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(iv) Dupuytren’s disease
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(iv) Dupuytren’s disease
between 40 and 60 years of Northern European descent such as Great Britain, North America and Eastern Europe where it has been estimated that the prevalence of Dupuytren’s Disease ranges from 2 to 42% of the population.2 Males are nine times more likely to have signs and symptoms necessitating surgical intervention. Women tend to experience a more quiescent form characterized by more nodule formation than cords.3 Dupuytren’s disease is believed to be inherited as an autosomal dominant trait with incomplete penetrance.4 This was supported by Ling during a study of 50 patients and their families with Dupuytren’s disease in Edinburgh. Prior to examining the family members, each patient was asked as to whether any of their relatives experienced this condition. Sixteen percent of patients (eight out of 50) reported a positive family history. After examination of 832 relatives, however, 34 out of the 50 patients were found to have relatives with Dupuytren’s disease. Ling further noted that a single gene was probably involved in this condition and that its expression was almost complete in males over 75 years of age but less so within the female population.5 In patients with genetic predisposition to Dupuytren’s disease, environmental factors have been shown to contribute to the development of the disease. In trying to elucidate potential environmental factors, Burge and colleagues investigated the relationship between the risk of Dupuytren’s contracture in smokers and alcoholics.6 In a case control study, 222 patients having an operation for this condition were matched for age, operation date and gender with control patients who were having other orthopaedic operations. Results showed that smoking (adjusted odds ratio 2.8) and heavy alcohol consumption, as defined as greater than 7 units per week (adjusted odds ratio 1.9), were associated with an increase risk of developing Dupuytren’s contracture in predisposed patients. Arafa et al.7 compared the incidence of Dupuytren’s disease between seizure and non-seizure patients and noted a two-fold increase in the incidence in the former group compared to controls, particularly in those aged 50 years or more. Controversy exists as to the mechanism by which this occurs and may be related to altered tissue metabolism secondary to drug therapy. Renard et al.8 examined the link between diabetes and the development of Dupuytren’s disease. One hundred and twenty diabetics (both type 1 and 2) were compared to age and sex matched controls. The prevalence of this condition was 35.0% and 30.0% respectively in the diabetic population compared to 6.7% within the controls. The prevalence of Dupuytren’s disease was found to be correlated with increasing age in both types of diabetes and may be related to microangiopathy and increased collagen production in these patients. Controversy exists between the relationship of manual labour and the onset of the disease. Liss and Stock9 reviewed the literature addressing the relationship between manual work and Dupuytren’s disease and found evidence suggesting that workers exposed to repetitive manual work were over five times more likely to develop the condition compared to those not exposed to such trauma. The authors also noted a dose response relationship between vibration exposure and contracture development compared to controls (odds ratio 2.3). Advances in molecular biology have improved our understanding as to the varied gene expression patterns seen in Dupuytren’s disease. Using DNA microassay technology, Pan
Sanjeev Kakar Jennifer Giuffre Kshamata Skeete Basem Elhassan
Abstract Dupuytren’s disease is characterized by contracture of the palmar and digital hand fascia resulting in characteristic nodule and/or cord formation within the hand. This results in flexion deformity of the digits with subsequent impaired function. The pertinent epidemiology, aetiology and pathophysiology of this disease are discussed in this review. In addition, current principles of treatment of the hand contracture that develops as a result of this disease, both conservative and surgical, are highlighted in this review.
Keywords collagenase; Dupuytren’s disease; fasciectomy; needle aponeurotomy
Introduction Dupuytren’s disease can be defined as the characteristic contracture of the finger secondary to the formation of nodules and cords within proliferative palmar fascia. First described by Felix Platter in 1614,1 it was not until the work of Guillaume Dupuytren in 1830’s did the condition become generally recognized. During his lecture series, Dupuytren detailed the pathology, clinical course, and surgical treatment of this condition and dispelled the notion that the disease involved flexor tendons.1 Since that time, the disease became progressively well known. In depth understanding of its pathophysiology has grown significantly over the past decade resulting in improved treatment algorithms of the contracture.
Epidemiology and aetiology This condition is exceedingly rare within the black population and certain parts of Asia. It is more often seen within adults aged
Sanjeev Kakar MD MRCS MBA Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Jennifer Giuffre MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Kshamata Skeete MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Basem Elhassan MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW Rochester, MN 55905, USA. Conflicts of interest: none.
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et al.10 and Lee et al.11 studied the expression patterns of over 40 000 genes within normal and Dupuytren’s disease palmar fascia. They found 23 genes, including fibronectin, collagen III, IV and VI, TGFb2, alpha-1 (COL8A1), contactin I (CNTN1), leucine-rich repeat containing 17 (LRRC17) and musculoaponeurotic fibrosarcoma oncogene homolog B (MafB), to be highly up-regulated compared to controls. MafB is a transcription factor and has been linked to the formation of colonies by chicken fibroblasts.12 Compared to normal fascia, immunohistochemical studies have shown an up-regulation of MafB in myofibroblasts in diseased cord tissue and, as such, may play an important role in disease progression. Further research is being conducted in this area trying to elucidate the potential mechanism that may exist.
