You are in: eMedicine Specialties > Plastic Surgery > HAND Hand, Dupuytren DiseaseArticle Last Updated: Nov 17, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: D Glynn Bolitho, MD, PhD, FACS, FRCSC, FCS(SA), Associate Clinical Professor, Department of Plastic Surgery, University of California at San Diego; Private Practice, LaJolla, California D Glynn Bolitho is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society for Aesthetic Plastic Surgery, American Society of Maxillofacial Surgeons, American Society of Plastic Surgeons, California Society of Plastic Surgeons, and Royal College of Physicians and Surgeons of Canada Editors: Milton B Armstrong, MD, FACS, Associate Professor of Clinical Surgery, Associate Professor of Clinical Orthopedics, Department of Surgery, University of Miami Miller School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; David W Chang, MD, FACS, Associate Professor, Department of Plastic Surgery, MD Anderson Cancer Center, The University of Texas; Nicolas (Nick) G Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center at Swedish Medical Center; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics Author and Editor Disclosure Synonyms and related keywords: dupuytren, Dupuytren contracture, fibroproliferative disorder, joint contracture, proximal interphalangeal joint contracture, PIP joint contracture, metacarpophalangeal joint contracture, MP joint contracture, knuckle pads, Garrod nodes, plantar fibromatosis, Lederhose disease, Peyronie disease EPIDEMIOLOGYSection 2 of 11
  Dupuytren disease is a fibroproliferative disorder of unclear etiology and pathogenesis. It is a disease that occurs primarily in men with Celtic ancestry. In Australia, as many as 20% of men older than 60 years are affected. This condition is rare in Asian individuals and very rare in African Americans. The male-to-female ratio is 5:1 and one third of patients have a positive family history. Usually, both hands are involved, with unilateral disease an uncommon variant. ETIOLOGYSection 3 of 11
A strong correlation exists with Peyronie disease. Dupuytren disease is not an etiologically uniform disease. Abnormal chromosomal patterns (mosaicism) may be the cause of a fundamental abnormality in fibrogenesis. The Murrell hypothesis states that localized fat hypoxia in the palmar tissue results in the release of oxygen free radicals by the reaction of xanthine oxidase and oxygen. Myofibroblasts have been demonstrated to proliferate after stimulation by oxygen free radicals. Treatment with allopurinol is suggested. Associated conditions include the following:
PATHOLOGYSection 4 of 11
The fibromatoses as a group are intermediate between benign fibrous lesions and fibrosarcoma. Dupuytren disease is a quasi-neoplastic proliferative disorder. The cell of Dupuytren is the fibroblast in the early stages, whereas the myofibroblast is the principal cell in the contractile phase. Fibrocytes occasionally are observed in cords of long-standing contracture. Myofibroblasts derive from pericytes following a hypoxic stimulus (luminal occlusion). In addition to having contractile properties, myofibroblasts are capable of manufacturing collagen and elastin. In Dupuytren disease, types III, V, and type I trimer collagen are increased (as in healing wounds). The collagen types are no different than those found in normal healing wounds. Biochemical changes (ie, increased chondroitin sulfate and dermatan sulfate) are found throughout the palmar fascia, underlying the multifocal nature of the disease. The biochemical changes may be secondary to mechanical loading of the soft tissues, rather than a primary abnormality. PATHOGENESISSection 5 of 11
The pathognomonic lesion is the nodule, which is usually adjacent to the distal palmar crease. It is the site of the contractile process and may be multiple. Three stages are as follows:
In 1985, Flint noted fibrous replacement of subdermal fat in Dupuytren disease and argued that this was the primary abnormality. Loss of cushioning resulted in damage to the longitudinal fibers of the fascia, and the fibrotic reaction in the palmar fascia represents a reparative response. Mechanism of contraction Myofibroblasts contain actin and myosin filaments and strong intercellular bonds that permit a synchronized contraction process. Once the dermis is fixed to the palmar fascia, the normal hand movements result in continuation of the process to a severe contracture. ANATOMYSection 6 of 11
The only two components of the palmar fascia to become involved are the pretendinous band and the natatory ligament. The former is the only structure that results in metacarpophalangeal (MP) joint contracture. According to Luck, normal fascial tissue is referred to as bands and diseased tissue is referred to as cords. The pretendinous bands of the palmar aponeurosis run longitudinally down the hand. The band to the index finger frequently ends in the skin on the radial side of the hand; the band to the thumb is inconsistent. The insertion of the pretendinous bands is to the skin distal to the distal palmar crease and by means of a bifurcate insertion into the side of the finger dorsal to the neurovascular bundle. The natatory ligament runs transversely across the hand distal to the MP joints into the first web space, giving fibers that blend with the lateral digital sheet to each digit and attaching to the superficial aspect of the flexor tendon sheath. The superficial transverse ligament lies deep to the pretendinous bands, proximal to the MP joints and the natatory ligament. In the fingers, only the superficial fascial structures become involved (Cleland and Landsmeer ligaments are spared), as follows:
