Dupuytrens disease

Dupuytrens disease
Dupuytren’s Disease

• Baron Guillaume Dupuytren, 1831
  – Described the condition of palmar fascial
    contraction
Epidemiology
•    Prevalence – Age, sex, Race, Geographical distribution
•    Increasing Age Peaks between 40-60
•    Men > Women 7-15 times
•    White Caucasians of North European descent
•    Genetics unclear autosomal dominant, variable penetrance
•    Associations
1.   Alcohol and liver disease Icelandic cohort study
2.   Smoking
3.   Manual work
4.   Diabetes
5.   Epilepsy
Strong Diathesis
•   Strong family history
•   Young patient
•   Bilateral disease with radial involvement
•   Diffuse dermal involvement
•   Lederhosen – soles of feet
•   Peyronie’ s disease – penile
•   Garrod’s knuckle pads – PIP joints
•   Recurrence and extension
Palmar Fascia Anatomy
•   Palmar           Aponeurosis
•   Hypothenar         Aponeurosis
•   Thenar            Aponeurosis
•   Digital Fascia
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
Pretendinous Bands
• McGrouther – three different insertions for the
  pretendinous bands
• Superficial layer – terminates into the dermis distal to
  the MCP joint midway between the distal palmar and
  proximal digital creases
• Intermediate layer – passes deep to the natatory
  ligament and the neurovascular bundles, merges with
  the lateral digital sheath. Spiral bands of Gosset and
  may attach to the retrovascular band
• Deep layer – passes vertically on either side
  FTS at the level of the A1 pulley and
  terminates in the vicinity of the extensor
  tendon
Pretendinous Bands
Palmar Aponeurosis - Vertical Fibers

•  Exist throughout
•  Superficially they connect the PA to the
   dermis
• Deep fibers are three types
1. Septa of Legueu and Juvara
2. McGrouther’s Fibers
3. Vertical septa between the lumbricals and
   flexor tendons
• Septa of Legueu and Juvara – well developed fibrous
  structures arising from the deep surface of PA at the
  level of the MC head and neck
• Pass down to the palmar plate and fascia over the
  interossei
• Most developed distally where they blend with the
  deep transverse intermetacarpal ligament
• They have a sharp proximal border lying 1cm distal
  to the superficial palmar arch and approx. 1 cm in
  length
• Eight septa, one on either side - four fibro
  osseous tunnels
• Each tunnel has three compartments
  containing the common neurovascular bundles
  and the lumbricals
• The radial nv bundle of index and the ulnar nv
  bundle of little are not included
Palmar Aponeurosis – Transverse
              Fibers
• Natatory Ligament (NL, Superficial transverse
  metacarpal ligament, STML)
• Transverse ligament of the palmar aponeurosis
  (TLPA)
The TLPA differs from the deep transverse
  intermetacarpal ligament It is a distinct part of
  the palmar aponeurosis and gives origin to the
  vertical fibers of L&J
Hypothenar Aponeurosis
• Covers the muscles of the hypothenar eminence
• Continuous with the ulnar border of the palmar
  aponeurosis
• Merges distally with the tendon of ADM and
  continues close to the lateral digital sheath
• Also attached to the palmar plate of the mcp joint,
  TLPA, ulnar saggital band while vertical fibers
  connect to the dermis
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
• The distal transverse commissural ligament –
  NL
• The proximal transverse commissural ligament
  - TLPA
Digital Fascia
• The digital fascia holds the skin in
  position as the fingers or thumbs are
  moved
1. Grayson’s ligament –        midaxial,
   palmar
2. Cleland’s ligament –         thicker,
   midaxial, dorsal
Digital Fascia
3. Lateral Digital Sheet – superficial fascia
   lateral to the nv bundles – NL , Spiral band
4. Retrovascular band – deep to the nv bundles
   longitudinal fibers
Pathologic Anatomy
• Normal fascial structures in the hand and
  digits are referred to as bands
• Diseased fascial structures in Dupuytren’s are
  referred to as cords
• Palm – Pretendinous cord resulting in MCPJ
  flexion Does not affect the nv bundles
     - Vertical cords can cause pain and
  triggering
Dupuytrens disease
Central cord ADM
Natatory & Central Cord
Spiral Band of Gosset
Pretendinous band, its
distal continuation, the
lateral digital sheet and
the Grayson’s ligament
May involve the
retrovascular band
Gradual contraction of
the spiral cord pulls the
nv bundle towards the
midline which may
come to lie transverse
to the long axis
Spiral Band of Gosset
Biology and Biochemistry
• Fibroblast proliferation, collagen deposition
• LUCK, Three Stages
• Proliferative Stage – increased number of cells during
  nodule formation
• Involutional Stage – longitudinal bands of collagen
  fibers – less biologically active
• Residual Stage – biologically quiescent
  disappearance of cells, contracted cords densely
  packed tough inelastic fibrotic palmar fascia
Biology and Biochemistry
• Biologically similar to wound repair
• Myofibroblasts – nodules
• Gelberman – myofibroblasts in nodules and
  fibroblasts with prominent microtubules in the
  fascia
