fully updated dupuytren's disease

1. GOOD MORNING

2. Dupuytren’s Disease
Dr. Indranil Dutta
DNB Trainee Plastic Surgery
Shija Hospitals & Research Institute
Imphal, Manipur, India

3. Introduction
• In 1831,Baron Guillaume Dupuytren
described the condition of palmar
fascial contraction (Dupuytren’s disease).
• It is a proliferative fibroplasia of the
subcutaneous palmar tissue, occurring in the
form of nodules and cords, that may result in
secondary progressive and irreversible flexion
contractures of the finger joints.

4. • Other changes include:
–thinning of the overlying subcutaneous fat
–adhesion to skin
–and later pitting or dimpling of the skin.

5. History
• Felix plater (1536-1614) gave the 1st description
of palmar fibromatosis.
• Henry Cline (1750-1836) described the anatomy
& recommended surgical release.
• Astley cooper (1768-1841) explained the etiology
as repeated trauma and described percutaneous
fasciotomy.
• Guillaume Dupuytrene (1834) gave detailed
anatomic pathology, C/F, natural history, surgical
technique, postop care, response, follow up.

6. Epidemiology
• Age: Incidence increases with increasing age and
peaks between 40-60 years
• Sex: Males > Females (3-7 times)
• Race: White Caucasians
• Geography: North European descent
• Genetics is Unclear (Autosomal dominant with
variable penetrance)
• Family h/o +ve often
• Usually B/L. (Rt>Lt)

7. Associated Conditions
1. Diabetes mellitus
2. Cigarette smoking
3. Alcoholism and liver chirrhosis
4. HIV infection
5. Epilepsy: Anti-epileptic drug - Phenobarbitone
6. Trauma
7. Manual labor
8. Rheumatoid disease
9. Previous myocardial infarction
10. Plantar fasciitis
11. Peyronie’s disease

8. 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.
• A/w Knuckles pad (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.

9. CLINICAL PRESENTATION

10. Chief Complaints
Patient complaints of fingers get in
the way with:
Washing face
Combing hair
Putting hand in pocket
Racquet sports
Playing Golf
Putting hand in glove

11. Symptoms
• First notice tender nodule or progressive
palmar cord development.
• May be painless, and may avoid care until
joint motion reduced.
• Symptoms may be present bilaterally, with
one hand occurring first (not necessarily
dominant hand).

12. • MCP joint affected first and then
PIP joint.
• Ring > small finger affected first,
thereafter palmar involvement.

13. CLINICAL FINDINGS

14. Palpable
Nodules and
Cords
• Firm nodules may be tender to palpation.
• Cords proximal to nodules painless.
• Atrophic grooves or pits in skin signify adherence to
the underlying fascia.
• Tender knuckle pads over dorsal aspect of PIP
joints--indicates aggressive disease.

15. ON EXAMINATION

16. • First step – assess patient’s functional status,
complaints, goals.
• Careful documentation of degree of
contracture of each finger.
• Quality of overlying skin to plan intervention
and likelihood of leaving the wound open or
needing skin graft.

17. Hueston Positive Table top Test:
The distance marked should be zero in
a normal hand with a negative table
top test.

18. Dynamic flexion contracture:
When MCP joint is at neutral, the PIP joint contracture
is more.
When MCP joint is flexed, the deformity at PIP is
reduced.
This is attributed to the Central Cord involvement.

19. Grading

20. • Grade I: Thickened nodule & band skin tethering
& puckering – full movements.
• Grade II: Pretendinous bands involved  extension
of fingers limited.
• Grade III: Flexion contracture.

21. TUBIANA SCORING
SYSTEM

22. • The hand is divided into 5 rays.
• For each ray, the total contracture (MP+PIP+DIP) is
measured.
• Number system- depending on deformity
0–No lesion
N(0.5)–Palmar or digital nodule without flexion
deformity
1-Total flexion deformity between 0-45 degrees
2-Total flexion deformity between 45-90 degrees
3-Total flexion deformity between 90-135 degrees
4-Total flexion deformity >135 degrees

23. • For each ray, palmar lesions denoted as P and
for digital lesions D.
• For thumb P stands for lesions of the first web
space.
• H indicates fixed hyper-extension of DIP joint.
• R – recurrence after operation, E – extension
of disease not operated, A- finger amputation

24. DIFFERNENTIAL DIAGNOSIS
1. Epidermoid cyst
2. Foreign body
3. Desmoid fibroma
4. Nodular fasciitis
5. Fibrosarcoma

25. Relevant
Anatomy

26. The Palmar Aponeurosis
• Thick triangular fascial
layer that covers the
lumbrical and flexor
tunnels between the
thenar and hypothenar
eminences
• Proximally: palmaris
longus
• Distally: Longitudinal
bands, called
Pretendinous Bands
• Bifurcates distally to
pass on either side of
the tendons

