Dupuytren Disease

Charles Eaton, MD

Neither Dr. Eaton nor any immediate family member has received anything of value from or has stock or stock options held in a commercial company or institution related directly or indirectly to the subject of this article.

Introduction

Dupuytren disease is the most common hereditary disorder affecting connective tissue. It is most common in senior Caucasian men and affects at least 10 million Americans. There is no biologic cure, all current treatments are palliative, and recurrence is common. The single biggest risk factor for developing Dupuytren disease is having a close relative with the condition—parent, grandparent, or sibling. The hallmarks of Dupuytren disease are nodules in the palm or on the extensor surfaces of the finger joints, and cords in the palm, which may cause finger deformity and limit finger extension.

Until a laboratory biomarker of Dupuytren disease is developed, the diagnosis can only be made clinically, after the development of secondary changes. The increasingly recognized association of Dupuytren disease with other medical issues (hypothyroidism, depression, cardiovascular disease, early mortality, cancer) underscores the need to study Dupuytren disease as a systemic disorder, not simply a local hand problem.

The basic biology is an abnormal response of the fascia to mechanical stress (Figure 25.1). The key cell is the myofibroblast, which is not usually present in a normal fascia, but appears in response to new mechanical strain in injury. Myofibroblasts have characteristics of both fibroblasts and smooth muscle cells. They manufacture and remodel collagen strands along lines of mechanical tension. This increases strength and stiffness of the fascia, which is the normal adaptive response to activity-related strain on supporting tissues. Myofibroblast tissue shortening involves cell contraction, rearrangement of individual collagen strands attached to myofibroblasts, and the action of extracellular enzymes (Figure 25.2). The end result of fibrotic tissue changes resembles scar tissue.

There is controversy as to whether, on a cellular level, Dupuytren tissue remodeling more resembles wound scar contracture or immobilization fibrosis (Figure 25.3). Most patterns of Dupuytren contracture bring the fingers into flexion resembling the resting posture of the hand. In contrast, the same biology affecting the extensor aspect of the fingers (dorsal Dupuytren nodules or “knuckle pads”) does not usually produce extension contracture, because that is not their resting posture—these joints rest in flexion.

The clinical presentation varies greatly. At one end of the spectrum, most patients with Dupuytren disease have mild involvement and never develop contractures. On the other end, some patients have aggressive biology with severe, recurrent contractures and all of the associated conditions. Patients with more severe biology are more likely to develop contractures and to require treatment. Patients referred for hand therapy after a procedure for Dupuytren contracture represent a subgroup of Dupuytren patients with more severe biology than average Dupuytren disease patients.

Patients diagnosed before the age of 50 years have a greater risk of progression to contracture and recurrence after treatment. Recurrent contractures are not uncommon, but not all recontractures are recurrent Dupuytren contracture. The achievable goal of Dupuytren contracture procedures is not to cure the patient of Dupuytren disease, but to improve deformity and trigger a period of prolonged disease stability before the contracture recurs. This goal is not always achieved. Postoperative rehabilitation plays an important role in the final outcome of surgical management of Dupuytren disease.

Anatomy

Most patterns of Dupuytren cords follow the course of normal anatomic structures (Figure 25.4). Common cord patterns are shown in Figure 25.5. Cords may be confined to the palm, the digit, or span both. Common central palm cords are the central palmar, spiral, and proximal first web. Common border palm cords are the natatory, distal first web, hypothenar, and thenar cords. Thenar and hypothenar cords are uncommon, and usually associated with diffuse disease or aggressive biology. The majority of metacarpophalangeal (MCP) joint contractures are due to the effect of an isolated central cord. In contrast, the majority of proximal interphalangeal (PIP) contractures have involvement of multiple cords in the digit. In the digit, the relationship of the neurovascular bundle to the cord is determined

by whether the cord is central, lateral, spiral, or retrovascular (Figure 25.5). Neurovascular bundles may be abnormally displaced by a spiral cord, which occurs in about a quarter of digital contractures.

Figure 25.1 Illustration of the components involved in myofibroblast contraction shown in Figure 25.2. The central structure is a myofibroblast. The pink arrows show how tension placed on tissues is transmitted to individual myofibroblasts through attachments (adhesions) of extracellular collagen strands to the myofibroblast cell membrane and internal cell structures. A, Large adhesion complex in the cell membrane, attached to both extracellular and intracellular stress fibrils. B, Focal adhesion in the cell membrane, attached to both extracellular matrix fibrils and subcellular stress fibrils. C, Subcellular stress fibrils responsible for periodic (weak, brief) contraction. D, Global stress fibrils responsible for isometric (strong, sustained) contraction. E, Extracellular matrix proteolytic enzyme and cross-linker that joins adjacent collagen strands. F, Collagen fibrils in the extracellular matrix.

