INTRODUCTION:
- Dupuytren contracture is a progressive disease of the palmar fascia which results in shortening, thickening and fibrosis of the fascia and aponeurosis of the palm.
- Dupuytren disease is predominantly a myofibroblastic disease that affects the hand/fingers and results in contracture deformities.
- The most commonly affected digits are the third and fourth digits.
- The disease begins in the palm as painless nodules that form along longitudinal lines of tension.
- The nodules form cords that produce contracture deformities within fascial bands and tissues of the hand.
- Dupuytren contracture is usually seen in whites and the disorder is often bilateral; when unilateral the right side is more likely to be involved compared to the left.
- In many individuals, there is a family history with males being more likely to be affected than females.
Anatomy:
- The palmar aponeurosis (palmar fascia) invests the muscles of the palm, and consists of central, lateral, and medial portions.
- The central portion occupies the middle of the palm, is triangular in shape, and of great strength and thickness.
- Its apex is continuous with the lower margin of the transverse carpal ligament, and receives the expanded tendon of the palmaris longus.
- Its base divides below into four slips, one for each finger. Each slip gives off superficial fibers to the skin of the palm and finger, those to the palm joining the skin at the furrow corresponding to the metacarpophalangeal articulations, and those to the fingers passing into the skin at the transverse fold at the bases of the fingers.
- The deeper part of each slip subdivides into two processes, which are inserted into the fibrous sheaths of the flexor tendons. From the sides of these processes offsets are attached to the transverse metacarpal ligament.
- By this arrangement short channels are formed on the front of the heads of the metacarpal bones; through these the flexor tendons pass. The intervals between the four slips transmit the digital vessels and nerves, and the tendons of the lumbricales.
- At the points of division into the slips mentioned, numerous strong, transverse fasciculi bind the separate processes together.
- The central part of the palmar aponeurosis is intimately bound to the integument by dense fibroareolar tissue forming the superficial palmar fascia, and gives origin by its medial margin to the palmaris brevis.
- It covers the superficial volar arch, the tendons of the flexor muscles, and the branches of the median and ulnar nerves; and on either side it gives off a septum, which is continuous with the interosseous aponeurosis, and separates the intermediate from the collateral groups of muscles.
- Lateral and medial portions
- The lateral and medial portions of the palmar aponeurosis are thin, fibrous layers, which cover, on the radial side, the muscles of the ball of the thumb, and, on the ulnar side, the muscles of the little finger; they are continuous with the central portion and with the fascia on the dorsum of the hand
Pathophysiology:
- The pathophysiology of Dupuytren disease involves abnormal myofibroblastic growth in the hand.
- Type III collagen predominates, which under a nondisease state would be Type I collagen.
- Dupuytren contracture progresses through three phases: (1) proliferative, (2) involution, and (3) residual. The proliferative phase has a characteristically high concentration of immature myofibroblasts and fibroblasts arranged in a whorled pattern.
- In the involution phase, fibroblasts become aligned in the longitudinal axis of the hand following lines of tension. In the residual phase, relatively acellular collagen-rich chords remain causing contracture deformity.
- The disorder is not always progressive and in at least 50-70% of patients, it may stabilize or even regress.
- Several cords can develop which can cause unique deformities of the hand.
- Pretentious cords cause skin pitting and metacarpal phalangeal (MCP) joint contracture.
- Natatory cords are responsible for web space contractures.
- Spiral cords are the most important in the disease process and can cause proximal interphalangeal (PIP) contracture.
- Risk factors for increased severity and recurrence of disease after treatment include: male gender; onset before age 50; bilateral disease; sibling/parent involvement; presence of Garrod pads, Ledderhose, or Peyronies diseases.
Symptoms:
- This condition most commonly begins with thickening of the skin on the palm, resulting in a puckering or dimpled appearance.
- As the condition progresses, bands of fibrotic tissue form in the palmar area and may travel distal toward the fingers. This tightening and shortening eventually leads to the affected fingers being pulled into flexion.
- Dupuytren contracture typically occurs bilaterally, with one hand being more severely affected than the other.
- Physical findings:
- Early Signs of Dupuytren’s Contracture
- First, the skin on the palm of the hand starts to thicken. The skin might appear puckered as knots (nodules) of hard tissue begin to form on your palm. These nodules might feel tender to the touch, but they’re usually not painful. The thickening of the skin usually happens very slowly. You don’t need treatment unless your symptoms bother you.
- Blanching of the skin when the finger is extended
- Proximal to the nodules, the cords are painless
- Pits and grooves may be present
- The knuckle pads over the PIP joints may be tender
- If the plantar fascia is involved, this indicates more severe disease (Ledderhose disease)
- The patient may not be able to place the palm flat on the table
Diagnosis:
- X-rays of the hand should be obtained to examine for other contributing, bony abnormalities that may contribute to the loss of range of motion.
