2. Patient Complaints
Fingers get in the way with:
Washing face
Combing hair
Putting hand in pocket
Racquet sports
Golf
Putting hand in glove
3.
4. 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.
5. 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.
6. 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.
8. 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
9. 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.
10. 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.
11. 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%).
12. Statistics
7:1 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.
13. 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.
14. 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.
15. History
Felix 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!
16. Pathology
Two 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
17. Cell Types
Two 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.
18. Histological Stages
Proliferative 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.
19. Proliferative stage
(fibroblasts present)
A=high cellular area (dark staining)
B=normal (less cellular)
1.
Moermans JP
Segmental apeunorectomy in Dupuytren's Disease
Dupuytren's Disease Edited by: R. M. McFarlane, D.A. McGrouther, M.H. Flint
Churchill-Livingstone pp 352-356 1990
20. Involutional Stage
Fibroblasts aligning themselves with
lines of stress
1.
Moermans JP, Segmental apeunorectomy in Dupuytren's Disease
Edited by: R. M. McFarlane, D.A. McGrouther, M.H. Flint
Churchill-Livingstone pp 352-356 1990
21. Pathology
Mechanical 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.
22. 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.
23. 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.
24.
25. 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 TGFbeta.
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.
26. 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.
27.
28.
29. Webspace anatomy
Grayson’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.
30.
31. Dupuytren Anatomy
Pathologic 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.
32. Pathological Cords
Central 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
33. 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.
34. 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.
35. 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.
36. Management of
Palmar Fascia
Methods include:
Radical vs. Selective vs. Segment
Fasciectomy
Fasciotomy
Amputation
Joint resection and arthrodesis
37. 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
38. 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
40. Direct Closure
Direct closure
Primary wound healing
No need for skin grafts
Simple post-op management
Increased incidence of
hematoma
Skin flap necrosis
41. 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
42. 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
43. 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.
44. Complications
Intra-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%.
45. Recurrence
Presence 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).
46. 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.
47. 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.
48. 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.
49. 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.
50. References
Niel Meeta
Alman, B.A.; Goldberg, M.J.;Jiranek, W.A.; Terek, R.M.; Wolfe, H.J.: The expression of PlateletDerived 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
