You are in: eMedicine Specialties > Orthopedic Surgery > HAND AND UPPER EXTREMITY Dupuytren ContractureArticle Last Updated: Apr 6, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: Steve Lee, MD, Physician in Plastic, Reconstructive, and Hand Surgery, The Samra Group Steve Lee is a member of the following medical societies: American College of Surgeons and American Society of Plastic Surgeons Coauthor(s): Michael Baytion, BS, Ohio State University College of Medicine; Mark F Hendrickson, MD, Chief, Section of Hand Surgery, Department of Plastic and Reconstructive Surgery, Cleveland Clinic Foundation; Derek L Reinke, MD, Consulting Staff, Cary Orthopedic and Sports Medicine Specialists; Yelena Bogdan, Stony Brook University Health Sciences Center School of Medicine (SUNY) Editors: Michael S Clarke, MD, Clinical Associate Professor, Department of Orthopedic Surgery, University of Missouri-Columbia School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Michael Yaszemski, MD, PhD, Associate Professor, Departments of Orthopedic Surgery and Bioengineering, Mayo Foundation, Mayo Medical School; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; Harris Gellman, MD, Consulting Surgeon, Broward Hand Center, Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine Author and Editor Disclosure Synonyms and related keywords: Dupuytren's disease, Dupuytren disease, fibromatosis, fibromatoses, Dupuytren's contractures, palmar contracture, palmar fascia INTRODUCTIONSection 2 of 9
  ProblemDupuytren contracture, a disease of the palmar fascia, results in the thickening and shortening of fibrous bands in the hands and fingers. This disease entity belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads). Guillaume Dupuytren received recognition for the condition that came to be associated with his name because of his expertise on the clinical findings, pathogenesis, prognosis, and treatment of this disease; however, Plater and Cline respectively provided the earliest known records of Dupuytren contracture and its surgical treatment (Verheyden, 1983). FrequencyRace, Sex, and Genetics The incidence of Dupuytren contracture is highest in Caucasians, historically those of Celtic descent. Ling (1963) demonstrated that the prevalence of Dupuytren disease in a Celtic family was 24%, which increased to 74% with the inclusion of close relatives. The disease affects men 7-15 times more often than it does women. Although the incidence of Dupuytren disease is higher in men, Hueston (1960) suggested that the male-to-female incidence rate may actually be less because the disease is less severe in women; therefore, this condition may go unnoticed until later in life. Most cases of Dupuytren disease occur in patients older than 50 years. Consequently, one may expect an increasing incidence as the aging population increases. Dupuytren disease has long been known to be transmitted in an autosomal dominant fashion with variable penetrance. Neumuller et al (1994) demonstrated an increased relative risk of 2.94 for individuals who express human leukocyte antigen (HLA)-DR3. Associated conditions The incidence of Dupuytren disease also increases with concurrent patient clinical conditions or factors such as diabetes, smoking, chronic alcoholism, seizures, and infection (Ross, 1999). (Rheumatoid arthritis is associated with a decreased incidence of Dupuytren contracture.) About 5% of individuals with Dupuytren disease are diabetic, with an increased prevalence that is proportional to the duration of the diabetes (Noble et al, 1984). One study showed a higher incidence of retinopathy and Dupuytren contracture in diabetic patients and attributes the cause to microangiopathic changes. Similarly, smoking is associated with microvascular changes that may contribute to Dupuytren disease (Burge et al, 1997). The prevalence of Dupuytren disease in those with chronic alcoholism increases in proportion to the amount of alcohol consumed (Bradlow and Mowat, 1986). Although the incidence of Dupuytren disease is 2-3 times higher in individuals with epilepsy, opinions about the cause differ. One group of investigators concluded that electroencephalogram (EEG) abnormalities were more common in patients with Dupuytren disease than in those with other clinical conditions. Another study showed a correlation between increased barbiturate medication and a higher occurrence of Dupuytren disease (Critchley et al, 1976), whereas other studies implicated a genetic link between the 2 diseases. Lastly, there are conflicting results about whether the human immunodeficiency virus (HIV) is implicated in some cases of Dupuytren disease (Ross). Bower et al (1990) demonstrated an increased incidence in patients with both Dupuytren contracture and HIV infection, whereas a study by French researchers showed no statistically significant