Dupuytren's contracture
Encyclopedia
Dupuytren's contracture (not to be confused with Dupuytren fracture), (also known as morbus Dupuytren, Dupuytren's disease or palmar fibromatosis) 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. 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 tendon
s 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.
s 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.
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.
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.
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, 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. 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 going to the limb. The skin is opened with a Zig-Zag incision. 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 recommended. Approximately 6 weeks after surgery patients are able to completely use their hand.
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.
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.
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 studie showed 85% recurrence rate after 5 years.
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.
(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. The cords are weakened through the injection of small amounts of the enzyme collagenase, which breaks peptide bonds in collagen
.
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.
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 that severe as the primary disease. 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.
treatment of Dupuytren's contracture with low energy x-ray
s, has shown some promising results in trials lacking 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 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. 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.
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.
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.
Actors David McCallum
and Bill Nighy
, politicians Bob Dole
, Ronald Reagan
, and Margaret Thatcher
, playwright Samuel Beckett
, pianist Misha Dichter
, 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,
and Bill Frindall
, who did have a finger amputated.
Flexion
In anatomy, flexion is a position that is made possible by the joint angle decreasing. The skeletal and muscular systems work together to move the joint into a "flexed" position. For example the elbow is flexed when the hand is brought closer to the shoulder...
contracture
Contracture
A muscle contracture is a permanent shortening of a muscle or joint.. It is usually in response to prolonged hypertonic spasticity in a concentrated muscle area, such as is seen in the tightest muscles of people with conditions like spastic cerebral palsy....
of the hand
Hand
A hand is a prehensile, multi-fingered extremity located at the end of an arm or forelimb of primates such as humans, chimpanzees, monkeys, and lemurs...
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
Palmar aponeurosis
The palmar aponeurosis invests the muscles of the palm, and consists of central, lateral, and medial portions.-Central portion:The central portion occupies the middle of the palm, is triangular in shape, and of great strength and thickness....
of the hand. It is named after Baron Guillaume Dupuytren
Guillaume Dupuytren
Guillaume Dupuytren, Baron was a French anatomist and military surgeon. Although he gained much esteem for treating Napoleon Bonaparte's hemorrhoids, he is best known today for Dupuytren's contracture which is named after him and which he described in 1831.- Birth and education :Guillaume...
, the surgeon
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
who described an operation
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
to correct the affliction in the Lancet in 1831.
The ring finger
Ring finger
The ring finger is the fourth digit of the human hand, and the second most ulnar finger, located between the middle finger and the little finger. It is also called digitus medicinalis, the fourth finger, digitus annularis, digitus quartus, or digitus IV in anatomy.- Etymology :According to László A...
and little finger
Little finger
The little finger, often called the pinky in American English, pinkie in Scottish English , or small finger in medicine, is the most ulnar and usually smallest finger of the human hand, opposite the thumb, next to the ring finger.-Muscles:There are four muscles that...
are the fingers most commonly affected. The middle finger
Middle finger
The middle finger or long finger is the third digit of the human hand, located between the index finger and the ring finger. It is usually the longest finger...
may be affected in advanced cases, but the index finger
Index finger
The index finger, , is the first finger and the second digit of a human hand. It is located between the first and third digits, between the thumb and the middle finger...
and the thumb
Thumb
The thumb is the first digit of the hand. When a person is standing in the medical anatomical position , the thumb is the lateral-most digit...
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 tendon
Tendon
A tendon is a tough band of fibrous connective tissue that usually connects muscle to bone and is capable of withstanding tension. Tendons are similar to ligaments and fasciae as they are all made of collagen except that ligaments join one bone to another bone, and fasciae connect muscles to other...
s connected to the fingers cannot move freely. The palmar fascia becomes hyperplastic and contracts. Incidence
Incidence (epidemiology)
Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the...
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.
