High Yield Topics

Dupuytren’s Contracture

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Ovid: 5-Minute Sports Medicine Consult, The


Dupuytren's Contracture
Thomas Trojian
Basics
Description
  • Contracture of the palmar fascia owing to fibrous proliferation and resulting in flexion deformities and loss of function. Similar changes rarely may occur in plantar fascia. Dupuytren contracture (DC) usually appears simultaneously.
  • Genetics:
    • Autosomal dominant with variable penetrance
    • 10% of patients have a positive family history.
  • System(s) affected: Musculoskeletal
  • Abbreviations: MP = metacarpophalangeal; PIP = proximal interphalangeal
Epidemiology
Incidence
  • Predominant age: 50 yrs for males; 60 yrs for females
  • Predominant gender: Male = Female (ranges from 2:1 to 10:1)
Prevalence
  • Prevalence is about 306/100,000.
  • Disease prevalence varies from 2–42% depending on population and risk factors.
  • Overall prevalence is 8.8% in Northern European population.
  • African Americans are less likely to get the disease.
  • Japanese and Taiwanese have a prevalence equal to that in Northern Europe. These patients usually present with a less severe form of the disease.
Risk Factors
  • Smoking (mean 16 pack-years; odds ratio = 2.8)
  • Alcohol intake
  • Both alcohol and cigarette use has a higher risk than individual use.
  • Increasing age (>40 yrs of age)
  • Male (increase in androgen receptors in nodules)
  • Northern European, Japanese, Taiwanese
  • Diabetes mellitus (1/3 affected, increases with time, usually mild; middle and ring fingers involved)
  • Epilepsy (may be medication or disease)
  • HIV infection (may or may not be associated)
  • Vibration work exposure (Some studies show increased risk, and other studies show no risk.)
  • Rheumatoid arthritis (lower risk)
Genetics
  • Autosomal dominant with variable penetrance; in one family, the gene has been mapped to chromosome 16q.
  • >30 unique genes were upregulated, and 6 unique genes were downregulated by 4-fold or greater.
  • Upregulated genes include ones that code for fibronectin, tenascin C, transforming growth factor β2 (TGF-β2), collagen III, collagen IV, and collagen VI.
  • All 3 forms of TGF-β are upregulated. TGF-β2 is upregulated 10–20 times. As in tendinopathy, it may be a TGF receptor lack of response, so TGF continues to be made.
  • Musculoaponeurotic fibrosarcoma oncogene homologue B, or MafB, in the family of oncogenes is found in the cord but not paracord fascia of normal control individuals.
General Prevention
None known; avoid risk factors when possible.
Etiology
  • Unknown
  • Ischemia to the fascia with oxygen free-radical formation
  • Possibly related to release of angiogenic basic fibroblast growth factor
  • Related to microhemorrhage and release of growth factors
Commonly Associated Conditions
  • Alcoholism
  • Smoking
  • Epilepsy
  • Diabetes mellitus
  • Occupational hand trauma (vibration white finger)
Diagnosis
Physical Exam
  • Signs and symptoms:
    • Typical:
      • Caucasian male aged 50–60 yrs
      • Bilateral with one hand more involved
      • Family history
      • Unilateral or bilateral (50%)
      • Right hand more frequent
      • Ring finger more frequent
      • Ulnar digits more affected than radial
      • Mild pain early
      • Later painless plaques or nodules in palmar fascia
      • Extends into a cordlike band in the palmar fascia
      • Skin adheres to fascia and becomes puckered.
      • Nodules can be palpated under the skin.
      • Digital fascia becomes involved as disease progresses.
      • Web space contractures
      • Dupuytren diathesis can involve plantar (Ledderhose 10%) and penile (Peyronie 2%) fascia.
      • Knuckle pads
    • Atypical:
      • No age, gender differences
      • No family history
      • May have systemic disease (see “Risk Factors”)
      • May have a history of trauma
      • More common unilateral
      • No ectopic manifestations (Ledderhose or Peyronie)
      • Nonprogressive
  • Physical examination:
    • A firm nodule in the palm of the hand proximal to the metacarpophalangeal (MCP) joint
    • Hueston tabletop test
    • Test is positive if patient is unable to flatten his or her hand on the table.
Diagnostic Tests & Interpretation
Lab
  • Drugs that may alter lab results: N/A
  • Disorders that may alter lab results: N/A
Imaging
  • Not needed except in rare cases
  • US can be used in diagnosis.
  • MRI can assess cellularity of lesions, which correlate with higher recurrence after surgery.
Diagnostic Procedures/Surgery
Rarely is a biopsy needed to differentiate Dupuytren nodule from a soft tissue tumor.
Pathological Findings
  • Myofibroblasts
  • 1st stage (proliferative): Increased myofibroblasts
  • 2nd stage (residual): Dense fibroblast network
  • 3rd stage (involutional): Myofibroblasts disappear.
Differential Diagnosis
  • Callus formation
  • Nodule on flexor tendon
  • Camptodactyly—early teens tight facial bands ulnar side of small finger
  • Soft tissue tumor

