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Dupuytren's Contracture

Introduction

  • Progressive scarring and thickening of the connective tissue that causes the fingers to remain in a flexed position and prevents their extension
  • Most commonly occurs in the palm of the hand
  • Most commonly affects the ring and small fingers

Anatomy

Describe the pertinent anatomy

Pathogenesis

  • Like all fascia, the palmar fascia contains a population of resident fibroblasts that allow for connective tissue renewal. The pathophysiological process underlying Dupuytren's contracture is fibroblast proliferation and collagen deposition.
  • Although it is not known why this uncontrolled proliferation of palmar fascia occurs, it is thought to be related to increased expression of growth factors, including:
    • Basic fibroblast growth factor
    • Platelet-derived growth factor
    • Transforming growth factor-beta
  • This process typically involves the longitudinal and vertical components of the fascia, but at times seems to exist apart from anatomically distinct fascia. The skin overlying the sclerosed fascia may fuse with it and become raised, or it may atrophy, acquiring a puckered appearance.
  • It is speculated that in the beginning, an idiopathic over-proliferation of fibroblasts begins the process of scarring and contraction. The hand, exposed to unusual forces and non-physiological traction of the phalanges, likely develops reactive inflammation (hence the lymphocytic infiltration of the intermediate-stage lesions) and consequently contributes to the activation of scar tissue deposition, acting synergistically with the initial idiopathic disease process
  • The histopathology of the disease changes as the condition progresses:
    • Early disease shows nodules with a predominance of fibroblasts and type III collagen
    • A contractile phase follows, during which fibroblasts are replaced by myofibroblasts and lymphocytes infiltrate the tissue. In this phase, the concentration of proteoglycans greatly increases
    • Advanced disease is relatively acellular, with a predominance of type I collagen in tendon-like cords
  • Of note, histological staging is a reliable method for predicting recurrence.
Aetiology
  • The exact cause of Dupuytren's contracture is not known. The disproportionate prevalence of the condition in Northern Europeans suggests a genetic component.
    • Studies suggest an autosomal dominant pattern of inheritance with variable penetrance.
    • HLA-B7 and HLA-DR3 have been identified in a number of patients, suggesting a possible immunologic influence as well.
  • Some researchers suggest that Dupuytren's contracture may result from an error in growth and regulation of the fibroblast, resulting from chromosomal changes similar to those seen in cells undergoing neoplastic change. Trisomy 8 has been identified in the fibroblasts excised from some patients. In support of this theory, early nodules display features of a benign neoplasm.
  • Because the changes occurring in the palmar fascia approximate the normal stages of wound repair and remodeling, but to an excessive and detrimental extent, another theory holds that this disease is a response to traum.
    • Repeated exposure to compression, stress, and other mechanical forces may trigger a reparative process in the palmar fascia.
    • The reason for the progression to severe joint contractures remains unknown.
  • In the author's opinion, these theories are not mutually exclusive; I tend to believe that the contracture is a symptom associated with diverse disease processes and that the palmar fascia is particularly sensitive to these disease processes, thus it is disproportionately affected.
  • I believe it is analogous to a cough in the sense that it is a symptom, but just like a cough, can be due to cancer, infection, heart failure, etc. Dupuytren's contracture is due to any process that cause dysregulation of connective tissue remodeling. Its association with cancer favors a neoplastic-like process, while its association with trauma supports the aberrant wound healing theory.
  • Furthermore, I believe that metabolic imbalances (such as those created by diabetes mellitus and liver failure) may dysregulate the exceedingly complex tissue remodeling signaling cascades, leading to fibroblast over-proliferation and subsequent scarring. The inflammatory state established by those diseases further favors scarring.
Nutritional factors
  • Nutrition affects the disease, insofar as diabetes and alcoholism both affect the likelihood of developing the disease.
  • Some studies reported that taking 200-2,000 IU of vitamin E per day for several months was helpful in the treatment of Dupuytren's contracture, suggesting a role for antioxidants in slowing the progression of the fibrosing process. Other studies on the effect of vitamin E, however, did not obtain the same promising results.
Risk factors
  • Being of northern European descent is a risk factor in the development of the disease
  • Male
  • Older than 50 y/o
  • Medical conditions that increase the risk of developing the disease, including:
    • Alcoholism
    • Smoking
    • Liver failure
    • Epilepsy
    • Trauma
    • Diabetes
Prevention
  • There are no proven strategies to prevent Dupuytren's contracture, although the role of antioxidant vitamins (such as vitamin E) has been proposed.
  • In light of the existence of early treatments to slow or halt the progression of the disease, awareness in predisposed groups and early diagnosis/treatment may be the best "preventive" options.
Psychosocial impact of the disease
  • A variety of activities can be affected:
    • Work and career can be greatly affected if Dupuytren's contracture occurs in a younger person who is still workin
    • Although the ability to write is not as affected if only the fourth and fifth fingers are contracted, the disease can change a person's handwriting.
    • The disease can affect the ability to paint, cook, play golf, or participate in any sport that uses the hands.
  • Aesthetically, Dupuyten's contracture can be disfiguring and can greatly affect the person's confidence and self-esteem.

