Dupuytren's Disease

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
- 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 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 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

Dupytrens Open Fasciectomy

Needle Aponeurotomy

Dupuytren's Open Fasciectomy