• Murell – compared fibroblasts in both tissues
  and found them identical Just more abundant
  in Dupuytren's
Biology and Biochemistry
• Increased levels of fibroblastic growth factors in the diseased
  fascia
• Macrophages – perivascular cuff early in disease
• Basic Fibroblastic growth factor (bFGF)
  bFGF – proliferation of myofibroblasts and endothelial cells
  which narrow the lumina of vs
• Platelet derived growth factor (PDGF)
  cellular signal for myofibroblast proliferation
  increase rate of type III collagen synthesis
• Transforming growth factor (TGF beta)
  stimulates growth of mesenchymal cells, fibroblasts
  increased deposition of extra cellular matrix
Pathogenesis
• Intrinsic theory- McFarlane 1974
• Diseased cords are as a result of the pathologic
  changes in the normal fascia
• Cords arise from defined fascial precursors
  and progress along predictable routes
  determined by normal fascial anatomy
• Does not offer a rational explanation for the
  central cord commonly seen in Dupuytren’s
Pathogenesis
• Extrinsic Theory – Hueston 1985
• The fibrotic process begins with nodules and
  progresses to cords
• Nodules arise de novo by metaplastic transformation
  of fibrofatty tissue and later spread as cords just
  superficial to the palmar aponeurosis
• Rational explanation for the presence of nodules,
  recurrence after fascial excision, lower recurrences
  after dermofasciectomy
Pathogenesis
• Synthesis Theory - Gosset’s 1985
• Cords and nodules represent different forms rather
  than different stages of the disease process
• Cords arise from palmar fascia and nodules de novo
• Anatomic studies Strickland and Leibovic support the
  synthesis theory as the best reconciliation between
  the intrinsic and extrinsic
Murell’s Free Radical Hypothesis
Localized ischemia             Increased
Free Radical                   Hypoxanthine
generation                     concentration

Microvs narrow                 Xanthine
lumina thickened               oxidase activity
endothelial cells              1990
Endothelial
xanthine oxidase
derived free
radicals
Stimulate
fibroblasts
localized damage
Pericyte damage
collagen deposition
further ischemia
Unifying Pathogenesis Andrew 1991
Clinical Presentation
               Anatomical
               Distribution
               Skin Involvement
               Contracture
               Digital Allen’s Test
               Maturity
               General Condition
Anatomical Distribution

             Normally Ulnar
             One or more digit
             Different stages of
             involvement
Skin Involvement
Nodules, Cords, Pits, Skin
Shortening
Non Operative Management
1. External Fixation and Distraction
• Progressive lengthening of the digital nv bundle
• Easier access to the diseased cords after distraction
   in the fingers that are very contracted
• Only useful in end stages
• Long distraction time
• Complications – infection, stiffness, pain, CRPS
   type I
• Contractures recur quickly without fasciectomy
Non Operative Management
• Collagenase – achieved full extension in 90%
  patients with a single injection and maintained
  9 mths after treatment
• Radiotherapy, dimethyl sulfoxide, ultrasound,
  steroids, colchicine, alfa interferon None has
  shown any significant benefit
Operative Management
•   Age
•   General Health
•   Motivation
•   Type of hand – Aesthetic , Workman’s
•   H/O CRPS
•   Type of involvement
•   Deformity and progression
Surgical Management
•  Formulation of a plan regarding the
   management of the skin, involved fascia,
   joints and extensor apparatus
• Management of Skin
1. Incision
Longitudinal
Spiral cord – The nv bundle is pulled towards
   the centre and may lie transversely just under
   the skin
Skin Management
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 replacement
Surgical Management
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
Skin Replacement
Skin Replacement
Skin Replacement
•    Flaps
1.   Cross finger
2.   Seagull
3.   Dorsal transposition flaps
4.   Dorsal Rotation Flaps
5.   Flag Flaps
Contracted Fascia Management
•    Fasciotomy
•    Fasciotomy and grafting Extensive
     Dupuytren’s --- Firebreak
•    Fasciectomy
1.   Segmental
2.   Complete Longitudinal fasciectomy
3.   Radical Palmar Fasciectomy
4.   Dermofasciectomy
Contracted Fascia
Contracted Fascia
Contracted PIP Joint
• Gentle passive manipulation
  40% peri articular adhesions which require GPM
• Long standing contractures may produce joint
  changes requiring surgical release
  Volar plate – check rein ligaments
  Accessory collateral ligaments
  Flexor tendon sheath release between A2-A4
  Vascular compromise
• Extensor apparatus – patients with 60 degree
  contracture, 80% will show central slip attenuation
Postoperative Rehabilitation
• Very important
• Commenced after early inflammatory phase
  (3-5 days)
• ROM exercises, short periods, repetitive
• Splinting, initial static for 2 wks, MCPJ 10-20
  deg. Flexion, PIPJ straight, DIPJ free then PIP
  splint at night – 8-10 wks.