Symptoms
In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick which can cause the fingerFinger
A finger is a limb of the human body and a type of digit, an organ of manipulation and sensation found in the hands of humans and other primates....
s 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 diseasePeyronie's diseasePeyronie's Disease Peyronie's Disease Peyronie's Disease (also known as "Induratio penis plastica", or more recently Chronic Inflammation of the Tunica Albuginea (CITA), is a connective tissue disorder involving the growth of fibrous plaques in the soft tissue of the penis affecting up to 10% of men...
- curvature of the penis - Ledderhose disease - callus under the foot and possible curling under of toes
- Garrod's knuckleKnuckle padsKnuckle pads are circumscribed, keratotic, fibrous growths over the dorsa of the interphalangeal joints...
- pads on the back of knuckles of fingers
Risk factors
Dupuytren's contracture is a non-specific affliction, but primarily affects:- People of ScandinaviaScandinaviaScandinavia is a cultural, historical and ethno-linguistic region in northern Europe that includes the three kingdoms of Denmark, Norway and Sweden, characterized by their common ethno-cultural heritage and language. Modern Norway and Sweden proper are situated on the Scandinavian Peninsula,...
n or Northern EuropeNorthern EuropeNorthern Europe is the northern part or region of Europe. Northern Europe typically refers to the seven countries in the northern part of the European subcontinent which includes Denmark, Estonia, Latvia, Lithuania, Norway, Finland and Sweden...
an ancestry; it has been called the "Viking disease" or "Celtic hand," though it is also widespread in some Mediterranean countries (e.g. Spain and Bosnia) and in Japan; - Men rather than women (men are ten times as likely to develop the condition);
- People over the age of 40;
- People with a family historyFamily history (medicine)In medicine, a family history consists of information about disorders from which the direct blood relatives of the patient have suffered. Genealogy typically includes very little of the medical history of the family, but the medical history could be considered a specific subset of the total history...
(60% to 70% of those afflicted have a genetic predisposition to Dupuytren's contracture); - People with liver cirrhosis.
Some suspected, but unproven, causes of Dupuytren's contracture include trauma
Physical trauma
Trauma refers to "a body wound or shock produced by sudden physical injury, as from violence or accident." It can also be described as "a physical wound or injury, such as a fracture or blow." Major trauma can result in secondary complications such as circulatory shock, respiratory failure and death...
, diabetes, alcoholism
Alcoholism
Alcoholism is a broad term for problems with alcohol, and is generally used to mean compulsive and uncontrolled consumption of alcoholic beverages, usually to the detriment of the drinker's health, personal relationships, and social standing...
, epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...
therapy with phenytoin
Phenytoin
Phenytoin sodium is a commonly used antiepileptic. Phenytoin acts to suppress the abnormal brain activity seen in seizure by reducing electrical conductance among brain cells by stabilizing the inactive state of voltage-gated sodium channels...
, and liver disease
Liver disease
Liver disease is a broad term describing any single number of diseases affecting the liver.-Diseases:* Hepatitis, inflammation of the liver, caused mainly by various viruses but also by some poisons , autoimmunity or hereditary conditions...
. 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.
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.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. 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
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. 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. 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.
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.During the treatment the patient is under regional or general anesthesia. Surgeons use a surgical tourniquet
Surgical tourniquet
Surgical tourniquets enable surgeons to work in a bloodless operative field by preventing blood flow to a limb. This allows surgical procedures to be performed with improved precision, safety and speed...
to prevent blood flow going to the limb. The skin is opened with a Zig-Zag incision. 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 recommended. Approximately 6 weeks after surgery patients are able to completely use their hand.
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. 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
Subcutaneous tissue
The hypodermis, also called the hypoderm, subcutaneous tissue, or superficial fascia is the lowermost layer of the integumentary system in vertebrates. Types of cells that are found in the hypodermis are fibroblasts, adipose cells, and macrophages...
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
Epidermis
Epidermis may refer to:* Epidermis , in plants, the outermost layer of cells covering the leaves and young parts of a plant* Epidermis , the outermost layer of the skin of a human...
and the entire thickness of the dermis
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular 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.
