Definition

A slowly progressive, painless thickening of the palmar fascia which results in a fixed flexion contracture of the fingers affected.

 

There is an increased incidence amongst Nothern Europeans (Scotland, Iceland, Norway) and the Scandinavians, and has been also nicknamed "Viking's Disease".

Associated Factors

 

  •  Family history (Autosomal dominant)
  •  Alcoholism
  •  Antiepilectics
  •  Peyronie’s disease
  •  Men are 10x more likely than women to be affected
  •  Age >40
  •  Liver cirrhosis
  •  Diabetes
  •  Hypercholesteraemia
  •  Smoking

 

Clinical Progression

 

The onset of Dupuytren's Contracture (DC) is gradual, and may begin as a painless nodule on the palm, which is fixed to the skin and deeper fascia. Over time, a cord develops, which gradually contracts with time.  The contraction of the cord pulls on the metacarpophalangeal joint (MCP) and proximal interphalangeal joints (PIP), resulting in a fixed flexion deformity of the fingers.

 

Pathophysiology

  •  Initial fibroblast proliferation occurs as the nodule is formed
  •  There is collagen deposition as the cord thickens and the contracture develops.
  •  The exact aetiology is uncertain

 

 Clinical Features

  •  45% bilateral
  •  Right hand is more common in unilateral cases
  •  May be associated with other conditions affecting connective tissues

Clinical Examination

 

  •  The degree of contraction of the MCP and PIP should be measured

 

Inspection

  •  Fixed flexion deformity of the affected finger will be present, together with a palpable, thickened cord
  •  Scars from previous surgery to release the contractures may be present
  •  Inspect for other areas that can be affected

 

Other areas that can be affected

  •  Knuckles - Thickened knuckle pads over the dorsum of the PIP joint (Garrod's knuckle pads)
  •  Soles of the feet - Plantar fibromatosis (Ledderhose disease)
  •  Penis - Peyronie's disease

 

Special tests

  •  Hueston’s tabletop test: The patient is unable to place his/her hand flat on the table

Differentials

 

 

  •  Ganglion
  •  Trigger finger
  •  Epithelioid sarcoma

 

Further investigations

  •  Liver function tests - Dupuytren's Contracture is associated with prolonged high alcohol intake

Clinical Management

 

Many patients live with this condition and do not develop significant disability. Treatment is considered if the contracture is causing deterioration in function. The aim of treatment is to restore function and prevent progression. Treatment may be categorized into the following:

  1. Surgical treatment
  2. Medical treatment
  3. Non-medical treatment

 

    Surgical Treatment

      Surgical options for releasing the contracture include the following:

      1. Fasciotomy (open or closed)
      2. Fasciectomy
      3. Finger Amputation

       

      Fasciotomy

      Closed fasciotomy (or percutaneous needle fasciotomy)

      •  May be performed as an outpatient procedure
      •  A needle is inserted percutaneously, and the thickened cord is divided using the bevel of the needle to weaken the cord
      •  The fingers are extended to snap the thickened, fibrous bands
      •  The wound is closed, and a dressing is placed on for 48 hours

      An open fasciotomy may be performed as a day case procedure. A longitudinal incision is made and the release is performed under direct vision

      Recurrence rates for the closed fasctiotomy at 3-5 years is 50%; this procedure may not be suitable for severe deformities, but has been recommended by NICE for patients unsuitable for major surgery.

       

      Fasciectomy

      •  May be segmental (removal of sections of the cord), regional (entire cord is removed) or a dermofasciectomy(the cord and overlying skin is removed, and a skin graft is placed above)
      •  This procedure is performed as a day case, and involves a regional block or general anaesthesia
      •  This is the most common procedure for treating Dupuytren's Contracture

       

      Finger amputation

      This is very rarely performed, and on very severe cases presenting late.

       

      Post-operative complications

      •  Infection
      •  Damage to surrounding digital nerves and vasculature
      •  Scar contraction
      •  Carpal tunnel syndrome
      •  Reflex sympathetic dystrophy

       

      Medical Treatment

      •  Steroids
      •  Topical Vitamin A
      •  Radiotherapy

       

      Newer medical treatment options:

      •  Collegenase enzymatic fasciotomy (Collegenase is injected into the thickened cord)
      •  Intralesional triamcinolone acetonide
      •  5-flurouracil
      •  Intralesional gamma inteferon

       

       Non-medical Treatment

      •  Splinting

       

      Historical Trivia

       

      This condition was named after Baron Dupuytren, who performed his first palmar fasciotomy in 1831. This condition has been described in 17th century European medical literature, and has been associated with playing Scottish bagpipes, horse-riding (due to grasping of the reins) and Vikings.

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