Dupuytren’s Contracture

Patient Complaints

Fingers get in the way with: Washing face Combing hair Putting hand in pocket Racquet sports Golf Putting hand in glove

Symptoms

First notice tender nodule or progressive palmar cord development. Painless, and may avoid care until joint motion reduced. Skin pitting and nodule formation near distal palmar crease. Symptoms may be present bilaterally, with one hand occurring first (not necessarily dominant hand). MCP joint affected first and then PIP joint.

Further development of Patient Signs

As nodules grow, cords can be palpated proximally and distally.

Ring and small finger affected first, after palmar involvement.

Patients may have quiescent findings for years--then increased nodule formation and cord extension rapidly.

Physical Exam

Firm nodules may be tender to palpation. Cords proximal to nodules painless. Skin blanching on active finger extension. Atrophic grooves or pits in skin signify adherence to the underlying fascia. Tender knuckle pads over dorsal aspect of PIP joints--indicates aggressive disease.

Differential Diagnosis

Trigger finger--usually pain w/ flexion Stenosing Tenosynovitis--history of overuse Callus Tumor--rule out epithelioid sarcoma Ganglion Cyst--usually moveable nodule Ulnar Nerve Palsy Prolapsed flexor tendon Fibromas/fibromatoses Camptodactyly--congenital contracture

Possible related conditions

Diabetes mellitus--microvascular Cigarette smoking--microvascular Alcoholism-liver disease HIV infection Epilepsy--anti-epileptic drug phenobarbitone Trauma Manual labor Rheumatoid disease Previous myocardial infarction Plantar fasciitis Peyronie disease

What is it?

Not a tumor/malignancy. Famous patients include Ronald Reagan, Margaret Thatcher, and creator of Captain Hook (inspiration for his claw hand). Abnormal thickening of fascia. May limit movement of one or more fingers. Cord forms beneath skin stretching from palm into fingers. Cord causes fingers to bend into palm preventing straightening.

Dupuytren’s Diathesis

Strong gene expression causing physical findings. Present earlier in life (20s and 30s). Aggressive cord development with high incidence of multi-digit and bilateral hand involvement. Knuckles (Garrod’s nodes), plantar fibromatosis (Lederhose’s disease), penile fascial involvement (Peyronie’s disease). High risk for poor surgical outcome due to higher recurrence rates, greater risk of surgical technical complications, and longer post-op care.

Statistics

US: common, reflecting immigration from northern Europe.

4-6% of Caucasian population. Approximately 5-15% of males older than 50 years are affected.

Internationally: Norway, 5.6% of >60 yo Australia, 26% of males and 20% of females > 60 yo

Less common in blacks and Asians (less than 3%). Least common among native Americans and Hispanics (<1%).

Statistics7:1 men:women.

Onset of disease earlier, more severe and rapid in males.

Mean age of disease onset:

Males=49 years; Females=54 years.

The mean age for surgical treatment: males=58 years; Females=62 years.

Viking’s Disease

Greatest concentration in Scandinavia and Great Britain (Ireland and Scotland)

Viking heritage in original gene pool and follows pattern of Viking travel (prevalence decreases as distance increases from Europe) High prevalence in Australia due to British occupation.

Curse of The MacCrimmons

First known to be prevalent in western isles of Scotland. MacCrimmons were musicians and pipers to the chieftains of the clan MacLeod of Skye

Contractures inhibited playing bagpipes.

HistoryFelix Plater of Basel, Switzerland (1614).

Noted palmar fibromatosis in autopsy findings (stone cutter who sustained injury to small finger and subsequent fixed flexion contracture of the digit.). Thought tendons were released from pulleys and raised cords were tendons bowing.

Henry cline, London (1808). Characteristic anatomy of palmar aponeurosis, noting contraction and thickening. Recommended surgical release of tightened palmar aponeurosis. Noted close proximity of nerves and arteries. Post op care included extension splint for fingers.

Astley cooper, London (1822) –cline’s apprentice. Chronic inflammation of palmar aponeurosis causing finger contractures. Speculated that repetitive trauma is causative factor. Surgical release of contracted fibers through percutaneous fasciotomy.

Guillaume Dupuytren (1834). Contracture due to hand overuse or trauma. Named after him since he was the most famous surgeon in Europe at the time. Thought to be good looking guy, great surgeon, but much to be desired for manner!