• Scar management
Complications
• 17-19 % 0verall
• Intra operative
  Nerve Injury
  Digital circulation
  Skin flap Thinning , Button hole
• Post operative
  Haematoma
  Skin loss
  Infection
  Edema
  Wound Dehiscence
Complications
• Dupuytren’s Flare – Inflammatory reaction occurring 2-3 wks
  after the surgery
  More common in women 20 %
  Acute carpal tunnel syndrome
  Redness, pain, edema, stiffness
  Sympathetic blockade, oral steroids, carbamazapine
• Reflex Sympathetic Dystrophy – 5 x more common in women
  (5 %)
  Pain, edema, stiffness, vasomotor symptoms
  Sympathetic blockade, oral steroids, carbamazapine
Recurrence and Extension
•  Recurrence is the reappearance of disease in the
   area of previous surgery
   26-80 %
• Extension is the appearance of new disease in an
   area not subjected to surgery
• Common causes of failure
1. Failure to remove all the involved tissues
2. Failure to correct PIP joint contractures at initial
   surgery
Recurrence
3. Failure to recognize central slip attenuation at initial
   procedure 80 % of patients with a 60 deg flexion
   contracture at PIPJ
4. Failure to recognize perivascular fibrosis leading to
   ischemia on full extension
5. Poor cooperation with hand therapy After excision
   of fascia haematoma and scarring is inevitable
   Vigorous hand therapy limits the effect of scarring
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Dupuytrens disease

  • 2. Dupuytren’s Disease • Baron Guillaume Dupuytren, 1831 – Described the condition of palmar fascial contraction
  • 3. Epidemiology • Prevalence – Age, sex, Race, Geographical distribution • Increasing Age Peaks between 40-60 • Men > Women 7-15 times • White Caucasians of North European descent • Genetics unclear autosomal dominant, variable penetrance • Associations 1. Alcohol and liver disease Icelandic cohort study 2. Smoking 3. Manual work 4. Diabetes 5. Epilepsy
  • 4. Strong Diathesis • Strong family history • Young patient • Bilateral disease with radial involvement • Diffuse dermal involvement • Lederhosen – soles of feet • Peyronie’ s disease – penile • Garrod’s knuckle pads – PIP joints • Recurrence and extension
  • 5. Palmar Fascia Anatomy • Palmar Aponeurosis • Hypothenar Aponeurosis • Thenar Aponeurosis • Digital Fascia
  • 6. 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
  • 7. Pretendinous Bands • McGrouther – three different insertions for the pretendinous bands • Superficial layer – terminates into the dermis distal to the MCP joint midway between the distal palmar and proximal digital creases • Intermediate layer – passes deep to the natatory ligament and the neurovascular bundles, merges with the lateral digital sheath. Spiral bands of Gosset and may attach to the retrovascular band
  • 8. • Deep layer – passes vertically on either side FTS at the level of the A1 pulley and terminates in the vicinity of the extensor tendon
  • 10. Palmar Aponeurosis - Vertical Fibers • Exist throughout • Superficially they connect the PA to the dermis • Deep fibers are three types 1. Septa of Legueu and Juvara 2. McGrouther’s Fibers 3. Vertical septa between the lumbricals and flexor tendons
  • 11. • Septa of Legueu and Juvara – well developed fibrous structures arising from the deep surface of PA at the level of the MC head and neck • Pass down to the palmar plate and fascia over the interossei • Most developed distally where they blend with the deep transverse intermetacarpal ligament • They have a sharp proximal border lying 1cm distal to the superficial palmar arch and approx. 1 cm in length
  • 12. • Eight septa, one on either side - four fibro osseous tunnels • Each tunnel has three compartments containing the common neurovascular bundles and the lumbricals • The radial nv bundle of index and the ulnar nv bundle of little are not included
  • 13. Palmar Aponeurosis – Transverse Fibers • Natatory Ligament (NL, Superficial transverse metacarpal ligament, STML) • Transverse ligament of the palmar aponeurosis (TLPA) The TLPA differs from the deep transverse intermetacarpal ligament It is a distinct part of the palmar aponeurosis and gives origin to the vertical fibers of L&J
