Dupuytren’s Contracture

Leon S. Benson, MD, Craig S. Williams, MD, and Marjorie Kahle, OTR/L

Abstract

DupuytrenÕs contracture is a fibroproliferative disorder of autosomal dominant ommended surgical release of the inheritance that most commonly affects men over age 60 who are of tightened palmar aponeurosis. He Scandinavian, Irish, or eastern European descent. Local microvessel ischemia also noted that the close proximity in the hand and specific platelet-derived and fibroblast growth factors act at the of nerves and arteries required cellular level to promote the dense myofibroblast population and altered collagen meticulous surgical technique and profiles seen in affected tissue. Surgical treatment depends to some degree on that postoperative care was facili- patient preference and a clear understanding of the possible complications and tated by use of an extension splint considerable postoperative therapy commitment. Operative management is for the fingers. appropriate when metacarpophalangeal or proximal interphalangeal joint con- One of ClineÕs apprentices was tracture exceeds 30 degrees. A volar zigzag Brunner incision in the digit and Astley Cooper (1768-1841). Cooper palm provides reliable exposure and leads to predictable healing in most cases. also described palmar fibromatosis The mainstay of postoperative hand therapy is early active-flexion range-of- prior to DupuytrenÕs formal writings motion exercises to restore grip strength. A nighttime extension splint is often (by about 10 years). In CooperÕs used for several months postoperatively to maintain the correction achieved in book, A Treatise on Dislocations and the operating room. Early recurrence of disease is most common in individuals Fractures of the Joints,2 published in with DupuytrenÕs diathesis; use of full-thickness skin grafts may be helpful for 1822, he noted that a chronic inflam- these patients. mation of the palmar aponeuro- J Am Acad Orthop Surg 1998;6:24-35 sis produces finger contractures. Cooper speculated that repetitive trauma might be a causative factor and that surgical release of con- The first description of Dupuy- esting to note, however, that care- tracted fibers should be performed trenÕs disease has been credited, ful translation of PlaterÕs original by means of a percutaneous fas- not to Guillaume Dupuytren, but Latin manuscript has been thought ciotomy. to Felix Plater of Basel, Switzerland to describe a traumatic rupture of (1536-1614).1 PlaterÕs description of the flexor pulley system. palmar fibromatosis was published Henry Cline (1750-1826), a sur- in his book cataloguing autopsy geon at LondonÕs St. Thomas Hos- Dr. Benson is Assistant Professor of Clinical findings (Observationum in Hominis pital, also contributed to the docu- Orthopaedic Surgery, Northwestern University Affectibus, published in 1614). mentation of palmar fibromatosis Medical School, Chicago. Dr. Williams is Plater described the case of a stone- before DupuytrenÕs publications. Assistant Professor of Clinical Orthopaedic Surgery, Northwestern University Medical cutter who sustained a severe trac- Most of ClineÕs work was not pub- School. Ms. Kahle is Senior Occupational tion injury to the small finger and lished formally but was recorded Therapist, Glenlake Hand Therapy, Glenview, subsequently presented with a by the note taking of his pupils. Ill. fixed flexion contracture of the During one of his lectures in 1808, digit. This case has been cited as Cline clearly described the charac- Reprint requests: Dr. Benson, Suburban the first record of DupuytrenÕs dis- teristic anatomy of the palmar Orthopaedic Associates (subdivision of Illinois Bone and Joint Institute, Ltd), Suite 300, 3633 ease, probably because PlaterÕs aponeurosis, noting that it Òsome- West Lake Avenue, Glenview, IL 60025. description focused on the progres- times becomes contracted and sive contraction of affected digits thickened.Ó2 Cline went on to Copyright 1998 by the American Academy of into the palm and the appearance describe the typical contracted Orthopaedic Surgeons. of palmar skin ridging. It is inter- appearance of the fingers and rec-