difference in these patients compared with the general population. Although results of previous studies that linked repetitive manual labor to Dupuytren disease were inconclusive, more recent studies show that the incidence of the disease is 5.5 times higher with such labor. Anecdotal evidence suggests that Dupuytren disease occurs after trauma. Regardless of these results, a positive family history may play a role in both occupational and traumatic cases of Dupuytren disease. Age and family history Younger individuals with a positive family history for the Dupuytren disease have been reported, although the disease most often affects people older than 50 years. In addition, they often have other fibromatoses, such as plantar fibromatosis, knuckle pads, and penile fibromatosis, although this diathesis occurs in less than 1% of the patients with Dupuytren disease. Among patients affected with Dupuytren disease, only 10-20% develops knuckle pads, and about 10% develop plantar fibromatosis. Factors such as family history, age younger than 40 years, presence of related fibromatoses, and disease on the radial side of the hand indicate an increased severity of the disease and an increased likelihood of recurrence. EtiologyDupuytren disease is an autosomal dominant fibroproliferative disease with variable penetrance. Therefore, the manifestation of this condition depends on genetic and environmental factors. Although Dupuytren contractures are associated with other diseases, such as diabetes, chronic alcoholism, and seizures, specific causal relationships have not yet been determined. A number of growth factors, immunologic mediators, and free radicals are implicated in the development of Dupuytren contractures. Most likely, an inciting disease or event in a genetically predisposed individual causes a cascade of events that may include processes that promote the formation of growth factors and free radicals that ultimately leads to abnormal fibroproliferation and the appearance of the characteristic Garrod nodule. Even when homeostasis is ultimately achieved and fibroblastic growth lessens, the pathologic nodule and cord remain. PathophysiologyInvestigators have proposed several hypotheses for the pathogenesis of Dupuytren disease. One cause may be localized ischemia and subsequent xanthine oxidase–derived free-radical formation from endothelial cells. Fibroblasts proliferate within the fascia, clustered around the microvasculature. Research has revealed that lower concentrations of free radicals cause fibroblast proliferation in laboratory cultures. Because active fibroblasts produce free radicals as well, the fibroblasts induce an autocrine positive-feedback effect on themselves, causing further ischemia to the microvasculature. Research has shown that growth factors such as basic fibroblast growth factor (FGF), platelet-derived growth factor (PDGF), and transforming growth factor-beta (TGF-â) may signal the overproduction of the myofibroblasts and/or myofibroblastic activity of the fibroblasts. In addition, high levels of TGF–â may hinder apoptosis, or cell death, of the active myofibroblasts, unlike normal tissue healing. The increased concentration and activity of the myofibroblasts not only increase the total amount of collagen leading to the pathologic nodule but also cause remodeling of the normal collagen matrix and an increase in the ratio of type I collagen to type III collagen. Ultimately, the excess deposition of type III collagen and the formation of cross-links along the lines of tension on the palm and proximal digits result in contractures. ClinicalThe typical patient with Dupuytren disease is aged 50 years or older and presents with a palmar nodule and cord adherent to the skin, as well as with a flexion contracture. The likelihood of Dupuytren disease also increases in patients with pitting over the nodule and/or nodules, which are present bilaterally. Dupuytren disease must be distinguished from several other conditions that affect the hand, including trigger finger, stenosing tenosynovitis, a ganglion cyst, or a soft-tissue mass. Unlike Dupuytren contracture, trigger finger typically involves pain with flexion followed by the inability to extend the affected digit. Stenosing tenosynovitis may be distinguished from Dupuytren disease by pain and a history of overuse or trauma. A small, movable nodule that is tender to palpation at the metacarpophalangeal (MCP) joint is likely a ganglion cyst. A soft-tissue mass must also be excluded from the diagnosis, especially if the patient is significantly younger than the typical patient with Dupuytren disease and if he or she has no other risk factors. A patient younger than age 40 years without involvement of the dorsal hand, foot, or penis is unlikely to have Dupuytren disease; however, the possibility of a sarcoma must be ruled out—although the pathologic findings of a biopsy will most likely reveal a benign etiology (eg, lipoma, inclusion cyst). RELEVANT ANATOMYSection 3 of 9
