Dupuytren's, other fibrous or stenosing hand, hand tumors! Flashcards
Define Dupuytren’s disease
- Benign fibroproliferative disorder of superficial palmar and digital fascia +/- dermis
Describe etiology of Dupuytren’s disease
- Combinatin of genetic, environment factors and altered connective tissue formation / cytokines
- Genetic:
- AD with variable penetrance
- strong association to HLA-DR3
- “Northern European / Celtic” (or just plain European) > 80%
- M>F 9:1
- Environmental - controversial RFs
- repetitive work (RR 5.5), DM, ETOH, smoking, (epilepsy, HIV, cancer)
- Altered connective tissue
- b-FGF - increased myofibroblast, endothelial c
- TGF-b - increased mesenchymal cells and myofibroblast, deposition of ECM
- PDGF - increased myofibroblast, protein (type III coll) deposition
- Free radicals - xanthine oxidase, hypoxanthine
Describe natural history of Dupuytren’s disease
Described by Luck (similar to 3 phases of wound healing)
- Proliferative - nodule formation; cellular phase, myofibroblasts, type III collagen
- Involution - cord formation; laying longitudinal bands of collagen, less cellularity and more organization
- Resolution - mature cord w/ contracture; hypocelllarity with organized collagen (type III)
Describe theories of pathogenesis for Dupuytren’s
4 theories
- Intrinsic - cords arise from normal tissues, and follow predictable routes of development
- Extrinsic - nodules arise de novo superficial to normal palmar structures
- Synthesis - combination of intrinsic and extrinsic, nodules and cords are different forms/subtypes of same disease
- Murrell’s hypothesis of positive feedback: microvessel narrowing - local ischemia - free radicals - myofibroblast - cord and contracture - more narrowing
What is the differential diagnosis for Dupuytren’s disease
- traumatic palmar fascitis
- DM fascial thickening
- Callous
- Camptodactyly
- Arthritis
- Tumour
- post-traumatic scar and joint stiffness
What is dupuytren’s diathesis
More severe form of disease characterized by:
- Bilateral, radial sided involvement
- Family history
- in men
- earlier age of diagnosis (< 50)
- with ectopic sites: Garrod knuckle pads, Lederhosen plantar involvement, Peyronie’s penile involvement
Discuss anatomic origin, diseased structure and clinical significance of anatomy and pathoanatomy in Dupuytren’s disease
Palm
- Pretendinous cord - from pre-tendinous band - causes MCP flexion
- Vertical cord - from Bands of Legue and Juvura - can cause painful triggering, tethering
Palmo-digital
- Spiral cord - from pre-tendinous band, spiral band, natatory ligament, Grayson’s ligament - MCP flexion, can displace NV bundle central, proximal, superficial
- Natatory cord - from natatory ligament - adduction contracture
Digit
- Central cord - digital extension of pre-tendinous cord (no normal anatomic origin) - PIPJ contracture
- Retrovascular cord (from retrovascular band) and lateral digital cord (from lateral digital sheet) PIPJ +/- DIPJ contracture
- ADM cord - from ADM tendon - D5 PIPJ contracure
Thumb
- proximal and distal commissural cord - from proximal and distal commissural ligament - web contracture
What structures are usually uninvolved in Dupuytren’s?
- superficial transverse fibres
- Cleland’s ligament
- deep transverse ligament
- Oblique retinacular ligament / Landsmeer’s ligament
List treatments for Dupuytren’s disease
- Non surgical modalities are largely ineffective
- Collagenase histolyticum - Xiaflex
- Needle aponeurotomy
- Open fasciotomy
- Open partial fasciectomy
- Dermatofascietcomy with skin grafting
- Radical fasciectomy
- Amputation
- Discuss the indication, proportion success, risk of recurrence, advantages, disadvantages and complications of needle aponeurotomy
- Ideal for palmar pretendinous cord that is discrete (ie for MCPJ contracture)
- Success ~ 50-55% < 5’ contracture
- Recurrence is ~ 60% at 3 yrs and 85% at 5 yrs, but studies do show variable recurrence
- Risks: tendon laceration, digital nerve laceration, skin laceration and delayed wound healing
- Advantages: quick, easy, symptom relief, low morbidity, multiple cords at once
- Disadvantages: (ealry) recurrence, difficult to teach and observe
Discuss the indication, proportion success, risk of recurrence, advantages, disadvantages and complications of collagenase injection for Dupuytren’s
- Indications: best for discrete cords in palm and proximal finger; MCP & PIP contracture > 20’
- Sucess: ~ 65% will have < 5’ flexion contracture
- Recurrence: ~ 35% at 3 years
- Risks: bruising, swelling, lymphadenopathy, tendon or pulley rupture, CRPS
- Advantages: less early recurrence, minimal morbidity, reasonable success
- Disadvantages: increased risk recurrence vs. open, cost, availability
Discuss the indication, proportion success, risk of recurrence, advantages, disadvantages and complications of open partial fasciectomy for Dupuytren’s
- Indications: limited function, difficulty w/ hygeien, MPC > 30’, PIP any, + table top, rapid progression; may be better for recurrence, revision, diffuse disease
- Risks: classic triad (hematoma, infection, flap necrosis), tendon injury, digital nerve/arterial injury, recurrence, scar, stiffness/pain
- Success: > 90% < 5’; recurrence: 5% @ 5 yrs (variable in literature)
- Advantages: least recurrence, easiest to teach/observe, currently thought to be safer modality for re-do
- Disadvantages: greatest morbidity
Define stenosing tenosynovitis
Tendon impingement of digital flexors as they pass through narrowed pulley and sheath; greatest impingement as flexors cross over MCP head and through A1 pulley
Discuss pathophysiology of trigger finger
- Overall - size mismatch between tendon and sheath
- Tendon - impaired nutrition / vascularity - tendon degeneration / fibrous proliferation
- Sheath - repeated use, friction, edema, fibrocartilaginous metaplasia
What are the important features when counselling regarding dupuytren’s
- clinically benign
- high recurrence and/or new disease progression
- no cure or prevention
- high treatment complications (espeically with open surgery)
- significant rsk