24 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al

Guillaume Dupuytren (1777- puytrenÕs disease, the myofibro- sue hypoxia.6 It is therefore postu- 1835) was one of the giants of blast is also located in palmar fas- lated that the pathogenesis of surgery for more than 30 years, cial nodules. DupuytrenÕs contracture is related from the end of the 18th century Biochemical characteristics have to local ischemia, which stimulates through the early 19th century. He also been carefully examined. A increased production of fibroblasts was a brilliant physician-scientist, a study of 10 major metabolic en- and related cell types. The fibro- captivating lecturer, and a de- zymes and 7 lysosomal enzymes of blasts tend to organize themselves manding, egocentric personality.3 fibroblasts in tissue from patients along lines of mechanical stress, While he was Chief Surgeon of the with DupuytrenÕs disease showed eventually creating typical cord- H™tel Dieu in Paris, he published a a higher total activity level com- type arrangements of palmar fas- description of two patients with pared with those in normal tissue.5 cia. All of the subsequent physical palmar fibromatosis in 1834.1 However, when the higher density and chemical characteristics of DupuytrenÕs writings reiterated of cells in the diseased tissue was DupuytrenÕs tissue are a direct many points of those before him, taken into account, the enzyme result of this increased population including CooperÕs theory that the activity per cell was not different of fibroblasts. The exact mecha- contracture was a result of overuse from that in normal tissue. nism of how ischemia stimulates or trauma. DupuytrenÕs name like- Collagen chemistry research has fibroblast production has been ly became affixed to the disease for shown that DupuytrenÕs fascia con- tied to the production of oxygen a variety of reasons, not the least of tains much higher concentrations free radicals that occurs in hypoxic which was his stature as the most of type III collagen, a higher ratio tissue. famous surgeon in Europe at the of type III collagen to type I colla- Local microvessel ischemia can time. DupuytrenÕs publications gen, increased amounts of total col- be the result of genetic, age, gen- described not only the anatomic lagen, and increased lysyl oxidase der, or environmental factors, pathology but also the clinical pre- activity.4 However, subsequent many of which are already known sentation, natural history, surgical studies that took into account the to be associated with the presenta- technique, postoperative care, increased cell density of the tissue tion of DupuytrenÕs disease. For response to treatment, and long- demonstrated that the pattern of example, alcohol consumption, term follow-up. collagen production could be relat- cigarette smoking, and human ed almost entirely to increased den- immunodeficiency virus infection sity of cells and not to any specific are all associated with Dupuy- Basic Science intrinsic biochemical behavior of trenÕs disease in varying degrees. an ÒabnormalÓ fibroblast.4 Even All of these conditions may be Although it was once thought that the higher concentration of gly- linked to DupuytrenÕs contracture a particular cell type was uniquely cosaminoglycans found in the col- through their ability to increase responsible for DupuytrenÕs dis- lagen has been shown to be propor- production of oxygen free radicals. ease, it is now clear that the two tional to the increased number of A self-perpetuating cycle can key cell types present in the dis- cells present.4 develop, as fibroblast strangula- eased fascia, the fibroblast and the The high cellularity seen in tion of microvasculature further myofibroblast, are not unique to DupuytrenÕs tissue may be caused increases local ischemia, which this condition. The myofibroblast by local ischemia at a microvascu- then additionally increases fibro- has received particular attention as lar level. Most of the abundant blast production and elevates the possibly the histologic hallmark of fibroblasts in DupuytrenÕs tissue concentration of oxygen free radi- DupuytrenÕs contracture; however, are clustered around partially or cals (Fig. 1). detailed electron-microscopic stud- totally occluded microvessels. A Myofibroblast proliferation has ies have shown that these myofi- similar pattern of fibroblast aggre- been shown to be responsive to broblasts, which share characteris- gation has been identified in the specific protein factors that operate tics of both typical fibroblasts and local ischemia seen in diabetic at a cellular level.7 Platelet-derived smooth muscle cells, are not ultra- patients.4 Furthermore, a study growth factor and basic fibroblast structurally different from those comparing lipid composition in growth factor have been shown to identified in normal tissues else- affected hands with that in normal stimulate cell growth.7-9 A third where in the body, such as the hands showed that the fascia in protein, transforming growth fac- umbilical cord, small intestine villi, the former displayed a lipid pro- tor B, has been shown to be a pow- and pulmonary alveoli.4 In Du- file consistent with mild local tis- erful stimulator of collagen produc-

Vol 6, No 1, January/February 1998 25 Dupuytren’s Contracture

Anatomy - O2 ATP • An understanding of the normal fascial organization of the hand will aid the clinician in unraveling the seemingly chaotic pathoanat- Hypoxanthine omy of DupuytrenÕs contracture. O Normal fascial structures are XD®XO 2 O - 2 referred to as bands and ligaments. H O 2 2 The two major forms of diseased Ischemia ¯ OH- Stimulation tissue are referred to as nodules Alcohol of fibroblast and cords. proliferation Uric acid The palmar aponeurosis is typi- cally a triangular thin sheet of fascial tissue that becomes more discretely organized distally into Òpretendi- nous bandsÓ that travel toward each digit. Some pretendinous band Narrowed microvessels fibers terminate in skin just distal to Factors: ¥ Age the distal palmar crease. However, ¥ Genetics a portion of the band continues on ¥ Environment and immediately bifurcates into two ¥ Gender strips that then pass deeper into the ¯ Collagen type I ® palm to wrap around both sides of • type IIIÐtype I ratio the metacarpal head, twisting 90 degrees in the process, partially In times of stress attaching to the metacarpopha- langeal (MCP) joint capsule, and Fig. 1 Schematic depicting MurrellÕs theory of the pathogenesis of DupuytrenÕs contrac- ture, illustrating the role that local microvascular ischemia plays in stimulating fibroblast blending into the web-space archi- proliferation.4 ATP = adenosine triphosphate; XD = xanthine dehydrogenase; XO = xan- tecture.13 These deeper twisting thine oxidase. extensions of the pretendinous bands are called the spiral bands. The pretendinous bands also give tion.10 It has been proposed that tion.11 Nodules produce digital rise to discrete fascial extensions DupuytrenÕs cells are more sensi- flexion contractures by pulling that travel sagittally in the palm and tive to the effects of these cellular through cords that have extended attach to the interosseous muscle modulators and that these agents past adjacent joints. Myofibro- fascia. These extensions, called the play a key role in initiation and blasts have been identified in der- septa of Legueu and Juvara, create progression of disease.7 mal and epidermal tissue that is seven compartments, which contain Diseased tissue presents in two close to, but separate from, dis- four sets of digital flexor tendons discrete forms, either as a nodule crete nodular foci.12 Recurrence of and three groups of neurovascular or as a cord. Each form has dis- disease after surgery may be structures (traveling toward the tinctive histologic features. Nod- attributable to residual myofibrob- three ulnarmost digital web spac- ules are dense cellular collections last populations that have migrat- es)13 (Fig. 2). of myofibroblasts, representing ed into the adjacent dermis and The superficial transverse pal- centers of intense metabolic activi- epidermis of the palm. The use of mar ligament is primarily a two- ty.11 DupuytrenÕs cords contain full-thickness skin grafts in ad- dimensional fascial structure in the no myofibroblasts but are highly vanced cases greatly diminishes coronal plane that runs approxi- organized collagen structures sim- the rate of recurrence, perhaps mately perpendicular to the pre- ilar to tendons. Electron-micro- because removal of both nodular tendinous bands across the distal scopic studies have demonstrated tissue and adjacent skin more third of the palm. The natatory lig- that the myofibroblast in the nod- thoroughly reduces myofibroblast ament is also a transversely orient- ule accounts for active contrac- populations. ed structure that runs roughly par-