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.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. 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
Surgical tourniquet
Surgical tourniquets enable surgeons to work in a bloodless operative field by preventing blood flow to a limb. This allows surgical procedures to be performed with improved precision, safety and speed...
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.
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. 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 studie showed 85% recurrence rate after 5 years.
Extensive Percutaneous Aponeurotomy and Lipografting
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 form 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.
Collagenase
In February 2010, the US Food and Drug AdministrationFood and Drug Administration
The Food and Drug Administration is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments...
(FDA) approved injectable collagenase extracted from Clostridium histolyticum
Clostridium histolyticum
Clostridium histolyticum is an anaerobic, motile, gram-positive bacterium that thrives in feces and soil. The ammonia and proteases it produces, including several collagenases, digest proteins outside its body into amino acids, which it eats. When Cl...
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
Pfizer
Pfizer, Inc. is an American multinational pharmaceutical corporation. The company is based in New York City, New York with its research headquarters in Groton, Connecticut, United States...
as Xiapex. The cords are weakened through the injection of small amounts of the enzyme collagenase, which breaks peptide bonds in collagen
Collagen
Collagen is a group of naturally occurring proteins found in animals, especially in the flesh and connective tissues of mammals. It is the main component of connective tissue, and is the most abundant protein in mammals, making up about 25% to 35% of the whole-body protein content...
.
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.
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 that severe as the primary disease. 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 therapyRadiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
treatment of Dupuytren's contracture with low energy x-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
s, has shown some promising results in trials lacking 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 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. 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.- QuercetinQuercetinQuercetin , a flavonol, is a plant-derived flavonoid found in fruits, vegetables, leaves and grains. It also may be used as an ingredient in supplements, beverages or foods.-Occurrence:...
- BromelainBromelainBromelain is a pineapple extract thought to be effective for reducing swelling , especially of the nose and sinuses, after surgery or injury. It may also be used for a variety of other effects that remain scientifically unconfirmed and not authorized by regulatory authorities like the Food and Drug...
- DMSODimethyl sulfoxideDimethyl sulfoxide is an organosulfur compound with the formula 2SO. This colorless liquid is an important polar aprotic solvent that dissolves both polar and nonpolar compounds and is miscible in a wide range of organic solvents as well as water...
- MSMMethylsulfonylmethaneMethylsulfonylmethane is an organosulfur compound with the formula 2SO2. It is also known by several other names including DMSO2, methyl sulfone, and dimethyl sulfone. This colorless solid features the sulfonyl functional group and is considered relatively inert chemically...
- AcetylcarnitineAcetylcarnitineAcetyl-L-carnitine or ALCAR, is an acetylated form of L-carnitine. It is a dietary supplement and naturally occurs in plants and animals.- Biochemical Production and Action :ALCAR is an acetylated derivative of L-carnitine...
Hcl - PABA4-Aminobenzoic acid4-Aminobenzoic acid is an organic compound with the formula H2NC6H4CO2H. PABA is a white grey crystalline substance that is only slightly soluble in water...
- NattokinaseNattokinaseNattokinase is an enzyme extracted and purified from a Japanese food called nattō. Nattō is a food made from fermented soybeans that has been eaten in Japan for many years. Nattō is produced by fermentation by adding the bacterium Bacillus natto, a beneficial bacteria, to boiled soybeans. The...
- Vitamin EVitamin EVitamin E is used to refer to a group of fat-soluble compounds that include both tocopherols and tocotrienols. There are many different forms of vitamin E, of which γ-tocopherol is the most common in the North American diet. γ-Tocopherol can be found in corn oil, soybean oil, margarine and dressings...
This was investigated in the 1940s - CopperCopperCopper is a chemical element with the symbol Cu and atomic number 29. It is a ductile metal with very high thermal and electrical conductivity. Pure copper is soft and malleable; an exposed surface has a reddish-orange tarnish...
