Dupuytren's Disease Flashcards
Dupuytren's disease
what is Dupuytren’s disease?
- A Benign Proliferative disorder CHARACTERISED by FASCIAL NODULES and CONTRACTURES of the hands
Can you describe the epidemiology of Dupuytren’s?
- AUTOSOMAL Dominant
- 5-7th decade of life
- 2:1 M:F
- Highest incidence in Caucasian males European descent
- Linked to Diabetes Mellitis
- Alcholism
- Epilepsy
- HIV
What is its pathology?
- **MYOFIBROBLASTS **are the dominant cell type found histologically
- Cytokine mediated Transformation of normal fibroblasts into mylofibroblasts
- Increase in type 3 collagen to type 1
- The disease orginates in longitudinally orientated fascial structures
- Early proliferative phase characterised by high no of immature fibroblasts & myofibroblasts in a whorled pattern- hostological nodule
What 3 stages of disease have been described?
By Luck ( himself)!!!!- NB LUCKy Pir
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
What does it using present with?
- A nodule in the palmar fascia maybe painful and progress insidiously -> diseased cords and finally digitally flexion contractions beginning at MCP and progressing distally
- Reduced rom MCPJ/PIPJ/DIPJ
what has duptyren’s been associated with?
- ETOH xs
- DM
- epilepsy
- chronic pulmonary disease
- TB
- HIV
Can you describe/draw the normal fascial structures that become involved?
- PRETENDINOUS BAND- palm
- SPIRAL BAND- palmodigital transition( underneath NV bundle)
- NATATORY BAND- palmodigital transition
IN DIGIT
- GRAYSON LIGAMENT (palmar to NV bundle, passes flexor sheath to the skin, maintain digitial skin position)
- LATERAL DIGITAL SHEET- formed from fibres form natatory & spiral band
Cleland ligaments relatively not involved in D.
What does the disease change the bands into?
Can you name them all?
Diseased CORDS
- Central
- Spiral
- Natatory
- **Lateral **
- Retrovascular
What is not involved in the disease process?
CLELAND ligaments (nb Clevland)
Can you name the palm cords?
Pretendinous cords
What is the SPIRAL CORD made up from?
- SPIRAL BAND
- PRETENDINOUS BAND
- LATERAL DIGITAL BAND
- NATATORY BAND/LIGAMENT
- GRAYSON LIGAMENT
(NB Simon Phillips Never Loves Grapes)
What does the spiral cord lead to ?
- PIPJ contraction
- NV bundle = As travels under NV bundle it displaces it MIDLINE and SUPERFICIAL( VOLAR) increased risk during surgical resection
- Best predictors of displacement = PIPJ flexion contracture ( 77% PPV), Interdigital soft tissue mass (71% PPV)
- Clinically the most important contracture as pipj flexion warrants surgery
What makes up the natatotory cord?
What is its function?
- Develops from fibres of natatory ligament, just under commissure skin
- causes Web space contracture
What is the central cord in the phalanx?
What does it cause?
- The distal extent of the pretendinous cord
- Fibres fro cord extend and insert into flexor sheath around PIPJ -> MCPJ contraction
- Not involved with neurovascular bundle
What makes up the retrovascular cord?
What does it cause?
- Can arise dorsal to the NV bundle taking origin fro the proximal phalanx and inserting onto the distal phalanx
- DIPJ flexion
What are the signs of D?
- Nodule in pretendinous band of palmar fascia
- involves commonly small or ring finger
- Postive Heuston Table top test- look for mcpj/pipj contracture
- look for bilateral involvement and ectopic
- Ledderhose disease- plantar fascia
- Peyronie’s disease- dartos fascia of penis
- Garrod’s disease ( kuckle pads)
What are the surgical options?
- Needle cordotomy- for frail patients- risk of nerve injury
- Regional palmar fasciectomy - Brunner incision, V to y or sequential z plasties can be used to lengthen the skin- removed disease tissue only in digits with preservation of skin- iatrogenic nerve injury 7%. early rom day 5-7, night tiemextension brace/splint
- Dermofasciectomy with full thickness skin graft or z plasty, v-y advancements or healing by secondary intention- recurrence is less with this procedure as remove skin
- Open palm technique- McCash- approach transverse skin incision at level of distal palm crease ( see pic)- may be left open with/ wout delayed wound healing- low risk of haematoma formation- useful older pts at risk of stiffnes/low risk of complx due to lack of haematoma formation
- Recurrent disease- dermofascectomy+FTSG / arthrodesis/ amputation maybe required