27. Vertical Fibers
• Superficially they connect the aponeurosis to
the dermis
• Deep fibers are of three types:
1. Septa of Legueu and Juvara
2. McGrouther’s Fibers
3. Vertical septa between the lumbricals and flexor
tendons

28. • Septa of Legueu and Juvara are well developed fibrous
structures arising from the deep surface of the
aponeurosis at the level of the Metacarpal head and neck
• Pass down to the palmar plate and fascia over the
interossei
• Eight septa, one on either side - four fibro osseous tunnels
• Each tunnel has three compartments containing the
common neurovascular bundles and the lumbricals

29. Transverse Fibers
• Natatory Ligament (NL, Superficial transverse
metacarpal ligament, STML)
• Transverse ligament of the palmar aponeurosis
(TLPA): It is a distinct part of the palmar
aponeurosis and gives origin to the vertical fibers
of Legueu and Juvara

30. Natatory Lig. & Central Cord

31. Pretendinous Bands
Three different insertions for the
pretendinous bands:
• Superficial layer: terminates into
the dermis distal to the MCP joint
• Intermediate layer: passes deep to
the natatory ligament and the
neurovascular bundles, merges
with the lateral digital sheath,
Spiral bands and may attach to the
retrovascular band
• Deep layer: passes vertically down
at the level of the A1 pulley and
terminates in the vicinity of the
extensor tendon

32. Hypothenar Aponeurosis
• Covers the muscles of the hypothenar
eminence
• Continuous with the ulnar border of
the palmar aponeurosis
• Merges distally with the tendon of
Abductor Digiti Minimi and continues
close to the lateral digital sheath

33. Thenar Aponeurosis
• Radial continuation of the palmar
aponeurosis, much thinner
• Skin over thenar aponeurosis more
mobile because there are a few
vertical fibers connecting it to the
dermis

34. Digital Fascia
• It holds the skin in
position as the fingers
or thumb move
1. Grayson’s ligament:
Midaxial, Palmar
2. Cleland’s ligament: Thicker,
Midaxial, Dorsal

35. 3. Lateral Digital Sheet: Superficial fascia
lateral to the Neurovascular bundles
4. Retrovascular band: Deep to the
Neurovascular bundles, longitudinal fibers

36. Spiral Band of Gosset:
Pretendinous band, the
lateral digital sheet and
the Grayson’s ligament
may involve the
retrovascular band
• Gradual contraction of the spiral cord pulls the
neurovascular bundle towards the midline which
may come to lie transverse to the long axis

37. Spiral Band of Gosset

38. Pathologic Anatomy
• Normal fascial structures in the hand and
digits are referred to as BAND.
• Diseased fascial structures in Dupuytren’s
are referred to as CORD.
• In Palm:
 Pretendinous cords are involved resulting in
MCP Joint flexion. Does not affect the
neurovascular bundles and are painless.
 Involvement of Vertical cords can cause pain
and triggering.

40. Basic Pathology
• Myofibroblasts are the histologic
hallmark of Dupuytren’s contracture
• Increase in:
–Type III collagen
–Total collagen
–Lysyl oxidase
–Glycosoaminoglycans
• Increase in cellularity (fibroblasts).

41. Pathogenesis
• Local ischemia at the microvascular
level  increase in fibroblast &
related cell types
• Fibroblasts then organize themselves
along line of stress  cords 
deformity

42. • Ischemia  free radicals 
increased cells (fibroblasts)
• Smoking, HIV, alcohol  promote
free radicals
• Increase Fibroblast 
Vasoconstriction  Ischemia (self
perpetuating cycle)

44. Role Of Protein Factors
• PDGF, FGF, TGF-B  increased
collagen production
• Myofibroblasts are more sensitive
• Nodules & Cords:
Major forms of diseased tissues
Two distinct histological tissues

45. Nodules
• Dense cellular collections of myofibroblasts: indicates
centers of high metaplastic activity.
• LUCK described 3 stages of progression of nodule:
1. Proliferative: Young nodules with non-stress
aligned fibroblasts, grows & fuses to skin
2. Involutional: Growth stops, Stress alignment of
fibroblasts, More collagen  Fascial hypertrophy
 Nodule cord units
3. Residual: Size reduces, Acelullar fibrous cords

46. Nodules, Pits, Skin Contractures

47. Cords
• No myofibroblasts
• Highly organised collagen structure similar
to tendon
• Nodules produce the contraction by
pulling the cords which expand across the
joints
Myofibroblasts found in dermal &
epidermal tissue cause recurrence

48. Treatment

49. Non-Operative Management
• No finger contracture / no pain – Reassurance.
• Collagenase Studies show good results in 90%
patients with a single injection and maintained 9
months after treatment.
• Dimethyl sulfoxide, Ultrasound, Steroids,
Colchicine, Alfa interferon: None has shown any
significant benefit.