P.133


Treatment
  • ∼10% of patients will have disease regression without treatment.
  • Intralesional steroid injection (triamcinolone) has been shown to reduce the need for surgery.
  • Although surgical intervention has been shown to be very helpful in cases of advanced DC, there are inherent risks to surgery, such as nerve injury, hematoma, skin necrosis, and infection.
  • Although partial fasciectomy has long been the mainstay of treatment for patients with advanced DC, office procedures such as percutaneous needle aponeurotomy and collagenase injections may represent alternatives to surgery in certain patients.
  • Whatever the method selected to treat the contracture, each technique carries with it a significant risk of recurrence.
Medication
First Line
Steroid injection (triamcinolone) for an acute tender nodule, painful knuckle pad
Second Line
Radiation therapy, 5-fluorouracil, and hyperbaric oxygen have been used.
Additional Treatment
General Measures
  • Steroid injection for acute tender nodule
  • Physiotherapy is ineffective alone.
  • Isolated involvement of palmar fascia can be followed.
  • MCP joint involvement can be followed if flexion contracture is 30 degrees.
Additional Therapies
Collagenase injections to the nodules and cords under US guidance may be approved for use. Phase III clinical trials have been completed.
Surgery/Other Procedures
  • Surgery: Partial fasciectomy:
    • Surgical referral should be made when MCP joint contractures reach 30 degrees or any degree of PIP joint contracture develops.
    • May require skin grafts for wound closure with severe cutaneous shrinkage
    • 80% have full range of movement if operated on early.
    • Continuous elongation technique is useful to prepare a severely contracted PIP joint for surgery. The digit frequently can be completely extended but will relapse if surgery is not performed.
    • Amputation of little finger, if severe and deforming (rare)
  • Needle aponeurotomy (NA), also called percutaneous needle fasciotomy (PNF), is a minimal invasive technique.
    • Done under local anaesthetic
    • Type I DC
    • Done through the skin usually blind with 18-gauge or special tool
    • US may be beneficial.
    • Recurrence rates can be high, but recovery is faster and procedure is inexpensive.
Ongoing Care
Follow-Up Recommendations
  • No activity restrictions
  • Physical therapy after surgery: Started 3–5 days after surgery (passive and active exercises, posterior dynamic extension splints)
Patient Monitoring
Follow patient in early stages of disease.
Diet
No special diet
Patient Education
  • Avoid risk factors, especially with a strong family history.
  • Regular follow-up by physician every 6 mos to 1 yr
Prognosis
  • Typical:
    • Unpredictable but usually slowly progressive
    • Patients likely to have aggressive disease (one or more): age 40 at onset, knuckle pads, positive family history, bilateral disease involving radial side of hand
    • Reports of clinical regression with continuous passive skeletal traction in extension and under a skin graft
    • Recurrence rate after surgery is 10–34%.
    • Prognosis better for MCP joint vs PIP joint after surgery.
  • Atypical:
    • Nonprogressive
    • Surgery rarely needed
    • Recurrence unlikely if surgery performed
Complications
  • Postoperative stiffness (common)
  • Postoperative recurrence or extension 46–80% (common)
  • Postoperative development of reflex sympathetic dystrophy (uncommon)
  • Postoperative hematoma or infection (rare)
  • Intraoperative neurovascular injury (rare)
  • Digital infarction (rare)
Additional Reading
Rayan GM. Dupuytren's disease: anatomy, pathology, presentation, and treatment. Instr Course Lect. 2007;56:101–111.
Burke FD, Proud G, Lawson IJ, et al. An assessment of the effects of exposure to vibration, smoking, alcohol and diabetes on the prevalence of dupuytren's disease in 97,537 miners. J Hand Surg Eur Vol. 2007;32:400–406.
Hueston J. Dupuytren's contracture. J Hand Surg [Br]. 1993;18:806.
Rayan GM. Dupuytren's disease: anatomy, pathology, presentation, and treatment. Instr Course Lect. 2007;56:101–111.
Saboeiro AP, Porkorny JJ, Shehadi SI, et al. Racial distribution of Dupuytren's disease in Department of Veterans Affairs patients. Plast Reconstr Surg. 2000;106:71–75.
Shaw RB, Chong AK, Zhang A, et al. Dupuytren's disease: history, diagnosis, and treatment. Plast Reconstr Surg. 2007;120:44e–54e.
Thurston AJ. Dupuytren's disease. J Bone Joint Surg Br. 2003;85:469–477.
Trojian TH, Chu SM. Dupuytren's disease: diagnosis and treatment. Am Fam Physician. 2007;76:86–89.
Codes
ICD9
728.6 Contracture of palmar fascia
Clinical Pearls
  • DC is an inherited disease of progressive fibrous tissue contracture of the palmar fascia.
  • The disease predominantly affects males of Northern European decent over the age of 40 yrs who are smokers, alcohol drinkers, or diabetics.
  • Patients present with a small lump or multiple lumps with pits in the palm of the hand progressing to contractures of the fingers.
  • Intralesional steroid injections have been shown to reduce the need for surgery.
  • Surgical referral should be made when MCP joint contractures reach 30 degrees or if any degree of PIP joint contracture is present.
  • Surgery usually is done in advanced disease in order to restore function to the hand.
  • Percutaneous needle aponeurotomy and the emerging therapy of collagenase injections are significant therapeutic alternatives to surgery.


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