and the retaining ligaments of the fingers. Their roles are to retain the flexor tendons, stabilize metacarpals, provide anchorage for palmar skin and support the skin against compressive and shear forces. The palmar fascia complex consists of the central, radial and ulnar aponeuroses (Figure 1). The central aponeurosis is a triangular based structure within the centre of the palm. It comprises longitudinal, transverse and vertical fibres (Figure 2). The longitudinal fibres are a distal continuation of the palmaris longus and pass to the small to index fingers and occasionally to the thumb. They are termed pretendinous bands as they run parallel to the deep flexor tendons and overlie the transverse fibres that are located over the metacarpophalangeal (MCP) joints. As they pass distal to the distal palmar crease, the longitudinal fibres divide into three distinct layers.20 Within layer 1, the most superficial region, fibres insert into the skin between the distal palmar and proximal digital creases. Upon passing from radial to ulnar, the insertion into skin is more distal, thereby explaining why contracture development is more readily apparent in the ulnar digits. Layer 2 passes on either side of the flexor sheath, below the neurovascular bundle, and continues distally as the spiral bands of Gosset to insert on the lateral digital sheath. The deeper longitudinal fibres, layer 3, pass deep to the flexor tendons on each side of the MCP joints. The transverse fibres of the central aponeurosis pass dorsal to the longitudinal cords from the ulnar border of the small finger to the radial side of the index finger. Between the index and thumb, this structure continues as the proximal commissural ligament. The distal part of this ligament is termed the distal commissural ligament and is more often involved in Dupuytren’s contracture compared to its proximal counterpart.18 Located deep to the transverse fibres are vertical septa of the fibres of Legueu and Juvara (Figure 2). They arise from the
Pathophysiology of Dupuytren’s disease Dupuytren’s disease occurs in three main stages, namely proliferative, involutional and residual processes.13 As popularized by Luck,13 the disease is characterized by the development of nodules that occur within specific locations within the longitudinal tension lines in the palmar and digital fascia. The proliferative stage is characterized by an abundance of myofibroblast activity, believed to be the precursor cell for Dupuytren’s disease, into a characteristic whorl like pattern. These cells express a smooth muscle actin which plays a role in cell contractility and synthesize fibronectin which connects the cells together as well as to the extracellular matrix via integrins.14 Several theories exist as to the stimulus for myofibroblast activity. Murrell et al. believed that a hypoxic insult within the tissues resulted in the generation of free radicals. These in turn caused damage to surrounding tissues resulting in fibroblast and myofibroblast proliferation. The resulting increase in collagen production (type III to type I)15 caused further ischaemia thereby causing further free radical formation.16 In addition to free radicals, numerous other factors like platelet derived growth factor, basic fibroblast growth factor and interleukins 1a and b have been linked to myofibroblast stimulation. Badalamente and colleagues investigated the role of transforming growth factor beta (TGFb) isoforms 1 and 2 in the three stages of Dupuytren’s disease.17 Results demonstrated that TGFb1 was active during all three stages of the disease process whereas TGFb2 was most active during the proliferative and involutional stages. Compared to TGFb1, TGFb2 had the most significant proliferative effect. The involutional stage is characterized by the alignment of myofibroblasts along tension lines within the fascia. This is an example of mechanical transduction whereby mechanical stresses have a direct effect at the cellular level, thereby perpetuating the disease process.18 This was demonstrated by Tomasek et al.19 where they noted, in cell culture experiments, that mechanical stress promoted the differentiation of fibroblasts into myofibroblasts. The final phase is the residual process. During this quiescent stage, the tissue is largely devoid of the myofibroblast cells and is predominantly tendon like with only thick bands of collagen tissue remaining.13,19
Figure 1 Palmar fascia arrangement within the hand. RA ¼ radial aponeurosis, UA ¼ ulnar aponeurosis, PA ¼ palmar aponeurosis, NL ¼ natatory ligament, TLPA ¼ transverse ligament of the palmar aponeurosis (Reprinted with permission from Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 1: 190e198).
Anatomy The retinacular system of the hand comprises the transverse carpal ligament, palmar fascia, natatory ligaments, pulley system
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Figure 2 Three-dimensional structure of the central aponeurosis. s ¼ skin, lf ¼ longitudinal fibres, tf ¼ transverse fibres, t ¼ flexor tendon, dtl ¼ deep transverse ligament, slj ¼ septa of the fibres of Legueu and Juvara, m ¼ metacarpal.
transverse ligament of the palmar aponeurosis and insert into a complex comprising of the palmar plate, sagittal band, A1 pulley, interpalmar plate and septa of Legueu and Juvara (Figure 3).14 The septae form fibro-osseous tunnels, four of which contain the flexor tendons and three webspace channels, house the lumbricals and neurovascular bundles (Figures 2 and 3). The radial aponeurosis consists of the fascia over the thenar musculature, a pretendinous band (that may be absent) and the proximal and distal commissural ligaments. Constituents of the ulnar aponeurosis are the hypothenar muscle fascia, pretendinous band to the small finger and abductor digiti minimi fascial strands. The digital fascia comprises the natatory ligaments, lateral digital sheaths, Grayson’s and Cleland’s ligaments (Figure 4). The former span the digital webspaces and lie superficial to the
Figure 4 a Components of normal digital fascia that produce b the spiral cord. 1 ¼ pretendinous fascia, 2 ¼ spiral band, 3 ¼ lateral digital sheet, 4 ¼ Grayson’s ligament. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
transverse metacarpal ligament. They are composed of transverse and curved fibres with the latter joining the lateral digital sheath. The lateral digital sheaths are believed to be thickenings of the superficial digital fascia on either sides of the finger and receive contributions from the spiral bands, natatory and Grayson’s ligaments. Grayson’s ligaments run from the skin to the flexor tendon sheath and prevent bowstringing of the underlying neurovascular bundle upon digital flexion.21 In contrast to Grayson’s ligaments, Cleland’s ligaments lie dorsal to the neurovascular bundles. They arise from either side of the proximal interphalangeal joints of the fingers (interphalangeal joint in the thumb) to the distal interphalangeal joints and insert into the skin.21
Pathological anatomy in Dupuytren’s contracture With a detailed understanding of normal palmar and palmodigital fascia of the hand, the lesions of Dupuytren’s disease can be seen to follow this well defined anatomy. Pathological cords include the pretendinous or vertical cords in the palm, the spiral and natatory cords extending from the palm of the hand into the fingers, and the central retrovascular and lateral cords in the digits (Table 1) (Figure 5). The pretendinous cord is a thickening of the pretendinous band (layer 1) of the palmar aponeurosis and is the most common cord in Dupuytren’s contracture. The cord attaches to the skin within the distal palmar crease causing MCP joint flexion contracture. Occasionally, rather than passing into a single digit, the cord bifurcates into a “y” cord with each limb attaching into an adjacent finger. Owing to its superficial course,
Figure 3 Soft tissue confluence and septa of Legueu and Juvara. IPPL ¼ interpalmar plate ligament, PA ¼ palmar aponeurosis (Reprinted with permission from Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 190e198).
