What is Dupuytren's Contracture?

Dupuytren's contracture is a progressive fibrous proliferation of the palmar fascia of the hand. It is a tumor (growth) but not a cancer.  Similar type fibrous fascia buildups may also occur in the fascia of the feet and is called called Ledderhose disease. In the penis it is called Peyronies disease. There is no known cause or etiology for Dupuytren'r and it is generally considerred an inherited condition.  Dupuytren's is often observed in persons of northern European descent, especially Scandanavians. It is sometimes called the "Viking" disease although the "Viking" theory of disease and spread is disputed by some authors:

http://www.ncbi.nlm.nih.gov/pubmed/12015711?dopt=Abstract

 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi??

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11357696&query_hl=5story

Many patients have a family history and the disease presents itself more often in men in about a 60/40 male to female ratio. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10473157&query_hl=24 There does not seem to be a specific relation to labor or work and it is not covered by workers compensation.  Dupuytren's is considered a genetic disease.

The cause of the disease is unknown and it usually presents later in life.  It has been reported in children and teenagers.   

It often starts as nodule in the palm that are composed of fibroblasts and type III collagen. After this, the nodules may start connecting and contracting. One will notice pits or grooves in the skin and there may be associated pain or tenderness. Microscopically, myofibroblasts may be found and these are the cells that start the contracture. At this stage, the composition shifts to more type III and type V collagen. Late stage Dupuytren's is characterized by metacarpophalangeal (MCP or first joint), proximal interphalangeal (PIP or middle joint), and rarely distal interphalangeal (DIP or distal joint) contractures.

Associated conditions

Peyronies disease, knuckle pads(Garrod nodes), plantar fibromatosis (Lederhose disease). Trigger fingers (stenosing tenosynovitis) may also occur in association with Dupuytren's.

Incidence

A large study in the US Veterans hospitals of 9938 patients found an incidence of 734 per 100,000 population in whites, 237 per 100,000 in Hispanics, 130 per 100,000 in blacks, and 67 per 100,000 in Asians.

In the United States the incidence in white populations is about 3%.

In Norway, the incidence is about 30%.    http://www.ncbi.nlm.nih.gov/pubmed/10050243?ordinalpos=4&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Onset

The onset of Dupuytren's is earlier in males than females. The disease is progressive and most operated patients have recurrent finger contractures http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11252689&query_hl=17

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=892619&query_hl=17

History

Dupuytren’s contracture, named after Baron Dupuytren the French Surgeon who detailed the condition in a lecture in 1831.  It has been an enigma for centuries. Due to the low incidence in Southern Europeans, no record of Dupuytren’s is found in ancient Greek and Roman medical books.  Dupuytren was not the first to discover this disease but the condition is named after Dupuytren as he clearly showed in anatomical dissections that the contracted fingers were not because of a tendon problem, but rather a build-up, fibrosis, and contracture of the palmar fascia..

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8345270&query_hl=45.   The first documented cases in theEuropean literature was the case reported by Plater in 1614 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9206674&query_hl=41. After Plater, it was Henry Cline (1750-1826) that first delineated this condition http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=3049855&query_hl=38 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=3074150&query_hl=38. Dupuytren is creditied with the name as he clearly delineated the problem in dissecting two cadavers with this condition.  Dupuytren recognized that it was a contracture of the palmar fascia or palmar aponeurosis. He proposed the term fasciotomy as an incsion of the contracted tissues. This approach, the release the fascia with small cuts, was the original technique used to treat Dupuytren’s and was proposed decades before Dupuytren’s description. Previous French surgeon, Alexis Boyer, described “crispatura tendinum” in 1814. He felt the contraccture problem was a disease of the flexor tendons. Astley Cooper, from England, a contemporary of both Boyer and Dupuytren also described contracted palms in 1822 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12864830&query_hl=35  Cooper also recommended fascial release with small cuts or aponeurotomies. Dupuytren, the man after which the condition is named lectured on this disease in 1832 and he described small transverse cuts in the palmar fascia as its treatment http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12864830&query_hl=35.

Surgical treatment of Dupuytren’s

Needle fasciotomy (otomy=opening, fasciotomy is opening of the fascia) also called (needle aponeurotomy, NA, or needle aponevrotomy) is a technique developed and refined in France by Rheumatologists Lermusiaux, Badois and others since the 1970's http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8054928&query_hl=31]. It involves cutting the tight contracted Dupuytren's cord under local anesthesia as on office procedure using hypodermic needles instead of a scalpel. Small needles are used to release the cords in multiple areas, then the cords are popped as the finger is straightened. This technique has been supported in the British Journal of Hand Surgery http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12954251&query_hl=3

They reported a low incidence of nerve injury (0.3%) and no tendon injuries. They also found significant gain in extension at the MCP joint and also good improvement at the PIP joint. The need for reoperation at average follow up of 3.2 years was only 24%.

