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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/tabletop.jpg
https://upload.orthobullets.com/topic/6058/images/mccash.jpg
https://upload.orthobullets.com/topic/6058/images/surgery soft tissue complications.jpg
  • Summary
    • Dupuytren's Disease is a benign proliferative disorder characterized by decreased hand function that begins as a painless nodule and can progress to form diseased cords and contractures of the palm and fingers 
    • Diagnosis can be made by physical examination which shows painless nodules in the palm with associated digital contracture, usually found at the MCP and PIP joints.
    • Treatment ranges from nonoperative through observation, collagenase injections, and needle aponeurotomy to operative open fasciectomy if the disease progresses or affects a patient's daily living.
  • Epidemiology
    • Incidence
      • common
        • ~30 per 100,000 annually
    • Demographics
      • 2:1 male to female ratio
        • more severe disease in men than women
      • most commonly occurs in 5-7th decade of life
        • presents earlier in men (mean 55y) than women (mean 65y)
      • ethnicity
        • most commonly in caucasian males of northern European descent
        • rare in South America, Africa, China
        • in Asian populations, palm more likely to be involved than digits
    • Genetics
      • autosomal dominant with variable penetrance
      • sporadic cases are more common
    • Anatomic location
      • ring > small > middle > index
  • Etiology
    • Pathophysiology
      • cytokine-mediated transformation of normal fibroblasts into abnormal myofibroblasts, turning normal fascial bands into pathological cords
        • contractile properties are abnormal and exaggerated
          • 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
      • increase in ratio of type III to type I collagen
      • increase in free radical formation
      • cytokines released by macrophages and lymphocytes have been implicated
        • TGFbeta1, TGFbeta2, epidermal growth factor, PDGF, connective tissue growth factor
    • Risk factors for increased severity and recurrence after treatment
      • male gender
      • onset before age 50
      • bilateral disease
      • sibling/parent involvement
      • Dupuytren's Diathesis
        • age <50, white men, bilateral hands, family history, ectopic disease outside the palm including Ledderhose, Peyronies, Garrod pads
    • Associated conditions
      • medical 
        • tuberculosis
        • chronic pulmonary disease
        • HIV/AIDS
        • tobacco use
        • alcohol use
        • diabetes
        • epilepsy
        • antiseizure medications
        • ectopic manifestations (Ledderhose, Peyronie's, Garrod)
          • Peyronie's disease (dartos fascia of penis) 2-8%
          • Garrod disease (knuckle pads) 40-50%
          • Ledderhose disease (plantar fascia) 10-30%
  • Anatomy
    • 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
        • 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
  • Classification
      • 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 tension lines and produce more collagen
      • Increase ratio of type III to type I collagen
      • Residual stage
      • Myofibroblast disappear (acellular) leaving fibrocytes as the predominate cell line
      • Leaves dense collagen-rich tissue/scar
  • Presentation
    • History
      • palpable nodules start in the palm, usually along the distal palmar crease
      • the nodules progress into palpable cords along the palm
      • as the cords thicken and shorten, they cause fixed flexion contractures at the MCP and PIP joints
      • patients rarely report pain
    • Symptoms
      • decreased ROM affecting ADL
      • painless nodules unless compressing nearby neurovasculature
    • Physical exam
      • painless nodule in the pretendinous bands of the palmar fascia
        • nodule beyond MCPJ is strong clue suggesting spiral cord displacing digital nerve midline and superficial
      • Garrod pads (knuckle pads over PIP joints) may be tender to palpation
      • most commonly involve small or ring finger
      • blanching of the skin with finger extension
      • pits and grooves in the palm
      • Hueston's tabletop test
        • ask patient to place palm flat on table
        • the test is positive if they are unable to straighten the fingers and keep hand completely flat
      • look for bilateral involvement and ectopic associations (plantar fascia)
        • indicative of more aggressive form (Dupuytren's diathesis)
  • Imaging
    • Radiographs
