Dupuytren’s Contracture

Introduction

Dupuytren’s contracture (Dupuytren’s disease) is a progressive fibroproliferative disorder of the palmar fascia, characterized by the formation of nodules and cords that produce progressive flexion contracture of the digits. First described by Baron Guillaume Dupuytren in Paris in the early 19th century, the condition remains the principal benign tumor-like disease of the hand and one of the most common indications for hand surgery. The disease is generally not associated with pain or significant functional limitation in its early stages but progresses to produce flexion contractures at the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints with associated functional disability. The chapter synthesizes content from Apley & Solomon’s, Miller’s Review, and the hand-surgery sections of the surgical-approach text.

Epidemiology and Genetics

Dupuytren’s contracture is predominantly a disease of patients of Northern European ancestry, with the highest prevalence in Scandinavian, Celtic, and British populations (the so-called “disease of the Vikings”). The prevalence rises with age, from approximately 1% in the third decade to over 30% in patients over 70 years of age in susceptible populations. Men are affected approximately 5-10 times more often than women (though women tend to develop the disease at a later age, with a less aggressive course). The pattern of inheritance is autosomal dominant with variable penetrance; multiple genetic susceptibility loci have been identified, with the WNT signaling pathway being a prominent contributor. Risk factors include positive family history (the strongest single predictor), age, male sex, smoking, alcohol use (particularly with liver disease), diabetes mellitus (the disease is more frequent in diabetic patients but tends to be less severe and less progressive than in non-diabetic patients), epilepsy (a historical association attributed in part to phenytoin therapy), and various other factors. The association with manual labor and repetitive trauma has been historically claimed but is not well-supported by modern evidence. The “Dupuytren diathesis” — the constellation of features predicting an aggressive and recurrent course — includes positive family history, bilateral disease, ectopic disease (Garrod’s pads on the dorsal aspects of the PIP joints, plantar fibromatosis of the foot — Ledderhose disease, Peyronie’s disease of the penis), and onset before the age of 40. Patients with the Dupuytren diathesis are more likely to have aggressive disease, bilateral involvement, and recurrence after surgery.

Pathology

The histopathology of Dupuytren’s disease passes through three stages described by Luck: proliferative (high cellularity with proliferating fibroblasts), involutional (alignment of fibroblasts and myofibroblasts in cord-like structures), and residual (acellular collagen). The myofibroblast — a cell with features of both fibroblast and smooth muscle, expressing

α-smooth muscle actin and contracting under appropriate stimuli — is the central cellular actor in the disease and is responsible for the progressive contracture. The disease begins in the palm with thickening and contracture of the palmar fascia, then extends into the digits. The progression involves transformation of normal anatomical fascial bands into pathological cords. The principal cord patterns include: the pretendinous cord (the commonest, arising from the pretendinous band and producing MCP joint contracture); the central cord (extending into the digit and contributing to PIP joint contracture); the spiral cord (which displaces the neurovascular bundle medially and toward the midline as it courses around the bundle — an important clinical consideration since aggressive surgical dissection may injure the displaced nerve and artery); the lateral cord; the natatory cord (involving the web spaces); and several others. The recognition of the cord patterns is critical for surgical planning and avoidance of neurovascular injury.

Clinical Features

The classical presentation is of a slowly developing nodule in the palm, typically at the level of the distal palmar crease and most commonly in the ring or little finger ray. The nodule is firm, mobile relative to deeper structures initially, and may be tender in early disease. Over months to years, the nodule extends distally as a palpable cord, and progressive flexion contracture develops at the MCP joint, then at the PIP joint. The DIP joint may develop hyperextension as a compensatory deformity. Patients typically present when the contracture interferes with function — inability to lay the hand flat on a table (the “tabletop test” of Hueston is the classical clinical sign — failure to place the entire palmar surface of the hand flat against a flat surface indicates a contracture requiring intervention), difficulty putting the hand in a pocket, difficulty washing the face. Pain is uncommon and severe pain should raise suspicion of an alternative diagnosis. Examination assesses: the location and size of palpable nodules and cords; the joints affected (MCP, PIP, DIP) and the degree of contracture at each joint (measured with a goniometer); the involvement of specific rays (the ring finger is most commonly affected, followed by the little, middle, index, and thumb); the involvement of the contralateral hand; the presence of Garrod’s pads (knuckle pads, characteristic of the Dupuytren diathesis); and the presence of associated ectopic disease. The classical clinical presentation has been classified into stages by various authors. Tubiana’s classification grades from Stage N (palpable nodule without contracture) through Stage 4 (greater than 135° total contracture).

