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
Describe technique of xiaflex
- 0.58ml per injection - 0.25 for MCP (palmar cord) and 0.20ml for digital
- alcohol skin prep
- inject direct into cord (can do passive dipj flxn to confirm not in tendon)
- 1/3 at entry, 1/3 distal and 1/3 proximal
- avoid > 4mm distal from proximal digital flexion crease
- direct manipulation 24 hrs later
- max 3 injections per digit
- splint x 4 mos
Describe management of PIPJ contracture
- pre-op stretching (using “Digit Widget”) with/without joint release show mean 55’ improvement compared with operative alone - mean 28’; level III (Davidson JHS european; reviewed in PRS EBM Dupuytren’s 2014)
- vs. operative joint release - steps:
- check rein ligament release anterolateral proximal volar plate
- accessory ligaments
- gentle manipulation
- +/- K-wire
Classify stenosing tenosynovitis
- Congenital
- Acquired - primary; female, single digit involvement
- Acquired - secondary; higher multiple digit involvement
- inflammatory - DM, RA, gout, renal disease
- space occupying lesion
or classify by severity
- I: pre-triggering - patient report but not observed
- II: reproducible triggering, no locking
- III: locks but manually reduces (or inability to completely flex)
- IV: cannot manually reduce
What is the differential diagnosis of trigger digit?
- Remote sites of tenosynovitis: A2, A3, A4 pulley, wrist (TCL)
- Trauma: partial tendon lac
- Infection: TB, fungi, N.gonorrhea
- Metabolic: DM (collagen deposits), crystal arthropathy, renal (Ca oxalate crystals), hypercholesterolemia (tendinous xanthoma)
- Neoplastic: space occupying lesion: fibroma, lipoma, GCT, cartilaginous / bony tumour
List and describe treatment options for acquired stenosing tenosynovitis
- Non operative:
- rest, splinting, NSAIDS - reasonable to start here for patients with grade I/II +/- III
- corticosteroid injection at A1 pulley
- varied response rates
- ~ 50% response; Shubert Hand 2013 80% success
- use K-10 (0.5cc; preferred) or K-40; max 2-3 injections
- Operative
- open A1 pulley release (only option for congenital thumb)
- note: if trigger at A2 then increase size of A2 with slip FDS or oblique/step-lengthen of pulley
- Kerrigan, JHS, 2011: cost effective protocol is 2 steroid injections followed by surgery
- percutaneous release
- complications: digital nerv einjury, tendon injury, incompelte release, bowstringing
- open A1 pulley release (only option for congenital thumb)
Define de quervain’s disease
- stenosing tenosynovitis of 1st dorsal extensor compartment
- (apl and epb at radial styloid)
discuss surgical anatomy relevent at 1st dorsal compartment when considering management of de quervain’s
- The 1st dorsal compartment is divided into subcompartments between APL and EPB in 20-30% (EPB in “sub-compartment”)
- APL has multiple slips in a proportion of patients
- Radial sensory branch crosses over radial wrist at styloid - possibly several branches
- Radial artery passes from volar to dorsal at this level deep to tendons
Describe pertinent findings on history and physical exam
- History: middle-aged female, repeated/sustained hand stress, pregnancy/lactation (non-op mngmt successful)
- Physical: tender & swelling at radial styloid / 1st DC, crepitus/snapping of tendons, +ve finklestein
- can isolate EPB from APL: by fixing thumb metacarpal in maximal abduction (eliminating APL motion) & flexing MCP to test EPB
Describe differential diagnosis and differentiating etiology and clinical findings
- 1st CMC OA: joint OA - +ve grind, +ve axial load and distraction, + joint tenderness
- Wartenberg syndrome (neuropathy of sensory branch radial nerve as it emerges from between BR & ECRB) - paresthesia sensory distribution, tinel, finklestein aggravates, radial/ulnar deviation aggravates
- Intersection syndrome (tenosynovitis of 2nd dorsal compartment - where APL and EPB cross ECRL & ECRB about 4cm proximal to wrist)
describe treatment of de quervain’s
- rest, thumb spika splinting, NSAIDS - 1st line in nearly all pts
- corticosteroid
- 2-3 times
- 0.5cc of k-10 (or k-40)
- risks: moderate risk of atrophy/depigmentation, tenderness, tendon rupture
- operative release
- chevron 1cm proximal to radial styloid
- identify and protect sensory branches
- incise the 1st dorsal compartment (brent graham does a step-lengthen)
- ensure to examine for subcompartments (higher proportion of sub compartment among patients who go on to require operative rleease)
- risks: nerve injury, subluxation, tendon injury, scar, incomplete release
what is intersection syndrome?