26 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al

the skin and fascia that create the web space. Palmar aponeurosis The web space is therefore a coa- lescence of fibers from the natatory ligaments, the spiral bands, and the septa of Legueu and Juvara (Fig. 3, A). Distal to the web space, fibers continue to create a fascial struc- ture known as the lateral digital sheet, which courses in the sagittal Deep transverse metacarpal ligament plane on either side of each finger. As the lateral digital sheet passes distally in the finger, it sends off Lumbrical Septa of Legueu and Juvara fibers that attach to the periosteum, the joint capsule, and the tendon Fig. 2 The seven compartments created by the septa of Legueu and Juvara. sheath. The fibers that pass volar to the neurovascular bundle are referred to as GraysonÕs ligaments; those passing dorsal to the neu- allel to the superficial transverse into soft-tissue attachments around rovascular bundle are termed palmar ligament and travels be- the MCP joints.13 The natatory lig- ClelandÕs ligaments (Fig. 3, B). tween the web spaces. However, aments have some deep fibers that As DupuytrenÕs disease devel- the natatory ligaments, like the spi- attach to the flexor tendon sheath ops, pathologic nodules tend to ral bands, have extensions that at the MCP joint; other fibers travel form in fatty zones, particularly be- travel in three planes and blend in the coronal plane to blend into tween the MCP and proximal inter-

GraysonÕs ligament ClelandÕs ligament

Neurovascular bundle

Lateral digital sheath Web-space coalescence Natatory ligament

Natatory ligament Common digital Spiral band Spiral artery bands Pretendinous band Common digital artery Superficial Septa of Legueu Common digital transverse and Juvara Digital nerves nerve palmar ligament

A B

Fig. 3 A, The web-space coalescence, consisting of the natatory ligament, the spiral bands, and the septa of Legueu and Juvara. B, The fascial structures in the digit and web space, including GraysonÕs and ClelandÕs ligaments.

Vol 6, No 1, January/February 1998 27 Dupuytren’s Contracture phalangeal (PIP) flexion creases. blend together as an entire longitu- and severity of disease may be The nodules contain myofibroblasts dinal unit that wraps completely related to variable penetrance of and are believed to be responsible around the neurovascular bundle as gene expression. Certain popula- for the contractile behavior of it travels from the palm through the tions show a particularly high DupuytrenÕs tissue. Controversy web space and into the digit. This prevalence of disease. The greatest exists as to whether nodules give explains why DupuytrenÕs tissue so concentrations of patients with rise to cords or whether nodule for- predictably encompasses the digital DupuytrenÕs disease are located in mation and cord formation proceed nerves and requires great care in its Scandinavia and Great Britain simultaneously and independently. removal. As the diseased cords (especially Ireland and Scotland).14 In either case, pathologic cord contract, the overall encircling path- Viking heritage seems to be part of structures form along the pathways way becomes more linear (produc- the original gene pool, not only of normal fascial anatomy. For ing a flexion contracture of adjacent because of the high Scandinavian example, pretendinous bands joints), while the entrapped neu- prevalence of the disease, but also become pretendinous cords, and rovascular bundle, which normally because the path of Viking con- spiral bands become spiral cords. travels in a straight line, becomes quests correlates with its world dis- There tends to emerge a blending of spiraled around the DupuytrenÕs tribution. Viking invasions includ- tissue that allows the pretendinous cord and is drawn toward the mid- ed campaigns through Iceland to cords, spiral cords, web-space tis- line of the digit (Fig. 4). Canada, along the western Euro- sue, and the lateral digital sheet to pean coast to the British Isles, and coalesce in a continuous cord. into southeastern Europe. Dupuy- Furthermore, DupuytrenÕs disease Clinical Features trenÕs disease becomes rarer as one does not present as a simple longi- travels farther south in Europe. tudinally oriented rope of collagen Demographics The high prevalence of disease in in the coronal plane, but follows the DupuytrenÕs disease is inherited Australia is almost certainly related three-dimensional path of the vari- as an autosomal dominant trait. to the influx of British and Irish set- ous fascial extensions in the hand. Factors such as age at presentation tlers early in its development.14 DupuytrenÕs cords are also usually anchored firmly in the sagittal plane by their attachments to the flexor tendon sheath, joint capsule, interosseous fascia, periosteum, and skin. Because of the various fascial attachments, the neurovascular GraysonÕs ligament (D) bundle in an involved digit or web zone becomes predictably and pro- D foundly intertwined with diseased Lateral digital sheet (C) Spiral cord = B-D tissue. For example, the pretendi- nous band normally is located volar Natatory and central with respect to the neu- ligament C rovascular bundle in the palm. As the spiral band proceeds distally, its fibers assume a position dorsal and B peripheral to the neurovascular Spiral band (B) bundle. The next fascial extension, Common digital nerve the lateral digital sheet, remains A peripheral and dorsal to the nerve until it gives rise to GraysonÕs liga- Pretendinous band (A) ment, which travels volar to the nerve to connect centrally with the Fig. 4 Left, Normal anatomy. Right, Formation of a spiral cord from four fascial struc- phalangeal periosteum and tendon tures: the pretendinous band, the spiral band, the lateral digital sheet, and GraysonÕs liga- sheath. When these fascial struc- ment. The involved digital nerve becomes spiraled around the cord, and the nerve is tures become diseased cords, they drawn toward the midline of the digit.

