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Dupuytren's Contracture

Dupuytren's contracture is a connective tissue disorder which causes thickening and shortening of palmar fascia of the hand, leading to fixed bending of the fingers towards the palm so that fingers cannot be fully straightened. Palmar fascia is the tissue under the skin of the palm, short and thick palmar fascia pulls and bends the fingers in toward the palm. It develops gradually and rarely causes pain. The Dupuytren's contracture can change the normal look of the hand and can make difficult to use affected fingers. Ring finger and small finger are the most commonly affected fingers, in advanced cases middle finger may be involved. Always, thumb and index finger are spared. Over a period of time, palmar fascia becomes hyper-plastic which restricts the movements of tendons connected to the fingers.

Incidence of Dupuytren's contracture increases after the age of 40 and occurs more often in men compared to women. Most of the times it affects both the hands and sometimes it may involve soles of the feet. The exact cause of the disease is not known, the probable cause might be hereditary tendency because it tends to happen in families. Risk factors for Dupuytren's contracture include alcohol, smoking and diabetes. Other suspected risk factors are trauma, anti-epileptic treatment with phenytoin and liver cirrhosis. Dupuytren's contracture has a high recurrence rates, especially in patients with Dupuytren's diathesis. Dupuytren's diathesis includes 4 factors: 1- history of Dupuytren's contracture in the family 2- patient aged below 50 years 3- involvement of both the hands 4- presence of ectopic lesions.

Signs and Symptoms-

Appearance of a small lump in the palm of the hand, usually at the junction of ring and small finger, is the first sign of Dupuytren's contracture. As disease progresses, a fibrous cord may develop in the palmar fascia. Usually, this cord extends to the ring or small finger and pulls fingers towards the palm causing Dupuytren's contracture. At some point, it may not be possible to fully extend the affected fingers or it may not be possible to flatten the hand on a table. Patient may find it hard to do things like washing hands, putting gloves or picking up things. The Dupuytren's contracture does not cause pain, in some cases pain appears in initial stages of the disease.

The palmar fascia, as a main function, increases the grip of the hand. In Dupuytren's contracture, hand loses ability to grip objects. In advanced stages of the disease, the palmar fascia becomes abnormally thick due to change of collagen type. Normal palmar fascia is of collagen type-1, in Dupuytren's contracture the collagen type-1 changes to collagen type-3. Collagen type-3 is a lot thicker than collagen type-1, therefore gradually contracture develops. Treatment is indicated when the table top test becomes positive.

Treatment-

The goal of the treatment is to keep or restore the hand function. Doing range-of-motion exercises may be able to keep hand function, in early stages of the disease. In some cases, splints or stretches are used to slow down the progress of the disease. Collagenase is the new medicine available to treat this disorder, collagenase injection dissolves tight tissue to some extent. If condition does not improve or disability continues, surgical release of the contracture is the best option. Non-surgical treatments such as Collagenase injections and Radiation therapy have shown promising results.

Surgical Procedures-

1- Limited Fasciectomy- The limited or selective fasciectomy is widely used procedure all over the world and it is considered as golden standard treatment for Dupuytren's contracture. Before the surgical procedure, surgical tourniquet is used to prevent blood flow to the hand. Under regional or general anesthesia, the skin is opened with a zig-zag incision and all diseased cords and fascia are excised. The cords and fascia should be excised precisely sparing neurovascular bundles. After the removal of all the tissue, the skin closed with stitches. For one week, hand should be wrapped in a light compressive bandage after surgery. As soon as anesthesia is resolved, the patient should start practicing bending and extending of fingers. After surgery, hand therapy is recommended and approximately after 6 weeks patient will be able to use the hand completely. Even after successful surgery, thick palmar fascia can develop again in the same place or in a new area of the hand.

2- Dermofasciectomy- This surgical procedure is indicated in recurrent Dupuytren's contracture. In dermofasciectomy, all the diseased cords and fascia are excised along with overlying skin and subcutaneous tissue. After the excision, the skin should be closed with a full thickness skin graft, which consists of epidermis and entire thickness of the dermis. Usually, the graft is taken from proximal inner side of the arm and the donor site can be closed with direct suture. The full-thickness graft is placed on the palm and sutured to the surrounding skin. After the surgery, hand should be protected with a dressing for one week, hand and arm should be elevated with a sling. After one week, dressing can be removed and patient can start careful mobilization of the hand. After two weeks of the surgery, the skin graft becomes stabilized and more intense mobilization can be achieved. After this procedure, the recurrence of the Dupuytren's contracture can be low.

3- Free Vascular Flaps- This surgical procedure is recommended in severe cases of Dupuytren's contracture, where the recurrence rate is too high.

Radiation Therapy-

Radiation therapy is a non-surgical treatment which has shown promising results while treating Dupuytren's contracture. In this therapy, low energy x-rays are used to prevent disease progression. Radiation therapy prevents disease progression effectively in early stages with mild or late side effects. With efficient dosage, the radiation therapy is given for 5 days in a row which will irradiate the nodules and cords. After the 5 days treatment, patient needs to weight for 6 weeks and again the 5 days course is repeated after 6 weeks. The initial proliferating stage of Dupuytren's contracture is most suitable for radiation therapy. The low energy x-rays prevent disease progression by affecting the development paths of the fibroblast and myofibroblast cells and reduce their growth rate. Slowing down of the growth of these cells can result in softening and shrinking of nodules.