Natural History

  • The prevalence of Dupuytren's contracture varies according to the ethnic makeup of a given country. It is most common in northern Europe and the United Kingdom, as well as countries inhabited by immigrants from these areas, such as Australia, Canada, and the United States.
  • In the United States, A 10-year retrospective study using the Department of Veterans Affairs computer system was conducted to determine the racial distribution of this disorder.
    • Approximately 5-15% of males older than age 50 years are affected.
    • Dupuytren's contracture is less common in blacks and Asians (incidence = 3%) than in whites.
      • The characteristics of the disease in blacks are similar to those in whites - late onset; predominantly affecting the ulnar digits; associated with alcoholism, smoking, liver failure, epilepsy, trauma and diabetes.
      • An important difference hinting at a genetic component to the pathogenesis of the disease is that the disease is rarely bilateral in blacks.
    • The lowest incidence (<1%) was among Indians, Native Americans, and individuals of Hispanic descent
  • Approximately 80% of affected individuals are male in all races studied.
    • Disease onset is earlier in males than in females, and the course of the disease is more rapid and severe in males.
  • The mean age of disease onset in males is 49 years; in females, it is 54 years.
  • The mean age among men who seek surgery to treat this condition is also earlier, at 58 years. The mean age among females is 62 years.

Clinical Presentation

  • Dupuytren's contracture has an unpredictable rate of progression, but the earlier it starts in life, the more destructive and recurrent it is apt to be.
  • It manifests itself most commonly in the palm by nodular or cordlike thickening. The skin may fuse with the underlying fascia and become raised and hard, or it may shrink, sometimes drawn into a deeply puckered crevasse. The disorder invades the palm at the expense of fat, but is never adherent to vessels, nerves, or musculotendinous structures (although it may be adherent to flexor tendon sheaths).
  • Dupuytren's contracture may involve any digit or web space, but it affects predominantly the ring and small fingers. Over time, the cord contracts, resulting in digital flexion contracture and reduced hand function. In long-standing cases, the fingers may be drawn tightly into the palm, resulting in secondary contracture of the joint capsule and ligaments, flexor sheaths, and atrophic muscles.
  • The condition is not usually associated with pain
  • There is a paucity of studies on the specificity/sensitivity of the clinical findings, as Dupuytren's contracture is a diagnosis based on surface anatomy findings and associated functional impairment. The findings are consistent with a progressive scarring process
Late presentation
  • Dupuytren's contracture has early, intermediate, and late phases:
    • Early phase: loss of normal architecture and formation of skin pits
    • Intermediate phase: nodules and cords
    • Late phase: contractures
  • The late phase tends to go through four stages of contracture:
    • Stage I: contracture of the MCP joint of the ring finger 
    • Stage II: contracture of the MCP and PIP joints of the ring finger and the MCP joint of the small finger
    • Stage III: contractures of the MCP and PIP joints of the ring finger, the MCP and PIP joints of the small finger, and the MCP joint of the long finger
