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Dupuytren’s Disease
Mark Bagg, MDMarch 24, 2017
Outline
• History
• Basic Science
• Anatomy
• Diagnosis
• Treatment
• Complications
Dupuytren’s Disease
• Unsolved Issues of the 21st Century– Cause
– Cure
History of Dupuytren’s
• Disease of the Vikings
• Felix Plater- 1614
• Cline/ Cooper- 1777
• Baron Dupuytren-1832– “Napoleon of Surgery”
– “First among surgeons, last among men”
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Basic Science
• DD is a fibroproliferative disorder affecting the palmar fascia.
• Shortening of the palmar fascia can be attributed to fibroblasts and myofibroblasts present in diseased tissue.
• These cells lead to the formation of a nodule followed by a cord that are the hallmark findings in DD.
Basic ScienceTomasek, et al JBJS-A 69:1987
• Myofibroblast - cell responsible for tissue contraction
• Fibronectin– migration
– differentiation
– adhesion
• TGF-B1
Dupuytren’s Is Progressive
• Palmar lesions• Nodule formation
• Cord formation• Digital contracture begins
• Contracted cords• Flexion deformities
cord formation
contractures
Progression of Dupuytren’s*
nodule
Luck JV. J Bone Joint Surg [Am]. 1959;41:635-664.
cord
Epidemiology
• Northern European descent- “disease of the Vikings”
• prevalence 2- 42%
• Mikkelsen’s study- 9.4% men/ 2.8% women
• Low prevalence in Blacks and Asians
• Autosomal dominant with incomplete penetrance
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Epidemiology
• Autosomal dominant with incomplete penetrance
• Gender issues– Men:Women- 6:1
• JHS Volume 32, Issue 9, November 2007, 1423-1428
– Men- peak onset about 10 years before women
– Women- Flare rxn after surgery more common (7-20%)
Etiology (Associations)
• Alcoholism (Noble, JHS-B 17:1992)• Smoking (An, JHS-Am 13:1988, Burge, JBJS-B
79:1997)• Diabetes (Noble JBJS-B 66:1984) • Epilepsy- role of anti-epileptics may play a role
– D/C phenobarbital- regression of cords and knuckle pads
• Injury- little conclusive evidence• Manual Work- conflicting data
– Exposure to vibration- positive correlation
Clinical Presentation
• Skin changes → Nodule formation → Cord formation → Contracture– Reilly, Stern JHS Am 2005 30(5)
– Skin changes can be the earliest manifestation of DD.• Loss of mobility, thickening, dimpling, and skin pits.
– The nodule is key for early diagnosis.• Firm soft tissue mass, well defined, and localized.• Located between distal palmar crease and PIP flexion crease.• Usually painless.
– The nodule often regresses giving way to the cord.• Normal bands are precursors of the cord.• Cord becomes prominent, tendon like, with an abundance of collagen tissue.
– Contracture: RF > SF > Thumb > MF > IF• Small size of pretendinous bands on radial side likely to be reason for this
distribution.
Dupuytren’s Diathesis• Diseased tissue outside the
hands:– Plantar fibromatosis
(Lederhose’s disease)– Penile fascial involvement
(Peyronie’s disease)– Knuckle pad keratosis (Garrod’s
nodes)
• Strong gene expression.• Disease in the 20’s and 30’s.• Very aggressive cord
development.• Higher risk for poor outcome.
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Anatomy• Longitudinal fibers:
– Pretendinous bands
– Spiral bands
– Lateral digital sheets
• Transverse fibers– Transverse ligament of PA (Skoog’s
fibers)
– Natatory ligament
– Grayson’s ligament
– Cleland’s ligament
• Vertical fibers– Superficial vertical bands
– Septa of Legueu and Juvara
Anatomy of the Palmar Fascial Complex
• Radial Aponeurosis
• Ulnar Aponeurosis
• Palmar (central) Aponeurosis– Retinaculum for flexor
tendons
– Stabilize the metacarpals
– Supports and anchors palmar skin
Anatomy of the Palmar Fascial Complex
• Pre-tendinous Bands
• Transverse fibers
• Vertical Fibers– superficial connect skin to
fascia
– deep fibers- Septa of Legueu and Juvara which form 7 distinct compartments
Anatomy of the Palmo- Digital FascialComplex
Spiral BandNatatory ligament
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Anatomy of the Digital Fascial Complex
• Digital Fascia– Lateral digital sheath
– Cleland’s ligament (dorsal)
– Grayson’s ligament (volar)
– Retrovascular Fascia
Patho-anatomy
• Nodules tend to form between the distal palmar crease and the PIP flexion crease.
