Dupuytren’s Contracture

Introduction

Dupuytren contracture is a benign myeloproliferative [1]progressive disease of the palmar fascia which results in shortening thickening and fibrosis of the fascia and aponeurosis of the palm.

• Dupuytren’s disease is primarily a myofibroblastic disorder affecting the hands/digits and causing contracture deformities.
• Usually the numbers affected are those furthest from the thumb, the fourth (ring) and fifth (pink) numbers.
• The disease begins on the palms as painless nodules that form along longitudinal lines of tension.
• The nodules form cords that create contracture deformities within the fascial bands and tissues of the hand.
• Dupuytren’s contracture is common in Caucasians, and the disease is usually bilateral. When unilateral, the right side is more likely to be affected than the left side.
• Many people have a family history, and men are more prone to the disease than women. [2][3]

Figure 1: Clinical presentation of Dupuytren’s contracture [4]

Etiology

Dupuytren’s disease is a genetic disorder expressed in an autosomal dominant manner, but most commonly has a multifactorial etiology. It is associated with diabetes, epilepsy, smoking, alcoholism, HIV, and vascular disease [5].

Ectopic manifestations other than the hands can be seen in plantar fibromatosis (plantar fascia) in 10% to 30%; Peyronie’s disease (Dartos fascia of the penis) in 2% to 8%; and Garrod disease (dorsal knuckle pads) ) 40% to 50%. [5]

Epidemiology

This condition is common in people of Nordic/Scandinavian ancestry. It is relatively uncommon in southern European and South American populations, and it is also rare in Africans and Asians. The disease affects men more severely than women. Males are affected 2:1 compared to females. Younger age of onset was also associated with increased severity of disease progression. In Asian populations, the palms are more affected than the fingers and therefore are often overlooked. [5]

Pathological Process

The pathophysiology of Dupuytren’s disease involves abnormal myofibroblast growth in the hands.

• Type III collagen predominates and in the non-disease state will be type I collagen.
• Dupuytren’s contracture goes through three phases: (1) proliferative phase (2) involutional phase and (3) residual phase. The proliferative phase has a characteristic high concentration of immature myofibroblasts and fibroblasts arranged in a wheel. During the degenerative phase, fibroblasts are aligned Follow the tension line on the longitudinal axis of the hand. In the residual stage, the relatively decellularized collagen-rich chordae still lead to contracture deformities.
• The disease is not always progressive and may stabilize or even regress in at least 50-70% of patients.

Several cords can form, which can cause a unique deformity in the hand.

• Fake cords can cause dimpling of the skin and contractures of the metacarpophalangeal (MCP) joints.
• Swimming ropes are responsible for cyberspace contractures.
• The helical cord is most important in the disease process, causing proximal interphalangeal (PIP) contractures.

Risk factors for increased disease severity and recurrence after treatment include: male sex; onset before age 50 years; bilateral disease; sibling/parental involvement; presence of Garrod pads Ledderhose or Peyronies disease. [2]

Clinical Presentation

Dupuytren’s contracture occurs slowly and usually progresses over several years, but can also develop more rapidly over weeks or months. [6]

It usually affects older males of European descent. The condition most commonly begins with thickening of the skin on the palms, resulting in a wrinkled or sunken appearance. As the disease progresses, bands of fibrotic tissue develop in the palmar region and may migrate distally to the fingers. This tightening and shortening eventually causes the affected finger to be pulled into flexion. Dupuytren’s contracture is usually bilateral, with one hand being more severely affected than the other.

Physical findings:

• whitening of the skin when the fingers are stretched
• The line close to the nodule is painless
• Pits and grooves may be present
• Knuckle pads on PIP joints may be soft
• If the plantar fascia is involved, it indicates a more serious disorder (plantar fibromatosis)
• Patients may not be able to keep their palms flat on the table [2]

Diagnostic Procedures

• X-rays of the hands should be taken to check for other skeletal abnormalities that could lead to loss of range of motion.
• Laboratory tests are recommended to rule out diabetes.
• Ultrasound may show thickening and nodules of the palmar fascia. [2]

Differential Diagnosis

Dupuytren’s disease should be distinguished from other disorders of the hand, including narrow flexor tenosynovitis, ganglion cysts, and soft tissue masses [2].