The most common complication of needle aponeurotomy is skin openings that occur with ruputure of the Dupuytren's cord. This occurs about 16% of the time and may require a suture or can be left open to heal http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12954251&query_hl=29. Another criticism is that the diseased Dupuytren's tissue is not released, but is only incised. Recurrence at 5 years is reported to be 50% http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8054928&query_hl=31. The techniqe may be repeated if recurrence develops. The French authors report a very low incidence of complications.

A great advantage of this techniuqe is the lack of hospitalization, outpatient surgery centers, or an anesthesiologist. The small needles provide little trauma to the tissues and if done in areas where the skin is pliable, skin tears do not occur. Healing is rapid. The initial bulky dressing may be removed after a day or two and them may only Band-Aids. Formal physical therapy is not usually necessary. Surgical removal of Dupuytren's frequently requires therapy. However, needle aponeurotomy only separates the bands. Scar tissue may reform and start the contracture again. It is important to do finger stretching frequently to maximize the results. In the beginning, this may be difficult due to the recent procedure. As weeks pass, aggressive hyperextension can prevent the severed cords from re-connecting.

Subcutaneous Fasciotomy

This technique was first suggested by Dr. Astley Cooper in 1822 for the treatment of fascial contracions of the finger ie Dupuytren's. Dr. Cooper stated: but when the aponeurosis is the cause of the contraction and the contracted band is narrow, it may be with advantage divided by a pointed bistoury (scalpel), introduced through a very small wound in the integument. The finger is extended and a splint is applied to preserve it in a straight position." From Cooper, A: Treatise on dislocations and on Fractures of the Joints, Ed. 2 p. 521 Longmans, London 1823. The open fasciotomy of Dupuytren involves cutting straight down through the skin and fascia and avoiding the tendons which are deep. The subcutaneous fasciotomy, like the needle aponeurtomy cuts the contracted tissues under the skin and doesn't usually leave any major skin openings. Articles reporting on the success of blind scalpel aponeurotomy with great success include the works of Kelly in 1959 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Search&db=pubmed&term=kelly+dupuytren+s&tool=fuzzy&ot=kellyu+dupuytrren%27s , and Luck http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=13664703&query_hl=3&itool=pubmed_docsum

Kelly's article from 1959 in the Plastic and Reconstructive Surgery Journal stated very strongly as to why are major surgical apporaches undtertaken when the results from subcutaneous release can be so good. The quote from Kelly is below"

"Subcutaneous fasciotomy" was first suggested for the treatment of flexion contracture of the fingers by Sir Astley Cooper in 1822. He wrote "... but when the aponeurosis is the cause of the contraction and the contracted band is narrow, it may be with advantage divided by a pointed bistoury, introduced through a very small wound in the integument. The finger is extended and a splint is applied to preserve it in a straight position". This procedure fell into disrepute through the years because it was indiscriminately applied to all cases of contracture; but Luck, 1958, has recently reintroduced it and it has achieved limited popularity. Having perused 20 papers, published since Skoog's monograph in 1948, on the surgical treatment of flexion contracture, we find that fasciotomy is either not mentioned at all or condemned. The radical fasciectomy, on the other hand, is given not merely as the procedure of choice but is considered the only satisfactory surgical treatment. This blind adherence to a single procedure for the treatment of a disease that has many individual variations and several stages of development shows an infatuation with a technical exercise that does not properly answer each patient's need.

The current techniqe of needle aponeurotmy, developed in France for the flexion contracture of Dupuytren is a type of subcutaneous fasciotomy.

Subcutaneous fascitomy was the predominant treatment for Dupuytren's till the 1900's.  At that time, with improvements in anesthesia and surgical technique, excision of Dupuytren's, fasciectomy, became the normal treatment.  Radical excision of Dupuytren's, removing all palmar fascia, both diseased and normal tissue as a prevention fell into disfavor in the 1960's due to the complications associated with this surgery.

Segmental Fasciectomy

A scalpel excision (removal or fasciectomy; ectomy=excise) of a short segment or piece of a contracted Dupuytren's cord. This technique was developed by Moermans http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1960487&query_hl=19

He has reported favorable long term results and this is the most non-invasive surgery http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8982932&query_hl=19 This technique may be the first surgery necessary and is the next step after needle releases become ineffective. Patients with previous surgery on Dupuytren contracture may need segmental surgical releases as needle fasciotomies or NA are less effective after previous surgery.