Treatment

The treatment of Dupuytren’s contracture is principally surgical or interventional rather than medical, as no medical therapy reliably prevents progression. The decision for intervention is based on the degree of contracture and functional limitation, with the classical threshold for intervention being: positive tabletop test, fixed MCP contracture of 30° or greater, or any fixed PIP contracture. The reasoning is that MCP joint contractures

can typically be corrected even at advanced stages (the MCP joint has stable bony anatomy and the collateral ligaments tolerate prolonged shortening), while PIP joint contractures become increasingly difficult to correct as they progress (the PIP joint develops volar plate and capsular contractures that may not be reversible). Three principal treatment modalities are available: Needle Aponeurotomy (Percutaneous Needle Fasciotomy, PNF) Needle aponeurotomy is the simplest and least invasive treatment. The procedure involves percutaneous division of the cord with a small-gauge needle under local anesthesia in the office setting. The cord is identified, the needle inserted at multiple sites along its length, and the cord is divided by gentle manipulation. The procedure produces immediate improvement in contracture with minimal disruption to surrounding tissue. Advantages include: outpatient office-based procedure; minimal recovery; immediate return to normal activities; low cost; and the ability to be repeated easily for recurrence. Disadvantages include: higher recurrence rate than open surgery (estimated 50-80% recurrence by 5 years); inability to address complex disease or sigificant PIP contractures; risk of skin tear (the skin is often thin over the cord and may tear during manipulation); and risk of digital nerve injury, particularly with the spiral cord. PNF is the procedure of choice for moderate disease with MCP joint contracture predominantly and a palpable discrete cord; it is less suitable for established PIP joint contracture or for complex multi-ray disease.

Collagenase Injection (Clostridial Collagenase) Collagenase clostridium histolyticum injection (Xiaflex, Xiapex) is an enzymatic treatment introduced in 2010. The procedure involves intra-cord injection of the bacterial collagenase enzyme, which selectively digests the collagen of the cord. After 24-48 hours, the patient returns for cord manipulation under local anesthesia, with audible “popping” of the cord as it ruptures and immediate improvement in contracture. Outcomes are similar to needle aponeurotomy for moderate disease, with comparable rates of contracture correction and recurrence. Disadvantages include the cost of the enzyme, the requirement for two visits, and the small but real risk of tendon rupture (the enzyme also affects collagen in normal tendons if accidentally injected outside the cord) and other complications. Surgical Fasciectomy Surgical fasciectomy — the open removal of pathological palmar and digital fascia — has been the historical mainstay of Dupuytren’s treatment and remains the procedure of choice for severe disease, recurrent disease, and disease with established PIP joint contracture. Three principal types of fasciectomy are described: Limited (regional) fasciectomy: Removal of the pathological cord with limited resection of surrounding fascia. The standard technique for primary disease. Performed through a