Outcome Measures

• Range of motion measurements of the metacarpophalangeal (MCP) proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints (measurements of flexion and extension and passive and active range of motion of these joints) should be recorded. as a baseline measurement and then throughout the The course of treatment can help stage the severity of the contracture.
• Hand function is measured with tests and measures such as the Disability of the Arm Shoulder and Hand Questionnaire (DASH) or its shorter version, the Quick Dash.

Medical Management

Indications for treatment are based on the impact of the disease on the patient’s quality of life. Many patients who test positive for a MCP contracture of 30 degrees or a PIP contracture of 15 to 20 degrees on the table test will opt for treatment.

Treatment options include observational needle aponeuriotomy with collagenase injection and/or surgical resection and fasciectomy.

Observation is appropriate for individuals with painless stable disease and no functional impairment. Follow-up can be done every 6 months to assess disease progression.

• Physical and occupational therapy, including ultrasound and heat therapy, can help in the early stages of the disease. Some patients may also benefit from a brace/splint to stretch the finger. Range of motion of the fingers is necessary to prevent sticking.
• Corticosteroid injections may benefit some patients, such as those with painful nodules. Steroid injections are not effective in all patients and a 50% relapse rate has been reported. Corticosteroid injections can cause lipoatrophic hyperpigmentation changes and possibly skin breakdown tendon.
• Other treatments that have been tried include tamoxifen; anti-tumor necrosis factor agents; 5-fluorouracil imiquimod; botulinum toxin. There is no evidence that any of these treatments are superior or work for everyone.
• Radiation therapy may only be effective in the early stages of the disease, but is also associated with numerous complications.
• Needle aponeurotomy is usually used for mild contractures. The procedure is minimally invasive and usually performed in an office setting.
• Collagenase injections offer a minimally invasive treatment derived from Clostridium histolyticum. The nightly extended splint was maintained for 6 months. Collagenase injections resulted in a 75% reduction in contractures and a 35% reduction in recurrence. Complications include edema skin tear tendon rupture Complex regional pain syndrome and pulley breaks. Images before and after collagenase treatment at R [7]
• Surgical fasciectomy can be limited or radical. The recurrence rate in 1 to 2 years is 30% to 15% in 3 to 5 years, and the recurrence rate in 10 years is less than 10%.
• A total palmar fasciectomy can also be performed, but is rarely used because it requires removal of all palmar and digital fascia, including non-diseased tissue.
• Complications of fasciectomy include skin necrohematoma (the most common complication), flare reactions, neurovascular injury, ischemic swelling of the digits, and infection.

Regardless of the treatment modality, the recurrence rate at 5 years is approximately 20-50%. [2]

Physical Therapy Management

Conservative Approach

Physical therapy may include ultrasound: heat (early stages of disease); braces/splints to stretch the finger; range of finger motion to prevent adhesions.

Postoperative Care/Rehabilitation

Patients often come into hand therapy to:

• Maintaining range of motion in the hands and fingers is important (for many activities in everyday life) see Hand Exercises
• Extended splints are often used in combination with other modalities.
• Odema and scar interventions.[8]
• It should be done for at least 3 months to prevent contractures.
• The greatest benefit of surgery will not be seen immediately, it will only become apparent after 6-8 weeks. [2]

The standard protocol for postoperative management of Dupuytren’s disease is shown below (Engstrand et al., 2009). [8]

• During the first 5 postoperative days, the main intervention is to educate the patient on the importance of reducing edema and performing a range of motion exercises on the unaffected fingers.
• After 5-7 days postoperatively, the main intervention shifts to a series of movement exercises and splints.
• The exercises are tailored to each subject’s individual goals and based on their impaired physical condition and abilities.
• The types of splints used include volar splints, dynamic extension splints, dynamic flexion splints, sports splints, and wrist splints.

The video below provides a good summary of the condition and physical therapy (less than 4 minutes)

[9]

CONCLUSION

The key fact to note is that not all patients require treatment.

• There are many treatments for Dupuytren’s contracture, but none are ideal or consistently effective.
• Treatment should only be offered to symptomatic patients, as all treatments have complications.
• Patients must be made aware of potential complications of treatment, which are more serious than the disease itself.
• Close communication between teams is critical to improving results.
• Overall, only a small number of patients achieve desirable results.
• In many cases, prolonged physical therapy is required to restore function [2]