Dupuytren's contracture
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| Dupuytren's contracture Classification and external resources |
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| Dupuytren's contracture of the fourth digit (ring finger). | |
| ICD-10 | M72.0 |
| ICD-9 | 728.6 |
| OMIM | 126900 |
| DiseasesDB | 4011 |
| MedlinePlus | 001233 |
| eMedicine | med/592 orthoped/81 plastic/299 pmr/42 derm/774 |
| MeSH | D004387 |
Dupuytren's contracture (also known as Morbus Dupuytren or Dupuytren's disease, and sometimes misspelled as Dupuytren's constricture) is a fixed flexion contracture of the hand where the fingers bend towards the palm and cannot be fully extended (straightened). It is named after the famous surgeon Baron Guillaume Dupuytren, who described an operation to correct the affliction.
The ring finger and little finger are the fingers most commonly affected; the middle finger may be affected in advanced cases, but index finger and thumb are nearly always spared. Dupuytren's contracture progresses slowly and is usually painless. In patients with this condition, the tissues under the skin on the palm of the hand thicken and shorten so that the tendons connected to the fingers cannot move freely. The palmar aponeurosis becomes hyperplastic and undergoes contracture. As a result, the affected fingers start to bend more and more and cannot be straightened.
Incidence increases after the age of 40; at this age men are affected more often than women. After the age of 80 the distribution is about even.
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[edit] Symptoms
In Dupuytren's disease, the tough connective tissue within one's hand becomes abnormally thick, which can cause the fingers to curl, and can result in impaired function of the fingers, especially the small and ring fingers. It usually has a gradual onset, often beginning as a tender lump in the palm. Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop. [1] These bands, which are the source of the reduced mobility commonly associated with the condition, are visible on the surface of the palm, and may appear similar to a small callus. It commonly develops in both hands, and has no connection to dominant- or non-dominant hands, nor any correlation with right- or left-handedness.
The contracture sets on very slowly, especially in women. However, when present in both hands, and when there is associated foot involvement, it tends to accelerate more rapidly.
[edit] Risk factors and possible causes
Dupuytren's disease is a very specific affliction, and primarily affects:
- People of Scandinavian or Northern European ancestry[2], particularly those of Viking descent.[3]
- Men rather than women (Men are ten times as likely to develop the condition)[4]
- People over the age of 40, and
- People with a family history, (60 to 70% of those afflicted have a genetic predisposition to Dupuytren's Contracture)[5]
Correlations have also been found between Dupuytren's contracture and:
- Smoking or drinking activity,
- Diabetes, Thyroid problems and Epilepsy
- Alcoholism and Liver Disease
- Pulmonary Tuberculosis
Surgery of the hand may trigger growth of Dupuytren nodules and cords if an inclination existed before. Dupuytren's contracture may accompany fibrosing syndromes such as Peyronie's disease, Ledderhose's disease and Riedel's thyroiditis.
There is no proven evidence that hand injuries or specific occupational exposures lead to a higher risk of developing Dupuytren’s disease although there is some speculation that Dupuytren's may be caused or at least the onset may be triggered by physical trauma, such as manual labor or other over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness casts some doubt on this claim. [6]. For an overview on research literature see also Dupuytren's work related?.
[edit] Treatment
- Surgery (in cases of severe contracture removes the contracture)
- Radiation therapy (specifically in early stages inhibits development of contracture)
- Needle aponeurotomy (releases the contracture)
- Triamcinolone (kenalog) injections provide some relief
Dupuytren's contracture is not a dangerous condition, and often no treatment is necessary. Even when treatment is used, there is no permanent way to stop or cure the infliction. If there is a painful lump present, an injection can often help alleviate the pain. If pain persists or the function of the hand becomes seriously impaired, surgery is an option.
Surgical management consists of opening the skin over the affected cords and excising (removing) the fibrous tissue. The fingers may then be brought out to length with the help of postoperative therapy. The procedure is not curative in that remaining non-affected fascia may still develop Dupuytren's disease later on, and therefore the patient may need repeat surgery. In addition, the thickened fascia often is near to or wrapped around the digital nerves and arteries, so there is some risk of nerve and/or arterial injury.
Needle aponeurotomy is a minimally invasive technique where the cords are weakened through the insertion and manipulation of a small needle. Once weakened, the offending cords may be snapped by simply pulling the finger(s) straight. The advantage claimed for needle aponeurotomy is the very small amount of surgery required and the very rapid return to normal activities, but the nodules are not removed and might start growing again.
Currently in phase III of Food and Drug Administration (FDA) approval is another promising therapy, the injection of collagenase. This procedure is similar to needle aponeurotomy, but the cords are weakened through the injection of small amounts of an enzyme that dissolves them.
Treatment of Dupuytren's disease with low-energy x-rays (radiotherapy) may cure the condition in the long term, specifically if applied in early stages of the disease.
[edit] Notable sufferers
Those of northern European descent are markedly at risk. Samuel Beckett, Ronald Reagan, Margaret Thatcher, and Bill Nighy are among the most famous patients. Interestingly, although this a predominantly caucasian disease, many Caribbean blacks can present with contractures, and all can trace a distant relative to Britain or Ireland.[citation needed]
