Autosomal dominant disease of variable penetrance characterised by Benign fibromatosis of palmar and digital fascia with nodule and cord formation
Demographics
M:F 4-10:1
Diabetes
Cholesterol
HIV
Usually unilateral
Young, b/l, ectopic disease with family history: Dupuytren’s diasthesis, a severe disease
Peyronie: dartos fascia
Ledderhose: plantar fascia
Garrod: knuckle pad
Pathology
Angiofibromatous metaplasia
Myofibroblast is the chief cell
Produce fibronectin
Type III collagen proliferation
My be subdermal origin/metaplasia of existing fasciae
Natural bands are converted in to cords
Three stages
Proliferation
Involution
Residual stage
Cords
Spiral cord: bring the NV structures medially to the midline
Pretendinous band
Central band
Lateral band
Greyson ligament
Commisural cord
Ab D Min cord
Natatory cord
Clinical features
History
Age, hand dominance, function, other area involvement
DM, epilepsy
Examination
Digits involved and the cords
sensation and digital allen test
MCPJ angle and PIPJ angle
Garrod pad
Hueston table top test
Indication for surgery
Functional impairment
MCPJ 30 degree contracture
PIP 15 degree contracture (PIP is bad as polar plate and collaterals contact permanently)
Consent
Aim of the disease is to improve function and the surgery is done under general anaesthetic/regional block. you will wake up with a bulky dressing on your hand, which will be reduced at 48 hours. Sutures will be removed at 10-12 days and we have to start a lengthy physio and scar management protocol with night splinting for 6 months.
Surgery is not curative, and the disease can recur and we may not get full correction at PIP joint. There is a small risk of skin grafting, this if required will be taken from inner forearm.
There is a also a small risk of digital nerve/vessel injury, cold intolerance and in a worst case scenario may result in amputation.
Treatment
Collagenase inj (AUX 1 and ii from Cl. Histolyticum )
one cord at a time
area where cord is easily palpable and away from tendon