The basis of Dupuytren disease lies in the nodule and the cord, the pathologic counterparts to the tendon and pretendinous bands. Most often, a nodule forms on either side of the distal crease of the palm. Later, nodules may form near the MCP joint or next to the PIP joint of the thumb and fourth and fifth digits. Contractures may then form along the normal fascial structures. In the palm, contractures occur in the pretendinous bands and natatory ligaments, which are subsequently called the pretendinous cord and natatory cord, respectively. In addition, a contracture may be formed by the attachment of the transverse fibers of the palmar aponeurosis, which is found at the crease between the index finger and the thumb. In the digits, normal fascial structures, including the volar superficial fascia and lateral digital sheets, effectively become the central cord and lateral cords, respectively. The pathologic spiral cord arises from 4 anatomic components: the lateral digital sheet, the pretendinous ligament, the spiral band, and the Grayson ligament. Fascia deep to the neurovascular bundle forms the retrovascular cord. The spiral band is located on the lateral aspects of the digits and is named such because it spirals around the neurovascular bundle. As progressive fibrosis of the spiral band forms the spiral cord, the structure shortens and pulls the normally lateral neurovascular bundle toward the midline of the affected digit. Knowledge of the neurovascular bundle displacement is critical so as not to sever it during surgery. CONTRAINDICATIONSSection 4 of 9
In younger individuals in whom Dupuytren disease is suspected, epithelioid sarcomas and fibromas must first be ruled out. WORKUPSection 5 of 9
Lab Studies
Imaging Studies
Histologic FindingsHistologic analyses of lesions resulting from Dupuytren contractures indicate physiologic characteristics of hypertrophic scar formation, including the following: myofibroblastic proliferation with increased water levels, type III collagen, chondroitin sulfate, and reducible cross-linkages. In addition, surrounding areas without clinical manifestations reveal newly formed type III collagen, reducible cross-links, and fibroblasts with myofibroblastic characteristics such as intracellular microfilaments, as well as basement lamina-like condensation. This evidence suggests that Dupuytren disease spreads by migrating along the altered collagen bundles and that clinically silent areas are also affected. TREATMENTSection 6 of 9
Medical therapyIn the past, nonsurgical approaches to Dupuytren contractures such as splinting, irradiation, ultrasonography, dimethylsulfoxide, vitamin E therapy, and allopurinol treatment were shown to be ineffective. Steroids produced only temporary therapeutic effects, and their use has been debated in the literature. Future nonoperative therapies include the use of calcium channel blockers or gamma-interferon, skeletal traction, and percutaneous needle fasciotomy, with the last of these showing the most promise with minimal adverse effects. Collagenase percutaneous needle fasciotomy is currently undergoing clinical trials. Preliminary results in a study by Badalamente and Hurst (1999) showed astonishing results of more than 90% correction of the MCP joint, 66% correction of the PIP joint, and minimal recurrence rates. Adverse effects included pain at the injection site, minimal swelling, and hematoma. Although collagenase also shows promise in clinical trials, surgical intervention for Dupuytren contractures is still considered the criterion standard. Surgical therapyAlthough the option for surgery in Dupuytren disease is considered on a case-by-case basis, guidelines for the timing of surgery exist. In general, surgery should be performed on an affected MCP joint if the contracture is 30° or greater. Such contractures most likely cause some debilitation for the patient. Usually, a limited fasciectomy of the pretendinous cord is sufficient to establish normal function in the MCP joint. McFarlane favors use of a regional fasciectomy of the pretendinous cord to prevent recurrence of Dupuytren contracture. For longitudinal incisions, Z-plasties or multiple Y-to-V advancements may adequately close the wound. A transverse incision may be necessary for more extensive disease; in such cases, a defect may require a full-thickness skin graft or necessitate the wound to heal secondarily. The evaluation of a PIP joint in Dupuytren disease is different from that of an MCP joint, and the prognoses differ as well. In PIP joint contractures, one should clearly define the method to be used in surgery, as well as discuss with patients their expectations, occupation, and