28 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al

Areas in which the condition is vir- it often has extensions that fan out those with DupuytrenÕs diathesis. tually unknown include Greece, toward multiple digits. Recurrence can be defined as the the Middle East, and the Orient. Progression of disease is unpre- presence of diseased tissue in a sur- dictable. Some patients may have gically treated field. These lesions Patient Presentation minimal findings that are quiescent may represent new foci of Dupuy- DupuytrenÕs disease is much for years and then demonstrate trenÕs disease that have, over time, more common in men than in increased nodule formation and progressed from an adjacent dis- women (by a ratio of at least 7:1) cord extension over a short period. eased zone that has not been treat- and commonly presents after age It has been speculated that trauma ed surgically. This may be the 40.15 Patients often first seek med- may be a stimulus for progression most common situation in diathesis ical attention after they notice a ten- in some cases, but there has been patients, who demonstrate aggres- der nodule or progressive palmar no formal scientific evidence sup- sive and extensive disease; in these cord development. In many cases, porting overuse trauma as a cause. patients, diseased tissue from the process is completely painless, ÒDupuytrenÕs diathesisÓ refers untreated areas is likely to extend and patients will delay seeking to a spectrum of physical findings into and reinvolve surgically treat- evaluation until joint motion has that is present in patients with par- ed territory. been seriously compromised. ticularly strong gene expression for It is also possible that recurrent Patients are frequently referred for the disease. These patients present contracture may be due to the pres- orthopaedic assessment by their several decades earlier in life than ence of areas of residual disease primary-care physician with a pre- those with DupuytrenÕs diease that were incompletely excised, sumptive diagnosis of trigger finger itself (often in their 20s or 30s) and with myofibroblasts persisting in or joint stiffness. Although any demonstrate very aggressive cord the skin or dermis. It has been soft-tissue mass must be carefully development with a high incidence observed that areas treated with assessed as a possible tumor, skin of multiple-digit and bilateral hand full-thickness skin grafts rarely pitting and nodule formation near involvement. Additional physical develop recurrent disease, perhaps the distal palmar crease are typical findings include knuckle pads (also due to the removal of all tissue lay- early findings that help confirm the called GarrodÕs nodes), plantar ers adjacent to the nodular foci diagnosis of DupuytrenÕs involve- fibromatosis (LederhoseÕs disease), (dermis and epidermis). Whether ment. Examination of the opposite and penile fascial involvement recurrence is truly due to disease hand may help confirm the diagno- (PeyronieÕs disease). Although progression or disease persistence, sis, as nodule formation and other these three findings rarely require patients should be aware that joint early changes may be present bilat- specific surgical intervention, they contractures may recur after surgi- erally. can serve to identify patients with cal treatment, especially in young As nodules grow, cords can usu- DupuytrenÕs diathesis. Diathesis patients and those with a strong ally be palpated extending proxi- patients are at a much higher risk diathesis. mally and distally. The ring and for a poor surgical outcome due to small finger are usually the first higher early recurrence rates, digits involved, and disease in the greater risk of technical complica- Nonoperative Treatment palm typically precedes digital tions due to complex disease in- involvement. In women, disease volvement, and longer postopera- Reports of treatment of Dupuy- expression seems to be more often tive rehabilitation. trenÕs contracture with a wide vari- less severe or atypical; isolated PIP ety of nonoperative modalities, joint involvement or first-web- Disease Recurrence including creams, lotions, cortico- space contractures may be the main It is important to remember that steroid injections, physical therapy, clinical finding. Nodule and cord DupuytrenÕs disease is controlled and splinting, are largely anecdo- maturation often follow different at the genome level; surgical exci- tal. No consistently favorable re- courses. Although the nodule sion of affected tissue does not sponse has been documented in a forms first and is a major center of effect a true cure from a histologic large group of patients who have biologic activity, the nodules may perspective, but rather improves been followed up prospectively. disappear as the disease progress- hand function by alleviating con- The course of disease progression es. Cord development is the more tractures. Recurrence of disease is is notoriously unpredictable, and striking physical finding. As the more common in patients who are the literature is replete with isolat- cord thickens and extends distally, young at first presentation and in ed reports of spontaneous regres-