Knuckle pads develop in the dorsal subcutaneous wrinkle ligaments of McGrouther. The spiral cord originates from the pretendinous band and winds around the neurovascular bundle in disease. The central, lateral, and spiral cords terminate in the tendon sheath and adjacent middle phalanx. One or more may be found in any patient, but seldom on both sides of the finger. The disease process in the finger results in the formation of the following 4 cords:
Spiral and lateral cords displace the neurovascular bundle toward the mid line, while the central cord encases the bundle and usually is directed toward one or the other side of the mid line of the finger. The spiral cord is made up of (1) the pretendinous band, (2) the spiral band, (3) the lateral digital sheet, and (4) Grayson ligament. The retrovascular cord of Thomine is an oft-neglected part of the disease and a common cause of postoperative failure. With increasing flexion contracture, the neurovascular bundle comes to lie more superficial and more proximal. The thumb may be involved by 1 of 3 fascial structures: the natatory ligament, the pretendinous band, or the superficial transverse ligament of the palm. In short, the cardinal features are the nodule, the cord, and the digital flexion contracture. TREATMENTSection 7 of 11
Incision or excision of the diseased fascia is the only available treatment to correct joint contracture. However, surgery does not cure the disease. Dupuytren disease is usually no more than an inconvenience, thus the need for treatment must be tailored to the individual. IndicationsThe presence of the disease does not constitute an indication for operation. This depends on the severity of the contracture and the joint involved. Tenderness is seldom a symptom since it soon resolves. When the tabletop test is positive, begin thinking about surgery. The goals of treatment, in order, are improvement of functional capability, reduction of deformity, and lessened recurrence. Presently, the latter is clearly unattainable although a skin graft prevents local recurrence. The incurability of the disease should be communicated clearly to the patient because it may affect the decision of initial operation.
Types of surgeryAll techniques fall into 1 of 3 groups: fasciotomy, regional fasciectomy, and extensive fasciectomy. Three decisions must be made: (1) type of operation needed, (2) type of incision required, and (3) method of wound closure employed. Two surgical principles are the release of longitudinal tension and the management of the skin. At the heart of the problem is the question of recurrence since curing Dupuytren disease is impossible. The continued ability to produce Dupuytren tissue within an operated area (ie, recurrence) or without (ie, extension) is dictated by the patient's Dupuytren diathesis. The second factor is ongoing tension on the cord. More often it seems that the patient's disease, not the surgeon, dictates recurrence. Fasciotomy
Many of these procedures end up as regional fasciectomies since minimal dissection results in further exposure and resection of the band. Bear in mind that an interposed skin graft functions as a virtual firebreak, with no possibility of recurrence beneath it (Gonzalez principle). Regional fasciectomy In this technique, previously known as limited fasciectomy, only the diseased fascia is excised (in the palm, the pretendinous cords and the natatory ligament; in the fingers, only those cords obviously involved). This procedure is flawed because Dupuytren disease is a multifocal disease and likely to recur in other sites. Nevertheless, good results can be obtained. Limited fasciectomy is the most commonly performed procedure for Dupuytren disease. Gonzalez's limited open fasciectomy plus graft (1985) falls into this category (incision and excision of the diseased tissue, with grafting of the defect). Whether dermofasciectomy has an advantage over this remains to be proven, with this technique preserving more vital palmar skin. Extensive fasciectomy In extensive fasciectomy, perform as near complete removal of the fascia as possible. In the palm, the entire palmar aponeurosis and natatory ligament are excised. In the fingers, all cords and bands are excised. McIndoe and Beare (1958) popularized the extensive palmar fasciectomy but hematoma formation with subsequent swelling and stiffness proved insurmountable. Currently, this is seldom performed. The more aggressive the fasciectomy, the more numerous the complications, and the risk of recurrence does not diminish with more aggressive surgery. McCash (1964) solved the hematoma problem by leaving the wound to close