Dupuytren's Contracture

Dupuytren contracture is a fibroproliferative disorder of the palmar fascia. Myofibroblasts proliferate in the fascia and produce nodules and cords that progressively tether the skin and flex the metacarpophalangeal and proximal interphalangeal joints. The ring and little fingers are most often involved, and disease is bilateral in about 45%.

Prevalence is 3 to 6% in northern European populations, rising to 20 to 30% after age 70 in some, and is much lower in people of African and Asian descent. Men are affected 3 to 6 times more often than women, and onset is usually after 40. Inheritance is autosomal dominant with variable penetrance.

Risk factors:

• Family history
• Diabetes, alcohol use, smoking
• Epilepsy and anticonvulsant treatment
• HIV infection

Dupuytren diathesis (an aggressive, recurrence prone form): onset before 50, male sex, bilateral disease, a family history, and ectopic lesions (Garrod knuckle pads, plantar fibromatosis or Ledderhose disease, and Peyronie disease).

The staging uses total flexion deformity of the finger: stage N nodule only, stage 1 up to 45 degrees, stage 2 45 to 90, stage 3 90 to 135, stage 4 over 135.

Progression is unpredictable. Complications are fixed flexion contracture (proximal interphalangeal contracture is the most disabling), skin maceration, loss of grip and recurrence after treatment.

• A painless firm palmar nodule, then a cord extending into the finger, with skin pitting and puckering
• Progressive inability to straighten the finger, with difficulty washing the face, putting on gloves and placing the hand in a pocket
• Positive table top test (the patient cannot lay the palm flat on a table)
• Knuckle pads on the dorsal proximal interphalangeal joints
• Red flags: rapid progression in a young patient, pain, skin ulceration, an unusually rapid or irregular mass (consider sarcoma), and sudden contracture after trauma

• Clinical diagnosis. Measure the deficit in extension at the metacarpophalangeal and proximal interphalangeal joints with a goniometer, and record the table top test.
• No imaging is needed. Ultrasound can define cords. Radiographs are only for arthritis.
• Blood tests when indicated: HbA1c, liver enzymes, and HIV testing in the appropriate setting.

Differentials: trigger finger, camptodactyly, flexor tendon adhesion, palmar ganglion, sarcoma, Volkmann ischaemic contracture, systemic sclerosis, diabetic cheiroarthropathy (prayer sign).

Observe nodules and mild disease with no functional loss. Treat when the metacarpophalangeal contracture exceeds 30 degrees, any proximal interphalangeal contracture is present, or the table top test is positive.

• Percutaneous needle aponeurotomy: a 25-gauge needle is used to divide the cord under local anaesthesia (lidocaine 1% 2 mL), with finger extension at the same session. Suited to metacarpophalangeal contracture. It is quick and has a low complication rate, but recurrence is common (about 50% at 3 to 5 years).
• Collagenase injection: collagenase clostridium histolyticum 0.58 mg injected into a palpable cord (metacarpophalangeal or proximal interphalangeal), then extension manipulation under local anaesthetic 24 to 72 hours later. May be repeated at 4-week intervals, up to 3 injections for a cord. Adverse effects: skin tears, bruising, swelling, lymphadenopathy, flexor tendon rupture (rare), complex regional pain syndrome. Use with care in anticoagulated patients.
• Surgery (proximal interphalangeal contracture, severe or recurrent disease, skin involvement, failure of other treatment):
  • Limited fasciectomy through a Bruner zigzag or midline incision with excision of the diseased cord, protecting the neurovascular bundles.
  • Dermofasciectomy with a full thickness skin graft for recurrent disease and a strong diathesis.
  • Segmental fasciectomy, or arthrodesis or amputation for very severe recurrent proximal interphalangeal contracture.
  • Cefazolin 2 g IV before incision (3 g if over 120 kg), with tourniquet control.
  • Postoperative: night extension splint for 3 to 6 months and hand therapy from week 1.
• Complications of surgery: digital nerve or artery injury (3 to 5%), skin flap necrosis, haematoma, infection, stiffness, complex regional pain syndrome (about 5%) and recurrence (up to 50% at 5 years).

Follow up at 2 and 6 weeks, then at 3 and 12 months. Counsel that treatment does not cure the disease, on the possibility of recurrence, and on checking the contralateral hand. Offer glycaemic and alcohol review.