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Origin of diseased cords in Dupuytren’s disease and their clinical significance. (Reprinted with permission from Shaw RB, Chong AKS, Zhang A Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis and treatment. Plast Recon Surgery 2007; 120(3): 44ee54e) Disease structure Palmar cords Pretendinous cord Vertical cord
Palmo-digital cords Spiral cord Natatory cord Digital cords Central cord Retrovascular cord Lateral cord Abductor digiti minimi cord
Thumb and first web Proximal commissural cord Distal commissural cord Thumb pretendinous cord
Origins Pretendinous band
Uncommon, from diseased vertical fibres of McGrouther or septa of Legueu and Juvara Pretendinous band, spiral band, lateral digital sheet and Grayson’s ligament Distal fibres of natatory ligament Extension of pretendinous cord in the finger Retrovascular band of Thomine Lateral digital sheet, often associated with pretendinous and natatory cord Abductor digiti minimi tendon Proximal commissural ligament Distal commissural ligament Pretendinous band
Clinical significance Most common cord in hand; causes MCP flexion contracture; does not displace neurovascular bundle May cause painful triggering Displaces the neurovascular bundle palmarly and to the midline, creating a “spiral nerve” Web space contracture limiting digital abduction Does not usually displace the neurovascular bundle Causes DIPJ contracture and prevents full correction of PIPJ contracture Flexion contracture of PIPJ and DIPJ; displaces neurovascular bundle to midline Flexion contracture of PIPJ First web contracture First web contracture MCP contracture
Table 1
the neurovascular bundles are not usually displaced by this cord. Vertical cords are diseased septa of Legueu and Juvara and are relatively uncommon. They form a small cord emanating from the pretendinous cords and run between the neurovascular bundle and flexor tendon sheaths.3 Disease affecting the natatory ligaments, natatory cords, within the palmo-digital fascia can be seen within the second to
fourth webspaces. The normal U shape of the webspace is lost, thereby limiting digit abduction. These cords tend to run volar to the neurovascular bundles thereby sparing any displacement. The spiral cord arises from the pretendinous band and follows layer 2 of the palmar fascia to the lateral digital sheath and attaches to the middle phalanx via Grayson’s ligament (Table 1 and Figure 5). From its origin, the spiral cord passes dorsal to
Figure 5 How Dupuytren’s disease affects normal digital fascia. Left: normal digital fascial components. Right: Diseased tissue that results in joint contracture. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
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the neurovascular bundle just distal to the MCP joint. It then passes lateral to the artery and nerve as it enters the digit and finally runs superficial as it attaches into Grayson’s ligament. Owing to this protracted course, with worsening flexion contracture of the proximal interphalangeal (PIP) joint, secondary to cord thickening and shortening, the neurovascular bundle displaces proximal, superficial and towards the midline of the digit (Figure 6). This displacement accounts for the increased susceptibility to injury during surgical contracture releases of this joint. Within the small finger, the spiral cord is known as the abductor digit minimi cord as it arises from this muscle and inserts at the base of either the middle or distal phalanges. Running volar to the neurovascular bundle, this cord can cause displacement of the neurovascular bundle from its usual lateral location. Within the digit, the central cord is an extension of the pretendinous cord of the palm and attaches to the ulnar base of the middle phalanx or flexor tendon sheath. Owing to its midline course, it rarely displaces the neurovascular bundle.22 The lateral cord arises from the lateral digital sheath and attaches via Grayson’s ligament to the palmar skin or flexor tendon sheath, thereby aiding in PIP joint contracture and occasionally distal interphalangeal joint flexion contracture (Figure 5). Often present with the pretendinous and natatory cords, the lateral cord can cause midline displacement of the neurovascular bundle.3 The retrovascular cord is located dorsal to the neurovascular bundle.14 Originating from the diseased retrovascular cord of Thomine, this cord needs to be excised to achieve full PIP joint contracture release. In the thumb, adductor contracture development of the first web space is related to disease of the proximal commissural ligament ( proximal commissural cord ) and distal commissural ligament (distal commissural cord ). Metacarpophalangeal joint contracture is primarily attributed to thumb pretendinous cord
development with a contribution from the thenar cord, a thickening of the thenar fascia.
Clinical presentation and differential diagnosis Dupuytren’s disease typically presents in a 50-year-old Caucasian male who has had palmar fascia contracture for several years that is beginning to affect his activities of daily living or the appearance of the hand. The disease usually starts proximally and progresses distally into the digits, affecting in decreasing order, the ring finger, followed by the small, middle, index fingers and the thumb. On the palmar surface, changes that can be seen include skin pits, nodules and cord formation. The former are related to thickening of the Grapow fibres which connect the dermis to the palmar fascia. The skin loses its mobility as it becomes increasingly tethered to the fascia. Commonly seen in the palm distal to the distal palmar crease, skin pits are the earliest sign of the disease. Dupuytren nodules originate from the superficial palmar or digital fascia and mark the intermediate stage of the disease.14 They are well defined thickenings of the underlying deep fascia and can be symptomatic if they abut upon the underlying flexor tendons inducing a tenosynovitis reaction. Palmar nodules usually arise at the distal palmar crease whereas digital nodules are seen at the base of the digit or the PIP joint.3 As nodules regress, they are typically replaced by pathologic cords (as discussed earlier). This marks the onset of the late phase of the disease, and as they mature, form distinct band like structures within the hand. Contracture development usually progresses from a proximal to distal direction, first affecting the MCP joint of the ring finger followed by ipsilateral PIP joint involvement. This is by no means absolute and can be confined to just the palm or digit. Dupuytren’s disease can manifest with dorsal skin changes such as Garrod’s node (PIP joint nodules) or knuckle pad
Figure 6 Neurovascular bundle displacement proximally, palmarly and towards the midline with increasing PIP joint contracture thereby increasing its susceptibility to injury during surgery. a By a spiral cord, b by spiral and central cords, c by the pretendinous, natatory and lateral cords, d by a communication between Grayson’s ligament and the central cord and e by the spiral and natatory cords. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
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(PIPJ fibroma like lesions) formation (Figure 7).23 They tend to be seen in patients with bilateral disease and in patients with similar lesions like on the feet or genitalia. Other conditions may mimic Dupuytren’s disease, especially early in the disease process. These include hyperkeratosis, callus formation, inclusion cysts, rheumatoid nodules, palmar fibromatosis, ganglions, soft tissue giant cell tumours (pigmented villonodular synovitis) and epithelioid sarcomas.