      • unnecessary for diagnosis but may help determine other etiologies for joint stiffness including arthritis or bony anomalies
    • Ultrasound
      • unnecessary for diagnosis
      • demonstrates thickened palmar fascia and nodules
  • Differential
    • Locked trigger finger 
      • painful condition with tenderness at A1 pulley
    • Pulley rupture with bowstringing
      • associated with finger flexion
      • may be associated with trauma 
    • Intrinsic Minus/Claw hand 
      • ulnar nerve palsy
      • MCP joint hyperextension and IP joint flexion of ulnar innervated digits 
      • loss of sensation to ulnar nerve distribution
    • Volkman's contracture 
      • history of painful compartment syndrome
      • characteristic deformity of wrist/hand 
        • wrist flexion
        • thumb adduction
        • MCP joints in extension
        • IP joints in flexion
  • Diagnosis
    • Clinical
      • diagnosis is made with careful history and physical examination
  • Treatment
    • Nonoperative
      • observation
        • indications
          • nodules alone
          • no functional impairment
      • hand therapy
        • indications
          • mild cases with minor contracture
      • corticosteroid injections
        • indications
          • painful nodules
        • outcomes
          • up to 50% recurrence rate
          • concerning adverse effects of fat atrophy, pigment changes, and tendon rupture
      • collagenase clostridium histolyticum (CCH) injections
        • Metalloprotease derived from Clostridium Histolyticum 
        • indications
          • treatment of adult patients with a palpable cord
        • outcomes
          • early efficacy seen with injections of clostridial collagenase into Dupuytren's cords
            • causes lysis and rupture of cords
          • able to correct MCP/PIP contracture to <5°
          • more successful at MCP correction than PIP correction
          • PIP recurrence more severe than MCP recurrence
          • disadvantage: cost and only able to address 2 cords per treatment visit
      • percutaneous needle aponeurotomy (PNA)
        • indications
          • mild contractures (at the MCP > PIP)
          • medical co-morbidities that preclude surgery
        • outcomes
          • more successful for MCP contracture than PIP
          • less improvement and higher recurrence rate than surgery (open partial fasciectomy)
          • disadvantages include iatrogenic injury to nerves and tendons and up to 58% recurrence rate
    • Operative
      • partial palmar fasciectomy
        • indications
          • MCP flexion contractures > 30°
          • PIP flexion contractures
          • painful nodules are not an indication for surgery
        • techniques
          • partial fasciectomy: removal of all diseased tissue 
          • open palm fasciectomy (McCash technique)
      • total/radical palmar fasciectomy 
        • indications
          • rarely needed for primary cases
          • severe, diffuse disease
          • multiple joint involvement
          • recurrences
        • technique
          • total/radical fasciectomy: removal/release of all diseased and non-diseased tissue 
      • salvage techniques
        • indications
          • for chronically recurrent and advanced disease
        • technique
          • dermofasciectomy: removal/release of all diseased and non-diseased tissue including the overlying skin  
            • skin grafting may be necessary
          • arthrodesis
          • amputation
        • outcomes
          • skin grafts rarely fail to "take" even if placed directly over neurovascular bundles/flexor sheath
          • Dupuytrens recurrence is uncommon beneath a skin graft 
  • Techniques
    • Hand therapy
      • Ultrasound and heat may be helpful in early stages of disease
      • Bracing/splinting to stretch digits 
        • may delay the progression and potentially improve the degree of flexion contractures of the PIPJ.
    • Collagenase Injections
      • technique
        • reconstitution of collagenase lyophilized powder with supplied diluent
        • inject 0.58 mg of collagenase into each palpable Dupuytren’s cord with a contracture of a metacarpophalangeal (MP) joint or a proximal interphalangeal (PIP) joint 
          • up to two joints or two cords in the same hand may be treated during a treatment visit 
        • followed by manipulation within 24-72h under local anesthesia
          • literature has shown that contracture correction is equivalent up to 7 days following collagenase injection
        • injections and finger extension procedures may be administered up to 3 times per cord at approximately 4-week intervals.
