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Dupuytren's Disease

Topic updated on 02/19/15 6:13pm
Introduction
  •  A benign proliferative disorder characterized by fascial nodules and contractures of the hand
  • Epidemiology & genetics
    • autosomal dominant with variable penetrance
    • 5-7th decade of life with 2:1 male to female ratio
    • highest incidence in caucasian males of northern European descent 
  • Pathophysiology 
    • myofibroblast is the dominant cell type 
    • cytokines have been implicated
    • ectopic manifestations
      • Ledderhose disease (plantar fascia)
      • Peyronie's disease (dartos fascia of penis)
      • Garrod disease (knuckle pads) 
  • Associated conditions
    • HIV, alcoholism, diabetes, antiseizure medications
  • Prognosis
    • long-term recurrence rate after operative management up to 50%
Pathoanatomy
  • Nodules and Cords make up the pathologic anatomy
  • Normal fascial bands become pathologic cords
    • Palmar
      • pretindinous cord
    • Palmodigital transition
      • natatory cord 
      • spiral cord
    • Digital
      • central cord - distal extent of the pretendinous cord 
      • lateral cord
      • digital cord
      • retrovascular cord
  • Different named cords include but are not limited to
    • spiral cord
      • clinically the most important 
      • cause of PIP contracture
      • typically inserts distally into the lateral digital sheet then into Grayson's ligament
      • involves several structures including
        • pretendinous band
        • spiral band
        • natatory band/ligament
        • lateral digital sheet
        • Grayson's ligament
      • travels under the neurovascular bundle
        • bundle is displaced central and superficial 
        • at risk during surgical resection  
        • best predictors of displacement are
        • PIP joint flexion contracture (77% positive predictive value)
        • interdigital soft-tissue mass (71% positive predictive value)
    • central cord
      • distal extent of the pretendinous band
      • not involved with neurovascular bundle
      • cause of MCP contracture
    • retrovascular cord
      • usual cause of DIP contracture
      • runs deep to the neurovascular bundle distally
      • displaces neurovascular bundly central and superficial
  • Cleland's ligament is the only retaining dermal ligament not involved with Dupuytren's disease
Histopathology
 
Stages of Dupuytren's (Luck)
Proliferative stage Hypercellular with a predominance of large myofibroblast 
Very vascular with many gap junctions
Minimal extracellular matrix
Involutional stage Dense myofibroblast network
Increase ratio of type III to type I collagen
Residual stage Myofibroblast disappear leaving fibrocytes as the predominate cell line

Presentation
  • Symptoms
    • decreased range of motion affecting activities of daily living
    • nodules may be painful
  • Physical exam
    • nodule in the pretendinous bands of the palmar fascia
    • most commonly involve small or ring finger
    • Hueston's tabletop test 
      • ask patient to place palm flat on table
      • look for MCP or PIP contracture
    • look for bilateral involvement and ectopic associations (plantar fascia)
      • indicative of more aggressive form (Dupuytren's diathesis)
Treatment
  • Nonoperative
    • range of motion exercises, injections
      • indications
        • may be attempted but condition will not spontaneously resolve
      • modalities
        • early efficacy seen with injections of clostridial collagenase into Dupuytren's cords 
          • causes lysis and rupture of cords
  • Operative
    • surgical resection/fasciectomy
      • indications
        • MCP flexion contractures > 30°
        • PIP flexion contractures 
        • painful nodules are not an indication for surgery
Surgical Techniques
  • Regional/limited palmar fasciectomy  
    • technique
      • removal of all diseased tissue only in involved digits
    • pros
      • most widely used surgical treatment
      • overlying skin is preserved
    • postoperative care
      • early active range of motion (starting postoperative day 5-7)
      • night-time extension brace or splint
  • Total/radical palmar fasciectomy
    • technique
      • release and/or excision of all palmar fascia including portions that do not appear involved in the disease process
    • cons
      • decreased utilization due to recurrence rate and complications
  • Open palm technique (McCash technique) 
    • approach
      • transverse skin incision at the distal palmar crease
        • may be left open with or without delayed skin grafting
    • pros
      • useful in older patients who are at risk for stiffness
      • low risk of complication due to lack of hematoma formation
Complications
  • Hematoma
    • most common surgical complication
    • can lead to flap necrosis 
  • Recurrence
    • long term recurrence up to 50%
  • Flare reaction
    • pain syndrome with diffuse swelling, hyperesthesia, redness and stiffness
    • increased risk with concomitant carpal tunnel release
  • Neurovascular injury
    • bundle at risk due to central and superficial displacement from spiral cord
    • identify prior to excising cord

 

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Qbank (6 Questions)

TAG
(OBQ12.245) A 50-year-old patient presents with stiffness in her hand. A clinical photo is shown in Figure A. During surgical exposure, the neurovascular bundle is identified and dissected. What is the clinically most important pathologic structure to identify and what is its location relative to the neurovascular bundle in the digit? Topic Review Topic
FIGURES: A          

1. Spiral cord which is central and superficial to the neurovascular bundle
2. Central cord which is midline and superficial to the neurovascular bundle
3. Retrovascular cord which is central and superficial to the neurovascular bundle
4. Spiral cord which is lateral and deep to the neurovascular bundle
5. Central cord which is lateral and deep to the neurovascular bundle

PREFERRED RESPONSE ▶
TAG
(OBQ10.110) Which of the following patients with Dupuytren's contracture would benefit the most from dermatofasciectomy and full-thickness skin grafting opposed to traditional fasciectomy? Topic Review Topic

1. 70-year-old sedentary male with small finger involvement isolated to the MCP joint
2. 50-year-old male systems analyst with ring and small finger involvement limited to the MCP joints
3. 65-year-old female golfer with ring and small finger involvement including MCP and PIP joints
4. 40-year-old female stenographer with middle, ring, and small finger involvement including MCP and PIP joints with 50 and 55 degree contractures of ring and small finger MCP joints, respectively
5. None of the above as no difference in outcome has been demonstrated between the two procedures

PREFERRED RESPONSE ▶
TAG
(OBQ04.133) All of the following have been implicated in the pathogenesis of Duputryen's contracture EXCEPT? Topic Review Topic

1. Fibroblast growth factor (FGF)
2. Transforming growth factor- beta (TGF-beta)
3. Myofibroblasts
4. Platelet-derived growth factor (PDGF)
5. CBFA-1

PREFERRED RESPONSE ▶
TAG
(OBQ04.267) What is the name of the pathologic structure, identified by the white arrow in Figure A, that displaces the digital neurovascular bundle and places it at risk during during surgical treatment of Dupuytren's disease? Topic Review Topic
FIGURES: A          

1. Pretendinous cord
2. Pretendinous band
3. Spiral cord
4. Spiral band
5. Natatory cord

PREFERRED RESPONSE ▶
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