- Tenosynovitis of 2nd dorsal compartment
- Pain & swelling at intersection of radial wrist extensors (ECRB, ECRL) as 1st compartment tendons cross over (APL, EPB), approximately 4 cm proximal to the wrist
- Associated with repetitive activity (eg. athletes)
- Treatment
- Rest, splinting, NSAIDs, local steroid injections
- Persistent pain ® operative release of 2nd dorsal compartment via longitudinal incision
- Post-op: splint wrist in neutral/slight extension x 10 days
what is EPL tenosynovitis / tendonitis
- Pain and swelling just distal to lister’s tubercle
- Associated with RA, OA, sequela of Colles’ fracture
- Rarely associated with overuse
- Treatment
- EPL prone to rupture (eg. RA) ® early operative release of 3rd dorsal compartment
- Subcutaneous transposition of EPL tendon radial to tubercle
What is FCR tenosynovitis?
- Pain & tenderness over FCR tunnel (area of scaphoid tubercle, trapezoid crest), worse with resisted wrist flexion & radial deviation
- Treatment:
- Rest, splinting, NSAIDs, local steroid injections
- Operative release of FCR tunnel for persistent pain (avoid injury to palmar cutaneous branch of median nerve)
List top 5 benign hand tumours
- Ganglion Cyst
- a) dorsal wrist b) volar wrist c) flexor sheath (palm) d) DIPJ (mucous cyst) e) MC boss
- Giant cell tumour, flexor sheath
- Epidermal inclusion cyst
- Glomus tumour
- Lipoma
what are the top malignant tumours in the hand
bcc & scc
what are the top malignant BONE tumours in the hand
- chrondrosarcoma
- osteosarcoma
- ewing sarcoma
list top tumours in hand by location
- DIPJ
- mucous cyst, Heberden’s node
- PIPJ
- Garrods knuckle pad, Bouchard nodule
- Digit
- GCT flexor sheath, ganglion cyst flexor sheath, inclusion cyst
- Palm
- Dupuytren’s cord / nodule, inclusion cyst, lipoma, GCT flexor sheath, ganglion
- Wrist
- Ganglion, schwannoma, neurofibroma, lipoma
list benign and malignant skin tumours on upper extremity
- Benign
- Verucca
- Benign pigmented lesions like nevus, freckle, lentigines
- FB granuloma
- inclusion cyst
- garrod pad, rheum nodules
- Heberden and Bouchard nodules
- Malignant
- BCC
- SCC
- melanoma
- Merkel cell
list benign and malignant tumours of fat in upper extremity
- Benign: lipoma, angiolipoma
- Malignant: liposarcoma
List benign and maligant hand tumours of fibrous tissue
- Benign:
- GCT of tendon sheat
- Dermatofibroma (bening fibrous histiocytoma)
- Fibroma
- Dupuytren’s (nodule)
- Nodular fasciitis
- Malignant
- Malignant fibrous histiocytoma
- DFSP
List benign and malignant nerve tumours of upper extremity
- Benign: schwannoma, neurofibroma, neuroma
- benign non-neural intraneural tumours: hemangioma, lipoma
- Malignant: malignant peripheral nerve sheath tumour
List benign and malignant vascular tumours of upper extremity
benign
- Hemangioma
- Vascular malformation - most common venous)
- Pyogenic granuloma
- Glomus tumour
- Aneurysm
- Fistula
Maligant
- angiosarcoma
list benign and malignant cartilage & bone tumours of upper extremity
- Benign bone: osteoid osteoma, osteoma, exostosis; cyst: unicameral, aneurysmal; GCT of bone
- Malignant bone: osteosarcoma, ewing sarcoma
- Benign cartilage: enchondroma, osteochrondroma, BPOP (benign perosteal osteochromatous proliferation), periosteal chondroma
- Malignant cartilage: chrondosarcoma