- Laboratory workup to rule out diabetes is recommended.
- Ultrasound may demonstrate thickened palmar fascia and the nodules.
- tabletop test: in which the patient put a hand, palm down, on a table to see if it lies flat. If it doesn’t, the patient may have a contracture.
Treatment
Medical Management:
- Indications for treatment are based on the effects of disease on the patient’s quality of life. Many patients with a positive tabletop test, MCP contracture of 30 degrees, or PIP contracture of 15 to 20 degrees will elect to have treatment.
- Treatment options consist of observation, needle aponeurotomy, collagenase injection, and/or surgical resection and fasciectomy.
- Observation is appropriate for individuals with painless stable disease and no impairment in function. Follow up every 6 months may be done to assess the progression of the disorder.
- Physio and occupational therapy including ultrasound waves and heat can help during the early stage of the disease. Some patients may also benefit from a brace/splint to stretch the digits. The range of motion of the fingers is necessary to prevent adhesions.
- Corticosteroid injections may be beneficial for some patients eg those with painful nodules. Steroid injections do not work in all patients and a 50% recurrence has been reported. Corticosteroid injections can lead to fat atrophy, pigmentation change and there is the potential to cause rupture of the tendons.
- Other treatments that have been tried include: tamoxifen; anti-tumor necrosis factor agents; 5 fluorouracil,; miquimod; botulinum toxin. No evidence exists to say any of these treatments are superior or work in everyone.
- Radiation therapy may work during the early phase of the disease only but is also associated with a significant number of complications.
- Needle aponeurotomy is typically reserved for mild contractures. The procedure is minimally invasive and is often performed in an office setting.
- Collagenase injections provide a minimally invasive treatment derived from Clostridium Histolyticum. Night extension splinting is maintained for 6 months. Collagenase injections result in a 75% contracture reduction with a 35% recurrence rate. Complications include edema, skin tearing, tendon rupture, complex regional pain syndrome, and pulley rupture.Before and after collagenase treatment.
- Surgical fasciectomy can be either limited or radical. The recurrence rate at 1 to 2 years is 30%, 15% at 3 to 5 years, and less than 10% after ten years.
- Total palmar fasciectomy can also be performed but is infrequently used as it requires resection of all palmar and digital fascia, including nondiseased tissue.
- Complications of fasciectomy include skin necrosis, hematoma (most common complication), flare reaction, neurovascular injury, digital ischemia, swelling, and infection.
- Irrespective of the treatment, recurrence is common with all of them, approaching 20-50% at 5 years.
Physiotherapy management:
- UltraSound is therapeutic modalities that generate ultrasound causes deep heat, provide micro-massage to soft tissue, increase flexibility, promotes healing of tissue as well improve localized blood supply to area.and ultimately pain relief.
- Heat can help relax and loosen tight musculature, prevents adhesion and maintain joint mobility.
- Stretching the shortened muscle can relax and lengthen this tight musculature that causes pain .brace/splint to stretch the digits; range of motion of the fingers to prevent adhesions.
- Try this stretch to help with pain relief and to improve the range of motion in your hands:Place your hand palm-down on a table or other flat surface.
- Gently straighten your fingers as flat as you can against the surface without forcing your joints.
- Hold for 30 to 60 seconds and then release.
- Repeat at least four times with each hand.
- Hold a soft ball in your palm and squeeze it as hard as you can.
- Hold for a few seconds and release.
- Repeat 10 to 15 times on each hand. Do this exercise two to three times a week, but rest your hands for 48 hours in between sessions. Don’t do this exercise if your thumb joint is damaged.
- Use this exercise to help increase the range of motion and flexibility in your fingers.Place your hand flat, palm down, on a table or other surface.
- Gently lift one finger at a time off of the table and then lower it.
- You can also lift all your fingers and thumb at once, and then lower.
- Repeat eight to 12 times on each hand.
- This exercise helps increase the range of motion in your finger.Start with your hand out in front of you, palm up.
- Extend your thumb away from your other fingers as far as you can. Then bend your thumb across your palm so it touches the base of your small finger.
- Hold for 30 to 60 seconds.
- Repeat at least four times with both thumbs
- Maintain the range of motion of the hand and fingers is important (for many activities of daily living),
- Extension splints often are used in conjunction with other modalities.
- Odema and scar interventions.
- Should be undertaken for at least 3 months to prevent contractures.
- Maximal benefits of surgery are not immediate, only become obvious after 6-8 weeks.
- Within the initial 5 days postoperative, the primary interventions are to educate the patient on decreasing edema and the importance of performing a range of motion exercises on the uninvolved fingers.
- After 5-7 days postoperative, the primary interventions shift to a range of motion exercises and splinting.
- The exercises are adapted to each subject’s individual goals and are based on their impairment, physical status, and competency.
- The types of splints used included volar splints, dynamic extension splint, dynamic flexion splints, exercise splints, and wrist splints.
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