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 contents of the 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 mechanism of action selectively target collagen types I and III 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