Dupuytren's Disease - Hand

Summary
- Dupuytren's Disease is a benign proliferative disorder characterized by decreased hand function caused by hand contractures and painful fascial nodules.
- Diagnosis can be made by physical examination which shows painful nodules in the palm with associated digital contracture.
- Treatment ranges from nonoperative passive stretching to injections, needle aponeurotomy, and 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
- Genetics
  - autosomal dominant with variable penetrance
- Anatomic location
  - ring > small > middle > index

Etiology
- 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

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
    
    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

                        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
- 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)

Diagnosis
- Clinical
  - diagnosis is made with careful history and physical examination

Treatment
- Nonoperative
  - range of motion exercises
  - injection of Clostridium histolyticum collagenase (Xiaflex)
    
    indications
    
    may be attempted but condition will not spontaneously resolve
    
    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
  - needle aponeurotomy
    
    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)
- Operative
  - surgical resection/fasciectomy
    
    indications
    
    MCP flexion contractures > 30°
    
    PIP flexion contractures
    
    painful nodules are not an indication for surgery
    
    techniques
    
    partial fasciectomy
    
    open palm fasciectomy (McCash technique)
    
    total/radial fasciectomy
  - surgical resection/fasciectomy 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
  - salvage techniques
    
    indications
    
    for chronically recurrent and advanced disease
    
    technique
    
    Hueston dermofasciectomy (excise skin + fascia)
    
    arthrodesis
    
    amputation

Techniques
- Injection of Clostridium histolyticum collagenase (Xiaflex)
  - technique
    
    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
    
    literature has shown that contracture correction is equivalent up to 7 days following collagenase injection
    
    repeat at 1 month if desired result not achieved
  - complications
    
    minor
    
    edema/contusion, skin tear, pain are most common
    
    major (1%)
    
    flexor tendon rupture, CRPS, pulley rupture
- Needle aponeurotomy
  - 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
- 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
- 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
  - risk factors
    
    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
- 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

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