PathologyTwo discrete forms differing in histologic features

Nodular tissue Cord tissue

Nodules are dense collections of myofibroblasts causing contraction Pull through cords that extend past adjacent joints, causing digital flexion contracture Follow longitudinal tension lines

Cords contain no fibroblasts but contain organized collagen structures similar to tendons

May be normal palmar fascial structure that hypertrophy and thicken in response to increased tension

Three histological stages Proliferative Infiltrative Residual

Cell TypesTwo cell types: fibroblasts and myofibroblasts.

Fibroblasts produce connective tissue of body Myofibroblasts contain actin (unlike fibroblasts) and are responsible for tissue repair (scar and contraction).

Myofibroblasts are responsible for synthesis of collagen type III. Same set of myofibroblasts found in hypertrophic scars, but not in normal granulation tissue. Higher total activity and density of cells in diseased tissue than in normal tissue. In residual phase, myofibroblasts replaced by fibroblasts.

Histological StagesProliferative stage:

Development of active nodule, fibroblasts present. Involutional (active contractile stage):

Cords develop proximal to nodules. Grooves or pits in skin are skin fixation to the underlying fascia. Myofibroblasts replace fibroblasts—show stress alignment according to stress lines in tissue.

Residual stage: Tissue largely acellular and tendon-like. Loss of nodule and placement of scar tissue. No myofibroblasts.

Proliferative stage (fibroblasts present)

A=high cellular area (dark staining) B=normal (less cellular) 1. Moermans JP

Segmental apeunorectomy in Dupuytren's DiseaseDupuytren's Disease Edited by: R. M. McFarlane, D.A. McGrouther, M.H. Flint Churchill-Livingstone pp 352-356 1990

Involutional Stage

Fibroblasts aligning themselves with lines of stress

1. Moermans JP, Segmental apeunorectomy in Dupuytren's DiseaseEdited by: R. M. McFarlane, D.A. McGrouther, M.H. Flint Churchill-Livingstone pp 352-356 1990

PathologyMechanical process:

Mechanical transduction-- cellular tissue once formed responds to physical forces (cyclic strain) and gene expression. Cell motility, shape, and differentiation production of matrix are influenced by forces on cell---respond to compressive and tensile load.

Remodeling of contractile tissue: Tension produced when fingers extended against contractive palmar forces---perpetuates disease process.

Collagen in Diseased Tissue

Type III Collagen—Skin, Muscles, Blood Vessels Type I Collagen—Skin, Tendon, Bone Higher concentration of type III collagen. Increased amounts of total collagen and increased lysyl oxidase activity (for lysine and hydroxylysine). Normal palmar aponeurosis type I collagen predominates, some type III present. Residual phase-- type I replaces type III collagen.

Ischemia Pathology

Fibroblasts cluster around microvessels and partially or totally occlude. Increased cellularity from local area microvascular ischemia (smoking, alcohol). Lipid composition shows profile consistent with mild tissue hypoxia. Postulated that local ischemia causes oxygen free radicals, stimulating fibroblasts. Self-perpetuating cycle.

Cell-mediated Pathology

Dupuytren’s cells are more sensitive to effects of cellular modulators for initiation and progression of disease. Two control factors postulated: mechanical stress and TGF-beta. Myofibroblast proliferation responsive to platelet derived growth factor, IL-1, and basic fibroblast growth factor. Contracture can be partially explained by cell response to cyclic strain mediated by PDGF-B. Dupuytren fibroblast also has higher levels of alpha smooth muscle actin and contractility than normal fibroblast. T-cell mediated autoimmune phenomenon. Autoantibodies association found between HLA-DR3 and types I-IV collagen.

Anatomy-Palmar aponeurosis

Palmar aponeurosis triangular thin sheet of fascial tissue made of “pretendinous bands”, traveling toward each digit. Pretendinous bands proceed towards webspace, wrapping both sides of metacarpal head and extending into MCP joint capsule—known as spiral bands. fascial extensions travelling sagitally in palm, attaching to interosseous muscle fascia-known as septa of Legueu and Juvara. 7 compartments containing four sets digital flexor tendons, and 3 neurovascular structures. Superficial transverse palmar ligament in coronal plane running perpendicularly to tendinous bands at distal third of palm. Natatory ligament-transverse oriented structure parallel to superficial transverse palmar ligament, between webspaces.

