Dupuytren’s contracture is the name for fibromatosis affecting the connective tissue of the palm of the hand. It is named after the French doctor Baron Guillaume Dupuytren (1777–1835) who conducted extensive research of the disease.
Between the skin of the palm of the hand and the finger, there is a fibrous layer of connective tissue known as the palmar aponeurosis. Dupuytren’s contracture causes changes in this layer of connective tissue and is characterised by the development of nodules and strands, which are typical of this disease, leading to notable skin retraction and possible encasement of the palmar arteries and nerves. This type of fibromatosis has a shrinkage propensity and causes progressive flexion contracture of the fingers, and in some cases, the thumb. The resulting functional limitation leads patients to seek consultation with a hand surgeon wishing for permanent restoration of function.
It is, therefore, a connective tissue disorder and not a “shortening of the flexor tendons” as was mistakenly assumed in the past. It is a disease that is exclusively benign. The incidence rate is estimated at between 3 and 9% with the incidence ratio between the sexes (men/women) reported at between 10:1 and 2:1. The disease commonly first manifests itself in patients in their 40s and 50s, culminating in the first operative procedures in men during their 50s and between 60 and 70 years of age for women.
The disease most commonly affects the region of the fourth and fifth digit rays but, in principle, in can affect all the fingers, and even the extensor digitorum. The strand-like changes to the connective tissue cause a shortening to develop, which leads to increasingly severe inward bending of the fingers toward the palm. The disease is classified based upon the extent to which the fingers are bent, in other words, the severity of the flexor contraction.
The progression of the disease is often intermittent, i.e. after a long period without change, a considerable exacerbation can occur very rapidly.
The cause of the disease remains unknown; however, it is nowadays suspected that genetic predisposition is involved. The fact that Dupuytren’s contracture is more prevalent in Central and Northern Europe, North America, and Australia points to a genetic cause.
There are related disease affecting the soles of the feet (Ledderhose disease) and the penis (Induratio penis plastica).
Examination and Diagnosis
An experienced hand surgeon can recognise the disease by simply looking at and palpating the hand. In some severe cases, x-ray images are required to display the joint contractures. An ultrasound examination, or in exceptional cases, an MRI of the hand can be useful to differentiate the nodules from tumours and cysts. Extensive or painful examinations are unnecessary. The disease progresses in stages and is classified in stages 1 to 4 using the staging system developed by the French hand surgeon Tubiana.
Stage 0 – No lesion
Stage N – Palmar nodule without presence of contracture
Stage 1 – Total flexion deformity 0–45°
Stage 2 – Total flexion deformity 45–90°
Stage 3 – Total flexion deformity 90–135°
Stage 4 – Total flexion deformity > 135°
The main difficulty in the treatment of Dupuytren’s contracture is that both conservative therapy and radical hand surgery do not always lead to permanent healing. In patients with a genetic predisposition, the disease can reoccur in certain areas of the hand or an expansion of the nodule and strand development can take place in another region.
Attempts at conservative therapy, such as the use of vitamin E and Allopurinol, did not produce any positive effects. Ultrasound treatment also appears to be unsuccessful. Local cortisone injections during the early stages of the disease can lead to the regression of the nodules lasting for a few years. Radiotherapy is also an option during the early stages. However, there are almost no useful scientific studies on this subject.
Collagenase (Enzyme Injection)
Collagenase treatment is a non-operative treatment developed in the USA where an enzyme (Collagenase) is injected into the Dupuytren cord and partially dissolves it. After 24 hours, the cord is mechanically stretched.
Collagenase treatment using Xiaflex® has been regularly used by practice founder, Prof. Sauerbier, since October 2011.
The pharmaceutical manufacturer Pfizer ended the distribution of Xiaflex® in Germany on 16th May 2012. Xiaflex® is now only available via international pharmacies. In Germany, statutory healthcare providers generally do not cover the cost of Xiaflex®. However, the treatment itself continues to be offered.
Percutaneous Needle Fasciotomy (PNF)
Because Dupuytren’s operations are associated with longer recovery periods due to the large wound area, in some cases, minimally invasive surgical interventions are used as these have far fewer adverse effects and a shorter healing period.
An example is Percutaneous Needle Fasciotomy (PNF). The Needle Fasciotomy procedure was developed by researchers in France and has been popular since its introduction more than 20 years ago. It is a minimally invasive technique and is carried out on an outpatient basis using only a weak local anaesthesia.
An operation should only be performed if a flexor contracture has developed, only limited spreading of the fingers is possible, or when the process extends across several digit rays in the palm of the hand.
The age of the patient plays no significant role as the operation is carried out using regional anaesthesia making it possible to carry out despite the existence of accompanying diseases, e.g., cardiovascular diseases.
Partial fasciectomy is the standard operative procedure with only the changed contracture tissue in the palmar aponeurosis removed. This should be carried out by an experienced hand surgeon aided by surgical magnifying spectacles.
Depending on the severity of the disease, it may be necessary to carry out extensive dissection to expose the vascular nerve fibres and possibly also find solutions for already existing joint stiffness. Any injuries to arteries or nerves during the operation are dealt with immediately using a surgical microscope.
The operative technique to be used is determined individually for each case during preoperative consultation between the patient and their physician. The practice founder, Prof. Sauerbier, takes a more defensive position toward indications for surgery and takes the individual symptoms of each patient into account when creating a treatment concept. Surgery is commonly indicated from Tubiana stage 2 and upward because the patient already has a flexor contracture of more than 45 degrees and is no longer able to put their hands in their pockets without problems, as well as having problems in daily life such as shaking hands or washing their face.
Scientific literature reports complication rates for the operative treatment of Dupuytren’s contracture of between 17 and 19%. The most severe cases can cause blood circulation disorders in the fingers, which are usually temporary.
To avoid secondary haemorrhaging, a suction drain is used for approx. two days after the operation. The suture material is removed after 12 to 14 days.
Wound healing disorders stemming from reduced blood flow to the skin are relatively common but normally present no serious issues when subject to correct aftercare. It is possible for the disease to reoccur at the area operated on or develop on previously unaffected fingers.
The operative intervention for Dupuytren’s contracture is often carried out on an outpatient basis. Depending on the wishes and condition of the patient, either general anaesthesia or regional arm anaesthesia can be used. Especially for cases classified as Tubiana stage 2 and above, or in the event of Dupuytren’s recurrences, the procedure is carried out on an inpatient basis with the use of a nerve block catheter inserted by a colleague from the Department of Anaesthesiology.
In the most severe cases, where blood circulation disorders affecting the fingers are to be expected, or when a skin graft is necessary, 2 to 3 days of inpatient monitoring will be required.
This also applies to patients with serious underlying conditions, including those with coronary heart disease, severe vascular disease, severe diabetes mellitus, renal insufficiency, bronchial asthma, or patients with a propensity to develop severe pain, among others.
The early initiation of physical therapy treatments is a fundamental factor in the success of the operation and plays a decisive role in the results of Dupuytren’s treatment. The treatment should begin on the second day following the operation and under the supervision of a hand therapist. The objective is to maintain the extension of the operated finger achieved by the hand surgeon during the operation. The early application of a night splint, fitted by a hand therapist, helps counteract any new contracture. During the course of treatment, massages and local silicone scar sheets can be used to treat the hardening of scar tissue. Usually, hardening and reddening of the operation scar tissue increases between weeks 2 and 6 after the procedure before subsiding by week 12. Hand therapy aftercare should continue regularly for at least 2 months.