    • Stage IV: contractures, plus DIP joint hyperextension of the ring or small finger
  • This progression is not universal; in fact, palmar fascial disease or contracture remains confined to the palm and does not progress enough to cause digital flexion deformity.
  • Palmar involvement usually precedes extension of the disease into the digits; however, the disease may also begin and remain in the digits.
  • A palpable interdigital soft-tissue mass is an indication that the neurovascular bundle is involved, but it is not a reliable indicator.
  • Complications of the late stage of the disease involve QOL factors (see below).
Differential diagnosis
  • Trigger finger
    • Typically causes pain with flexion
    • Inability to extend the affected digit
  • Palmar tendinitis
  • Stenosing tenosynovitis
    • Characterized by pain
    • History of overuse or trauma
  • Ganglion cyst
    • A small, movable nodule (unlike a more fixed fibrous nodule of Dupuytren's)
    • Tender to palpation
    • Usually at the MCP joint
  • Soft-tissue mass
    • A soft-tissue mass must be excluded from the diagnosis, especially if the patient is significantly younger than the typical patient with Dupuytren's.
    • A patient younger than age 40 years without involvement of the dorsal hand, foot, or penis is unlikely to have Dupuytren's, and the concerning possibility of a sarcoma must be ruled out.
    • The pathologic findings of a biopsy may mistakenly pose as a benign aetiology, such as lipoma or an inclusion cyst.
  • A firm scar may also be a simple overuse callus, although the history would point to that.
  • The more advanced phase may be confused with a "hand of benediction," a neuropathic claw hand condition caused by median or ulnar nerve injuries.
    • Median claw hand will show hyperextension of the MCP and flexion at the IP joints of the 2nd and 3rd digits (index and middle fingers).
    • Ulnar claw hand will show hyperextension of the MCP and flexion at the distal and proximal (IP) joints of the 4th and 5th digits (ring and little fingers).
    • In both conditions, clawing will become most obvious during finger extension.
    • Unlike median and ulnar claw hands, MCP joints are flexed in Dupuytren's.
    • Also, unlike the aforementioned neuropathies, the muscles have normal strength in Dupuytren's, but are limited by scarring.

Imaging and Diagnostic Studies

  • Because Dupuytren's is a surface disease, imaging is not typically obtained. Research on imaging has shown, however, that MRI is accurate for detecting Dupuytren's contracture, defining its palmar involvement and depicting its extent.
    • The cords of intermediate and late disease have a uniformly low signal intensity similar to that of tendons on both T1- and T2-weighted images.
    • Nodules have an intermediate signal intensity similar to that of muscle on both T1- and T2-weighted images, with focal areas of lower signal intensity.
    • The signal characteristics of the lesions correlate with the degree of cellularity of the lesions as seen histologically.
    • Prognostically, highly cellular lesions tend to have higher rates of recurrence after surgery than do hypocellular lesions.
Laboratory evidence
  • There are no laboratory studies that are commonly done to detect or stage the disease. However, testing for associated conditions may be useful in addressing the patient's overall health:
    • CAGE screening for alcoholism
    • LFTs for liver failure
    • Fasting blood glucose or glucose leading test plus HbA1C for diabetes