50. Operative Management
• Indications:
–A Positive Table Top Test: correlates with
MCP contracture of > 30-40°
–MCP joint contracture ≥ 40°
–Treatment of other digits on the same hand
should be considered when their MCP
contracture are 20-30° or more.
–PIP joint release if PIP joint contracture > 30°

51. • Important to distinguish true PIP joint
contracture from apparent contracture (due to
spiral cord)
• MCP joint contracture is measured with PIP
joint held in extension
• PIP joint contracture is measured with MCP
joint in flexion

52. Management Of Palmar Fascia
• Treatment options include:
–Radical vs. Selective vs. Segmental
Fasciectomy
–Fasciotomy
–Amputation
–Joint resection and arthrodesis

53. 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 is 50%
– Need for another surgery: 15%
– Recurrence due to undetectable diseased fascia
remaining

54. • 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

55. Incisions (Basic Principles)
• No incision should cross a flexion crease at
right angles on wound closure

56. • Thin potentially avascular flap should be
avoided.
• Dissection start in normal anatomy and
proceed distally.
• Start cord release in palm and identify Neuro
Vascular Bundle>> then palmar-digital skin
>>then digital.

57. Skin Management
• Digital Skin Shortening can be
corrected by:
–Release of skin corrugations by division
of the vertical fibers running up to the
dermis
–Multiple Z plasties
–Open palm technique
–Skin grafting

58. Skin Replacement
• Skin shortage due to dermal contracture
• Prophylactic firebreak to separate the ends of
contracted fascia
• Recurrent disease
• Electively excised as Hueston’s
dermofasciectomy
• Skin graft
• Flap

59. Management of Volar Skin
• Three types:
–Direct closure
–Full-thickness skin grafting
–Open technique with wound
contraction

60. • Direct closure:
–Primary wound healing
–No need for skin grafts
–Simple post-op management
–Increased incidence of Hematoma and
Skin flap necrosis

62. • 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

64. • 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 pre-contracture
length
– Less hematoma, wound edge necrosis, and infection
– Inconvenience during 3-5 weeks for closure

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

66. Joint Resection- Arthrodesis
• Severely contracted PIP joint.
• Avoids the potential for recurrent
PIP joint contracture and potential
amputation neuroma.

67. Amputation
• Rare.
• May be indicated:
–In Flexion contracture of PIP joint, especially
little finger, when cannot be corrected
enough to make finger useful.
–In case of vascular compromisation.

68. Newer Treatment
Modalities

69. Collagenase
• Newly approved 1st line drug.
• Collagenase reconstitution according to
package insert.
• Currently FDA approved- costly.
• Injected straight into nodule.
• Minimally invasive.
• Manipulation of finger done after 24hrs
& before 7 days.

70. Needle Aponeurotomy
• Fascia contractures sectioned
percutaneously with sharp-edged bevel of
local anesthetic 25G needle.
• The treatment is performed in Europe
mainly, primarily France, now international
acceptence.
• Minimally invasive procedure.
• Day Care Procedure and requires no physical
therapy.
• High recurrence rate.

71. Gamma Interferon
• Gamma-interferon is a 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.

72. Postoperative Rehabilitation
• Commenced after early inflammatory phase (3-
5 days)
• ROM exercises for short periods, repetitive
• Splinting:
– Initially static for 2 weeks with MCP in 10-20°
Flexion, PIP straight and DIP joint free
– After 2 weeks PIP splint at night for 8-10 weeks
• Scar management

73. 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%.

74. In Case Of Intra Operative Arterial
Insufficiency
Due to-direct trauma, traction and vasospasm
 Flex the finger
 Warm the finger with warm irrigant solution
 Apply topical papavarine (30 mg/mL) / lignocaine
 Be patient. Allow the relaxation, warming, and
antivasospasm interventions time to work. The artery may
require up to 10 minutes for the restoration of perfusion
 If arterial insufficiency persists beyond 10 minutes, explore
the digital artery throughout the extent of dissection.
Repair of a partial or complete laceration should be
performed under the operating microscope. A vein graft
may be necessary if undue tension is present

75. 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.

76. • Recurrence rates are more in 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.

77. OUTCOMES
• Outcome is variable according to severity of
disease, treatment type and joint affected.
• MCP joint contracture responds better than PIP
joint contracture for all type of treatment.
• Severe the disease, the more is risk of
complications.
• Release of contracted fingers does improve
function.
• No treatment changes the presence of disease
and recurrence/progression is expected.

78. FUTURE DIRECTIONS
• Focused on prevention of contracture.
• Radiation is being investigated for prevention of
disease progression.
• Success rate is 69%.
• No major long-term side effects.

79. Summary
• Dupuytren’s contracture is a genetic disease.
• Patient should counsel for that the disease is part
of their genetic makeup and surgeon can only treat
the symptoms and recurrence is expected.
• Newer treatment more medical and less surgical,
with eventual cure to be at genomic level.

80. Thank You