Dupuytren’s (Palmar Fibromatosis)
Dupuytren’s Disease is named after Baron Dupuytren, who become Professor of Surgery in Paris in the early 1800’s and wrote extensively about the condition.
Dupuytren’s Disease is characterised by progressive thickening, lumpiness and shortening of the palmar fascia (the dense protective fibrose layer of tissue lying between the skin of the hand and the underlying flexor tendons). The shortening (contracture) leads to the affected fingers being progressively pulled down into the palm of the hand.
Related fibromatosis can occur much less commonly in the plantar fascia of the foot (Ledderhose Disease) and even more rarely in the penis (Peyronie’s Disease).
Dupuytren’s results in 2 primary problems
- Fingers pulled into a bent position make for difficult hand use –eg. a finger pulled into the palm makes holding objects difficult, it tends to get caught when reaching for things
- Fingers held in a bent position too long undergo secondary changes ie. they want to stay in their bent position even when the causative Dupuytren’s band is removed, as the other structures in the finger have become stiff as a result of being held in a bent position for a long time.
It is a condition principally of inherited origin – occurring most commonly in males of North European descent. It is hence often nicknamed ‘Viking’s disease’ although this specific connection is probably false.
Dupuytren’s cannot be cured but its effects (mainly severely bent fingers) can be corrected, minimised and/or controlled.
Given it cannot be cured as such, treatment too early is generally unnecessary but late presentation can lead to severe secondary deformities which can be difficult to correct.
INJECTIONS – Steroids have been purported to be of some limited benefit.
‘Collagenase injections’ (an injection of enzymes which break down collagen) were introduced to the NZ market in 2017 under the name ‘XIAFLEX’. The treatment is almost as expensive as surgery, there are risks to adjacent structures which can be inadvertently injected and its long term effect has not yet been evaluated. It is however an option.
SURGERY – Surgery is normally advised once the patient can no longer put a hand flat on a level surface.
It is best performed before significant joint contracture occurs.
The more severe the joint contracture at the time of surgery then the:
- more difficult the surgery
- greater the risk of damage to digital nerves and blood vessels
- longer and more intrusive the post operative rehabilitation to correct secondary changes
- less chance of full correction of any secondary deformity
Having said this – it is seldom ‘too late for surgery’. Even though difficult, very severe contractures can usually be very significantly improved, if not fully corrected with a combination of an experienced surgeon, an experienced post operative hand therapist and a determined patient
Surgery is occasionally appropriate early if the diagnosis is in doubt. The condition can be confused at its early nodule stage with other soft tissue tumours and inflammatory nodules in the hand. Dupuytren nodules are typically painless and are associated with puckering and pit formation of the overlying skin. It can usually be distinguished clinically although sometimes biopsy is appropriate.
FASCIOTOMY – A very old type of intervention –the tight bands are simply divided and the finger straightened within the limits of the secondary changes. This can be done with a needle (needle aponeurotomy) or a scalpel. The procedure is usually done under a simple local anaesthetic injection. Hand therapy splintage is used to maintain and possibly further optimise the gains made. The bands will always reconnect at some time and the process will continue. It is a good procedure in very elderly or medically compromised patients in whom surgery might be contraindicated – giving reasonable straightening and usually getting the fingers out from the palm
FASCIECTOMY – Sections of the diseased tissue are partially or nearly completely removed. The fingers are straightened. The skin is closed in a zig-zag fashion to avoid a straight scar over a joint which in itself might shrink and pull the finger down and the hand is held straight in a splint. This procedure is normally performed under a general anaesthetic or an arm block. The hand is kept elevated in a sling for about 3 days. The hand is then increasingly useable. Hand therapy and splintage however is often necessary for intermittent periods over the next month and can be ongoing for 3 months, depending on the severity of the contractures before surgery.