secondarily (open palm technique). A delayed skin graft appears to be a better solution. The extensive technique is used only when the entire palm is involved. Dermofasciectomy This is a more radical approach in which the fascia and overlying skin are excised completely and full-thickness skin is grafted. This approach is recommended for recurrence and aggressive primary disease. Hueston recommends dermofasciectomy for digital disease. McFarlane criticizes this because it does not address the retrovascular cord, the unfavorable flexor sheath is left as the graft bed, and the diseased fascia can be separated from the overlying skin. Hueston feels the only difference between conservative and radical surgery is in the degree of palmar dissection; the digital dissection remains the same in either. Proximal interphalangeal joint contracture If complete extension is not present following fasciectomy, the options are to rely on splintage ( <30°) or to perform a joint release (>30°). In the latter option, the flexor sheath is incised and accessory collateral ligaments and the proximal attachment of the volar plate are released, in that order, as necessary. Most consider capsulotomy meddlesome at the time of the original operation. Amputation is rarely, if ever, necessary, since prophylactic resurfacing with skin grafts has provided permanent local control of the disease. For severe PIP joint contracture, consider a Swanson arthroplasty if the contracture is due to scar and not Dupuytren disease. The concomitant shortening results in improvement of the contracture. PIP arthrodesis is not a good option since it further limits function. Distal interphalangeal joint hyperextension Hyperextension of the distal interphalangeal (DIP) joint usually occurs secondary to long-standing PIP joint contracture. No inherent disease is present in the DIP joint. The Landsmeer ligament becomes foreshortened. If passively correctable, it usually corrects with PIP correction. If not, division of Landsmeer ligaments corrects the deformity. DIP may not flex due to dorsal knuckle pads. Incisions and wound closure The best location for the skin incision is along the line of greatest dermal involvement (flaps are then thicker at their bases). Incisions are transverse or longitudinal, depending on the type of operation. McFarlane states the following:
Technical points Use loupe magnification and sharp dissection and change blades frequently. Most wounds are closed by direct suture or Z-plasty. The Z-plasty flaps are not designed until the time of closure so that they are placed in an area where skin viability is guaranteed. A midline scar along the volar aspect of the DIP joint never contracts. Full-thickness skin graft (FTSG) from the inner arm is preferred to split-thickness skin graft (STSG) because of contraction in the latter. Transverse wounds may be left open in the manner of McCash. Dissection of the fascia off the skin may leave very thin skin flaps, particularly in the digits. The neurovascular bundle is best located distally. A midline digital incision is least likely to damage the neurovascular bundle. Dissecting retrovascular disease is important. Hueston always immobilizes in a plaster and does not release the tourniquet. The lateral stationary line of the finger is the line from which no flexion contracture can develop: grafts should be placed well back to this line. In recurrent disease, if the digital nerve has been severed by clinical testing, assume that the artery also has been severed and ensure that the contralateral artery is not damaged. A dermofasciectomy is the treatment of choice for recurrent Dupuytren disease. Begin the operation by dividing the natatory ligament of the thumb contracture and releasing by Z-plasty, lest this be forgotten later. Brachial block is preferred. POSTOPERATIVE MANAGEMENTSection 8 of 11
Postoperative management can be performed on an outpatient basis, although one must be sure that the hand is going to be elevated. Swelling, stiffness, and reflex sympathetic dystrophy (RSD) are unlikely to occur if the hand is elevated for the first 48 hours. A static splint maintains the surgical gains during the healing phase. It is removed for active and passive ROM exercises. Splintage also can be used to improve on the ROM gained at surgery. At least 3 months' splintage is necessary. Postoperative regimen
COMPLICATIONS AND RESULTSSection 9 of 11
Overall, 20% of patients develop a complication. Early complications These are surgeon dependent and include the following:
Late complications These generally reflect the Dupuytren diathesis and include the following:
Results The patient and surgeon should judge results subjectively, with function and joint ROM evaluated. MP contracture invariably is corrected (86% excellent), while good results for PIP contracture are less frequent (40% excellent result in middle, R fingers; only 20% in L finger). DIP has approximately 50% excellent results. The L finger is the most difficult to correct. MULTIMEDIASection 10 of 11
REFERENCESSection 11 of 11
Hand, Dupuytren Disease excerpt Article Last Updated: Nov 17, 2006 | |||||||||||||||||||||||||||||||||
