Updated: 2/21/2019

Dupuytren's Disease

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https://upload.orthobullets.com/topic/6058/images/dupy.jpg
https://upload.orthobullets.com/topic/6058/images/garrods.jpg
https://upload.orthobullets.com/topic/6058/images/Digital Fascia McFarlane_moved.jpg
https://upload.orthobullets.com/topic/6058/images/tabletop.jpg
https://upload.orthobullets.com/topic/6058/images/mccash.jpg
Introduction
  •  A benign proliferative disorder characterized by fascial nodules and contractures of the hand
  • Epidemiology & genetics
    • genetics
      • autosomal dominant with variable penetrance
    • age
      • 5-7th decade of life 
      • earlier onset = more aggressive disease with higher recurrence post resection
    • sex
      • 2:1 male to female ratio
      • presents earlier in men (mean 55y) than women (mean 65y)
      • more severe disease in men than women
    • ethnicity
      • Caucasian males of northern European descent 
      • uncommon in south Europe, south America
      • rare in Africa and China
    • location
      • ring > small > middle > index
  • Pathophysiology 
    • myofibroblast is the dominant cell type 
      • differs from fibroblast as the myofibroblast has INTRACELLULAR ACTIN filaments aligned along long axis of cell
      • adjacent myofibroblasts connect via EXTRACELLULAR FIBRONECTIN to act together to create contracted tissue
    • type III collagen predominates (> type I collagen)
    • cytokines have been implicated
      • TGFbeta1, TGFbeta2, epidermal growth factor, PDGF, connective tissue growth factor
    • ectopic manifestations
      • Ledderhose disease (plantar fascia) 10-30%
      • Peyronie's disease (dartos fascia of penis) 2-8%
      • Garrod disease (knuckle pads)  40-50%
  • Associated conditions
    • HIV, alcoholism, diabetes, antiseizure medications
Pathoanatomy
  • Nodules and Cords make up the pathologic anatomy
    • nodules appear before contractile cords
  • 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  
      • most important cord
      • cause of PIP contracture
      • typically inserts distally into the lateral digital sheet then into Grayson's ligament
      • components
        • pretendinous band
        • spiral band
        • lateral digital sheet
        • Grayson's ligament
      • travels under the neurovascular bundle displacing it 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
      • from disease involving pretendinous band 
      • inserting into flexor sheath at PIPJ level and causes MCP contracture
      • forms palmar nodules and pits between distal palmar crease and palmar digital crease
      • NOT involved with neurovascular bundle
    • retrovascular cord
      • runs dorsal to the neurovascular bundle distally
      • originates from proximal phalanx, inserts on distal phlanx
      • causes DIP contracture
    • natatory cord (from natatory ligament)
      • causes web space contracture
  • NOT involved in Dupuytren's disease
    • Cleland's ligament 
    • transverse ligament of the palmar aponeurosis
      • disease only involves longitudinally oriented structures
Histopathology
 
Stages of Dupuytren's (Luck)
Proliferative stage Hypercellular with large myofibroblasts and immature fibroblasts - this is a nodule
Very vascular with many gap junctions
Minimal extracellular matrix
Involutional stage

Dense myofibroblast network
Fibroblasts align along tenion lines and produce more collagen
Increase ratio of type III to type I col

Residual stage Myofibroblast disappear (acellular) leaving fibrocytes as the predominate cell line 
Leaves dense collagen-rich tissue/scar