Non-operative treatment of Dupuytren’s disease Observation is indicated for patients whose Dupuytren’s disease is relatively inactive with mild contracture or limited functional compromise. In this case, patients need to be monitored since the disease is one of gradual progression at various rates.25 Other modalities such as the use of intermittent splinting, radiation and vitamin E are limited in terms of their applicability and lack data demonstrating efficacy. More recently, there has been a resurgent interest in the use of intralesional injection therapy for early and late disease. Ketchum and Donahue26 evaluated the efficacy of steroid injections to soften and flatten Dupuytren nodules. Using triamcinolone acetonide intralesional injections, the authors reported after an average of 3.2 injections per nodule, 97% of the hands showed regression of disease. This effect appeared to be temporary, however, as half of the patients developed reactivation of the disease in the nodules 1e3 years after the last injection. Given the excessive collagen deposition seen within this condition, efforts have been made to use targeted enzymatic collagen degradation as a potential treatment modality. This was popularized by Badalamente and Hurst27 who tested the safety and clinical efficacy of intralesional clostridial collagenase injections as a non-surgical treatment of Dupuytren’s disease. Twenty-nine patients received collagenase injections (10 000 units) into contractures of 34 MCP joints, nine PIP joints and one thumb. Results showed that 82% of MCP and 44% of PIP joint contractures corrected to full extension within 2 weeks of injection. In a recent prospective randomized double blind controlled trial,28 23 patients with flexion deformities of the MCP and/or PIP joints of 20 or more were randomized to receiving placebo or collagenase injections. Clinical success, as defined as joint contracture being within 5 of full extension, was achieved in 70% of patients receiving one injection and in 91% patients receiving three injections. None of the placebo treated patients achieved joint correction. Currently, the Food and Drug Administration is evaluating the potential of collagenase for widespread clinical use as concerns exist regarding potential complications such as local extravasation, haematoma, swelling and toxicity to adjacent tendons, neurovascular structures and skin. Percutaneous needle aponeurotomy can be used as a one stage or part of a staged procedure (with weekly intralesional steroid injections) for cord disruption within the office setting. Popularized by French rheumatologists, this procedure is gaining increasing demand due to perceived limited invasiveness, good outcomes, fast recovery and overall patient satisfaction. This was further highlighted by Van Rijssen and co-workers who conducted a randomized controlled trial comparing needle aponeurotomy (88 digits) to limited fasciectomy (78 digits).29 At 6 weeks post treatment, patient satisfaction between both treatments was similar but function, as demonstrated by improved DASH scores, and complication rates were better in the percutaneous needle fasciotomy group. At longer follow-up, recurrence of deformity was more common after percutaneous treatment. This was further demonstrated by van Rijssen and Werker30 who reported immediate contracture improvement in over 75% of patients who underwent 74 percutaneous needle releases. By 32 months, however, recurrence was noted in 65% of patients demonstrating that this procedure has good short-term effects and may be suitable
Treatment options of hand contracture in patients with Dupuytren’s disease Numerous treatment options exist in the management of this disease ranging from simple observation to radical palmar fasciectomy. When balancing the advantages and disadvantages of any treatment option, it is important to consider several factors including the fingers’ deformity, loss of function, rate of disease progression and patient factors like occupation and comorbidities.18 The deformity of the fingers may be cosmetic only or could be significant enough to affect activities of daily living. Though surgical correction has been widely advocated for a flexion contracture of more than 30 at the MCP joint and/or any contracture at the PIP,24 treatment principles are better guided on the patient’s functional difficulty and the rate of deformity progression. A patient with a quiescent contracture may be more amenable to non-operative treatment compared to one with clear deterioration.
a and b Photograph demonstrating knuckle pads on the dorsum of the hand. Figure 7
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for patients who desire a minimally invasive approach or can be used to delay formal surgical fasciectomy. Of note, two patients experienced evidence of partial digital nerve injury but did elect to undergo any further intervention. Given these encouraging results, caution needs to be given as to which cords are amenable to this technique. Complications such as digital nerve injury, flexor tendon damage, infection, recurrence, complex regional pain syndrome (CRPS) and skin breakdown mean that this procedure may be best suited for isolated palmar or well defined central digital cords as opposed to the digits containing spiral cords.