Webspace anatomyGrayson’s ligament--fibers passing volar to neurovascular bundle.

Cleland’s ligament—dorsal fibers.

Both form lateral digital sheet on either side of finger.

Fibers attach to periosteum, joint capsule, and tendon sheath.

Dupuytren AnatomyPathologic nodules form in fatty zones, between MCP and PIP flexion creases. Pathologic cords form along paths of normal fascial anatomy. Blending of cords to form united cord among structures (between pretendinous cords, spiral, web-space tissue, and later digital sheet. 3 dimensional path following hand fascial extensions. Anchored firmly in sagittal plane by attachments to flexor tendon sheath, joint capsule, interosseous fascia, periosteum, and skin. Neurovascular bundle can be intertwined, encompassing digital nerves, wrapping spirally and being pulled toward midline of digit.

Pathological CordsCentral cord- longitudinal pretendinous band Spiral cord- spiral band, pretendinous band, lateral digital sheet, vertical band & Grayson's ligament Lateral cord - lateral digital sheet Retrovascular cord arises from digital fascia dorsal to neurovascular bundle Cord combinations : central-lateral cord is commonest

Non-Operative Treatment

Include vitamin E creams, lotions, corticosteroid injections, physical therapy, splinting.

No consistent favorable therapy.

Difficult to study as many cases spontaneously regress.

Aims of surgical treatment

Wide resection not needed as in oncologic surgery. Achieve biomechanics change of hand. Release of joint contractures.

Actual removal of Dupuytren’s tissues. Interposing healthy tissue between ends of cords to prevent linking up again.

Various surgeries differ in three ways: Management of Palmar fascia. Treatment of Volar skin. Incisions.

Operative Indications

“Table top” test—place palm of hand flat on table top. (+) When patient no longer able to do so. Correlates with MCP contracture of 30-40 degrees; >40 degrees requires surgery.

Other digits treated when MCP contracture 20-30 degrees. Patient preference, painless and slow progress--surrounding loss of function. Previously thought PIP contracture correction difficult to accomplish.

Surgical indication when any PIP joint contracture noted. However current trend is to wait until 30 degree contracture.

Must differentiate between true PIP contracture and MCP spiral tendons extending towards PIP joint. Solitary nodules not indications for surgery, and may spontaneously regress.

Management of Palmar Fascia

Methods include: Radical vs. Selective vs. Segment Fasciectomy Fasciotomy Amputation Joint resection and arthrodesis

Surgical Fasciectomy

Radical Fasciectomy-- mostly abandoned All palmar fascia removed High amounts of wound complications, and recurrence

Selective Fasciectomy-- most commonly used Removal of all diseased fascia in palm/finger Indicated when only ulnar one or two fingers involved Rate of recurrence-50%, need for another surgery-15% Recurrence due to undetectable diseased fascia remaining

Segmental Fasciectomy Removal of one or more segments of diseased fascia through multiple small incisions in palms and fingers or through transverse/longitudinal plasties, with skin grafts

Other Surgical Techniques

Fasciotomy Diseased tissue incised but not removed Used mainly in elderly patients or severe disease when unable to comply with post-operative rehabilitation protocol

Amputation Rare, but may be indicated in flexion contracture of PIP joint, especially little finger, when cannot be corrected enough to make finger useful Or in vascular compromise

Joint resection-arthrodesis Severely contracted PIP joint Avoids the potential for recurrent PIP joint contracture and potential amputation neuroma

Management of Volar Skin

Three types: Direct closure Full-thickness skin grafting Open technique with wound contraction

Direct Closure

Direct closure Primary wound healing No need for skin grafts Simple post-op management Increased incidence of hematoma Skin flap necrosis

Full thickness skin grafting

Full thickness skin grafting Pros: Less recurrence where full thickness graft used, modulating effect on underlying fascia Cons: Recurrence still possible beyond areas of graft Graft loss Hematoma formation Immobilization may cause stiffness Altered sensation on graft Altered wear patterns on graft

Open wound technique

Open wound technique Transverse incision in palm at level of midpalmar crease and extensions in fingers Transverese incision is left open and covered with non-adherent dressing Daily dry dressing changes, healing in weeks No granulation or epithelialization, instead transverse wound contracts to precontracture length Less hematoma, wound edge necrosis, and infection Inconvenience during 3-5 weeks for closure

Post-operative Treatment

Splinting, active and passive ROM excercises, wound care, scar and edema management, education and strengthening. Compliance with home exercise program—early active motion and restoration of grip, especially at PIP joint. Therapy 2-5 days post op, Forearm volar splint (2/3 splint) with wrist in neutral position and fingers extended in position. Nighttime extension splinting for 3-6 months. Scar management--massage and silicone gel. Strengthening 3-4 weeks after wound closure, 4 weeks after skin grafting, and 6 weeks after open-palm healing.

