There are very few surgical conditions mired in as much history and controversy as Dupuytren’s disease. Since time of the first auditorial dissection of the disease by the aristocratic French Baron, much has been written about this mysterious hand problem. Despite the plethora of information, a depth of understanding has provided us little in terms of a tangible cure. All current treatments target symptom control and restoration of function.
It is fairly well established that the disease occurs in the form of abnormal scar tissue within the normal fascia and fascial planes of the palm and digits. We know that the cause is predominantly hereditary with a reasonable estimate being that 80% of the causation is hereditary. And we know that these hereditary factors are located within the genome of those with Anglo Saxon descent.
The precise biochemical and signalling disruption which causes the disease is poorly understood, as is the nature of genomic penetrance and expression. Individual and inter-individual variation of disease is so vast that our only reliable predictive modality is observation
The result of all of this is that we have a disease, which is common but incurable and benign, yet may have significant impact on hand function. Presentation can vary from the presence of palmar nodular disease to full-blown contracture and digital deformity. The limitation on hand function can be significant and disabling.
In the presence of a common, potentially disabling, yet poorly understood problem, there is extremely fertile soil for proliferation of information (be it factual or fictitious). Patients who are inclined to “research” Dupuytren’s disease with Dr Google can present with all manner of potential interventions and causative theories. Indeed, self-directed research is an extremely confusing exercise for affected patients.
Although there is much mystery surrounding the disease and its treatment we are able to achieve good results more often than not if the disease is seen early enough.
When To Refer?
The classical descriptions regarding referral include parameters such as:
- MCP joint contracture greater than 30°
- Inability to place hand flat on a table (tabletop test)
- Any contracture of the PIP joint.
While these referral criteria are valid, patients can often have problems with more minor disease, particularly if they have large palmar nodules. As mentioned above, it is also very confusing for patients with Dupuytren’s disease to make sense of the plethora of conflicting information available to them.
A patient may have significant palmar nodules, which are getting in the way of their function in power grip or they may simply have early stage disease and a background concern regarding the significance of the problem in future. A consultation in this situation can at least reassure the patient in the setting of very minor disease or potentially intervene if there is a problematic nodule. In many cases, initial referrals do not result in surgical intervention and merely consist of a discussion and provision of information.
Current Treatment
In general terms international consensus is that conservative management should progress as long as possible. In an asymptomatic patient who is functioning normally the hand should be left alone and the disease process monitored over time. The importance of the hand and hand function dictates an imperative that no intervention should be initiated in the absence of symptoms. The principle of “first do no harm” applies.
Should the problem be symptomatic and limiting hand function, an indication for treatment may arise. A few of the more common and controversial interventions are listed below:
Steroid Injection
Although favoured for quite a period of time, an injection of steroid in and around the Dupuytren’s cord has not been shown to provide significant long-term release. It may slow disease progression in some circumstances but any cord rupture is more likely due to percutaneous fasciotomy rather than chemical actions. No significant retraction or firebreak is achieved within the disease and recurrence is common and significant.
In favour however, steroid injection is cheap and non-invasive. It is still practiced in some circumstances for minor indications.
Percutaneous Needle Fasciotomy
A more widely studied minimally invasive technique is percutaneous needle fasciotomy (PNF). In the hands of an experienced surgeon PNF can be useful in rupturing significant tense cords causing MCP joint contracture. On the downside however, there is risk of tendon and neurovascular injury as the procedure is blind to these vital structures. It also does not create any significant firebreak and disease recurrence can be relatively rapid.
Minimal Access Fasciotomy/Segment Fasciectomy
This procedure is one step up from the needle fasciotomy and is performed through a small incision over the cords. The clear advantage is the open technique allows for visualisation of important structures and the potential for segment fasciectomy places a large fire-break in the palm in an effort to prevent rapid recurrence. In general this provides a good balance between risk and reward in a patient not able or willing to undergo any more invasive operations.
Radiotherapy
In recent times there has been a corporate push to prove the validity of radiation in the treatment of benign disease. Research studies are being developed targeting asymptomatic Dupuytren’s to prove that disease progression and recurrence has decreased at two years post irradiation.
The concerns regard more the secondary effects and long term risk of radiation in the hand, not least of which is loss of appendageal structures which will affect grip, and later endarteritis and the potential for malignancy. There is not sufficient comforting information that radiation for asymptomatic disease is worthwhile or safe in the long term. Safe modalities exist.
Clostridial Collagenase
The recent TGA approval of Clostridial Collagenase for treatment of Dupuytren’s disease is a safer, non-invasive intervention. The Collagenase dissolves a section of the cord providing for a rupture and chemical firebreak. Current information from trials show recurrence rates equivalent to surgery. The main limitation of Collagenase at present is its price, as it is not listed on the PBS. Despite the cost, however patients undergoing cord rupture or nodule treatment with Collagenase express a great deal of satisfaction with their outcomes. The major benefit is the lack of wounds (in most cases) and the minimal downtime required for treatment and rehabilitation. In many cases, patients do not have any time off work.
Surgical Fasciectomy
Should all else fail, the historical mainstay of treatment is surgical fasciectomy. The extent of the fasciectomy is dictated by the extent of the disease and can range from relatively routine, in the setting of isolated MCP contracture, up to extremely complicated, with profound contracture of multiple digits involving multiple joints. Severe contractures often warrant local flap closure and significant dermal involvement pushes the surgeon towards dermo-fasciectomy and grafting.
On the upside, surgical intervention can relieve and excise severe disease with restoration of joint range of motion and full clearance of pathology. Whilst surgery can relieve many of the worst contractures, it is a particularly aggressive undertaking and the subsequent rehabilitation can take several months. So the downside is the rehabilitation and extensive scarring within the digit.
With appropriate health insurance there is often no gap.
In most cases hand surgeons will only proceed to surgical fasciectomy with a solid functional indication.
In Summary
Dupuytren’s disease is a conundrum of epidemiology and prevention but has been extensively studied in terms of treatment over the years. While surgery is the mainstay of treatment, a number of non-surgical options exist and will likely be chosen in preference until surgery is absolutely indicated. A number of potential interventions are possible but patients can become significantly confused without appropriate guidance.
Whilst historical referral has been limited to the development of significant and problematic contracture, it may be worthwhile considering early referral to discuss the disease and its natural history. This will provide the patient with clear information in the muddy waters which are currently pervasive.