covered with full thickness grafts. This technique is best indicated for recurrent and severe primary disease when the skin is adherent to Dupuytren tissue. Multiple options exist for skin incisions and can be categorized into the longitudinal and transverse types. The longitudinal incisions can be divided into a straight line incision with multiple Z plasties, Brunner type incisions, multiple V-Y advancement flaps and Moerman’s small curved incisions.18 They have the advantage over transverse incisions as they provide extensile exposure and address the skin shortage secondary to contracture.18,31 When raising the skin flaps, caution should be followed to ensure that the flaps are not too thin for fear of skin necrosis. At the palmo-digital junction, great care should be practised to try and identify the neurovascular bundles before cutting down deep onto the pathological cords. Dissection should be carried out from proximal and distal directions, thereby allowing an easier and safer way to identify the neurovascular bundles and prevent their inadvertent injury. Transverse incisions across the palm and/or digits permit access to the diseased tissue without providing a pathway for secondary scar contracture. Commonly known as the open palm technique, these wounds are either left open and allowed to heal via secondary intention or closed with a full thickness skin graft. This technique is primarily indicated for recurrence associated with considerable contracture and induration of the palm rather than for primary disease. Several different options exist for management of the fascia (Table 2). These range from simple fasciotomy to more radical fasciectomy. Fasciotomy is aimed at releasing the longitudinal fascia tension lines without removal of diseased tissue. Advised for patients with isolated palmar longitudinal central cords or in whom anaesthesia is best avoided, this procedure is associated with a high recurrence rate (up to 43%), injury to nerves and complex regional pain syndrome.14,35 Limited fasciectomy is a procedure whereby a portion of the diseased cord is excised. As reported by Rodrigo et al.35, postoperative stiffness and haematoma formation were common as well as the high recurrence rate. Sixty-three percent of patients reported recurrent deformity of which 15% required additional treatment. In light of these high recurrence rates, the most widely used procedure is regional fasciectomy. Through a longitudinal incision, full thickness skin flaps are raised from the palm to the digit. The longitudinal central cord is dissected as far proximally as needed, divided and
Surgical treatment of Dupuytren’s disease When surgical correction is planned, the patient must have clear expectations and understanding of the nature of the disease and the postoperative and rehabilitative course involved. There is no such surgery that cures the disease, but the main purpose of the surgery is to correct the deformity whenever possible. The postoperative rehabilitation may take at least 3 months before patients regain their hand function depending on the severity of the contracture before surgery and type of patients. For instance, patients with comorbidities such as cerebrovascular accident or Parkinson’s disease may lack compliance towards their hands’ rehabilitation compared to other patients. A very common postoperative complication that all patients should also be informed about is the recurrence rate after surgery which can be as high as 50% after 10 years.18 Once the decision has been made to proceed to surgery, the plan must target three fundamental areas, namely management of the skin, the fascia and the joints.18,31 In terms of the first, the surgeon needs to decide whether the type of skin incision will allow adequate exposure of the diseased tissue or whether the contracture is so far advanced that skin excision is preferable. Skin excision or dermofasciectomy is a technique whereby the diseased fascia and overlying skin are excised en bloc and combined with skin grafting. Popularized by Hueston,32,33 this technique serves three purposes: replace myofibroblast populated dermis with normal skin, replace the skin shortage and also interpose a piece of healthy skin between diseased tissue, thereby disrupting lines of longitudinal tension (so called “firebreak”). Ketchum and Hixson34 reported on their 15-year experience of this technique and noted no recurrence of disease in the palms and digits that were
Fascial treatment options. (Reprinted with permission from Shaw RB, Chong AKS, Zhang A Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis and treatment. Plast Recon Surgery 2007; 120(3): 44ee54e) Surgical technique Fasciotomy Local fasciectomy Regional fasciectomy Radical fasciectomy Open palm technique (McCash) Dermo-fasciectomy
Description Division of diseased cords without excision; it can be performed percutaneously Removal of some diseased tissue More extensive dissection and removal of involved tissues Removal of all the palmar fascia with extension into the involved fingers; the extensive dissection and skin flaps required increases the risks of complications Transverse skin incision and division of the aponeurosis, leaving the palm wound open; requires prolonged postoperative splintage Removal of the skin and fascia followed by coverage with skin grafts
Table 2
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elevated in a proximal to distal direction. Knowledge of the three layers of the palmar fascia becomes crucial to ensure all the diseased tissue is excised and the cord raised in a safe manner, with care taken to identify and protect the displaced neurovascular bundles. Within the digit, the cord is followed to its distal attachment and divided. If the joint contracture is released, surgery is discontinued. If contracture persists, however, additional cords need to be excised and consideration given to joint release. Radical fasciectomy is the process whereby all diseased fascia as well as uninvolved tissue is excised to prevent disease recurrence. The aim in the palm is to remove all of longitudinal fibres to the digits. This entails the raising of large skin flaps with the subsequent risk of increased wound healing complications and postoperative stiffness. Because of these reported risks, this procedure is advocated in patients who have extensive disease or an increased diathesis. Residual joint contracture should be addressed if the digit remained contracted after the skin is released and the fascia excised. Interestingly, almost all MCP joint contractures tend to be corrected after the fascial release procedure as this joint can tolerate prolonged immobilization in flexion due to the CAM effect of the collateral ligaments. For this reason, MCP flexion contracture is not an urgent indication for surgery. The PIP joint, however, is very intolerant to prolonged flexion and simple fascial excision may not be sufficient to resolve this contracture. MacFarlane conceptualized PIP joint involvement in Dupuytren’s disease as either primary or secondary flexion contractures.36 Primary contractures were directly attributed to fascial disease whereas secondary flexion contractures were due to either periarticular adhesions, contracture of the flexor sheath, shortening of the palmar skin or flexor muscles, contracture of the palmar plate, checkrein ligaments, collateral or accessory collateral ligaments.36,37 In patients with greater than 60 PIP joint contracture, Smith and Breed38 reported that this may be due to central slip attenuation. If the joint remains contracted after fasciectomy, splinting of the digit in full extension may help to restore the tone of the central slip. Draviaraj and Chakrabarti39 reported on 30 patients who underwent MCP and PIP joint corrective surgery over a 1 year time frame. The average preoperative MCP and PIP joint deformity was 31 and 35 respectively. Postoperatively, this corrected to 19 at the MCP and 16 at the PIP joint. Hand function tests demonstrated improved function between the total correction, PIP joint correction, and hand function up to 1 year, but not with MCP joint correction. Despite the theoretical causes of PIP joint flexion contracture, Weinzweig et al.40 failed to show any advantage to capsuloligamentous release in addition to fasciectomy in treating severe PIP joint contracture (greater than 60 ). Eighteen patients underwent fasciectomy alone and ten patients underwent capsulotomy in addition to fasciectomy. In the non-capsulotomy group, preoperative contracture averaged 78 and improved to 36 postoperatively. In the capsulotomy group, preoperative measurements averaged 82 and averaged 36 after surgery. No statistically significant difference was seen in the percentage of contracture correction in the capsulotomy group compared with the non-capsulotomy group at final follow up. In addition, the complication rate was higher in the capsulotomy group. Interestingly, the authors reported that the degree of correction
