Dupuytrens Contracture

Article Author:
Joel Walthall
Article Author:
Prashanth Anand
Article Editor:
Uzma Rehman
Updated:
5/29/2020 2:25:16 PM
PubMed Link:
Dupuytrens Contracture

Introduction

Dupuytren's disease is predominantly a myofibroblastic disease that affects the palmar and digital fascia of the hand and results in contracture deformities. The most commonly affected digits are the third (ring) and fourth (small or pinky) 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. [1]

Dupuytren's contracture is a benign disorder that also includes Peyronie disease, Ledderhose disease, and Garrod's disease. 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. 

Etiology

Dupuytren's disease is a genetic disorder inherited as an autosomal dominant condition. It is associated with diabetes, seizure disorders, smoking, alcoholism, HIV, and vascular disease.[2] Occupation and activities have not been shown to be risk factors. Ectopic manifestations beyond the hand can be seen in Ledderhose disease (plantar fascia), 10% to 30%; Peyronie disease (Dartos fascia of the penis), 2% to 8%; and Garrod's disease (dorsal knuckle pads), 40% to 50%.

Epidemiology

This condition is most commonly seen in populations of Northern European/Scandinavian descent.[3] It is relatively uncommon in Southern European and South American populations and is rare in Africans and Asians. Males are affected by a 2:1 ratio compared to women, with the disease also affecting men more severely. Younger age of onset is also associated with increased severity of disease progression.

In Asians, the palm is more likely to be involved than the digits and thus it is often not noticed.

Pathophysiology

The pathophysiology of Dupuytrens disease involves abnormal myofibroblastic growth in the hand. Type III collagen predominates, which under a nondisease state would be Type I collagen. The numerous cytokines involved include interleukin-1, transforming growth factor beta 1, transforming growth factor beta-2, epidermal growth factor, platelet-derived growth factor, and connective tissue growth factor. Dupuytren's 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.

Transformation of the normal fascial bands into pathological cords is what causes unique deformities of the hand. Central cords originate from the pretendinous bands and cause skin pitting and metacarpal phalangeal (MCP) joint contracture. Natatory cords are responsible for webspace contractures. Spiral cords are the most important in the disease process and can cause proximal interphalangeal (PIP) contracture. A spiral cord originates from four main structures: (1) pretendinous band, (2) spiral band, (3) Lateral digital sheath, and (4) Grayson ligament.  The spiral cord also causes displacement of the neurovascular bundle centrally, superficially, and proximally.[4] Of note, the Cleland ligament and the transverse ligament of the palmar aponeurosis are not involved in Dupuytren disease.

Risk factors for increased severity and recurrence of disease after treatment include male gender, onset before age 50, bilateral disease, sibling/parent involvement, or the presence of Garrod pads, Ledderhose, or Peyronies diseases.

Histopathology

Histologic analysis reveals myofibroblasts and fibroblasts.[5] Myofibroblasts have contractile actin microfilaments which align with the long axis of the cell. Myofibroblasts interconnect themselves with fibronectin and extracellular fibrils. Type III collagen predominates in the extracellular matrix.

History and Physical

Dupuytren's disease starts as a palpable nodule in the palm, usually at the distal palmar crease. The nodules enlarge into cords and early in the disease, patients may present with just palpable cords along the palm.  As the cords thicken and shorten, they cause fixed flexion contractures of the fingers at the MCP and PIP joints. At this stage, patients typically present with loss of range of motion of the hand and palpable cords in the palm extending into the affected digits. Nodules, cords, and finger contractures are pathognomic of Dupuytren's disease.

The 4th digit is most often affected, followed by the 5th digit. Even when the disorder is bilateral, the severity may not be symmetrical. In most cases, palpation of the nodules does not elicit pain unless the ulnar nerve is compressed. Further, the nodules may become tender in the presence of tenosynovitis.

Physical findings:

  • 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 tabletop test (Hueston) is performed by having the patient attempt to place the palm flat on the exam table. If there is any flexion contracture deformity, the patient will be unable to straighten the fingers, resulting in a positive test. [6]

Evaluation

X-rays of the hand are not needed but may be obtained to examine for bony abnormalities like arthritis 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.

Treatment / Management

Indications for treatment are based on the effects of the 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 conservative management, needle aponeurotomy, collagenase injection, and/or surgical resection and fasciectomy.[7] 

Conservative management: 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.