- Vitamin CVitamin CVitamin C or L-ascorbic acid or L-ascorbate is an essential nutrient for humans and certain other animal species. In living organisms ascorbate acts as an antioxidant by protecting the body against oxidative stress...
- MassageMassageMassage is the manipulation of superficial and deeper layers of muscle and connective tissue to enhance function, aid in the healing process, and promote relaxation and well-being. The word comes from the French massage "friction of kneading", or from Arabic massa meaning "to touch, feel or handle"...
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. 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. 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.
Notable sufferers
- See :Category:People with Dupuytren's contracture
Actors David McCallum
David McCallum
David Keith McCallum, Jr. is a Scottish actor and musician. He is best known for his roles as Illya Kuryakin, a Russian-born secret agent, in the 1960s television series The Man from U.N.C.L.E., as interdimensional operative Steel in Sapphire & Steel, and Dr...
and Bill Nighy
Bill Nighy
William Francis "Bill" Nighy is an English actor and comedian. He worked in theatre and television before his first cinema role in 1981, and made his name in television with The Men's Room in 1991, in which he played the womanizer Prof...
, politicians Bob Dole
Bob Dole
Robert Joseph "Bob" Dole is an American attorney and politician. Dole represented Kansas in the United States Senate from 1969 to 1996, was Gerald Ford's Vice Presidential running mate in the 1976 presidential election, and was Senate Majority Leader from 1985 to 1987 and in 1995 and 1996...
, Ronald Reagan
Ronald Reagan
Ronald Wilson Reagan was the 40th President of the United States , the 33rd Governor of California and, prior to that, a radio, film and television actor....
, and Margaret Thatcher
Margaret Thatcher
Margaret Hilda Thatcher, Baroness Thatcher, was Prime Minister of the United Kingdom from 1979 to 1990...
, playwright Samuel Beckett
Samuel Beckett
Samuel Barclay Beckett was an Irish avant-garde novelist, playwright, theatre director, and poet. He wrote both in English and French. His work offers a bleak, tragicomic outlook on human nature, often coupled with black comedy and gallows humour.Beckett is widely regarded as among the most...
, pianist Misha Dichter
Misha Dichter
Misha Dichter is a classical pianist who was born in Shanghai to Polish-Jewish parents who fled Europe at the outbreak of World War II.-Biography:...
, 16th-century slave trader John Hawkins
John Hawkins
Admiral Sir John Hawkins was an English shipbuilder, naval administrator and commander, merchant, navigator, and slave trader. As treasurer and controller of the Royal Navy, he rebuilt older ships and helped design the faster ships that withstood the Spanish Armada in 1588...
, cricketers Jonathan Agnew
Jonathan Agnew
Jonathan Philip Agnew is an English cricket broadcaster and former professional cricketer. He was born in Macclesfield, Cheshire and educated at Uppingham School. He is nicknamed "Aggers", and, less commonly, "Spiro"....
, David Gower
David Gower
David Ivon Gower OBE is a former English cricketer who became a commentator for Sky Sports. Although he eventually rose to the captaincy of the England cricket team during the 1980s, he is best known for being one of the most stylish left-handed batsmen of the modern era. Gower played 117 Test...
, Graham Gooch
Graham Gooch
Graham Alan Gooch OBE DL is a former cricketer who captained Essex and England. He was one of the most successful international batsmen of his generation, and through a career spanning from 1973 until 2000, he became the most prolific run scorer of all time with 67,057 runs...
, wrongly reported in the Daily Mail as having had a finger amputated,
and Bill Frindall
Bill Frindall
William Howard Frindall, MBE was an English cricket scorer and statistician. He was familiar to cricket followers from his appearances on the BBC Radio 4 programme Test Match Special, nicknamed the Bearded Wonder by Brian Johnston for his ability to research the most obscure cricketing facts in...
, who did have a finger amputated.