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obtained at surgery using either method was not maintained during follow up. This was similarly reported by Donaldson and co-workers.41 Fifty-two patients were followed prospectively for 18 months after undergoing fasciectomy for Dupuytren’s disease. Of the 42 MCP joints, full intraoperative correction was obtained in 41. Of these, 37 had full correction at final follow up. Of the 58 PIP joints treated, full intraoperative correction was obtained in 35 cases. After 18 months, less than 38% maintained their correction. The authors concluded that the degree of preoperative deformity and intraoperative correction, especially for the PIP joint, were significant predictors of loss of surgical correction and recurrence of the deformity after surgery. Postoperative rehabilitation The postoperative rehabilitation program is an integral and essential part of the management of Dupuytren’s disease after surgical correction. It is directed towards restoring hand function and preventing complications after surgery. In the immediate postoperative period, the hand is immobilized with the MCP and PIP joints fully extended or in the position of function with the MCP joints flexed and the PIP joints extended. After 3e5 days, once the early inflammatory process has subsided, active motion is begun with night splinting to preserve extension. Early involvement of a hand therapist is advised to aid with range of motion exercises, oedema control, scar massage and early treatment of CRPS. When PIP joint correction has been incomplete, dynamic extension splintage can be introduced. Rives et al.42 treated 23 joints (contracture greater than 45 ) with surgical correction and 6 months of splinting. After 2 years, a 44% improvement was noted in PIP joint contracture suggesting that the soft tissues are able to remodel with time. Postoperative complications Despite the meticulous technique that is usually applied in Dupuytren’s surgery, the reported complication rates are still as high as 17%.18 They are more likely to be encountered in patients with severe or recurrent disease and include intraoperative complications like neurovascular injury and buttonholing of the skin or postoperative complications like haematoma, skin necrosis, infection, dehiscence and CRPS. The incidence of neurovascular injury is approximately 3% and is increased in patients where the bundle is displaced due to a spiral cord or in revision surgery due to abundant scar formation.14,18 This risk is decreased if one approaches the diseased tissue from proximal to distal and distal to proximal directions to ensure the neurovascular bundle can be identified and isolated. Loss of digital artery circulation can result from spasm secondary to stretch, intimal haemorrhage, direct injury or vessel rupture due to overzealous joint contracture correction. When such a compromise is due to spasm or intimal damage, the digit is repositioned to its original resting attitude and the artery bathed in warm saline and a topical vasodilator such as papaverine. However, if direct arterial damage is identified, then a primary repair or use of an interpositional vein graft, should be performed. Postoperative haematoma formation can be addressed by deflating the tourniquet and use of bipolar cautery for haemostatsis. The use of adjuncts such as thrombin spray and drains may lessen their formation. If a haematoma develops, urgent evacuation is recommended to decrease the risk of skin necrosis or infection.
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13 Luck JV. Dupuytren’s contracture; a new concept of the pathogenesis correlated with surgical management. J Bone Joint Surg Am 1959 Jun; 41-A: 635e64. 14 Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 190e8. 15 Badalamente MA, Hurst LC. The biochemistry of Dupuytren’s disease. Hand Clin 1999; 15: 35. 16 Murrell GA, Francis MJ, Bromley L. Modulation of fibroblast proliferation by oxygen free radicals. Biochem J 1990 Feb 1; 265: 659e65. 17 Badalamente MA, Sampson SP, Hurst LC, Dowd A, Miyasaka K. The role of transforming growth factor beta in Dupuytren’s disease. J Hand Surg Am 1996; 21(2): 210e5. 18 McGrouther DA. Dupuytren’s contracture. In: Green DP, Hotchkiss RN, Pederson WC, Wolfe SW, eds. Green’s operative hand surgery. New York: Churchill Livingstone, 2005: 159e85. 19 Tomasek JJ, Vaughan MB, Haaksma CJ. Cellular structure and biology of Dupuytren’s disease. Hand Clin 1999; 15: 21e34. 20 McGrouther DA. The microanatomy of Dupuytren’s contracture. Hand 1992; 14: 215e36. 21 Milford LW. Retaining ligaments of the hand. Philadelphia: WB Saunders, 1968. 22 Barton. Dupuytren’s disease arising from the abductor digiti minimi. J Hand Surg Br 1984; 9: 265e70. 23 Garrod AE. On an unusual form of nodule upon the joints of the fingers. St Bartolomews Hosp Rep 1893; 29: 157e61. 24 Smith AC. Diagnosis and indications for surgical treatment. Hand Clin 1991 Nov; 7: 635e42. 25 Rayan GM. Nonoperative treatment of Dupuytren’s disease. J Hand Surg Am 2008; 33: 1208e10. 26 Ketchum LD, Donahue TK. The injection of nodules of Dupuytren’s disease with triamcinolone acetonide. J Hand Surg Am 2000; 25: 1157e62. 27 Badalamente MA, Hurst LC. Enzyme injection as nonsurgical treatment of Dupuytren’s disease. J Hand Surg Am 2000 Jul; 25: 629e36. 28 Badalamente MA, Hurst LC. Efficacy and safety of injectable mixed collagenase subtypes in the treatment of Dupuytren’s contracture. J Hand Surg Am 2007; 32: 767e74. 29 van Rijssen AL, Gerbrandy FS, Ter Linden H, Klip H, Werker PM. A comparison of the direct outcomes of percutaneous needle fasciotomy and limited fasciectomy for Dupuytren’s disease: a 6-week follow-up study. J Hand Surg Am 2006; 31: 717e25. 30 van Rijssen AL, Werker PM. Percutaneous needle fasciotomy in Dupuytren’s disease. J Hand Surg Br 2006; 31: 498e501. 31 Saar JD, Grothaus PC. Dupuytren’s disease: an overview. Plast Reconstr Surg 2000; 106: 125e34. 32 Hueston JT. Dermofasciectomy for Dupuytren’s disease. Bull Hosp Jt Dis Orthop Inst 1984; 44: 224e32. 33 Hueston JT. ‘Firebreak’ grafts in Dupuytren’s contracture. Aust N Z J Surg 1984; 54: 277e81. 34 Ketchum LD, Hixson FP. Dermofasciectomy and full-thickness grafts in the treatment of Dupuytren’s contracture. J Hand Surg Am 1987; 12: 659e64. 35 Rodrigo JJ, Niebauer JJ, Brown RL, Doyle JR. Treatment of Dupuytren’s contracture. Long-term results after fasciotomy and fascial excision. J Bone Joint Surg Am 1976; 58: 380e7. 36 MacFarlane RM. Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Displacement of the neurovascular bundle. Plast Reconstr Surg 1974; 54: 31e44. 37 Crowley B, Tonkin MA. The proximal interphalangeal joint in Dupuytren’s disease. Hand Clin 1999; 15: 137e47.