Physical and occupational therapy including ultrasonic 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, especially those with painful nodules. Unfortunately, the steroid injections do not work in all patients and recurrence of up to 50% have been reported. More important, 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 the use of tamoxifen, anti-tumor necrosis factor agents, 5 fluorouracil, imiquimod, and botulinum toxin. There is no evidence that 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 aponeurectomy: Needle aponeurotomy is typically reserved for mild contractures. The procedure is minimally invasive and is often performed in an office setting. A needle is used to break up the cord. The digit is then manipulated, and the patient wears a night extension splint.  Metacarpal-phalangeal joint contractures have the greatest improvement. The benefit of this procedure is that it is minimally invasive and has the potential for immediate results. Disadvantages include iatrogenic injury to nerves and tendons and 58% recurrence rates[8].

Collagenase injection: Collagenase injections provide a minimally invasive treatment derived from Clostridium Histolyticum. The injected enzyme is a metalloprotease that lyses collagen (sparing Type IV collagen which is needed in the basement membrane of blood vessels and nerves). Treatment typically consists of 0.25 mL for MCP and 0.20 mL for PIP contractures delivered subcutaneously directly into the cord with a needle. The affected digit is manipulated under local anesthesia at 24 to 48 hours after injection. Night extension splinting is maintained for 6 months. Collagenase injections have been shown to result in a 75% contracture reduction with a 35% recurrence rate. Complications of injections include edema, skin tearing, tendon rupture, complex regional pain syndrome, and pulley rupture.

Surgery: Surgical fasciectomy can be either partial or total. Partial palmar fasciectomy entails the limited resection of diseased tissue within a ray.  Dissection generally is performed from proximal to distal as this usually allows for the identification of neurovascular structures before encountering the spiral cord. Incisions are customized to each patient in a Brunner zigzag pattern. Also, V-Y incision and Z-plasty can be used to lengthen contracted skin. Care must be taken to preserve the neurovascular bundles. 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 of the palmar and digital fascia, including nondiseased tissue. It is indicated in chronic digital cords, infiltrating disease, or recurrence after a partial surgical procedure.

Complications of fasciectomy include skin necrosis, hematoma (most common complication), flare reaction, neurovascular injury, digital ischemia, swelling, and infection. [9]

Irrespective of the treatment, recurrence is common with all of them, approaching 20-50% at 5 years and this should be a critical part of the preoperative discussion with the patient.

Differential Diagnosis

Dupuytren disease should be distinguished from other diseases of the hand including stenosing flexor tenosynovitis, ganglion cysts, and soft tissue tumors.

Prognosis

Dupuytren contracture has variable morbidity depending on the severity of the disease. Contractures of the PIP and MCP joint can interfere with daily living activities and lifestyles. In some patients, the nodules may be painful. In others, there may also be associated with Peyronie disease or involvement of the knuckle pads- these associations usually tend to be associated with severe disease.

Complications

Complications of surgical treatment include wound edge necrosis, hematoma, nerve injury, digital ischemia, infection, swelling, recurrence, and postoperative flare. Digital ischemia can be due to direct damage to the vascular supply of the digit or because of stretch injury to vessels in digits with a history of prolonged flexion contracture.

Dupuytren flare reaction is pain with diffuse swelling, hyperesthesia, redness, and stiffness. Treatment of this complication consists of steroids, cervical sympathetic block, therapy, and A1 pulley release. There is no increased risk of complex regional pain syndrome (CRPS) if carpal tunnel release is performed with fasciectomy. [10]

Postoperative and Rehabilitation Care

Postoperatively, patients are entered into hand therapy to help maintain the range of motion of the hand. Extension splints often are used in conjunction with other modalities.

The physical therapy should be undertaken for at least 3 months to prevent contractures. Maximal benefits of surgery are not immediate and only become obvious after 6-8 weeks.

Enhancing Healthcare Team Outcomes

The management of Dupuytren's contracture is with an interprofessional team that may consist of a dermatologist, orthopedic/hand surgeon, hand therapist, and the patient's primary care physician. There are multiple treatment options available. Open techniques offer more comprehensive removal of diseased tissue while directly visualizing critical neurovascular structures.  Injection based therapy has the benefit of being minimally invasive, but there is a greater risk of damage to surrounding structures and incomplete release of contractures. Recurrence of the disorder is common with all treatments, but highest with non-operative and injection-based options.

The follow up of these patients is usually by the treating surgeon and the patient's primary care provider.  The primary care physician can help the patient manage blood glucose, reduce alcohol consumption, and discontinue smoking.

Only symptomatic patients with limitations in motion should be offered treatment because all treatments have potential complications. Close communication between the healthcare team is essential in order to improve outcomes.  Hand therapists also play a pivotal role in restoring motion in the treatment of this disease.[11] (Level V)


References

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