Figure 25.2 Illustration of contracting mechanism that shortens collagen. A, Isometric contraction of the global stress fibrils deforms the matrix, pulling collagen strands at each end of the myofibroblast and creating slack in strands in each side of the cell. B, C, Periodic contractions of the subcellular stress fibrils remove collagen strand slack beyond the cell, creating collagen strand loops on the side of the cell. D, While in this position, extracellular proteolytic and cross-linking enzymes divide overlapping collagen loops and join segment ends. E, Isometric contractions end, and the cell shape re-equilibrates. F, Newly shortened collagen strands sustain the matrix deformity created by active cell contraction.

Figure 25.3 Illustration comparing Dupuytren contracture biology concepts. The blue lines in the diagram represent connective tissues affected by Dupuytren disease. Top: Fascia accommodates full finger flexion and extension by conformational changes similar to folding and unfolding. Fingers rest in flexion. Left: Active contracture concept of tissue shortening, similar to that of the healing and scarring that draws closed an open wound. The turnbuckle represents tissue contraction independent of the posture of the finger. Right: Passive contracture concept of tissue remodeling, similar to that of immobilization fibrosis. At rest, depending on the posture of the finger, shortening conformational changes from tissue slack are made permanent by tissue remodeling, which removes posture-related slack in individual collagen strands. Bottom: The end result of each mechanism has the same tethering effect, limiting finger extension.

Patient Evaluation

The extent of Dupuytren involvement varies considerably from minimal contracture and deformity to severe involvement. Measurements of contractures are affected in two ways specific to Dupuytren disease. First, although the cords themselves are not elastic, they are often anchored to elastic tissues. This may lead to significant differences between active and passive measurements and to examiner bias in passive range of motion (PROM) measurements. Second, cords that span multiple joints may produce dynamic contractures, in which measurements of one joint are
influenced by the position of the adjacent joint. Dynamic contractures spanning the palm and the carpometacarpal (CMC) joints can result in large variations in measurements (Figure 25.6). A Dupuytren-specific diagram designed to allow documentation of findings and measurements is shown in Figure 25.7.

Figure 25.4 Illustration of normal fascial anatomy. A, Abductor digiti minimi fascia. B, Abductor digiti minimi tendon. C, Abductor pollicis brevis fascia. D, Cleland ligament. E, Deep pretendinous fibers. F, Distal first web space ligament. G, Extensor tendon. H, Flexor tendon sheath. I, Flexor tendons. J, Grayson ligament. K, Lateral band. L, Natatory ligament. M, Neurovascular bundle. N, Palmar anchoring fibers. O, Palmaris longus tendon. P, Pretendinous band. Q, Proximal first web space ligament. R, Proximal phalanx. S, Proximal pretendinous band coalescence. T, Retinacular ligaments. U, Septum of Legueu and Juvara. V, Spiral band. W, Superficial pretendinous fibers. X, Superficial transverse palmar ligament. Y, Transverse metacarpal ligament.

Figure 25.5 Illustration of common cord patterns. 0, central digital; 1, central palmar; 2, distal first web space; 3, hypothenar; 4, lateral digital; 5, natatory; 6, proximal first web space; 7, retrovascular; 8, spiral; 9, thenar.

Surgical Management

Despite pilot studies suggesting use of splinting as the sole treatment for Dupuytren contracture in compliant patients, there is no agreement on the effectiveness of preventive or nonoperative therapy for Dupuytren disease. Studies of the effect
of occupation and rock climbing on the incidence of Dupuytren contracture are consistent with cell biology research in that high-stress/shear manual activities may provoke the core biology. This is difficult to translate into lifestyle or activity recommendations for all patients, but should be discussed.

Figure 25.6 Photpgraphs demonstrating dynamic contractures with carpometacarpal (CMC) fasciodesis. Patients use a trick motion to compensate for a tight fascia. When cords span the MCP and PIP, patients flex their MCP to extend their PIP and vice versa. The same is true with cords extending into the proximal palm, in which flexing the ring and small CMC joints increases MCP extension. Left: CMC flexion allows 10° of active MCP hyperextension. When CMC flexion is blocked, active MCP extension is limited to 20°, which, in turn, improves active PIP extension by 10°. Right: Blocking CMC flexion changes passive MCP extension from 0° to 65°.

Figure 25.7 This diagram is based on common zones of involvement and allows standard documentation of the location of physical findings, procedures, and joint measurements. PDF versions of this as evaluation and procedure forms are available at http://Dupuytrens.org/forms.

There are three types of procedures used to treat primary Dupuytren contracture (Figure 25.8). Minimally invasive procedures disrupt cords without removing tissue. Examples of this are percutaneous needle fasciotomy (PNF) and enzymatic fasciotomy with collagenase Clostridium histolyticum (Xiaflex®, Auxilium Pharmaceuticals, Inc., Chesterbrook PA). Fasciectomy, the most common procedure, is removal of some or all of the affected cord tissue. Dermofasciectomy is removal of affected cord tissue and the overlying skin and resurfacing the area with skin graft. Salvage procedures for failed surgery include flap reconstruction, PIP arthrodesis, and amputation. The three procedures for primary disease have similar outcomes in terms of initial correction of deformity. Recovery time, complication rate, and average time before recurrence are least for minimally invasive procedures and greatest for dermofasciectomy.

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