Complex regional pain syndrome (CRPS) has been reported as high as 5% and is more common in woman and in patients undergoing simultaneous carpal tunnel release. The aetiology is uncertain and may be due to overzealous handling of the neurovascular bundles during surgery. Once diagnosed, treatment is directed to manage the CRPS with hand therapy, oral medications like steroids or carbamazepine, or sympathetic blockade.14,31,43 Recurrence of contracture is by far the most common complication with ranges from 2% to 60%.43 It is more commonly seen in patients with PIP joint contracture, multiple digit involvement, small finger involvement, advanced disease stage, comorbidities such as diabetes and in patients with Dupuytren diathesis.14,31,43
Conclusion Management of hand contracture in patients with Dupuytren’s disease still poses numerous challenges to hand surgeons, despite advances in medical science and surgical techniques. Surgical correction remains the mainstay of treatment and can result in excellent improvement in hand function. Less invasive techniques like needle aponeurotomy is an alternative to surgery and to date is largely patient driven because of quicker recovery times. Collagenase treatment offers promise for the future, either as a stand alone treatment or as an adjunct to other techniques. A
REFERENCES 1 Elliot D. The early history of Dupuytren’s disease. Hand Clin 1999; 15: 1e19. 2 Ross DC. Epidemiology of Dupuytren’s disease. Hand Clin 1999; 15: 53. 3 Shaw RB, Chong AKS, Zhang A, Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis and treatment. Plast Reconstr Surg 2007; 120(3): 44ee54. 4 MacFarlane RM. Progress in Dupuytren’s disease. J Hand Surg Br 1991; 16: 237e9. 5 Ling RS. The genetic factor in Dupuytren’s disease. J Bone Joint Surg Br 1963; 45: 709e18. 6 Burge P, Hoy G, Regan P, Milne R. Smoking, alcohol and the risk of Dupuytren’s contracture. J Bone Joint Surg Br 1997 Mar; 79: 206e10. 7 Arafa M, Noble J, Royle SG, Trail IA, Allen J. Dupuytren’s and epilepsy revisited. J Hand Surg Br 1992; 17: 221e4. 8 Renard E, Jacques D, Chammas M, et al. Increased prevalence of soft tissue hand lesions in type 1 and type 2 diabetes mellitus: various entities and associated significance. Diabete Metab 1994; 20: 513e21. 9 Liss GM, Stock SR. Can Dupuytren’s contracture be work-related?: review of the evidence. Am J Ind Med 1996; 29: 521e32. 10 Pan D, Watson HK, Swigart C, Thomson JG, Honig SC, Narayan D. Microarray gene analysis and expression profiles of Dupuytren’s contracture. Ann Plast Surg 2003; 50: 618e22. 11 Lee LC, Zhang AY, Chong AK, Pham H, Longaker MT, Chang J. Expression of a novel gene, MafB, in Dupuytren’s disease. J Hand Surg Am. 2006; 31: 211e8. 12 Kataoka K, Fujiwara KT, Noda M, Nishizawa M. MafB, a new Maf family transcription activator that can associate with Maf and Fos but not with Jun. Mol Cell Biol 1994; 14: 7581e91.
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38 Smith P, Breed C. Central slip attenuation in Dupuytren’s contracture: a cause of persistent flexion of the proximal interphalangeal joint. J Hand Surg Am 1994; 19: 840e3. 39 Draviaraj KP, Chakrabarti I. Functional outcome after surgery for Dupuytren’s contracture: a prospective study. J Hand Surg Am 2004; 29: 804e8. 40 Weinzweig N, Culver JE, Fleegler EJ. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease: combined fasciectomy with capsuloligamentous release versus fasciectomy alone. Plast Reconstr Surg 1996; 97: 560e6.
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41 Donaldson OW, Pearson D, Reynolds R, Bhatia RK. The association between intraoperative correction of Dupuytren’s disease and residual postoperative contracture. J Hand Surg Eur Vol 2010; 35: 220e3. 42 Rives K, Gelberman R, Smith B, Carney K. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease: results of a prospective trial of operative correction and dynamic extension splinting. J Hand Surg Am 1992; 17: 1153e9. 43 Boyer MI, Gelberman RH. Complications of the operative treatment of Dupuytren’s disease. Hand Clin 1999; 15: 161e6.