  • 14. Hypothenar Aponeurosis • Covers the muscles of the hypothenar eminence • Continuous with the ulnar border of the palmar aponeurosis • Merges distally with the tendon of ADM and continues close to the lateral digital sheath • Also attached to the palmar plate of the mcp joint, TLPA, ulnar saggital band while vertical fibers connect to the dermis
  • 15. 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 • The distal transverse commissural ligament – NL • The proximal transverse commissural ligament - TLPA
  • 16. Digital Fascia • The digital fascia holds the skin in position as the fingers or thumbs are moved 1. Grayson’s ligament – midaxial, palmar 2. Cleland’s ligament – thicker, midaxial, dorsal
  • 17. Digital Fascia 3. Lateral Digital Sheet – superficial fascia lateral to the nv bundles – NL , Spiral band 4. Retrovascular band – deep to the nv bundles longitudinal fibers
  • 18. Pathologic Anatomy • Normal fascial structures in the hand and digits are referred to as bands • Diseased fascial structures in Dupuytren’s are referred to as cords • Palm – Pretendinous cord resulting in MCPJ flexion Does not affect the nv bundles - Vertical cords can cause pain and triggering
  • 22. Spiral Band of Gosset Pretendinous band, its distal continuation, the lateral digital sheet and the Grayson’s ligament May involve the retrovascular band Gradual contraction of the spiral cord pulls the nv bundle towards the midline which may come to lie transverse to the long axis
  • 23. Spiral Band of Gosset
  • 24. Biology and Biochemistry • Fibroblast proliferation, collagen deposition • LUCK, Three Stages • Proliferative Stage – increased number of cells during nodule formation • Involutional Stage – longitudinal bands of collagen fibers – less biologically active • Residual Stage – biologically quiescent disappearance of cells, contracted cords densely packed tough inelastic fibrotic palmar fascia
  • 25. Biology and Biochemistry • Biologically similar to wound repair • Myofibroblasts – nodules • Gelberman – myofibroblasts in nodules and fibroblasts with prominent microtubules in the fascia • Murell – compared fibroblasts in both tissues and found them identical Just more abundant in Dupuytren's
  • 26. Biology and Biochemistry • Increased levels of fibroblastic growth factors in the diseased fascia • Macrophages – perivascular cuff early in disease • Basic Fibroblastic growth factor (bFGF) bFGF – proliferation of myofibroblasts and endothelial cells which narrow the lumina of vs • Platelet derived growth factor (PDGF) cellular signal for myofibroblast proliferation increase rate of type III collagen synthesis • Transforming growth factor (TGF beta) stimulates growth of mesenchymal cells, fibroblasts increased deposition of extra cellular matrix
  • 27. Pathogenesis • Intrinsic theory- McFarlane 1974 • Diseased cords are as a result of the pathologic changes in the normal fascia • Cords arise from defined fascial precursors and progress along predictable routes determined by normal fascial anatomy • Does not offer a rational explanation for the central cord commonly seen in Dupuytren’s
  • 28. Pathogenesis • Extrinsic Theory – Hueston 1985 • The fibrotic process begins with nodules and progresses to cords • Nodules arise de novo by metaplastic transformation of fibrofatty tissue and later spread as cords just superficial to the palmar aponeurosis • Rational explanation for the presence of nodules, recurrence after fascial excision, lower recurrences after dermofasciectomy
  • 29. Pathogenesis • Synthesis Theory - Gosset’s 1985 • Cords and nodules represent different forms rather than different stages of the disease process • Cords arise from palmar fascia and nodules de novo • Anatomic studies Strickland and Leibovic support the synthesis theory as the best reconciliation between the intrinsic and extrinsic