Vol 6, No 1, January/February 1998 29 Dupuytren’s Contracture sion of contractures, further casting surgical intervention, patient pref- trenÕs contracture. Most surgeons doubt on a cause-and-effect rela- erence also plays a role in treat- tend to select one method over tionship between nonoperative ment choice, especially since another on the basis of their train- treatments and favorable response. DupuytrenÕs disease is often pain- ing, experience, and philosophical Perhaps the most valid nonop- less and only slowly progressive. approach to the disease. The vari- erative treatment consists of educa- Patients must be educated about ous procedures differ in three tion of both patients and primary- potential postoperative complica- ways: management of the palmar care givers. Frequently, patients tions, the required commitment to fascia, treatment of the volar skin, with early disease or mild contrac- postoperative therapy, the degree and design of incisions. tures will present with fears or of risk of recurrence, and the possi- misconceptions regarding the eti- ble need for additional procedures, Management of the Palmar ology of the nodules and cords in such as skin grafting. Good com- Fascia their palms. This situation can be munication between patient and Methods of treatment for the compounded by primary-care surgeon is essential so that appro- palmar fascia include radical physicians who do not recognize priate expectations are created. fasciectomy, selective fasciectomy, the disease process as DupuytrenÕs The PIP joint contracture war- segmental fasciectomy, and fas- contracture. Patients are often re- rants special consideration. Because ciotomy. With the exception of assured when the nature of the complete correction of a PIP joint radical fasciectomy, each method condition is explained to them, and flexion deformity is difficult to has staunch proponents, and all they may also be educated as to achieve and maintain, operative probably have a role for selected what signs warrant reevaluation of intervention has generally been rec- patients. the affected hand. Education of ommended when any PIP joint con- Once a mainstay of DupuytrenÕs primary-care physicians will like- tracture is noted. However, a recent surgery, radical fasciectomy has wise help ensure that referral is multicenter study16 demonstrated largely been abandoned. This recommended before severe con- that while the surgical outcome for approach grew out of the philoso- tractures become established. severe PIP joint contractures was phy that resection of all the palmar notable improvement, the outcome fascia would prevent disease recur- for milder contractures (less than 30 rence and extension. However, Operative Indications degrees) was frequently either no recurrence was still reported, and improvement or worsening of the the extensive nature of the proce- The Òtable topÓ test provides a contracture. McFarlane17 advocates dure led to an unacceptable rate of good guideline for consideration of DupuytrenÕs contracture release wound complications. operative intervention. The test is only when the PIP joint contracture Selective fasciectomy is the positive when the patient can no is 30 degrees or more. method most commonly used. longer place the palm of the affect- It is important to distinguish a This procedure involves the resec- ed hand completely flat on a hard true PIP joint flexion contracture tion of all diseased fascia in the surface. This test serves as a sim- from an apparent one. A spiral palm and finger; adjacent normal ple reference by which patients can cord that originates proximal to the fascia is left. Because some dis- check for themselves the progres- MCP joint and terminates distal to eased fascia (which macroscopical- sion or significance of a contrac- the PIP joint has the potential to ly appears normal) will inevitably ture. The test typically correlates produce flexion of both joints. remain, disease may develop in with MCP joint contractures that Therefore, MCP joint contractures areas not treated surgically. measure more than 30 to 40 should be measured with the PIP Segmental fasciectomy and fas- degrees. An MCP joint contracture joint passively extended, and PIP ciotomy are limited procedures of 40 degrees or more is severe joint contractures should be mea- that achieve a partial or complete enough to justify operative inter- sured with the MCP joint held in correction. Segmental fasciectomy vention in most cases. Treatment flexion. is the removal of one or more seg- of other affected digits on the same ments of diseased fascia through hand should be considered when multiple small incisions in the their MCP joint contractures are 20 Operative Treatment palm and fingers. Alternatively, it to 30 degrees or more. can be performed through trans- While these guidelines are help- There are several operative proce- verse incisions or longitudinal inci- ful in identifying a threshold for dures for the correction of Dupuy- sions converted to Z-plasties.

30 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al

Segmental fasciectomy may also be skin has been replaced with full- is rare with the open-palm tech- combined with multiple small full- thickness skin grafts. Although nique. The major disadvantage to thickness skin grafts overlying the control of recurrence is a worthy the open-palm technique is the areas of cord excision, with the goal, it must be recognized that use inconvenience to the patient during intent that these grafts will prevent of skin grafts does not control the 3 to 5 weeks it may take for the recurrent disease beneath them. extension of disease beyond the wound to close. Limited open fasciotomy and per- grafted areas and that recurrence is cutaneous fasciotomy have been possible at the margins of the graft. Incisions used to obtain modest correction in Potential disadvantages of skin Fasciotomy and segmental less severe contractures or partial grafting include complete or partial fasciectomy may be performed per- correction of severe contractures in graft loss; hematoma formation; cutaneously or through transverse debilitated or very elderly patients prolonged immobilization for graft incisions, longitudinal incisions and in patients unable to comply incorporation, possibly resulting in converted to Z-plasties, or small with the postoperative rehabilita- stiffness; altered sensibility over curvilinear incisions. The various tion protocol. the grafted areas; and altered wear incision types may be used in com- Selective fasciectomy is prefer- characteristics of the grafted skin bination. Three types of incisions able for most patients because this compared with normal palmar are commonly used to provide technique provides the best correc- skin. wide exposure to the diseased fas- tion with acceptable rehabilitation An alternative to direct wound cia: a longitudinal midline incision requirements and complications. closure or skin grafting is the open- with Z-plasty closure, a Brunner For very elderly patients and wound technique, advocated by zigzag incision, and a zigzag inci- patients who cannot comply with McCash19 in 1964. The technique sion with V-Y advancement. therapy, fasciotomy is preferred. most frequently involves a trans- In a finger with a marked con- verse incision in the palm at the tracture, a longitudinal incision in Management of the Volar Skin level of the midpalmar crease com- the palm extending into the digit in Three methods of treatment are bined with additional incisions in its midaxial line provides excellent most commonly used: direct clo- the fingers to complete the fasciec- exposure and is simple to perform sure after fascial excision, skin exci- tomy. At the conclusion of the pro- compared with other incisions. sion followed by full-thickness skin cedure, the transverse incision, Although the digital nerve is grafting, and an open technique in which will be gapped open, is cov- drawn proximally, centrally, and which a portion of the volar skin is ered with a nonadherent dressing palmarly by a contracted cord, it is left open to close subsequently by and left open. Once motion is initi- rarely drawn completely to the wound contraction. ated, the wound is simply covered midline of the digit; therefore, a Advantages of direct closure with a dry dressing, which is longitudinal midline incision is rel- with or without skin-flap rear- changed daily. The wound will atively safe. When the fasciectomy rangement include primary wound gradually heal over a period of sev- is completed, the longitudinal inci- healing, no need for skin grafts, eral weeks. Interestingly, Lubahn sion is converted to multiple Z- and simple postoperative wound et al20 demonstrated that the open plasties, optimally placed over the management. Disadvantages of wound does not heal by granula- flexion creases, and the flaps are this technique are increased inci- tion and epithelialization; rather, closed. This provides the addition- dence of hematoma formation and the transverse wound contracts to al benefit of lengthening the skin-flap necrosis and a possible its precontracture length. Other wound. The flaps may be planned need for skin-flap rearrangements portions of the wound may also be but should not be made before to provide length. left open as long as their orienta- completion of the fasciectomy. The use of full-thickness skin tion is transverse, not longitudinal. This allows creation of flaps that grafts in the palm and digits has There are two significant advan- are not compromised by the dissec- many proponents. Hueston18 has tages to the use of an open-palm tion. championed this approach, believ- technique. The first is a lower com- The use of Brunner incisions and ing that the palmar skin has a mod- plication rate, specifically with re- the use of zigzag incisions with V- ulating effect on the underlying gard to the development of hema- Y flaps are similar except that the palmar fascia. This belief is sup- toma and wound-edge necrosis. latter provides some lengthening of ported by evidence that recurrence The second is early postoperative the wound. Both types of incisions is rare beneath areas where palmar patient comfort. Wound infection provide excellent exposure.

