Dupuytren's Contracture

This PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful.

Dupuytren's contracture is a progressive disorder that affects the palmar fascia causing the fibrous tissue to shorten and thicken.1 This contracture results in flexion deformity of the fingers and loss of hand function.

Clinical picture2

  • Most commonly affects the ring finger, followed by the little finger and then the middle finger. Can affect any digit.
  • Commonly bilateral (45%).3 Unilateral cases more often affect the right hand. Hand dominance is not a factor.
  • Starts with pitting and thickening of the palmar skin and underlying subcutaneous tissue with loss of mobility of the overlying skin.
  • Next a nodule forms which is firm and painless and fixed to the skin and deeper fascia. The nodule is palpable and later becomes visible.
  • A cord (a linear thickening that can resemble a tendon) then develops which begins to contract over months to years.
  • The contraction of the cord pulls on the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints and leads to a progressive flexion deformity in the fingers.
  • Deformity can affect activities of daily living and occupation. The stage at which the patient presents will depend upon tolerance of this and is likely to depend upon the degree to which work or hobbies may be affected.
  • A more aggressive form can occur in which there is early age of onset, a strong family history, bilateral hand involvement and associated ectopic disease (see below).

DUPUYTREN'S CONTRACTURE (OM1213b.jpg)

Epidemiology

  • Typically affects Northern European men (especially from Scotland, Iceland and Norway).4
  • Also common in Australia.4
  • Onset usually in mid 50s.
  • Women can also be affected but onset tends to be in mid 60s.
  • Autosomal dominant inheritance of varied penetrance suggested.
  • It may affect as many as 2 million people in the UK but only a small number will require surgery.2

Pathophysiology2

  • The aetiology is uncertain.
  • There is initial fibroblast proliferation as the nodule forms. This then slows down and leads to connective tissue assembling to form the cord. There is also collagen deposition.
  • Dupuytren's and wound healing seem to have many similar features.5 The initial triggers in Dupuytren's are unclear.

Risk factors

  • Smoking
  • Alcohol excess (but most patients are not alcoholics)4
  • Diabetes, especially if insulin-dependent6
  • Hypercholesterolaemia
  • Anticonvulsant drugs/epilepsy (controversial)7
  • Hand trauma and manual labour (also controversial, studies show no consensus; history of manual labour has worse prognosis)8

Examination

  • MCP joint contracture should be measured whilst applying passive extension to the PIP joint.
  • PIP joint contracture is measured while the MCP joint is held in flexion.
  • When there is 30 degrees of flexion deformity at the MCP joint, the patient is unable to place their palm flat against a hard surface, for example a table. This is known as the Hueston tabletop test.1
  • If there are knuckle pads on the dorsal PIP surfaces, there is more aggressive disease (see below).
  • Swan-neck deformities and boutonnière deformities may rarely occur.

Associated diseases9

Other areas of the body can be affected (also known as ectopic disease):

  • The knuckles: Garrod's knuckle pads (44-54%) are thickened knuckle pads over the dorsal surface of the PIP joints
  • The soles of the feet: Plantar fibromatosis, or Ledderhose disease (6-31%)
  • The penis: Penile fibromatosis, or Peyronie's disease (2-8%)

Differential diagnosis

Investigations

  • Usually a clinical diagnosis.
  • Due to the association with excess alcohol intake it may be judicious to take a drinking history and check liver function tests.
  • If there is any suggestion of undiagnosed diabetes then fasting blood glucose should be checked.

Management

Many patients with Dupuytren's contracture do not develop significant disability and will not need treatment, but:

  • Treatment is usually considered when (or ideally before) functional disability occurs. It aims to restore hand function and prevent progression.
  • A positive Hueston tabletop test is a good indication for referral.1
  • If the patient is amenable to surgery, early referral is recommended (i.e. ≥30° of contracture at the MCP joint and any degree at the PIP joint).1,2 This is because contracture and disability can become irreversible due to ligament remodelling, and early referral means surgeons are then best placed to decide on the timing of any interventions.

Surgical treatment

The exact timing of surgery varies. It is usually performed when the MCP joint contracture is >40° or when PIP joint contracture is >20°.1 Procedures include:

  • Fasciotomy
    • This can be done as either an open or closed procedure.
    • Closed fasciotomy (or percutaneous needle fasciotomy) may be done in the outpatient clinic and has received a lot of press recently.
    • The contracted cord is divided in the palm, the finger, or both, using a blade or the bevel of a needle. Once partial section has been achieved the finger is extended causing the fibrous band to snap. A dry bandage is then secured by elastic tape and placed over the injection site for at least 48 hours.10
    • With closed fasciotomy, there may be short-term benefit but by 3 to 5 years the recurrence rate can be around 50%.10
    • It is suggested that closed fasciotomy is not suitable for everyone and that it is unlikely to be of benefit in the long term if there is severe deformity.11
    • It may be more suitable for those with a prominent cord and predominant metacarpophalangeal contracture12 or for those who want minimally invasive surgery.
    • It may have a place in delaying fasciectomy.13
    • It has been reviewed and approved by NICE who consider that it may be of benefit for older patients unsuitable for more major surgery.10
    • Some people are concerned about the risk of nerve damage and tendon injury with percutaneous needle fasciotomy.10,13
  • Fasciectomy
    • This can be a segmental fasciectomy (short segments of the cord are removed), a regional fasciectomy (the entire cord is removed) or a dermofasciectomy (the cord and overlying skin is removed followed by skin grafting).
    • The procedure is usually done under regional block or general anaesthetic as a day case.
    • Regional fasciectomy is the most common procedure undertaken for Dupuytren's contracture.14
  • Finger amputation
    • This is rarely done.
    • Used if severe (usually because presentation has been very delayed).