  • 30. Murell’s Free Radical Hypothesis Localized ischemia Increased Free Radical Hypoxanthine generation concentration Microvs narrow Xanthine lumina thickened oxidase activity endothelial cells 1990 Endothelial xanthine oxidase derived free radicals Stimulate fibroblasts localized damage Pericyte damage collagen deposition further ischemia
  • 32. Clinical Presentation Anatomical Distribution Skin Involvement Contracture Digital Allen’s Test Maturity General Condition
  • 33. Anatomical Distribution Normally Ulnar One or more digit Different stages of involvement
  • 35. Nodules, Cords, Pits, Skin Shortening
  • 36. Non Operative Management 1. External Fixation and Distraction • Progressive lengthening of the digital nv bundle • Easier access to the diseased cords after distraction in the fingers that are very contracted • Only useful in end stages • Long distraction time • Complications – infection, stiffness, pain, CRPS type I • Contractures recur quickly without fasciectomy
  • 37. Non Operative Management • Collagenase – achieved full extension in 90% patients with a single injection and maintained 9 mths after treatment • Radiotherapy, dimethyl sulfoxide, ultrasound, steroids, colchicine, alfa interferon None has shown any significant benefit
  • 38. Operative Management • Age • General Health • Motivation • Type of hand – Aesthetic , Workman’s • H/O CRPS • Type of involvement • Deformity and progression
  • 39. Surgical Management • Formulation of a plan regarding the management of the skin, involved fascia, joints and extensor apparatus • Management of Skin 1. Incision Longitudinal Spiral cord – The nv bundle is pulled towards the centre and may lie transversely just under the skin
  • 41. 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 replacement
  • 43. 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
  • 46. Skin Replacement • Flaps 1. Cross finger 2. Seagull 3. Dorsal transposition flaps 4. Dorsal Rotation Flaps 5. Flag Flaps
  • 47. Contracted Fascia Management • Fasciotomy • Fasciotomy and grafting Extensive Dupuytren’s --- Firebreak • Fasciectomy 1. Segmental 2. Complete Longitudinal fasciectomy 3. Radical Palmar Fasciectomy 4. Dermofasciectomy
  • 50. Contracted PIP Joint • Gentle passive manipulation 40% peri articular adhesions which require GPM • Long standing contractures may produce joint changes requiring surgical release Volar plate – check rein ligaments Accessory collateral ligaments Flexor tendon sheath release between A2-A4 Vascular compromise • Extensor apparatus – patients with 60 degree contracture, 80% will show central slip attenuation
  • 51. Postoperative Rehabilitation • Very important • Commenced after early inflammatory phase (3-5 days) • ROM exercises, short periods, repetitive • Splinting, initial static for 2 wks, MCPJ 10-20 deg. Flexion, PIPJ straight, DIPJ free then PIP splint at night – 8-10 wks. • Scar management
  • 52. Complications • 17-19 % 0verall • Intra operative Nerve Injury Digital circulation Skin flap Thinning , Button hole • Post operative Haematoma Skin loss Infection Edema Wound Dehiscence
  • 53. Complications • Dupuytren’s Flare – Inflammatory reaction occurring 2-3 wks after the surgery More common in women 20 % Acute carpal tunnel syndrome Redness, pain, edema, stiffness Sympathetic blockade, oral steroids, carbamazapine • Reflex Sympathetic Dystrophy – 5 x more common in women (5 %) Pain, edema, stiffness, vasomotor symptoms Sympathetic blockade, oral steroids, carbamazapine
  • 54. Recurrence and Extension • Recurrence is the reappearance of disease in the area of previous surgery 26-80 % • Extension is the appearance of new disease in an area not subjected to surgery • Common causes of failure 1. Failure to remove all the involved tissues 2. Failure to correct PIP joint contractures at initial surgery
  • 55. Recurrence 3. Failure to recognize central slip attenuation at initial procedure 80 % of patients with a 60 deg flexion contracture at PIPJ 4. Failure to recognize perivascular fibrosis leading to ischemia on full extension 5. Poor cooperation with hand therapy After excision of fascia haematoma and scarring is inevitable Vigorous hand therapy limits the effect of scarring