Background

Definition Dupuytrens

 

Palmar Fibromatosis 

 

Aetiology

 

AD with variable penetration

 

Pathogenesis

 

Murrell's Theory of Pathogenesis

 

1. Microvascular ischaemia

 

2. Leads to conversion of 

- ATP to Hypoxanthine

- Endothelial Xanthine Hydrogenase to Xanthine Oxidase

 

3. Xanthine Oxidase converts Hypoxanthine to Uric Acid

- gives off OH-

 

4 OH- releases Free Radicals 

- stimulate fibroblast proliferation & increased Type III Collagen

 

5  Fibroblasts strangle microvessels

- Vicious Cycle  

 

Luck's three stages of Dupuytren's contracture 

 

1st stage (proliferative) stage 

- increased cellularity

- number of large myofibroblasts

 

2nd (involutional) stage 

- dense myofibroblast network aligned to long axis of collagen bundles

- the ratio of Type III collagen to Type I collagen is inc

 

3rd (residual) stage 

- myofibroblasts disappear 

- fibrocytes are dominant cell type

- dense collagen cord remains

 

Myofibroblasts 

 

Cell of origin for the nodular myofibroblast is unknown 

- fibroblast / smooth muscle cell / pericyte

- Contractile cell

- nodules composed of myofibroblasts 

- No myofibroblasts in cords

 

Dupuytren's diathesis 

 

Aggressive early-onset form of the disease which involves the multiple areas

- usually have family history

- disease recurs rapidly following treatment

 

Feet (Ledderhose, 1897)

Penis (Peyronie) 

Garrod knuckle pads on dorsum PIPJs

 

Associations

 

Chronic alcoholism 

- ? metabolic effect on fat and prostaglandin metabolism

 

Diabetes mellitus 

- may be related to the diabetic microangiopathy

 

Epilepsy 

- likely effect of antiepileptic drugs on collagen metabolism

 

Smoking

 

Chronic pulmonary disease

 

Occupational hand trauma 

- controversial 

- probably only aggravation due to traumatizing an early nodule

 

Epidemiology

 

Age 50-70

Male 7:1

 

Caucasians

- especially celtics / vikings heritage

- rare in blacks & asians

 

Anatomy

 

A.  Involved anatomy

 

1.  Pre-tendinous Bands

- part of the palmar aponeurosis in palm

- common site of disease

- palpable nodule is pathognomonic of Dupuytren's

 

2.  Spiral Band

- continuation of pre-tendinous band into finger

- spirals deep to NV bundle then becomes superficial to bundle

 

3.  Natatory Ligament

- pass between the web spaces

- frequently diseased and prevents abduction

 

4.  Lateral Digital Sheet

- condensation of superficial fascia on either side of the finger

- receives fibres from the natatory ligament, spiral band, Grayson's and Cleland's ligaments

 

5.  Grayson's Ligaments

- hold skin during flexion and extension

- pass from fibrous tendon sheath to the lateral digital sheet

- volar to the NV bundle

- almost always involved in Dupuytren's

 

B.  Not involved anatomy

 

Skoog's fibres 

- transverse palmar fibres 

- run from flexor sheath to flexor sheath at the level of the A1 pulley

- the nerve is always deep to the fibres

- part of palmar aponeurosis

- deep to pre-tendinous band

- don't become diseased

 

Cleland's Ligaments

- hold skin during flexion and extension

- firm fascial structures 

- pass from the side of the phalanges to the skin

- dorsal to the neurovascular bundle

- involved in Dupuytren's only through mingling with the lateral digital sheet

 

MEM: Dave Christie Goes Volar

(Dorsal Cleland's, Grayson's Volar)

 

Site

 

LF / RF most commonly affected

MF / IF are sometimes affected

1st web sometimes affected

 

Pathology

 

5 Major Pathological cords

 

1.  Pretendinous cord

 

In palm / other 4 in finger

- diseased pretendinous band

- causes MCPJ deformity

 

2.  Central cord 

 

Diseased central fibrofatty tissue

- large nodule often present in cord just proximal to PIPJ

- causes PIPJ deformity

 

3.  Spiral cord 

 

Pathological spiral band

- usually connects to the P2 (bone and tendon sheath)

- displaces neurovascular bundle volarly

 

Difficult to predict presence

- associated with more severe contractures

 

4. Lateral Cord 

 

Diseased lateral digital sheath

- intimately adherent to skin (sharp dissection required)

- contributes to DIPJ +/- PIPJ

 

5. Natatory Cord 

 

Diseased Natatory ligament

- causes web contracture

 

3 Minor Cords

 

1.  Retrovascular Cord 

 

Involves longitudinal fibers dorsal to the bundle

- commonly seen in combination with other cords

- causes DIPJ extension with lateral cord

 

2.  Abductor Digiti Minimi Cord 

 

Cord arises from abductor digiti minimi

- from MT junction 

- to ulnar side of the base of P2

- commonly adheres to the lateral skin

 

3.  Intercommissural Cords / 1st Web 

 

Pathological changes in 

- pre-tendinous band (radial longitudinal fiber)

- superficial transverse fibers of the palm (proximal transverse commissural ligament)

- the first web natatory ligaments (Grapow's ligament)

 

Contractures

 

1.  PIPJ Contracture 

 

4 components

- Central cord 

- Spiral cord 

- Lateral cord 

- Retrovascular cord

 

Correction sequence

- resection pathological cords

- capsulotomy, release check rein ligaments

- release of accessory collateral ligaments performed

- release of volar plate 

 

2.  MCPJ Contracture

 

Always correctable by removal of central band

- Flexion deformity does not lead to collateral shortening 

 

3.  DIPJ Hyperextension

Occurs in advanced disease

- contracture of retro-vascular + lateral cord

 

History

 

Usually mildly painful nodules to begin

- palm of RF and LF rays

- very short lived

 

Severe night pain

- suspect fibrosarcoma

 

Progressive contracture of MCP, then PIPJ

- nodule over PIPJ warning of impending PIPJ contracture

 

Difficulty putting hands in pockets

- difficult gripping

- poke themselves in the eye

 

Diasthesis

- foot, penis

 

Examination

 

Nodules / dimples / pits

- palm, fingers

 

Contractures

- MCPJ

- PIPJ

- DIPJ extension

- web space contractures / natatory cords

 

PIPJ Contracture

- Examine PIPJ with MCPJ flexed

- eliminate effect of cord

- establish if any joint contracture

 

Diasthesis

- feet, Garrod's pads

 

Hueston Table Top Test

- Royal Melbourne hospital

- palm down on table

- positive if can slide pen under

- MCPJ contracture 30-40o