Classification

Include a list with links to relevant conditions

Treatment

Conservative management
  • A number of non-surgical techniques have been attempted to treat Dupuytren's contracture, including:
    • Splinting
    • Stretching devices
    • Ultrasonic therapy
    • Steroids
  • None of these methods has been proven to benefit patients on a long-term basis.
Enzymatic fasciotomy
  • Enzymatic fasciotomy is performed with local injections of collagenase clostridium histolyticum (Xiaflex).
  • A randomized, double-blind, placebo-controlled trial has reported an 87% success rate in 62 joints treated during the placebo-controlled and open-label phases. Contracture recurrence was seen in five joints, one before 12 months, two at 12 months and two at 24 months follow-up. There were no serious treatment-related adverse reactions.
Radiotherapy
  • In early-stage Dupuytren's contracture, radiotherapy is applied to prevent disease progression.
  • Radiation therapy acts in a similar way as it does when applied to the treatment of sarcomas and other tumors.
Percutaneous needle fasciotomy
  • The recurrence rate for percutaneous needle faciotomy may be as high as 65%.
  • Some researchers have deemed this procedure an "alternative treatment for elderly patients with severe comorbidity or for those patients who do not want open surgery."
Surgical therapy
  • Surgery is indicated when the disorder has progressed sufficiently:
    • >30° of flexion at the MCP joint
    • Any flexion contracture of the PIP joint
  • Fasciectomy is the surgical procedure that gives the best long-term results, although in selected cases, subcutaneous fasciotomy done through a small longitudinal incision may release a contracture, indicated where:
    • Only the longitudinal pretendinous fascial band is involved
    • The skin moves freely over it
  • Unfortunately, the degree of contracture of the 5th digit may be so severe to require amputation.
Skin graft
  • The degree of atrophy of the skin overlying the contracture determines whether a skin graft is required for wound closure after fasciectomy.
  • A skin graft may have the added benefit of substituting the diseased overlying dermis, which has been implicated as an inductive mechanism in this process, thus lowering the recurrence rate in severe cases.
Postoperative management
  • Motion should be started within 3 to 5 days after surgery.
  • Dynamic splints and postoperative injection of corticosteroids into the joints and the operated areas may increase the patient's adherence to the physical therapy plan.
  • In the occasional case with acute and rapid onset of a tender nodule, local triamcinolone may be used for symptomatic relief.
  • DMSO (dimethyl sulfoxide) applied to the affected area may reduce pain by inhibiting transmission of pain messages, and may also soften the abnormal connective tissue. Its safety and effectiveness, however, remain unproven.

Outcome

  • In terms of early treatment, a study of long-term outcomes (13 years) of radiotherapy showed it is effective in preventing disease progression and improving patients' symptoms in the early stage.
  • Another study showed that regardless of the individual stage, radiotherapy led to stabilization of the disease in about 50% of patients (with an even greater percentage for earlier stages); 10% improved.
  • In case of disease progression after radiotherapy, a "salvage" fasciotomy is still feasible with good outcomes, despite the tissue changes associated with radiation therapy.
  • A retrospective study of the outcome of carpal tunnel release followed patients for a median of 10 months:
    • The outcome was considered good in 27% (pain, weakness, and numbness essentially resolved), fair in 42% (most of the symptoms improved), and poor in 32% (symptoms persisted or worsened).
    • Patients whose preoperative work activity was considered physically strenuous had a slightly but significantly poorer outcome (60% good or fair) compared to those in light work or with no employment (89% good or fair).
    • Proportionately fewer patients returned to their original work when they previously engaged in strenuous activity, ranging from 27% for those using air guns to 80% in light work.
    • The highest chance of a poor outcome from carpal tunnel release occurs in patients who have either associated symptoms of thoracic outlet syndrome or physically strenuous work activities.
  • Another study of postoperative management of Dupuytren's patients with prevention of applied mechanical tension in the early phases of wound healing showed that this intervention decreases complications after fasciectomy and that no digital motion is lost to this protective intervention.

Complications

  • The potential complications of surgery include:
    • Wound breakdown (loss of skin flaps)
    • Hematoma
    • Fibrosis
    • Stiffness
    • Digital nerve injury
    • Recurrence of contractures
    • Digital ischemia secondary to digital artery injury
  • Reflex sympathetic dystrophy, a painful, debilitating neurologic disorder of the hand, can occur after surgery and must be treated aggressively.
  • In general, the functional reward for the patient is great at any age.
  • A Cochrane review showed that although surgery can successfully correct deformity, it does not cure Dupuytren's and recurrence is common after surgery.
    • Recurrence rates following surgery - 27% to 34% within the first few years, between 41% and 71% after 5 years
  • The effectiveness of surgery when the contracture involves the MCP joint of only one finger is high. However, when two or more joints are involved complete correction is less likely.
  • At the PIP, the improvement rate is reduced. PIP contractures in isolated cords improved by 50% after surgery, but this improvement is often lost during the healing phase
  • Other postoperative complications of Dupuytren's contracture include:
    • Sympathetic flare
    • Hypertrophic scarring
    • Pain
    • Joint stiffness
    • Loss of digital extension
    • Reduction of function

Pearls and Pitfalls

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Controversy

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References


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