Dupuytren’s Contracture - Human & Disease

Dupuytren’s Contracture


DEFINITION

Dupuytren’s contracture is a benign, slowly progressive fibroproliferative disease of the palmar fascia that has no clear etiology or pathogenesis.


SYNONYMS

Dupuytren’s disease

Palmar fasciitis

Palmar fibromatosis

Viking disease

Dupuytren’s contracture is a benign, slowly progressive fibroproliferative disease of the palmar fascia that has no clear etiology or pathogenesis.    SYNONYMS  • Dupuytren’s disease  • Palmar fasciitis  • Palmar fibromatosis  • Viking disease
Dupuytren's-Contracture










ETIOLOGY

Dupuytren’s contracture is an autosomal dominant disease, characterized by fibroblast proliferation and collagen deposition. It is not known why the uncontrolled proliferation of palmar fascia begins and why it continues to the point of debilitating flexion contractures.

The pathognomonic feature of Dupuytren’s contracture is a firm nodule in the palm, often found near the distal palmar crease. Multiple nodules are common and may or may not be tender to palpation.

Nodules are a result of fibroblast and myofibroblast proliferation.The palmar skin becomes pitted and thick with the progressive contracture of the underlying subcutaneous tissue.

With disease progression, cords begin to develop proximal to the nodules.

Advanced disease is characterized by the regression of nodules and progression of MCP and PIP joint contractures. The fibrosis of the palmar fascia creates tendon-like cords adhering to underlying structures such as tendon sheaths.


EPIDEMIOLOGY AND 

DEMOGRAPHICS

Males are more likely to be affected than females.The male disease also tends to be more severe.

Dupuytren’s disease is very common in northern Europe, the United Kingdom and in countries inhabited by immigrants from these areas such asAustralia, Canada, and the US.

Approximately 5% to 15% of males older than 50 years of age are affected in the US, reflecting immigration from Northern Europe.

In Norway, approximately 5.6% of individuals older than 60 years are affected.

In Australia, 26% of males and 20% of females older than 60 years are affected.

Incidence of Dupuytren’s disease is less than 3% in blacks and Asians.

Among Indians, Native Americans, and individuals of Hispanic descent, the incidence of Dupuytren’s disease is less than 1%.

Increased severity of disease is associated with an earlier age of initial presentation.

Dupuytren’s contracture most often affects the ring and small fingers.


MECHANISM OF INJURY

There is no common physical reason to develop Dupuytren’s disease, since it has a genetic predisposition. However, it can be triggered by a fall onto the hand.


COMMON SIGNS AND 

SYMPTOMS

Nodules in the hand are normally not painful.

Flexion contracture of the MCP or PIP joints.

Dupuytren’s contracture is a benign, slowly progressive fibroproliferative disease of the palmar fascia that has no clear etiology or pathogenesis.    SYNONYMS  • Dupuytren’s disease  • Palmar fasciitis  • Palmar fibromatosis  • Viking disease
Dupuytren's-Contracture









AGGRAVATING ACTIVITIES

Patient with MCP contractures of 30 degrees or more complain of the

following:

 Difficultly shaking hands.

 Difficulty getting their finger in and out of pockets.


EASING ACTIVITIES

Attempts to stop the progression of this contracture via splinting have failed.


PAST HISTORY FOR THE REGION

Some research has shown trauma to the hand may trigger development of a contracture.


PHYSICAL EXAMINATION

Firm nodules that may be tender to palpation.

Painless, tendon-like cords proximal to the nodules.

Skin blanching on active finger extension.

Atrophic grooves or pits in the skin, denoting adherence to the underlying fascia.

Presence of MCP or PIP joint contractures.

Dupuytren’s contracture is a benign, slowly progressive fibroproliferative disease of the palmar fascia that has no clear etiology or pathogenesis.    SYNONYMS  • Dupuytren’s disease  • Palmar fasciitis  • Palmar fibromatosis  • Viking disease
Dupuytren's-Contracture







IMPORTANT OBJECTIVE TESTS

Table-top test

 Place the palm on a table. If the hand can flatten onto the table,then no surgical intervention should be needed. If the palm cannot reach the table, MCP or PIP joint contractures are demonstrated,indicating that surgical intervention should be considered.


DIFFERENTIAL 

DIAGNOSIS

Palmar tendinitis.