Bruner zigzag incision or multiple Z-plasty incisions on the palmar surface, with careful dissection along the diseased cord, identification and protection of the neurovascular bundles (particularly with attention to the spiral cord displacement), and excision of the cord with overlying or surrounding diseased fascia. Dermofasciectomy: Removal of the cord together with the overlying skin, followed by full- thickness skin grafting. Used for severe disease, recurrent disease, and in patients with the Dupuytren diathesis where the skin itself is involved in the disease. The procedure removes the substrate for recurrence and has substantially lower recurrence rates than fasciectomy alone, particularly in aggressive disease. Total (radical) palmar fasciectomy: Removal of the entire palmar fascia, including macroscopically normal tissue. Historical procedure with high complication rates, largely abandoned in modern practice. The principles of surgical management include: careful preoperative planning of incisions (Bruner zigzag, multiple Z-plasties, or longitudinal incisions with Z-plasty closure to prevent linear scar contracture); careful intraoperative identification and protection of the neurovascular bundles (particularly the radial digital artery and nerve, which can be displaced superficially by the spiral cord); identification and excision of all macroscopically diseased fascia; full mobilization of the contracted PIP joints when involved (sometimes requiring volar plate release in established contractures); and postoperative splinting and aggressive hand therapy to maintain extension. Outcomes after fasciectomy include immediate correction of the contracture in the great majority of cases, with recurrence rates at 5-10 years of approximately 30-50% for limited fasciectomy and 10-20% for dermofasciectomy. Complication rates include hematoma (5- 10%), wound healing problems (5-10%), nerve injury (1-3%), arterial injury (1-2%), infection (1-2%), CRPS (1-5%), and recurrence.

Salvage Procedures For severe recurrent or unsalvageable disease, several salvage procedures are described: PIP joint arthrodesis (in fixed severe PIP contracture not amenable to other treatment); amputation of the affected digit (rarely required, generally for severe little finger or ring finger contracture with neurovascular compromise); and various combined procedures.

Special Considerations

Recurrence Recurrence of disease — typically defined as new cord formation or worsening of contracture in the treated area — is common in Dupuytren’s disease and is one of the principal long-term concerns. The risk of recurrence is substantially higher in patients with the Dupuytren diathesis. Management of recurrence depends on the severity and the previous treatment: needle aponeurotomy can be repeated easily, while surgical revision becomes progressively more difficult with each previous procedure.

Ectopic Disease Ledderhose disease (plantar fibromatosis) is the analog of Dupuytren’s contracture on the plantar fascia. The condition produces nodules in the plantar fascia of the foot, often bilaterally, with progressive thickening and sometimes contracture. Unlike Dupuytren’s disease, plantar disease rarely produces significant functional contracture, since the plantar fascia is not anatomically attached to flexible digital structures in the same way as the palmar fascia. Treatment is principally conservative; surgical management is reserved for symptomatic mass effect. Peyronie’s disease is the analog of Dupuytren’s contracture on the tunica albuginea of the penis. The condition produces fibrous plaques with penile curvature and erectile dysfunction. Management is the domain of urology. Knuckle pads (Garrod’s pads) are subcutaneous nodules over the dorsal aspect of the PIP joints, associated with the Dupuytren diathesis. They are typically asymptomatic and require no treatment.

Summary and Take-Home Points

Dupuytren’s contracture is a progressive fibroproliferative disorder of the palmar fascia, predominantly affecting men of Northern European descent in middle to old age, producing palpable nodules and cords with progressive flexion contracture of the digits. The Dupuytren diathesis — strong family history, young age at onset, bilateral disease, ectopic disease — predicts aggressive and recurrent disease. The decision for intervention is based on functional limitation, with the tabletop test as a useful clinical threshold and the conventional indications being significant MCP joint contracture or any PIP joint contracture. Three principal treatments are available: needle aponeurotomy (simple, office-based, but with higher recurrence); collagenase injection (similar outcomes to aponeurotomy with the convenience of enzymatic dissolution); and surgical fasciectomy (the gold standard for established disease, with limited fasciectomy for primary disease and dermofasciectomy for aggressive or recurrent disease). The principal long-term concerns are recurrence and the management of established PIP joint contracture, which becomes progressively more difficult to correct as it persists. Across all treatment modalities, the principles of careful patient selection, anatomical knowledge of the cord patterns (particularly the displacement of the neurovascular bundle by the spiral cord), and meticulous surgical technique produce the best outcomes.