• Cords form along the pathways of normal fascial anatomy.
• Normal bands become pathologic cords.
• The NV bundle becomes intertwined with diseased tissue.
• As the cords contract, the encircling pathway becomes more linear causing the NV bundle to spiral.
Pathoantomy of the Palmar Fascial Complex
• Palmar Cords-– from pretendinous band
• Vertical cord– from vertical fibers of
Legueu/Javara
• Abductor Digiti Minimi Cord
Fascial structures NOT involved in Dupuytren’s disease
– Transverse Ligament of Palmar Aponeurosis (Skoog)
– Cleland’s ligament
– Transverse retinacular ligament
– Oblique retinacular ligament
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Treatment Options
• Surgical options– Open (fasciotomy, fasciectomy)
– Closed (needle aponeurotomy)
• Non-surgical options– In February 2010, the FDA approved
XIAFLEX®, anonsurgical treatment option for adults with Dupuytren’s contracture with a palpable cord
Efficacy: MP and PIP Joints
n=133 n=70n=20 n=25n=69 n=34n=11 n=10
Percentage of patients achieving a reduction in contracture to 0°to 5° of normal 30 days after up to 3 injections
Hand Center MPJ – 76 %
Efficacy By Severity of Joint Contracture Before Treatment
MP Joints PIP Joints
(20°-50°) (>50°-100°) (20°-40°) (>40°-80°)
n=81 n=10 n=52 n=10 n=21 n=5 n=49 n=20
Percentage of patients achieving a reduction in contracture to 0°to 5° of normal 30 days after up to 3 injections
n=43 n=7 n=26 n=4 n=9 n=2 n=25 n=8
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• When do I use Collagenase now– Isolated single digit MPJ contracture >30 degrees
with a DISTINCT pre-tendinous cord with associated PIP contracture less than 30 degrees.
– Isolated single digit PIPJ contracture between 20-40 degrees with DISTINCT raised cord
Indications for Surgery
• MCPJ contracture > 30°– McFarlane RM, In McCarthy’s:
Plastic Surgery 1990
• PIPJ contracture > 30°– McFarlane RM, In Green’s:
Operative Hand Surgery, 1993
• Hueston’s Table Top Test
Treatment- Fasciotomy
• May be useful in patients with limited life expectancy
• As a prelude to more definitive mgmt
Treatment- Fasciectomy
• Limited (Moerman, Gonzalez)– Disease likely to progress– May not require additional surgery
• Regional (partial)– most commonly done procedure– removes only the diseased tissue
• Extensive (radical)– Removes all palmar fascia– increased complication/ stiffness
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Treatment - Incisions
Treatment- Open Palm
• Originally proposed by Dupuytren
• McCash popularized
• Lubahn (JHS, 9A: 1984)– Retrospective comparison of open vs close palm
– Open palm did better
– Better ROM, no hematomas
– Recurrence rate similar for both groups
Treatment - Skin Grafting
• Thought to prevent recurrence of disease – Hueston JT, Br J Plastic Surg 1970
• “Firebreaks”
• Dermofasciectomy with FTSG
• Risks: Hematoma, graft loss, stiffness
• May be indicated in young person with diathesis
Treatment - Joint Contracture
• MPJ- usually corrects with fasciectomy
• PIPJ – Tight structures: skin, flexor sheath, palmar plate
adhesion, assessory collateral ligament, intra-articular changes
– Attenuation of the central slip
– Secondary ctx release less predictable (Weinzweig, JHS, 1996)
– Gentle passive manipulation (Breed, et al. JHS, 1996)
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Cost Analysis
Societal standard for cost effective treatment: $50,000/ QALY Open partial palmar fasciectomy- $820,114/ QALY
Percutaneous Needle Aponeurotomy $96,474/QALY
Without anesthesia $49,631/QALY
Collagenase