Vol 6, No 1, January/February 1998 31 Dupuytren’s Contracture

For all incision types, flaps must contracture, especially with regard predictor in gaining improvement be raised with great care, and flap to outcome. How much correction was compliance with the postoper- retraction must be performed gently. should be sought and how aggres- ative splinting program. It can be helpful to mark the skin sively joint release should be pur- Although it is preferable to at- first to ensure precise placement of sued remain controversial. Clearly, tempt release of the PIP joint in the incision with respect to digital whatever correction can be achieved cases of severe contracture, it is flexion creases and to ensure flap by resection of diseased fascia crucial that the patient be informed symmetry. To avoid flap necrosis, should be accomplished. Beyond preoperatively that maintenance of the base of the flap must be protect- this, Hueston18 advocates no further improvement is difficult and labor- ed from compromise due to dissec- release for a contracture of 40 intensive. Near-normal PIP joint tion or rough retraction. The apex of degrees or less. This is predicated motion is a highly unlikely result, each flap is often quite thin and ten- on his belief that hyperextension at and the use of dynamic splinting uous and should also be protected the MCP joint can accommodate for a prolonged period postopera- against vigorous manipulation. this degree of contracture and allow tively may be the most important Nylon retention sutures (5-0 or 6-0) the finger to be extended to a pal- detail in maintaining operative are helpful for retracting flaps; the marly flat position. Furthermore, correction. Furthermore, it is sutures should be placed completely resection or opening of the flexor essential to recognize that release in the subcuticular layer so that if sheath may compromise the ability of the PIP joint adds additional they inadvertently pull out, the epi- of a full-thickness skin graft to be insult to the digit and increases the dermis will not tear. Regardless of easily incorporated. chance of complications related to incision type, the digital neurovas- Others have recommended that dramatic swelling, skin-flap com- cular bundle will require exposure complete PIP joint extension be promise, and prolonged postoper- to adequately protect it as well as to obtained in the operating room. ative pain. The exposure required free it from diseased tissue. However, although obtaining full for PIP joint release requires viola- A Brunner incision is preferred correction may be appealing in tion of the flexor-tendon pulley for most patients. This incision is theory, there is evidence to suggest system, which adds the potential simple to plan, is reliable in healing that PIP joint release cannot be eas- complication of altering flexor-ten- and appearance, and usually ily maintained postoperatively. don mechanics. requires no modification in the Rives et al21 prospectively evaluat- In summary, careful patient patient with mild to moderate ed 23 PIP joints treated with surgi- selection and great technical focus involvement. If the contracture is cal release for a DupuytrenÕs con- are essential when considering PIP particularly severe in the digit, part tracture of 45 degrees or more. joint release in patients with severe of the incision can be left open, and The operative procedure included contracture. Not every patient is a full-thickness skin grafts can be volar joint capsulotomy, release of candidate; the ability to understand used to fill in the gaps. For patients the checkrein ligaments, and potential complications and to par- who have particularly severe release of accessory collateral liga- ticipate in a meticulous dynamic involvement in the palm, the pal- ments. Postoperative management splinting program is critical. mar portion of the Brunner incision consisted of dynamic extension Exposure of the flexor tendons and can be substituted for a transverse splinting for 6 months. At the 2- release of the PIP joint adds a palmar approach; the incision is year follow-up, only a 44% im- marked recovery burden that nota- then left open and managed with provement in PIP joint extension bly increases surgical morbidity. the open-palm technique. Al- was present (59% in the subgroup Furthermore, and most important, though the open-palm technique of patients who complied with the loss of PIP joint flexion and grip certainly has a low wound-compli- postoperative splinting program). strength can result and ultimately cation rate and the wound heals They noted the same degree of cor- make the digit and hand perma- well, some patients prefer not to rection for moderately contracted nently worse than the preoperative manage an open wound. (45 to 60 degrees) and severely state. contracted (60 to 100 degrees) PIP Joint joints. This experience under- Recurrent Disease and Salvage The PIP joint deserves special scores the difficulty of improving Procedures consideration. It undoubtedly con- severe PIP joint contractures, even Recurrent contracture severe stitutes the most difficult aspect of when aggressive surgical release is enough to warrant reoperation con- surgical treatment of DupuytrenÕs performed. The most important stitutes a formidable surgical chal-

