand to partially open up or may delay healing in an already open incision. This is not a significant concern and these areas will heal in on their own with daily dressing changes.
Duration of rehabilitationRehabilitation usually continues for several weeks after surgery and the use of night splints may continue for even longer. As long as the patient is receiving benefit from therapy the therapist will usually elect to continue following the patient. Although frequent visits with a therapist may not be necessary since patients can do most of their therapy on their own at home it is important to continue to be followed by a hand therapist until he/she feels that therapy is no longer necessary.
Returning to ordinary daily activitiesPatients are encouraged to use their hand early in the postoperative period. In general after the sutures have been removed (usually 2 weeks after surgery) patients can return to most activities. If some incisions have been left open the presence of dressings may interfere with daily work or recreational activities until the wounds have healed.
Long-term patient limitationsOnce patients have recovered and the period of rehabilitation is over there are no restrictions placed on the patient in terms of activities they can or can't do.
Summary of palmar fasciectomy for dupuytren's diseaseSeveral different factors including genetics are felt to be involved in the development of Dupuytren's disease. The condition cannot be prevented or cured and currently surgical treatment represents the standard of care. The condition can be quite stable over time or progress rapidly. Surgery is warranted when the contractures are severe enough that they interfere with the patient's daily activities or are painful. As with all operations there are potential complications. The condition may recur with time (approximately 50% of cases though not always requiring surgery again). Working with a hand therapist after surgery is vital to obtaining optimal results.
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