@ $250.00 $31,856/QALY
@ $945.00 $49,995/QALY
@ $3250.00 $166,268/QALY
Cost Effectiveness of Open Partial Fasciectomy, Needle Aponeurotomy, and Collagenease Injection for Dupuytren Contracture, Chen, et. al. JHS Vol 36 A 1826
Complications• Overall complication rate – 20% McFarlane RM, In
McCarthy’s: Plastic Surgery 1990
– Nerve – spiral nerve– Arterial injury – redo contractures– Hematoma formation– Flap necrosis– Infection– Loss of flexion/ decrease ROM– Reflex sympathetic dystrophy “flare”
• 4% males• 8% females• Rate of 58% noted with simultaneous carpal
tunnel release????– McFarlane RM, Dupuytren’s Disease: Biology and Treatment 1990
– Recurrence• 2% to 74%
– McFarlane RM, Dupuytren’s Disease: Biology and Treatment 1990
Dupuytren’s Recurrence• Surgery- 2% to 60%, with an average of 33% depending
on the type of surgery (fasciectomy, fasciotomy, or needle aponeurotomy)
• Collagenase- 19.3%– Joint contracture to ≥20° in the presence of a palpable
cord in a joint that previously achieved a 0°-5° contractureimprovement or (b) a joint which underwent surgicalcorrection to treat contracture in that joint
• Update of original diathesis factors– Positive family history– Male gender– Early age
(<50 years) of onset– Bilateral involvement– Garrod’s pads
Hindocha S et al. J Hand Surg. 2006;31A:1626-1634.Rayan GM. Dupuytren’s disease: anatomy, pathology, presentation, and treatment. J Bone Joint Surg Am. 2007;89A(1):190-198.Tubiana R et al. Dupuytren’s Disease. London: Martin Dunitz Ltd.; 2000;243.
Predictive risk of recurrence 22% when no factors are present 71% when all 5 factors are present
Rehabilitation
• Begin AROM and AAROM at 3 days
• Extension night time splint beginning 3 days for 6 months
• Scar mgmt
• Edema Control
• MOTION is LOTION!!
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RehabilitationSCIENTIFIC ARTICLE
• The Effect of Night Extension Orthoses Following Surgical Release of DupuytrenContracture: A Single-Center, Randomized, Controlled Trial
• Julie Collis, MSc (Hons), Shirley Collocott, BSc, Wayne Hing, PhD, Edel Kelly, MSc (Hons)
• JHS, 38A 2013
– randomized, controlled trial
– No difference at three months • therapy and nighttime splinting
• therapy alone
Questions?
SITE LAYOUT
Basic Science• Theory of local microvessel ischemia which
stimulates proliferation of fibroblasts and related cell types.
• Related growth factors – myofibroblast proliferation:
– TGF-1, TGF-– PDGF– bFGF
• Cytokines responsible for myofibroblast contraction:
– PGF2 alpha– LPA– Angiotensin II– Serotonin
• ratio of type III to type I collagen increased
• IFN-Ca++ Channel blockers counter effect of TGF-1
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OITE
• Surgical intervention is first indicated for DD when which of the following findings is present?
– MCPJ contracture > 60° and a PIPJ contracture of any degree
– MCPJ contracture and a PIPJ contracture > 40°
– MCPJ contracture > 30° or PIPJ contracture of any degree
– MCPJ contracture of any degree and a PIPJ contracture > 30°
– Any contracture of the MCPJ or PIPJ
Cellular StructureTomasek, et al JBJS-A 69:1987
• Myofibroblast - cell responsible for tissue contraction
• Fibronectin– migration
– differentiation
– adhesion
• TGF-B1
OITE
• When performing palmar fasciectomy for DD, which of the following should not be performed at the same time?
– Trigger finger release
– Intra-operative digital nerve laceration repair
– Knuckle pad excision
– PIPJ arthrodesis
– Carpal tunnel release
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