ComplicationsIntra-operative.

Digital nerve division. Hematoma formation. Wound healing difficulties (flaps). Vascular compromise of a digit.

Post-operative. Patient compliance. Reflex sympathetic dystrophy (flare reaction). 1-8% prevalence, 2x more common in women.

Recurrence up to 63%.

RecurrencePresence of diseased tissue in surgically treated field. Cure at genome level---surgical excision improves hand function. Recurrence more common at young ages and in Dupuytren’s diathesis. Most commonly diseased tissue from untreated areas extends into treated areas. Presence of residual tissue incompletely excised, leaving behind myofibroblasts in skin---(full skin grafts rarely recur, due to complete removal of all nodular area in dermis and epidermis).

New Treatments: Collagenase

Enzymatic percutaneous fasciotomy of residual stage disease. Collagenase diluted in calcium chloride, developed by Biospecifics technologies corporation. Currently treatment only available at stony brook medical center, under FDA “orphan drug status” in phase III trials. Injected straight into nodule. Minimal side effects: tenderness at injection site, hematoma, edema. Preliminary results by Badalamente and Hurst show results of more than 90% correction of MCP joint, 66% correction of the PIP joint, and minimal recurrence rates. Although collagenase is showing promise in clinical trials, surgery is still considered the standard of care.

Needle Aponeurotomy

Fascia contractures sectioned percutaneously with sharp-edged bevel of local anesthetic needle. The treatment is only performed in Europe, primarily France—”FRENCH CONNECTION.” Outpatient, $150 for 20 minute session and requires no physical therapy. Temporary treatment, not cure.

Gamma Interferon

Gamma-interferon is cytokine produced by t-helper lymphocytes.

Shown to decrease fibroblast replication, alpha-smooth-muscle actin expression, and collagen production. Fails to have long term disease free effect.

Summary

Dupuytren’s contracture is genetic disease.

Patients must understand that surgery is not a cure, and has potential side effects.

Future treatment more medical and less surgical, with eventual cure to be at genomic level.

ReferencesNiel Meeta Alman, B.A.; Goldberg, M.J.;Jiranek, W.A.; Terek, R.M.; Wolfe, H.J.: The expression of Platelet-Derived Growth-Factor Gene in Dupuytren Contracture. The Journal of Bone and Joint Surgery, 77A-1,1-9, Jan 1995. Alman, B.A.; Greel, D.A.; Ruby, L.K.; Goldberg, M.J.; Wolfe, H.J.: Regulation of proliferation and platelet-derived growth factor expression in palmar fibromatosis (Dupuytren Contracture) by mechanical strain. Journal of Orthopaedic Research, 14(5),722-728, Sep 1996. Alman, B.A.; Wolfe, H.J., et al: Molecular genetic and immunohistochemical analysis of the tumor suppressor genes Rb and p53 in palmar and aggressive fibromatosis. Diagnostic Molecular Pathology, 5(3):194-200, Sep 1996. Badalamenta, M.A.; Hurst, L.C.; Hentz, V.R.: Collagen as clinical target: Non-operative treatment of Dupuytren’s disease. J Hand Surg. 2002 Sep;27(5):788-98 Benson, L.S.; Williams, C.S.: Dupuytren’s Contracture, Journal of the American Academy of Orthopaedic Surgeons, 6(1),23-35, Jan/Feb 1998. Canale, S.T.: Campbell’s Operative Orthopaedics, 10th edition, 2003. Volume 4, 3751-3759 Green, D.P.; Hotchkiss, R.N; Pederson, W.C.: Green’s operative hand surgery, 4th edition, 1999. Volume 1, 563-591. Thurston, A.J.: Dupuytren’s Disease [Review Article], Journal of Bone and Joint Surgery, 85-B(4),469-477, May 2003