Splinting and hand physiotherapy are needed after surgery. A night splint is normally worn for 3 months.

Medical treatment

A large number of medical treatments has been tried with variable effects including:

  • Radiotherapy
  • Splinting
  • Steroids
  • Topical vitamin A

More promising newer treatments include:

Complications

During surgery there is a risk of nerve, tendon or blood vessel injury. Possible post-operative complications include:19

Prognosis

  • The course of the disease is variable in terms of how fast and how far it will progress.
  • 10% of cases can regress without treatment.20
  • Most patients do not require surgery.
  • Surgery, when performed, improves function21 but postoperative recurrence may be up to 50%.

Historical note

Dupuytren's contracture is noted in European medical literature as early as the 17th century. Medical historians have associated it with the Vikings, the Scottish bagpipe-playing MacCrimmon clan and the Papal Sign of Benediction.22

Baron Dupuytren performed his first palmar fasciotomy in 1831 and his name has become associated with the condition ever since. Dupuytren was born in 1777 and was later appointed surgeon at the Hotel-Dieu in Paris. He was created a baron by Louis XVIII. He died in 1835 at the age of 58, having suffered a stroke.


Document references

  1. Trojian TH, Chu SM; Dupuytren's disease: diagnosis and treatment. Am Fam Physician. 2007 Jul 1;76(1):86-9. [abstract]
  2. Townley WA, Baker R, Sheppard N, et al; Dupuytren's contracture unfolded. BMJ. 2006 Feb 18;332(7538):397-400.
  3. Dupuytren's Contracture, Wheeless' Textbook of Orthopaedics
  4. Hart MG, Hooper G; Clinical associations of Dupuytren's disease. Postgrad Med J. 2005 Jul;81(957):425-8. [abstract]
  5. Fitzgerald AM, Kirkpatrick JJ, Naylor IL; Dupuytren's disease. The way forward? J Hand Surg
    . 1999 Aug;24(4):395-9. [abstract]
  6. Godtfredsen NS, Lucht H, Prescott E, et al; A prospective study linked both alcohol and tobacco to Dupuytren's disease. J Clin Epidemiol. 2004 Aug;57(8):858-63. [abstract]
  7. Geoghegan JM, Forbes J, Clark DI, et al; Dupuytren's disease risk factors. J Hand Surg
    . 2004 Oct;29(5):423-6. [abstract]
  8. McFarlane RM; Dupuytren's disease: relation to work and injury. J Hand Surg . 1991 Sep;16(5):775-9. [abstract]
  9. Rayan GM; Clinical presentation and types of Dupuytren's disease. Hand Clin. 1999 Feb;15(1):87-96, vii. [abstract]
  10. Needle fasciotomy for Dupuytren's contracture, NICE (2004)
  11. Bryan AS, Ghorbal MS; The long-term results of closed palmar fasciotomy in the management of Dupuytren's contracture. J Hand Surg (Br). 1988 Aug;13(3):254-6. [abstract]
  12. Foucher G, Medina J, Navarro R; Percutaneous needle aponeurotomy: complications and results. J Hand Surg (Br). 2003 Oct;28(5):427-31. [abstract]
  13. van Rijssen AL, Werker PM; Percutaneous needle fasciotomy in dupuytren's disease. J Hand Surg (Br). 2006 Oct;31(5):498-501. Epub 2006 Jun 12. [abstract]
  14. The British Society for Surgery of the Hand; Dupuytren's disease. Updated 7th August 2008.
  15. Pittet B, Rubbia-Brandt L, Desmouliere A, et al; Effect of gamma-interferon on the clinical and biologic evolution of hypertrophic scars and Dupuytren's disease: an open pilot study. Plast Reconstr Surg. 1994 May;93(6):1224-35. [abstract]
  16. Badalamente MA, Hurst LC, Hentz VR; Collagen as a clinical target: nonoperative treatment of Dupuytren's disease. J Hand Surg . 2002 Sep;27(5):788-98. [abstract]
  17. Ketchum LD, Donahue TK; The injection of nodules of Dupuytren's disease with triamcinolone acetonide. J Hand Surg . 2000 Nov;25(6):1157-62. [abstract]
  18. Bulstrode NW, Mudera V, McGrouther DA, et al; 5-fluorouracil selectively inhibits collagen synthesis. Plast Reconstr Surg. 2005 Jul;116(1):209-21; discussion 222-3. [abstract]
  19. Bulstrode NW, Jemec B, Smith PJ; The complications of Dupuytren's contracture surgery. J Hand Surg . 2005 Sep;30(5):1021-5. [abstract]
  20. Gudmundsson KG, Arngrimsson R, Jonsson T; Eighteen years follow-up study of the clinical manifestations and progression of Dupuytren's disease. Scand J Rheumatol. 2001;30(1):31-4. [abstract]
  21. Draviaraj KP, Chakrabarti I; Functional outcome after surgery for Dupuytren's contracture: a prospective study. J Hand Surg . 2004 Sep;29(5):804-8. [abstract]
  22. Elliot D; The early history of Dupuytren's disease. Hand Clin. 1999 Feb;15(1):1-19, v. [abstract]

Internet and further reading

Acknowledgements

EMIS is grateful to Dr Michelle Wright for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2010.
Document ID: 2077
Document Version: 22
Document Reference: bgp1213
Last Updated: 18 Mar 2009
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