      • complications
        • minor
          • edema/contusion, skin tear, pain are most common
        • major (1%) 
          • flexor tendon rupture, CRPS, pulley rupture
        • has low activity against type IV collagen (in basement membrane of blood vessels and nerves) explaining the low neurovascular complication rate
    • Percutaneous needle aponeurotomy (PNA)
      • technique
        • perform in office using 22G or 25G needle
        • followed by manipulation and night orthosis wear
    • 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
    • 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
      • rehabilitation and Postoperative Care
        • hand therapy for several 1-3 months, starting 3-5 days postoperatively
          • Paraffin wax treatment
          • Scar and edema management
          • Dynamic and static splinting
          • Phonophoresis and iontophoresis
    • 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)
  • Complications
    • Wound complications
      • incidence
        • 23% 
      • risk factors
        • poor nutrition
        • total fasciectomy +/- dermatofasciectomy
      • treatment
        • local wound care
        • irrigation and debridement
        • skin graft or flap
    • Incisional scar pain
      • incidence: 17.4% 
      • risk factors
        •  larger incisions
      • treatment
        • hand therapy 
    • CRPS
      • incidence
        • 5.8% 
      • risk factors
        • female sex
        • long-term disability status
        • more extensive excision
      • treatment
        • hand therapy
        • pharmacotherapy (short-course steroids, bisphosphonates, gabapentin, botulinum toxin, and ketamine)
        • sympathetic blockade
        • spinal cord stimulator
    • Hematoma 
      • incidence  
        • 2.1% 
      • risk factors
        • larger incisions 
        • local vascular injury
      • treatment
        • compression, ice, elevation
        • decompression if skin at risk for necrosis
    • Skin tearing (with manipulation after CCH treatment)
      • incidence 
        • 12% 
      • risk factors
        • age
          • a 10-year increase in age results in a 1.5 times increase in the odds of skin tearing. 
        • amount of contracture correction
          • a 30° increase in contracture correction results in a 1.8 times increase in the odds of skin tearing. 
      • treatment
        •  local wound care
    • Flare reaction
      • Incidence
        • 3.5%
      • presentation
        • pain syndrome with diffuse swelling, hyperesthesia, redness and stiffness
      • risk factors
        • cord burden requiring significant tissue excision
      • prevention
        • minimize by not splinting immediately postop; apply splints at first follow-up
      • treatment
        • cervical sympathetic blockage, progressive stress-loading in therapy
        • A1 pulley release
    • Neurovascular injury
      • incidence
        • 2-3% in primary, 20% in recurrent
      • risk factors
        • spiral cord due to being midline + superficial displacement of NV bundle
          • prevent by identifying neurovascular bundle prior to excising cord
        • percutaneous needle aponeurotomy
      • treatment
        • immediate neurorrhaphy (nerve repair)
    • Recurrence
      • incidence
        • varies based on treatment method
          • CCH: approximately 50% at 5 years
          • PNA: approximately 50^% at 3 years
          • Fasciectomy: approximately 3.5%-20% at 4 years 
            • literature has recurrence rates from 3.5-73% based on the definition of recurrence 
        • PIP joints develop contractures of secondary structures that may need more comprehensive surgical release
          • volar plate
          • accessory collateral ligaments
          • flexor sheath
      • risk factors 
        • higher recurrence with non-operative measures (needle aponeurotomy and collagenase injection)
        • Dupuytren diathesis patients may need more aggressive followup and treatment
        • PIP disease
        • small finger contracture
      • treatment
        • revision fasciectomy, usually to a more invasive approach
    • Infection
      • incidence 
        • fasciectomy: 7%    
        • collagenase: 0% 
        • percutaneous needle aponeurotomy: 1.1%  
      • risk factors
        • DM
        • PVD
      • treatment
        • oral antibiotics for superficial infection
        • surgical drainage for deep infection



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