CONTRIBUTING FACTORS

Risk factors may include prior hand trauma, alcoholism, smoking, diabetes mellitus, and thyroid conditions.


TREATMENT

SURGICAL INDICATORS

MCP joint contracture of 30 degrees or more.When the MCP joint is involved, surgical intervention is not urgent because even long standing and severe contractures of the MCP joint are usually corrected readily after surgery and usually do not recur.

PIP joint contractures do not carry the same prognosis because more than one fascial band causes this contracture. Removing the involved fascia may not correct the joint contracture, particularly those of long duration. Patients should be informed that surgery can improve but may not completely correct the contracture.

Functional disability is a subjective symptom that may be an indication for surgery.It is essential the patient clearly understandsthe potential morbidity and that the process is occasionally exacerbated by the operation.


SURGICAL OPTIONS

The goal of surgical intervention is to excise the diseased fascia to make the hand functional for the patient. This treatment does not cure the disease but is meant to prevent progression to severe debilitating joint contractures.

Fasciotomy is a surgery performed with local anesthesia.A stab wound is made, which blindly cuts the contracted fascia. This procedure can be more thorough when an incision over the diseased cord is made to visualize and dissect the diseased fascia.

Regional fasciotomy involves removing the involved fascia and may provide short-term relief but is also associated with a very high recurrence rate. This procedure may correct an MCP joint contracture but almost certainly will not correct a PIP joint deformity. This procedure should be reservedfor elderly or debilitated patients who are unable to tolerate a more lengthy procedure.

Extensive fasciectomy involves removing as much fascia as possible,including the grossly normal.Today,this procedure is not commonly performed because of the increased associated morbidity, including hematoma risk and prolonged postoperative edema and stiffness.Some surgeons prefer to leave the skin wound open to heal by secondary intention as a means of decreasing hematoma risk.

Dermo-fasciectomy removes the diseased fascia and the overlying skin. Resurface the wound with a full-thickness skin graft. Recurrence rates are quite low with this approach. Because of the radical nature of this procedure, it is usually reserved for patients with recurrent or severe disease.


SURGICAL OUTCOMES

Long-term overall recurrence is approximately 50% and can be in the same area of the hand or in a new area.

MCP joint contractures are readily corrected with surgery and usually do not recur.

PIP joint contractures are usually not completely corrected and are occasionally exacerbated by surgery.


REHABILITATION

Postsurgical

 Preoperative hand therapy management of Dupuytren’s contracture with modalities or splinting is not supported by scientific evidence.

 Postoperative rehabilitation is a gradual process of increasing activity and decreased splinting to achieve optimal restoration of movement. Frequent visits to a hand therapist help restore preoperative flexion and maintain extension gained at the time of surgery is vital to the recovery process. Edema control, wound care, and scar management are also essential.

 A hand-based dorsal or volar splint is used to keep the fingers and hand open while the wound heals. This should be removed three times per day for AROM and PROM of the fingers.

 Postoperative splinting can also be used to statically progress remaining flexion contractures at the PIP joint.

 After surgery the splint must be worn 24 hours a day. It should only be removed for wound care and ROM. After 3 weeks, weaning from the splint may begin at the discretion of the hand therapist and surgeon. Be sure that the patient is provided with adequate oral analgesics to promote comfort and therapy compliance.

 Final results are realized in approximately 6 weeks. After this period, patients should wear the splint only at night for an additional 3 to 6 months, at the discretion of the hand therapist and surgeon. This is important to maintain extension and prevent scar contracture. Silicone gel sheets and scar massage are useful adjuncts to promote scar softening and maturation.


PROGNOSIS

Experiments are being performed with enzyme injections that may be able to break down the tough bands and improve motion without surgery. Early results are promising, but these injections are not available for general use at this time.


SIGNS AND SYMPTOMS 

INDICATING REFERRAL 

TO PHYSICIAN

If a painful lump is present, an injection from a physician may help diminish the pain.

If contractures have developed that cause the patient to lose functional use of the hand, the patient should be referred to a physician.


SUGGESTED READINGS:

1- Shaw RB Jr, Chong AK, Zhang A, Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis, and treatment. Plast Reconstr Surg. 2007;120(3):44e–54e.


2- Trojian TH, Chu SM. Dupuytren’s disease: diagnosis and treatment. Am Fam Physician. 2007;76(1):86-89.


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