Dupuytrens Contracture

All about Dupuytren's disease and contracture
History, presentation, treatment and recent advances

1. Dupuytren’s Disease Dr. Apoorv Jain D’Ortho , DNB Ortho [email protected] [phone]

2. Introduction In 1831,Baron Guillaume Dupuytren described the condition of palmar fascial contraction ( Dupuytren 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 .

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

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

5. Epidemiology Age: Incidence increases with increasing a ge and peaks between 40-60 years Sex: Males > Females (7-15 times) Race: White Caucasians Geography: North European descent Genetics is U nclear ( Autosomal dominant with variable penetrance)

6. 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 population.

7. 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.

8. Famous patients include Ronald Reagan, Margaret Thatcher, and creator of Captain Hook (inspiration for his claw hand).

9. Associated with: Diabetes mellitus Cigarette smoking Alcoholism and liver disease HIV infection Epilepsy: Anti-epileptic drug P henobarbitone Trauma Manual labor Rheumatoid disease Previous myocardial infarction Plantar fasciitis Peyronie disease

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. Patient Complaints Fingers get in the way with: Washing face Combing hair Putting hand in pocket Racquet sports Golf Putting hand in glove

12. 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 ).

13. MCP joint affected first and then PIP joint. Ring and small finger affected first, after palmar involvement .

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

16. 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.

17. Grading

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

19. Relevant Anatomy

20. 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

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

22. 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

23. 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

24. Natatory & Central Cord

25. 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

26. 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

27. 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

28. Digital Fascia It holds the skin in position as the fingers or thumb move Grayson’s ligament: M idaxial , Palmar Cleland’s ligament: Thicker, M idaxial , Dorsal

29. 3. Lateral Digital Sheet: S uperficial fascia lateral to the Neurovascular bundles 4. Retrovascular band: D eep to the Neurovascular bundles, longitudinal fibers

30. 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

31. Spiral Band of Gosset

32. 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 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.

33. 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).

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

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

36. Role Of P rotein F actors PDGF, FGF, TGF-B  increased collagen production Myofibroblasts are more sensitive Nodules & Cords: Major forms of diseased tissues Two distinct histological tissues

37. Nodules Dense cellular collections of myofibroblasts : indicates centers of high metaplastic activity. LUCK described 3 stages of progression of nodule: Proliferative : Young nodules with non-stress aligned fibroblasts, grows & fuses to skin Involutional : Growth stops, S tress alignment of fibroblasts, More collagen  F ascial hypertrophy  Nodule cord units Residual : Size reduces, A celullar fibrous cords

38. Nodules, Pits, Skin Contractures

39. 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

40. Treatment

41. Non Operative Management Collagenase Studies show good results in 90% patients with a single injection and maintained 9 months after treatment Radiotherapy, Dimethyl sulfoxide , Ultrasound , Steroids , Colchicine , Alfa interferon: None has shown any significant benefit

42. 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°

43. 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

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

45. Surgical Fasciectomy Radical Fasciectomy : M ostly abandoned All palmar fascia removed High amounts of wound complications, and recurrence Selective Fasciectomy : M ost commonly used Removal of all diseased fascia in palm/finger Indicated when only ulnar one or two fingers involved Rate of recurrence is 50% N eed for another surgery: 15 % Recurrence due to undetectable diseased fascia remaining

46. 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

47. Incision ( B asic Principles) No incision should cross a flexion crease at right angles on wound closure

48. 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.

49. 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

50. 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

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

52. Direct closure: Primary wound healing No need for skin grafts Simple post-op management Increased incidence of H ematoma and Skin flap necrosis

53. 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

54. 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

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

56. Joint R esection- Arthrodesis Severely contracted PIP joint Avoids the potential for recurrent PIP joint contracture and potential amputation neuroma

57. Amputation Rare M ay be indicated: In Flexion contracture of PIP joint , especially little finger, when cannot be corrected enough to make finger useful Or in case of vascular compromise

58. Newer Treatment Modalities

59. Collagenase Enzymatic percutaneous fasciotomy of residual stage disease Collagenase diluted in calcium chloride Currently treatment only available at stony brook medical center , under FDA “orphan drug status” in phase III trials Injected straight into nodule

60. 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

61. Needle Aponeurotomy Fascia contractures sectioned percutaneously with sharp-edged bevel of local anesthetic needle. The treatment is only performed in Europe , primarily France. Outpatient, $150 for 20 minute session and requires no physical therapy. Temporary treatment, not cure.

62. 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

63. 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

64. 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%.

65. 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

66. 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 .

67. Recurrence rates are more in presence of residual tissue incompletely excised, leaving behind myofibroblasts in skin. F ull skin grafts rarely recur, due to complete removal of all nodular area in dermis and epidermis.

68. Summary Dupuytren’s contracture is a 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.

69. Thank You