It is very useful on the thumb, as it is difficult to release thick thumb cords which lie over the digital nerves. In many cases, a small incision, under local anesthesia, will break up the contracting cord.A bigger surgery than needle releases, recovery will take weeks or even a month or two. Formal physicial therapy is not usually requred as it is after a limited fasciectomy.

Limited fasciectomy

The most common surgical procedure performed in the USA and also very common in France. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15993524&query_hl=24 An excisional technique removing diseased areas of Dupuytren’s fasciitis. It is normally performed under general anesthesia or nerve blocks and a mechanical tourniquet.

The McCash technique is a limited fasciectomy that is left partially open in order to prevent postoperative bleeding http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1769993&query_hl=25. It is less often used nowdays http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9746881&query_hl=19.

Dermofasciectomy

A technique that involves cutting the diseased tissue and the overlying skin. The wound is covered with a skin-graft. It was popularized by Hueston http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=6099169&query_hl=17 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=6380478&query_hl=17 as the new skin seems to prevent recurrence of Dupuytren’s in the underlying tissues. This surgery can be very useful in scarred and recurrent Dupuytren’s. Another technique developed by Hueston is the “firebreak” graft which is a skin-graft placed between excised or ruptured bands of Dupuytren’s to prevent the cord from redeveloping http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=6380478&query_hl=15.

This procedure requires excison of the Dupuytren's cords then coverage with a skin graft. It is based on the fact that Dupuytren's does not readily recur under a skin graft. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10697321&query_hl=29 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9149986&query_hl=29 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9158254&query_hl=29 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7952813&query_hl=29

Radical Fasciectomy

A mostly historical techniuqe. It was developed and proposed by McIndoe http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=13487940&query_hl=22

It involves cutting out all disease Dupuytren's cords and normal palmar aponeurosis. This is to prevent recurrence of the disease by removing all palmar fascia which could turn into contracted Dupuytren's nodules or fibrosis. The operation has a high complication rate and is rarely performed nowdays.

Medical (non-surgical) Treatment of Dupuytren's

Enzymatic dissolution of Dupuytren's via the product Xiaflex (collagenase) is near FDA approval. The FDA medical panel voted 12-0 for approval in September 2009.  Full FDA approval is pending.  This treatment will require injections of the product followed by breaking of the contracitng cords of Dupuyten's.  The product is made by Auxiliium Pharmaceuticals http://www.auxilium.com/ProductPipeline/DuputrensContracture.aspx .  Auxilium has the product under licence from Biospecifics, the makers of Santyl ointment.  The first use of clinical collagenase was for burns.  Now, Auxilium has prepared it in an injectible form for Dupuytren's, and soon for Peyronies disease, and possibly patients afflicted with frozen shoulder.

Collagenase (Xiaflex) will work best in the palmar area of the hand and use in the PIP joint (middle joint) has been assocated with more complications such as tendon ruptures.  Fortunately, collagenase spares nerves, but can dissolve tendons.

Alternative Medications

Some medications that have been tried or have been reported anecdotally are allopurinol http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1769989&query_hl=13 , colchicine http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11309227&query_hl=9http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1568169&query_hl=9, vitamin E http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10050246&query_hl=3, calcium channel blockers http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8969433&query_hl=6, interferon http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10050246&query_hl=3, and DMSO http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=5552618&query_hl=1. None have been shown to be consistently effective. Radiotherapy has been reported to reduce the progression of Dupuytren’s in the feet http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14652674&query_hl=2

In Germany, radiotherapy has been reported to be successful in preventing further contractues http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11757183&query_hl=4

Radiotherapy should be done when the fingers are straight and therefore preliminary needle aponeurotomy treatment would be necessary to straighten the fingers.  Radiotherapy would then be performed to prevent recurrence.

Ledderhose Disease 

A condiiton related to Dupuytren's of the hand but it occurs on the fibrous fascia of the feet. It also may be treated with needle release. Since there are usually no contracted toes the results are not as dramatic as one finds in the NA of Dupuytren's of the fingers. Ledderhose disease needle aponeurotomy requires optimally two visits separated 2-3 weeks apart to perform NA and settle down the painful nodules.

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For more information visit the Dupuytren contracture forum:

http://www.dupuytren-online.info/Forum_English/board/dupuytren-0.html

or Auxilium's website:

http://www.dupuytren-online.info/Forum_English/board/dupuytren-0.html