DUPUYTREN CONTRACTURE

Joshua L. Eaton, MD, FAWM • Alex Nguyen, MD

 BASICS

DESCRIPTION

•  Palmar fibromatosis; caused by progressive fibrous proliferation and tightening of the fascia of the palms, resulting in flexion deformities and loss of function

•  Not the same as “trigger finger,” which is caused by thickening of the distal flexor tendon

•  Similar change rarely occurs in plantar fascia, usually appearing simultaneously.

•  System(s) affected: musculoskeletal

•  Synonyms: morbus Dupuytren; Dupuytren disease; “Celtic hand”

EPIDEMIOLOGY

Prevalence

•  Increases with age; mean prevalence in western countries: 12%, 21%, and 29% at ages 55, 65, and 75 years, respectively. Norway: 30% of males >60 years; Spain: 19% of males >60 years

•  More common in Caucasians of Scandinavian or Northern European ancestry

ETIOLOGY AND PATHOPHYSIOLOGY

Unknown; possibly a T-cell–mediated autoimmune disorder. Occurs in three stages:

•  Proliferative phase: proliferation of myofibroblasts with nodule development on palmar surface

•  Involutional stage: spread along palmar fascia to fingers with cord development

•  Residual phase: spread into fingers with cord tightening and contracture formation

Genetics

•  Autosomal dominant with incomplete penetrance:

–  Siblings with 3-fold risk

•  68% of male relatives of affected patients develop disease at some time.

RISK FACTORS

•  Smoking (mean: 16 pack-years; odds ratio: 2.8)

•  Increasing age

•  Male/Caucasian; male > female (1.7:1)

•  Workers exposed to vibration—risk doubles if regular (weekly) exposure

•  Diabetes mellitus (increases with duration of DM, usually mild; middle and ring finger involved)

•  Epilepsy

•  Chronic illness (e.g., pulmonary tuberculosis, liver disease, HIV)

•  Hypercholesterolemia

•  Alcohol consumption

GENERAL PREVENTION

Avoid risk factors, especially if a strong family history.

COMMONLY ASSOCIATED CONDITIONS

•  Alcoholism

•  Epilepsy

•  Diabetes mellitus

•  Chronic lung disease

•  Occupational hand trauma (vibration)

•  Hypercholesterolemia

•  Carpal tunnel syndrome

•  Peyronie disease

 DIAGNOSIS

HISTORY

•  Caucasian male aged 50 to 60 years

•  Family history

•  Mild pain early:

–  Begins in palm and spreads to digits

•  Unilateral or bilateral (50%)

•  Right hand more frequent

•  Ring finger more frequent

•  Ulnar digits are more affected than radial digits

•  Flexion contracture of metacarpophalangeal (MCP) before proximal interphalangeal (PIP) joint

PHYSICAL EXAM

•  Painless plaques or nodules in palmar fascia

•  Cordlike band in the palmar fascia

•  Skin adheres to fascia and becomes puckered.

•  Palpable subcutaneous nodules

•  Reduced flexibility of MCP and PIP joints

•  No sign of inflammation

•  Web space contractures

•  Ectopic Dupuytren can involve plantar (Ledderhose—10%) and penile (Peyronie—2%) fascia.

–  Knuckle pads over PIP:

  Garrod nodes associated with severe disease

–  Disease stages:

  Early: skin pits (can also be seen in nevoid basal cell cancer and palmar keratosis)

  Intermediate: nodules and cords. Nerves and vessels can be entwined in cords.

  Late: contractures

DIFFERENTIAL DIAGNOSIS

•  Tendon abnormalities

•  Camptodactyly: early teens; tight fascial bands on ulnar side of small finger

•  Diabetic cheiroarthropathy: all four fingers

•  Volkmann ischemic contracture

DIAGNOSTIC TESTS & INTERPRETATION

Diagnostic Procedures/Other

MRI can assess cellularity of lesions that correlate with recurrence after surgery.

Test Interpretation

•  Myofibroblasts predominate

•  Nodules: lumps fixed to skin hypercellular masses

•  Cords: organized collagen type III arranged parallel and hypocellular

•  First stage (proliferative): increased myofibroblasts

•  Second stage (residual): dense fibroblast network

•  Third stage (involutional): Myofibroblasts disappear.

 TREATMENT

GENERAL MEASURES

•  Physiotherapy alone is ineffective:

–  Intermittent splinting is unlikely to be effective.

–  Continuous splinting may help pre- and postop.

•  Follow isolated involvement of palmar fascia conservatively.

•  MCP joint involvement can be followed conservatively if flexion contracture is <30 degrees.

MEDICATION

First Line

•  Clostridial collagenase injections (FDA-approved in 2010):

–  Degrades collagen to allow manual rupture of diseased cord

–  Best for isolated cord of MCP joint

–  5-year recurrence rate of 47%; comparable with surgical recurrence rates (1)[B]

–  More rapid recovery of hand function compared to limited fasciectomy with fewer serious adverse events (2)[B]

–  Complications: injection site reaction, skin tear

–  Can do two cords concurrently

–  Can be effective for postsurgical recurrence

•  Steroid injection:

–  Can treat acute nodules or painful knuckle pads

–  Serial triamcinolone injections improved long-term outcomes when combined with needle aponeurotomy (3)[B].

