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Dupuytrens disease1


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Dupuytrens disease1

  1. 1. Dupuytren’s Disease• Baron Guillaume Dupuytren, 1831 – Described the condition of palmar fascial contraction
  2. 2. 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• Associations1. Alcohol and liver disease Icelandic cohort study2. Smoking3. Manual work4. Diabetes5. Epilepsy
  3. 3. 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
  4. 4. Palmar Fascia Anatomy• Palmar Aponeurosis• Hypothenar Aponeurosis• Thenar Aponeurosis• Digital Fascia
  5. 5. 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
  6. 6. 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
  7. 7. • Deep layer – passes vertically on either side FTS at the level of the A1 pulley and terminates in the vicinity of the extensor tendon
  8. 8. Pretendinous Bands
  9. 9. Palmar Aponeurosis - Vertical Fibers• Exist throughout• Superficially they connect the PA to the dermis• Deep fibers are three types1. Septa of Legueu and Juvara2. McGrouther’s Fibers3. Vertical septa between the lumbricals and flexor tendons
  10. 10. • 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
  11. 11. • 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
  12. 12. 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
  13. 13. 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
  14. 14. 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
  15. 15. Digital Fascia• The digital fascia holds the skin in position as the fingers or thumbs are moved1. Grayson’s ligament – midaxial, palmar2. Cleland’s ligament – thicker, midaxial, dorsal
  16. 16. Digital Fascia3. Lateral Digital Sheet – superficial fascia lateral to the nv bundles – NL , Spiral band4. Retrovascular band – deep to the nv bundles longitudinal fibers
  17. 17. 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
  18. 18. Central cord ADM
  19. 19. Natatory & Central Cord
  20. 20. Spiral Band of GossetPretendinous band, itsdistal continuation, thelateral digital sheet andthe Grayson’s ligamentMay involve theretrovascular bandGradual contraction ofthe spiral cord pulls thenv bundle towards themidline which maycome to lie transverseto the long axis
  21. 21. Spiral Band of Gosset
  22. 22. 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
  23. 23. 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 Dupuytrens
  24. 24. 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
  25. 25. 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
  26. 26. 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
  27. 27. 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
  28. 28. Murell’s Free Radical HypothesisLocalized ischemia IncreasedFree Radical Hypoxanthinegeneration concentrationMicrovs narrow Xanthinelumina thickened oxidase activityendothelial cells 1990Endothelialxanthine oxidasederived freeradicalsStimulatefibroblastslocalized damagePericyte damagecollagen depositionfurther ischemia
  29. 29. Unifying Pathogenesis Andrew 1991
  30. 30. Clinical Presentation Anatomical Distribution Skin Involvement Contracture Digital Allen’s Test Maturity General Condition
  31. 31. Anatomical Distribution Normally Ulnar One or more digit Different stages of involvement
  32. 32. Skin Involvement
  33. 33. Nodules, Cords, Pits, SkinShortening
  34. 34. Non Operative Management1. 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
  35. 35. 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
  36. 36. Operative Management• Age• General Health• Motivation• Type of hand – Aesthetic , Workman’s• H/O CRPS• Type of involvement• Deformity and progression
  37. 37. Surgical Management• Formulation of a plan regarding the management of the skin, involved fascia, joints and extensor apparatus• Management of Skin1. IncisionLongitudinalSpiral cord – The nv bundle is pulled towards the centre and may lie transversely just under the skin
  38. 38. Skin Management
  39. 39. 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
  40. 40. Surgical Management
  41. 41. 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
  42. 42. Skin Replacement
  43. 43. Skin Replacement
  44. 44. Skin Replacement• Flaps1. Cross finger2. Seagull3. Dorsal transposition flaps4. Dorsal Rotation Flaps5. Flag Flaps
  45. 45. Contracted Fascia Management• Fasciotomy• Fasciotomy and grafting Extensive Dupuytren’s --- Firebreak• Fasciectomy1. Segmental2. Complete Longitudinal fasciectomy3. Radical Palmar Fasciectomy4. Dermofasciectomy
  46. 46. Contracted Fascia
  47. 47. Contracted Fascia
  48. 48. 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
  49. 49. 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
  50. 50. Complications• 17-19 % 0verall• Intra operative Nerve Injury Digital circulation Skin flap Thinning , Button hole• Post operative Haematoma Skin loss Infection Edema Wound Dehiscence
  51. 51. 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
  52. 52. 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 failure1. Failure to remove all the involved tissues2. Failure to correct PIP joint contractures at initial surgery
  53. 53. Recurrence3. Failure to recognize central slip attenuation at initial procedure 80 % of patients with a 60 deg flexion contracture at PIPJ4. Failure to recognize perivascular fibrosis leading to ischemia on full extension5. Poor cooperation with hand therapy After excision of fascia haematoma and scarring is inevitable Vigorous hand therapy limits the effect of scarring