Dupuytren Flashcards
Dupuytren
occurs through myofibrolasts in the palm and digits
contractile elements
TGF-ß responsible for proliferation and fibroblasts differentation
predominantly Type III collagen
(normal is Type I collagen)
occur through
genetic predisposition
environmental factors and local tissue environment
Genetics
- european white males
- autosomal dominant, variable penetrance
- siblings - 3 times greater risk for Dupuytren
- no single polymorphism
- Chromosom 16q - downregulation of collagen breakdown upregulation of collagen synthesis
local tissue environment
- cords and nodules (type III collagen)
- heterogenic mix of static and dynamic contractile elements
- actively contracting tissue shortens and transform into a static, acellular maxtrix - fixed flexion contracture
- increased cellularity in disease is predictable for recurrence rates up to 50%
environmental factors
- trauma (microruptures of faszia) - swelling and secondary ischemia
- aging
- smokers
- diabetes
- alcohol
- phenobarbitone
- epileptic
- HIV
- vascular disorders
Pathophysiology
- Ischemia leads to free radicals and release of IL-1
- IL-1 activates Growth factors
- (TGF-ß, EGF, FGF, PDGF) - mostly TGF-ß
- this leads to myofibroblasts (differentation, proliferation, contraction and collagen III synthesis)
other factors:
- increased tissue inhibitors of metalloproteinases (TIMP)
- tenascin and periostin proteins biomarkes in nodules and cords could be indentify
- increased lysophosphatidic acid (LPA)
- BMP-4 is absent in diseased tissue
disease phases by LUCK
1) PROLIFERATIVE- hypercellular, large myofibroblasts, minimal extracellular matrix
2) INVOLUTIONAL -dense myofibroblast network increase collagen 3 cf 1
3) RESIDUAL - myofibroblasts DISAPPEAR leave FIBROCYTES as predom cell
anatomy
3 fascial zones:
palm - pretendinous band
palmodigital - spiral band
digital - lateral band
lateral digital sheath - (LDS) lateral to the neurovascular bundle with contact to the spiral band and the natatory ligaments
neurovacular bundle
volar - Grayson ligament
dorsal - Cleland ligament
lateral - LDS
medial in the palm - vertical fibers of Legueu
superficial transversal ligament proximal to the spiral band - (Skoog’s ligament)
pathoanatomy
palm - pretendinous cords:
most common, attaches to skin, do not displace the NV-Bundle
natatory cord:
cause web space contracture
spiral cord:
pretendinous band, spiral band, lateral digital sheat and the Grayson ligament (volary to NV-Bundle) - responsible for NV-Bundle displacement - NV comes to midline, superficial and proximal - variable in anatomy
central cord - goes from the palm (pretendinous cord) to the base of the middle phalanx
ligaments are spared:
Cleland’s ligament (dorsal to the NV in digit)
Transverse fibers of the palmar aponeurosis (Skoog’s Ligament)
contractures
MCP: spiral cord and central/pretendinous cord
PIP: spiral cord, central cord, lateral cord
DIP: lateral cord, retrovacular cord
clinic
most common is the D4, than D5
after D3 and D2 - D1
table top test is positive
Garrod nodes on the dorsum of the PIP-Joint - nodules to the extensor tendon (Knuckle pads) - more common in Dupuytren disease with M. Ledderhose and Peyronie
Dupytren Diathesis:
- male
- onset <50 age
- one or more affected siblings or parents
- garrod pads
- northern european
one positive - worse prognosis
all positive - recurrence rate from about 71% vs 23% in normal population
treatment
non surgical
indication for surgery:
contracture MCP 30°, PIP 15-20°, depending on the individual situation of the patient
Injections:
Triamcolon: knuckle pads
(decreases alpha2-macroglobulin - potent inhibitor of collagenase acitivity)
97% - softening or flattering the nodules
50% recurrence
collagenase:
only effected to collagen I and III - not normally collagen IV (NV-Bundle)
common events: lymphadenopathy and skin tears - not so common - tendon rupture or injury of the pulley system
85-93% of patient develop anti-collagenase Antibodies
Recurrence: US-datas
47% (worsening about 20°)
MCP: 39%
PIP: 66%
32% (worsening about 30°)
MCP: 26%
PIP: 46%
Percutaneous needle fasciotomy (PNA)
for patients with comorbidities excellent - can be done in local anaesthesia
correction:
MCP: 99% - PIP: 89%
3 years correction:
MCP: 73% - PIP: 31%
recurrence: < 20°
MCP: 80%
PIP: 35%
PNA vs collagenase
1y MCP equivalent
2y 32% (PNA) vs 8% (collagenase) clinical improvement of PIP contractures
treatment II
surgical
most common is the partial fasciectomy
skin tension predicts recurrence rates
50% with primary closure
Z-plasty only 15%
recurrence rate: 35-65%
PIP- contracture after fasciectomy
- volar plate release
- checkrein ligament release
- be careful not to unstable the joint
- maybe pinning for weeks
- maybe tendon sheath incision - than A3 pulley
secondary surgery
- 10 times more risk for damage of the NV-Bundle
- may need dermatofasciectomy (especially in younger patients to decrease the recurrence rate) in severe cases
total fasciectomy or resection of the whole Dupuytren disease makes no better outcome for recurrence
complications
worse clinic:
- decreased rates of correction
- higher complication rates
- worse grip strength
- worse functional outcome
PIP-contracture recurrence:
non-compliance with therapy - higher with initial PIP-contracture
outcome:
fasciectomy vs. dermatofasciectomy
no difference in normal cases - in severe cases dermatofasciectomy is better because of lower recurrence rate (all Dupuytren fibers in the skin are removed)
Brunner vs Z-plasty
no difference (if there is the same skin tension in closure!!!)
complications II
complication rate after surger - 15%
wound healing: 22,9%
pain: 18%
digital artery: 2%
digtital nerve: 3,4%
infection: 2,4%
recurrence up to 50% in 5y - if patient will seen over a longer time and age - all patients will get a recurrence - surgery do not heal the disease
recent literature:
collagenase for anticoagulated patients
no difference
collagenase is better for digits than for the thumb
cost effectiveness
fasciectomy: not effective
PNA: effective
collagenas: effective if less than 945$