activities that may require use of their hands. Given the difficulty of correcting severe disease, fasciectomy is indicated for any amount of PIP joint contracture. Unfortunately, recurrence is common. The procedures of choice in the PIP joint are dermatofasciectomy or extensive fasciectomy. Interestingly, Hueston (1984) discovered no recurrence with a full-thickness skin graft in dermatofasciectomy. Therefore, this technique is used for patients with an increased diathesis and an increased likelihood for recurrence. Extensive fasciectomy prevents recurrence because the entire diseased fascia is removed, along with the central, lateral, spiral, natatory, and retrovascular cords, as well as any normal fascia that may later be affected. Preoperative detailsThe surgeon and/or patient may choose either regional (local, median, or ulnar nerve block) or general anesthesia for the procedure. Regional anesthesia performed more proximally decreases tourniquet-related discomfort. Hurst uses Marcaine (bupivacaine HCl; Cook-Waite, Cambridge, Ontario, Canada) without epinephrine for its longer duration of nerve blockage. (Note: Regional anesthesia should not be used if the patient has any of the following conditions: coagulopathy, psychosis, peculiar and/or unstable personalities, or progressive neurologic disease.) Intraoperative detailsLoupe magnification should be used for the procedure to aid visualization. The use of a tourniquet around the affected upper extremity also aids visualization of the lesion and control of blood loss. Depending on the location of the lesion, a transverse palmar incision may be used with a zigzag incision or a limited straight-line incision with Z-plasties over the affected digits. The skin over the distal palm remains intact, with continued excision of the underlying fascia. For procedures in the PIP joint, excision of the palmar aponeurosis is most often performed, and the cords are removed. For dermatofasciectomy, a full-thickness skin graft is used to replace the diseased fascia and overlying skin. The surgeon must exercise care not to inadvertently excise the digital nerves. Before the wound is closed, the tourniquet is removed, and electrocautery is performed for hemostasis. Postoperative detailsProper postoperative care is essential for a successful surgical outcome. The protocol includes splinting in extension and an exercise regimen with a therapist for instituting range-of-motion exercises within the first week after surgery. Patients who undergo PIP joint surgery require 6 weeks of continual splinting, including splinting at night, for as long as 3 months to minimize secondary scar contractures. COMPLICATIONSSection 7 of 9
Complications occur most often in patients who require extensive fasciectomy because of severe disease; McFarlane and McGrouther (1990) reported a complication rate as high as 17-19%. During surgery, complications may include severing of the digital nerves, most often the neurovascular bundle; the inadvertent creation of a buttonhole through the skin flaps during their separation between the skin and the fascia; and circulatory compromise secondary to trauma to the digital arteries. Postoperative complications include loss of flexion, hematoma, skin sloughing, infection, edema, and reflex sympathetic dystrophy. The most common PIP joint postoperative complication is loss of flexion, which occurs in 6% of patients. The triad of hematoma, infection, and skin loss occurs in 3% of patients. Hematomas most often form in the palm, and they may be prevented by meticulous hemostasis, by removing the tourniquet before the wound is closed, and by rapid evacuation of hematomas, which prevents necrosis of tissue and skin and decreases the risk of infection. Elevation of the hand can prevent postoperative edema. Reflex sympathetic dystrophy more commonly occurs in patients with extensive fasciectomies and, thus, more aggressive disease. This idiopathic pain syndrome, which often occurs 3-4 weeks after the surgery, consists of pain, edema, stiffness, and vasomotor disturbances. Reflex sympathetic dystrophy occurs in 5% of patients, affecting 3% of men and 7% of women; treatment includes sympathetic blockade for symptomatic relief. FUTURE AND CONTROVERSIESSection 8 of 9
Dupuytren disease has been researched extensively since its initial discovery in the 16th and 17th centuries. However, debate regarding the exact cause of the disease continues. Future nonoperative therapies may include percutaneous needle fasciotomy, skeletal traction, therapy with calcium channel blockers, and treatment with gamma-interferon. The last of these treatments shows the most promise. Until any of these treatments is proven to be therapeutic, surgery is still the only option for a cure. REFERENCESSection 9 of 9
Article Last Updated: Apr 6, 2007 |