32 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al lenge. Selective fasciectomy fol- course and the time required in a use of the hand. Nighttime exten- lowed by excision and grafting of formal therapy program. sion splinting should be continued all of the palmar skin overlying the Therapy begins 2 to 5 days post- for 3 to 6 months, with periodic recurrent contracture is a technique operatively with fabrication of a checks to ensure proper position- advocated by many. Unlike prima- volar forearm-based splint with the ing. ry DupuytrenÕs surgery, the anato- wrist in neutral and the fingers Scar-management techniques, my is not generally predictable, extended as much as possible. The such as massage and use of silicone and the risk of complications, thumb is splinted in extension to gel, can be helpful in optimizing including neurovascular injury, is minimize web-space contracture. the appearance and texture of the greater. Previous surgery may A forearm-based two-thirds splint scar. Strengthening is initiated have resulted in occult injury to a is recommended instead of a hand- after wounds are healed, usually 3 digital artery, and injury to the based splint because the former to 4 weeks after primary closure, 4 remaining artery during the course provides a longer lever arm for sta- weeks after skin grafting, and 6 of reoperation may have dire con- bility of finger extension. weeks after the open-palm tech- sequences. A preoperative digital The frequency and duration of nique. Allen test is helpful in identifying splint usage are dictated by the digits with impaired circulation. extent of the surgical procedure In some cases of severe recurrent and the propensity of the patient to Complications contracture or even severe primary lose motion. Not all patients will disease, PIP joint fusion or amputa- require, or even benefit from, The complications of DupuytrenÕs tion may be a reasonable alterna- splinting. Although extension surgery are numerous and may be tive. Joint fusion usually requires splinting is commonly emphasized related to underestimation of the partial contracture release and bone in an effort to maintain the correc- complexity of the procedure or shortening. Candidates for PIP tion achieved surgically, it is im- unfamiliarity with the requirements joint fusion include patients with portant to immediately start active of postoperative care. The most severe flexion contracture (more range-of-motion exercises for both obvious intraoperative pitfall is than 90 degrees), recurrent disease, flexion and extension. It is possible inadvertent division of a digital PIP joint arthritis, and inability or to inadvertently concentrate on nerve. A thorough understanding unwillingness to comply with the extension posture while allowing of DupuytrenÕs anatomy is the best required postoperative therapy. the patient to actually lose motion defense against this problem, Indications for amputation include in flexion and subsequently suffer although in patients with severe severe recurrent contracture, a PIP permanent loss of grip strength contracture (especially those with joint flexion contracture greater and terminal digital flexion. The DupuytrenÕs diathesis), the pathway than 90 degrees, a dysvascular essential ingredient of the postop- of the digital nerve can be madden- digit, a painful or insensate digit, erative program is early active ingly complex. Loupe magnifica- and patient preference. motion and restoration of grip. tion and great patience are essential Active range-of-motion exercises to safe unraveling of the nerve. are initiated at the patientÕs first The preferred technique begins Postoperative Treatment therapy visit. Passive stretching is with identification of the neurovas- also performed according to the cular bundle in the palm, just distal The goals of postoperative treat- patientÕs pain tolerance. Particular to the superficial transverse palmar ment are to maintain the correction attention is directed to the PIP joint ligament. Each nerve is then dis- achieved by surgery, to reduce the because collateral ligament tight- sected distally, taking care never to effects of postoperative edema and ness and capsular contraction must cut any structure without having scarring, and to restore preopera- be overcome. Joint-blocking exer- the nerve and the artery clearly in tive flexion and strength. Manage- cises are often required to regain view and protected. An optical ment involves splinting, active and distal interphalangeal joint flexion, illusion frequently occurs in which passive range-of-motion exercises, especially if hyperextension of the the nerve appears to just stop in a wound care, scar and edema man- joint was present before surgery. mass of diseased fascia; in reality, agement, education, and strength- Usually by the second postopera- the nerve has usually traveled ening. The patientÕs compliance tive week the patient can be gradu- deeper into the fascia, turning 90 with a home exercise program ally weaned from splinting during degrees away from the surgeonÕs determines the postoperative the daytime to facilitate functional visual perspective and giving the

Vol 6, No 1, January/February 1998 33 Dupuytren’s Contracture appearance of a dead end. Another sary to accept less deformity cor- disease and extension of disease, a important rule is not to excise any rection in favor of allowing ade- few principles are clear. Disease tissue until the digital nerve has quate blood flow to the affected recurrence rates are much higher in been identified on both sides of the finger. patients with DupuytrenÕs diathe- excision zone. Once the digital Compromise may be related to sis; use of full-thickness skin grafts nerves have been completely dis- the patientÕs inability to comply seems to protect against the return sected free of diseased fascia, exci- with therapy or to care for the heal- of diseased fascia in grafted areas. sion of the DupuytrenÕs tissue can ing incisions. The goals of therapy be quickly and easily accomplished. may need to be suspended tem- Hematoma formation is another porarily in deference to wound Summary common technical problem. Re- care. It can be helpful to identify lease of the tourniquet before those patients less likely to tolerate DupuytrenÕs contracture is a fibro- wound closure is advisable so that rigorous therapy or wound care proliferative disorder that most meticulous hemostasis can be estab- and perhaps limit their surgical commonly affects men over age 60 lished. Hematoma formation can intervention to the smaller, less who are of Scandinavian, Irish, or easily produce wound necrosis demanding incisions of a segmen- eastern European descent. Opera- because the skin flaps are often thin. tal fasciotomy. tive management is appropriate Wound healing difficulties can Reflex sympathetic dystrophy in when metacarpophalangeal or result from poorly planned inci- DupuytrenÕs patients has been proximal interphalangeal joint con- sions or imprecise technique. Care referred to as a ÓflareÓ reaction. Its tracture exceeds 30 degrees. How- must be taken when designing zig- prevalence varies from 1% to 8%, ever, the choice of surgical treat- zag incisions so that all flaps have although in one series22 a rate of ment is dependent to some extent adequate blood supply and web- 58% was noted in patients who on patient preference and a clear space skin is not compromised underwent simultaneous carpal understanding of the possible com- when surgery involves adjacent tunnel release. It is at least twice as plications and the considerable digits. DupuytrenÕs disease may common in female patients with postoperative therapy commit- directly involve the skin, most obvi- DupuytrenÕs disease as in male ment. A volar zigzag Brunner inci- ously in the case of nodules and pit- patients. As is the case with any sion in the digit and palm provides ting, and it is easy to inadvertently surgical patient, a reflex sympa- reliable exposure and leads to pre- buttonhole the skin when attempt- thetic dystrophy event can ruin the dictable healing in most cases. The ing to elevate a flap. Small defects results of treatment. Optimal man- mainstay of postoperative hand typically do not result in healing agement involves early recognition therapy is early active-flexion problems; it is preferable to leave and immediate treatment with range-of-motion exercises to them open if any tension is created hand therapy and adjunctive restore grip strength. A nighttime by attempting to suture the defect. modalities, such as nerve blocks extension splint may be used for Vascular compromise of a digit and medication when appropriate. several months postoperatively to is possible, especially in patients Although not truly a complica- maintain the correction achieved in who have undergone previous tion of treatment, recurrence defi- the operating room. Early recur- surgery. In any case of severe con- nitely spoils the result of surgical rence of disease is most common in tracture, it is wise to carefully intervention. Reported recurrence individuals with DupuytrenÕs examine the vascularity of surgical- rates vary from 2% to 63%.22 Al- diathesis; use of full-thickness skin ly treated digits when the tourni- though controversy surrounds the grafts may be helpful in this set- quet is released. It may be neces- distinction between recurrence of ting.

