Dupuytren Contracture

Pathogenesis

The cause of Dupuytren disease is unknown; however, the cellular and connective tissue changes that occur with this disorder have some similarity to malignant tumors despite being a benign process. Dupuytren contracture begins with increased fibroblast proliferation followed by type III collagen deposition resulting in uncontrolled palmar fascia growth ultimately causing flexion contractures. Dupuytren disease has been compared with wound healing in that myofibroblasts, which produce type III collagen, are dominant in both granulation tissue and Dupuytren tissue. In addition, growth factors and their receptors have been shown to have increased expression in diseased fascia, especially transforming growth factor-β and basic fibroblast growth factor. Transforming growth factor-β has been shown to induce differentiation of fibroblasts into myofibroblasts. An increase in glycosaminoglycans, matrix metalloproteinase activity, the presence of fibrofatty tissue between the skin and fascia, trauma, and microtrauma caused by free radicals also have been theorized as playing a role in the development of Dupuytren contracture.

Dupuytren disease progresses through several stages: proliferative, involutional, and residual. In the proliferative stage, nodules, composed of type III collagen and fibroblasts, develop and expand to displace the subcutaneous tissue and fuse to the skin. The nodules typically appear at the distal palmar crease over the MP joints and distally over the PIP joints but not over the distal interphalangeal joints. They eventually stop growing and begin to contract in the involutional stage. Stress alignment of the fibroblasts occurs, and more collagen is produced. Myofibroblasts then replace the fibroblasts as the predominant cell type, producing type III collagen and causing contraction. Nodule expansion places tension on the normal fascia proximally, producing fascial hypertrophy and nodule-cord units. In the residual phase, the nodules decrease in size and may become acellular fibrous cords. Contractures of the MP and PIP joints and displacement of digital neurovascular bundles result from predictable patterns of fascial cord involvement.

The fascial structures that may become involved in the fibroproliferative process have been clearly outlined by McFarlane ( Fig. 75.3 ). Thomine described a longitudinally oriented fascia located dorsal to the neurovascular bundle, which he termed the retrovascular cord. This structure often is involved in the disease and may be implicated as a cause for recurrent PIP contractures. The Cleland ligament generally is believed to be spared. The pretendinous cord nearly always is responsible for primary contracture of the MP joint. It may attach to the distal palmar crease skin, base of the proximal phalanx, or the tendon sheath at this level, or it may extend to attach to the flexor tendon sheath over the middle phalanx or the skin in this area. A spiral cord occurs when four normally existing structures (pretendinous band, spiral band, lateral digital sheet, and Grayson ligament) become diseased. The spiral cord runs dorsal to the neurovascular bundle proximally and volar to it distally. When the spiral cord is contracted, the neurovascular bundle is drawn superficially and toward the midline of the finger ( Fig. 75.4 ). Neurovascular displacement is found most commonly on the ulnar aspect of the little and ring fingers, and tedious dissection is required to prevent digital nerve injury.

The lateral digital cord may extend distally and contribute to a flexion contracture of the distal interphalangeal joint. The plane between this cord and the overlying skin is minimal and must be developed sharply. The retrovascular cord is not believed to contribute significantly to flexion contracture of the PIP joint; however, it may be responsible for some residual flexion contracture or recurrence if not excised.

MP and distal interphalangeal (DIP) joint contractures seem to result from pretendinous and lateral cord development. PIP joint contractures may develop from isolated digital cords in addition to central, spiral, or retrovascular cords ( Figs. 75.5 and 75.6 ). The diseased tissue in this unusual form of Dupuytren contracture most commonly affects the small finger; however, any digit may be involved. The cord originates from the periosteum of the proximal phalanx and fascia overlying the intrinsic muscles and distally courses dorsal to the neurovascular bundles, inserting into the middle phalanx or the overlying flexor tendon sheath volar to the neurovascular bundles. This digital cord frequently displaces the neurovascular bundle superficially and to the midline of the finger, similar to a spiral cord.

Skoog suggested that, of the palmar fascia components, only the longitudinal pretendinous bands are involved and that the superficial transverse palmar ligaments are always spared. We agree with McFarlane that the superficial transverse ligaments, in addition to natatory cord prominence, may be involved and require excision especially in symptomatic thumb web space contractures.

Prognosis

The prognosis in Dupuytren contracture seems to depend on the following factors, which may determine the appropriate intervention:

• • 1.

    Heredity. A family history of the disease indicates that the lesion is likely to progress more rapidly than usual, especially if the onset is early.

  • 2.

    Sex. The lesion usually begins later and progresses more slowly in women, who often accommodate better to the resulting deformity; however, long-term results after operation are worse in women than in men, with postoperative flare reaction being twice as likely.

  • 3.

    Epilepsy. Despite earlier statements positively associating Dupuytren contracture with epilepsy, Geoghegan et al. concluded that neither epilepsy nor antiepileptic medications were associated with the disease.

  • 4.

    Diabetes mellitus. Diabetes mellitus is a risk factor for Dupuytren disease, especially in patients requiring medical management compared with patients with diet-controlled diabetes mellitus. According to Geoghegan et al., patients taking insulin were more likely to have Dupuytren disease than patients taking metformin or sulfonylureas.

  • 5.

    Alcoholism or smoking. The lesions are more severe, progress more rapidly, and recur more frequently when associated with these conditions. Godtfredsen et al. found a dose-dependent relationship to alcohol intake and smoking and concluded that the combination of these two factors conveys a high risk for the development of Dupuytren disease.

  • 6.

    Location and extent of disease. When the disease is bilateral and especially when it is associated with knuckle pads and nodules in the plantar fascia, progression is more rapid and recurrence is more frequent. Progression is more rapid on the ulnar side of the hand.

  • 7.

    Behavior of disease. How the disease has behaved in the past, whether treated or not, is an indication of its probable behavior in the future.

Patients need to know that Dupuytren disease is not a curable disorder and that interventions are designed to manage functional deficits resulting from the nodules and cords. Contracture management usually focuses on isolated deformities; however, recurrence in the areas treated is common and more widespread disease may follow (disease extension) and be a cause for patient concern. Hence, counseling patients regarding disease recurrence and extension is important prior to engaging various treatment regimens ( Fig. 75.7 ).

Nonoperative Treatment

Various nonoperative treatment regimens for Dupuytren disease continue to focus on the fundamental disease histopathology ( Box 75.1 ).