Second Line

Surgery for contracture >30%

ISSUES-FOR-REFERRAL

•  Any involvement of PIP joints

•  MCP joints contracted >30 degrees

•  Positive Hueston tabletop test: When the palm is placed on a flat surface, the digits cannot be simultaneously placed fully on the same surface as the palm because of flexion contractures.

ADDITIONAL THERAPIES

•  Percutaneous and needle fasciotomy:

–  Best for MCP joint; improvement of 93% versus 57% for PIP joint (4)[B]

–  Recurrence common; 50%

–  Shown to be effective for recurrent disease (4)[B]

–  Better for MCP joints in patients with comorbid conditions; lower complication rate, but higher recurrence (5)[C]

•  Continuous elongation (atraumatic elongation using an external device, typically on 4th and 5th digits prepares a severely contracted joint for surgery (6)[B].

SURGERY/OTHER PROCEDURES

•  Dermofasciectomy/limited fasciectomy/segmental aponeurectomy:

–  Greater initial correction over nonincisional treatment; higher complication rates (5)[C]

–  Night extension orthosis in combination with standard hand therapy no different maintaining finger extension than hand therapy alone in the 3 months following surgical release (6)[B].

•  Indications:

–  Any involvement of the PIP joints

–  MCP joints contracted at least 30 degrees

–  Positive Hueston tabletop test

•  May require skin grafts for wound closure with severe cutaneous shrinkage

•  80% have full range of movement with early surgery.

•  Amputation of 5th digit if severe and deforming

•  MCP joints respond better to surgery than PIP joints, especially if contracted >45 degrees.

 ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Regular follow-up every 6 months to 1 year

PATIENT EDUCATION

•  Avoid risk factors (alcohol, vibratory exposure, etc.), especially if strong family history.

•  Mild disease: Passively stretch digits twice a day and avoid recurrent gripping of tools.

PROGNOSIS

•  Unpredictable but usually slowly progressive

•  10% may regress spontaneously.

•  Dupuytren diathesis predicts aggressive course. Features include ethnicity (Nordic), family history, bilateral lesions outside of palm, age <50 years—all factors with 71% risk of recurrence compared to baseline 23% without any risk factors.

•  Prognosis is better for MCP versus PIP joint after surgery and collagenase injection.

COMPLICATIONS

•  Reflex sympathetic dystrophy postsurgery

•  Operative nerve injury

•  Postoperative recurrence in 46–80%

•  Postoperative hand edema and skin necrosis

•  Digital infarction

REFERENCES

1.  Peimer CA, Blazar P, Coleman S, et al. Dupuytren contracture recurrence following treatment with collagenase Clostridium histolyticum (CORDLESS [Collagenase Option for Reduction of Dupuytren Long-Term Evaluation of Safety Study]): 5-year data. J Hand Surg Am. 2015;40(8):1597–1605.

2.  Zhou C, Hovius SE, Slijper HP, et al. Collagenase Clostridium histolyticum versus limited fasciectomy for Dupuytren’s contracture: outcomes from a multicenter propensity score matched study. Plast Reconstr Surg. 2015;136(1):87–97.

3.  McMillan C, Binhammer P. Steroid injection and needle aponeurotomy for Dupuytren disease: long-term follow-up of a randomized controlled trial. J Hand Surg Am. 2014;39(10):1942–1947.

4.  van Rijssen AL, Werker PM. Percutaneous needle fasciotomy for recurrent Dupuytren disease. J Hand Surg Am. 2012;37(9):1820–1823.

5.  Henry M. Dupuytren’s disease: current state of the art. Hand (N Y). 2014;9(1):1–8.

6.  Collis J, Collocott S, Hing W, et al. The effect of night extension orthoses following surgical release of Dupuytren contracture: a single-center, randomized, controlled trial. J Hand Surg Am. 2013;38(7):1285.e2–1294.e2.

ADDITIONAL READING

•  Ball C, Pratt AL, Nanchahal J. Optimal functional outcome measures for assessing treatment for Dupuytren’s disease: a systematic review and recommendations for future practice. BMC Musculoskelet Disord. 2013;14:131.

•  Eaton C. Evidence-based medicine: Dupuytren contracture. Plast Reconstr Surg. 2014;133(5):1241–1251.

•  Lanting R, Broekstra DC, Werker PM, et al. A systematic review and meta-analysis on the prevalence of Dupuytren disease in the general population of Western countries. Plast Reconstr Surg. 2014;133(3):593–603.

•  Michou L, Lermusiaux JL, Teyssedou JP, et al. Genetics of Dupuytren’s disease. Joint Bone Spine. 2012;79(1):7–12.

•  Sweet S, Blackmore S. Surgical and therapy update on the management of Dupuytren’s disease. J Hand Ther. 2014;27(2):77–84.

 CODES

ICD10

M72.0 Palmar fascial fibromatosis [Dupuytren]

CLINICAL PEARLS

•  Dupuytren contracture is a fixed flexion deformity of (most commonly) the 4th and 5th digits due to palmar fibrosis. 90% of cases are progressive.

•  Refer patients with involvement of the PIP joints or MCP involvement with contractures of >30 degrees.

•  Both surgical and enzymatic fasciotomy have high rate of recurrence.