References

1. Verheyden CN: The history of DupuytrenÕs Disease: Biology and 4. Murrell GAC: The role of the fibro- DupuytrenÕs contracture. Clin Plast Treatment. Edinburgh: Churchill blast in DupuytrenÕs contracture. Surg 1983;10:619-625. Livingstone, 1990, pp 1-9. Hand Clin 1991;7:669-681. 2. Elliot D: The early history of contrac- 3. Boyes JH: On the Shoulders of Giants: 5. DelbrŸck A, Reimers E, Schšnborn I: ture of the palmar fascia, in McFarlane Notable Names in Hand Surgery. Phil- A comparative study of the activity of RM, McGrouther DA, Flint MH (eds): adelphia: JB Lippincott, 1976, pp 30-37. lysosomal and main metabolic path-

34 Journal of the American Academy of Orthopaedic Surgeons Leon S. Benson, MD, et al

way enzymes in tissue biopsies and roles in DupuytrenÕs contracture. J Operative Hand Surgery, 3rd ed. New cultured fibroblasts from DupuytrenÕs Hand Surg [Am] 1995;20:101-108. York: Churchill Livingstone, 1993, vol disease and palmar fascia: On the 11. Rudolph R, Vande Berg J: The myofi- 1, pp 563-591. pathobiochemistry of connective tissue broblast in DupuytrenÕs contracture. 18. Hueston JT: DupuytrenÕs contracture, proliferationÑI. J Clin Chem Clin Hand Clin 1991;7:683-694. in Jupiter JB (ed): FlynnÕs Hand Biochem 1981;19:931-941. 12. McCann BG, Logan A, Belcher H, Surgery, 4th ed. Baltimore: Williams & 6. Rabinowitz JL, Ostermann AL Jr, Bora Warn A, Warn RM: The presence of Wilkins, 1991, pp 864-889. FW, Staeffen J: Lipid composition and myofibroblasts in the dermis of 19. McCash CR: The open palm technique de novo lipid biosynthesis of human patients with DupuytrenÕs contracture: in DupuytrenÕs contracture. Br J Plast palmar fat in DupuytrenÕs disease. A possible source for recurrence. J Surg 1964;17:271-280. Lipids 1983;18:371-374. Hand Surg [Br] 1993;18:656-661. 20. Lubahn JD, Lister GD, Wolfe T: 7. Alioto RJ, Rosier RN, Burton RI, Puzas 13. Strickland JW, Leibovic SJ: Anatomy Fasciectomy and DupuytrenÕs disease: JE: Comparative effects of growth fac- and pathogenesis of the digital cords A comparison between the open-palm tors on fibroblasts of DupuytrenÕs tis- and nodules. Hand Clin 1991;7:645- technique and wound closure. J Hand sue and normal palmar fascia. J Hand 657, 659-660. Surg [Am] 1984;9:53-58. Surg [Am] 1994;19:442-452. 14. Hueston JT, Seyfer AE: Some medi- 21. Rives K, Gelberman R, Smith B, 8. Badalamente MA, Hurst LC, Grandia colegal aspects of DupuytrenÕs con- Carney K: Severe contractures of the SK, Sampson SP: Platelet-derived tracture. Hand Clin 1991;7:617-634. proximal interphalangeal joint in growth factor in DupuytrenÕs disease. 15. Smith AC: Diagnosis and indications DupuytrenÕs disease: Results of a J Hand Surg [Am] 1992;17:317-323. for surgical treatment. Hand Clin prospective trial of operative correc- 9. Lappi DA, Martineau D, Maher PA, et 1991;7:635-643. tion and dynamic extension splinting. al: Basic fibroblast growth factor in 16. McFarlane RM, Botz JS: The results J Hand Surg [Am] 1992;17:1153-1159. cells derived from DupuytrenÕs con- of treatment, in McFarlane RM, 22. McFarlane RM, McGrouther DA: tracture: Synthesis, presence, and McGrouther DA, Flint MH (eds): Complications and their management, implications for treatment of the dis- DupuytrenÕs Disease: Biology and in McFarlane RM, McGrouther DA, ease. J Hand Surg [Am] 1992;17:324-332. Treatment. Edinburgh: Churchill Flint MH (eds): DupuytrenÕs Disease: 10. Kloen P, Jennings CL, Gebhardt MC, Livingstone, 1990, pp 387-412. Biology and Treatment. Edinburgh: Springfield DS, Mankin HJ: Trans- 17. McFarlane RM: DupuytrenÕs contrac- Churchill Livingstone, 1990, pp 377- forming growth factor-b: Possible ture, in Green DP, Hotchkiss RN (eds): 382.

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