Presentation
  • Symptoms
    • decreased ROM affecting ADL
    • painful nodules 
  • Physical exam
    • nodule in the pretendinous bands of the palmar fascia
      • nodule beyond MCPJ is strong clue suggesting spiral cord displacing digital nerve midline and superficial
    • 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 
    • injection of Clostridium histolyticum collagenase (Xiaflex)
      • indications
        • may be attempted but condition will not spontaneously resolve
      • technique/characteristics
        • has low activity against type IV collagen (in basement membrane of blood vessels and nerves) explaining the low neurovascular complication rate
        • minimum dose is 10,000 units
        • use 0.25ml for MCP, and 0.20ml for PIP
        • followed by stretch manipulation within 24-48h under local anesthesia
        • repeat at 1mth if desired result not achieved
      • modalities
        • early efficacy seen with injections of clostridial collagenase into Dupuytren's cords 
          • causes lysis and rupture of cords
      • outcomes
      •  
        • able to correct MCP/PIP contracture to <5° 
        • more successful at MCP correction than PIP correction
        • PIP recurrence more severe than MCP recurrence
      • complications
        • minor
          • edema/contusion, skin tear, pain are most common
        • major (1%)
          • flexor tendon rupture, CRPS, pulley rupture
    • needle aponeurotomy
      • indications
        • mild contractures (at the MCP > PIP)
        • medical co-morbidities that preclude surgery
      • technique
        • perform in office using 22G or 25G needle
        • followed by manipulation and night orthosis wear
      • outcomes
        • more successful for MCP contracture than PIP
        • less improvement and higher recurrence rate than surgery (open partial fasciectomy)
  • Operative
    • surgical resection/fasciectomy
      • indications
        • MCP flexion contractures > 30°
        • PIP flexion contractures 
        • painful nodules are not an indication for surgery
    • with skin graft
      • rarely needed for primary cases
      • indications
        • severe, diffuse disease
        • multiple joint involvement
        • recurrences
      • technique
        • full thickness skin graft
      • outcomes
        • rarely fail to "take" even if placed directly over neurovascular bundles/flexor sheath
        • Dupuytrens recurrence is uncommon beneath a graft
Surgical Techniques
  • Regional/limited/ partial palmar fasciectomy  
    • technique
      • removal of all diseased tissue only in involved digits
      • dissect from proximal to distal
      • incision options - Brunner zigzag, multiple V-Y, sequential Z-plasties
    • 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
    • infrequently used
    • technique
      • release/excision of all palmar and digital fascia including non-diseased fascia
    • cons
      • high complication rate
      • little effect on recurrence rate (also high)
  • Open palm technique (McCash technique) 
    • approach
      • leave a transverse skin incision open at the distal palmar crease
    • pros
      • reduced hematoma formation
      • reduced risk for stiffness
    • outcome
      • longer healing
      • greater recurrence than if the palmar defect were covered with transposition flap or FTSG
  • Salvage techniques (for recurrent/advanced disease)
    • Hueston dermofasciectomy (excise skin + fascia)
    • arthrodesis
    • amputation
Outcomes
  • Recurrence
    • 30% at 1-2y, 15% at 3-5y, 10% at 5-10y, and <10% after 10y
    • higher recurrence with non-operative measures (needle aponeurotomy and collagenase injection)
    • PIP develop contratures of secondary structures that may need more comprehensive surgical release
      • volar plate
      • accessory collateral ligaments
      • flexor sheath
    • risks
      • Dupuytren diathesis (age <50, white men, bilateral hands DD, family history, ectopic disease outside the palm including Ledderhoses, Peyronies, Garrods pads)
        • patients with Dupuytren diathesis may need more aggressive followup and treatment
      • PIP disease
      • small finger contracture
Complications
  • Wound edge necrosis/slough
  • Hematoma
    • most common surgical complication
    • can lead to flap necrosis 
  • Flare reaction
    • pain syndrome with diffuse swelling, hyperesthesia, redness and stiffness
    • minimize by not splinting immediately postop; apply splints at first follow-up
    • treatment
      • cervical sympathetic blockage, progressive stress-loading in therapy
      • A1 pulley release
    • no increase risk of CRPS with fasciectomy + carpal tunnel release
  • Neurovascular injury
    • because of midline + superficial displacement of NV bundle by spiral cord
    • identify prior to excising cord
    • risk is 5-10x higher for recurrent disease
    • treatment
      • immediate neurorrhaphy (nerve repair)
  • Digital ischemia
    • most common reason is correction of longstanding joint contracture and vessels have inadequate elasticity
    • less commonly traction, transection, spasm, intimal hemorrhage, rupture
    • minimize by not splinting immediately postop and apply splints at first follow-up visit
    • treatment
      • allow joint to relax, warm the digit
      • topical lidocaine and papaverine
      • if thrombosed segment is identified, use interpositional vein graft
  • Postop swelling
    • contributes to stiffness, poor wound healing
  • PIP complications
    • stiffness, instability, flexion contracture
  • Infection
    • increased risk with DM and PVD
    • oral antibiotics for superficial infection
    • surgical drainage for deep infection
 

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(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? Review Topic

QID: 4605
FIGURES:
1

Spiral cord which is central and superficial to the neurovascular bundle

18%

(647/3638)

2

Central cord which is midline and superficial to the neurovascular bundle

13%

(480/3638)

3

Retrovascular cord which is central and superficial to the neurovascular bundle

1%

(34/3638)

4

Spiral cord which is lateral and deep to the neurovascular bundle

62%

(2273/3638)

5

Central cord which is lateral and deep to the neurovascular bundle

5%

(179/3638)

ML 4

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PREFERRED RESPONSE 4

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

QID: 1238
1

Fibroblast growth factor (FGF)

2%

(18/900)

2

Transforming growth factor- beta (TGF-beta)

7%

(59/900)

3

Myofibroblasts

3%

(30/900)

4

Platelet-derived growth factor (PDGF)

19%

(170/900)

5

CBFA-1

69%

(618/900)

ML 2

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PREFERRED RESPONSE 5

(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? Review Topic

QID: 3204
1

70-year-old sedentary male with small finger involvement isolated to the MCP joint

2%

(46/2609)

2

50-year-old male systems analyst with ring and small finger involvement limited to the MCP joints

2%

(43/2609)

3

65-year-old female golfer with ring and small finger involvement including MCP and PIP joints

2%

(57/2609)

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

40%

(1033/2609)

5

None of the above as no difference in outcome has been demonstrated between the two procedures

54%

(1407/2609)

ML 4

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PREFERRED RESPONSE 5

(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? Review Topic

QID: 1372
FIGURES:
1

Pretendinous cord

12%

(240/2031)

2

Pretendinous band

4%

(77/2031)

3

Spiral cord

73%

(1475/2031)

4

Spiral band

6%

(118/2031)

5

Natatory cord

6%

(112/2031)

ML 2

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PREFERRED RESPONSE 3
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