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(iv) Dupuytren’s disease
between 40 and 60 years of Northern European descent such as Great Britain, North America and Eastern Europe where it has been estimated that the prevalence of Dupuytren’s Disease ranges from 2 to 42% of the population.2 Males are nine times more likely to have signs and symptoms necessitating surgical intervention. Women tend to experience a more quiescent form characterized by more nodule formation than cords.3 Dupuytren’s disease is believed to be inherited as an autosomal dominant trait with incomplete penetrance.4 This was supported by Ling during a study of 50 patients and their families with Dupuytren’s disease in Edinburgh. Prior to examining the family members, each patient was asked as to whether any of their relatives experienced this condition. Sixteen percent of patients (eight out of 50) reported a positive family history. After examination of 832 relatives, however, 34 out of the 50 patients were found to have relatives with Dupuytren’s disease. Ling further noted that a single gene was probably involved in this condition and that its expression was almost complete in males over 75 years of age but less so within the female population.5 In patients with genetic predisposition to Dupuytren’s disease, environmental factors have been shown to contribute to the development of the disease. In trying to elucidate potential environmental factors, Burge and colleagues investigated the relationship between the risk of Dupuytren’s contracture in smokers and alcoholics.6 In a case control study, 222 patients having an operation for this condition were matched for age, operation date and gender with control patients who were having other orthopaedic operations. Results showed that smoking (adjusted odds ratio 2.8) and heavy alcohol consumption, as defined as greater than 7 units per week (adjusted odds ratio 1.9), were associated with an increase risk of developing Dupuytren’s contracture in predisposed patients. Arafa et al.7 compared the incidence of Dupuytren’s disease between seizure and non-seizure patients and noted a two-fold increase in the incidence in the former group compared to controls, particularly in those aged 50 years or more. Controversy exists as to the mechanism by which this occurs and may be related to altered tissue metabolism secondary to drug therapy. Renard et al.8 examined the link between diabetes and the development of Dupuytren’s disease. One hundred and twenty diabetics (both type 1 and 2) were compared to age and sex matched controls. The prevalence of this condition was 35.0% and 30.0% respectively in the diabetic population compared to 6.7% within the controls. The prevalence of Dupuytren’s disease was found to be correlated with increasing age in both types of diabetes and may be related to microangiopathy and increased collagen production in these patients. Controversy exists between the relationship of manual labour and the onset of the disease. Liss and Stock9 reviewed the literature addressing the relationship between manual work and Dupuytren’s disease and found evidence suggesting that workers exposed to repetitive manual work were over five times more likely to develop the condition compared to those not exposed to such trauma. The authors also noted a dose response relationship between vibration exposure and contracture development compared to controls (odds ratio 2.3). Advances in molecular biology have improved our understanding as to the varied gene expression patterns seen in Dupuytren’s disease. Using DNA microassay technology, Pan
Sanjeev Kakar Jennifer Giuffre Kshamata Skeete Basem Elhassan
Abstract Dupuytren’s disease is characterized by contracture of the palmar and digital hand fascia resulting in characteristic nodule and/or cord formation within the hand. This results in flexion deformity of the digits with subsequent impaired function. The pertinent epidemiology, aetiology and pathophysiology of this disease are discussed in this review. In addition, current principles of treatment of the hand contracture that develops as a result of this disease, both conservative and surgical, are highlighted in this review.
Keywords collagenase; Dupuytren’s disease; fasciectomy; needle aponeurotomy
Introduction Dupuytren’s disease can be defined as the characteristic contracture of the finger secondary to the formation of nodules and cords within proliferative palmar fascia. First described by Felix Platter in 1614,1 it was not until the work of Guillaume Dupuytren in 1830’s did the condition become generally recognized. During his lecture series, Dupuytren detailed the pathology, clinical course, and surgical treatment of this condition and dispelled the notion that the disease involved flexor tendons.1 Since that time, the disease became progressively well known. In depth understanding of its pathophysiology has grown significantly over the past decade resulting in improved treatment algorithms of the contracture.
Epidemiology and aetiology This condition is exceedingly rare within the black population and certain parts of Asia. It is more often seen within adults aged
Sanjeev Kakar MD MRCS MBA Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Jennifer Giuffre MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Kshamata Skeete MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Conflicts of interest: none. Basem Elhassan MD Department of Orthopaedic Surgery, Mayo Clinic, 200 1st Street SW Rochester, MN 55905, USA. Conflicts of interest: none.
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et al.10 and Lee et al.11 studied the expression patterns of over 40 000 genes within normal and Dupuytren’s disease palmar fascia. They found 23 genes, including fibronectin, collagen III, IV and VI, TGFb2, alpha-1 (COL8A1), contactin I (CNTN1), leucine-rich repeat containing 17 (LRRC17) and musculoaponeurotic fibrosarcoma oncogene homolog B (MafB), to be highly up-regulated compared to controls. MafB is a transcription factor and has been linked to the formation of colonies by chicken fibroblasts.12 Compared to normal fascia, immunohistochemical studies have shown an up-regulation of MafB in myofibroblasts in diseased cord tissue and, as such, may play an important role in disease progression. Further research is being conducted in this area trying to elucidate the potential mechanism that may exist.
and the retaining ligaments of the fingers. Their roles are to retain the flexor tendons, stabilize metacarpals, provide anchorage for palmar skin and support the skin against compressive and shear forces. The palmar fascia complex consists of the central, radial and ulnar aponeuroses (Figure 1). The central aponeurosis is a triangular based structure within the centre of the palm. It comprises longitudinal, transverse and vertical fibres (Figure 2). The longitudinal fibres are a distal continuation of the palmaris longus and pass to the small to index fingers and occasionally to the thumb. They are termed pretendinous bands as they run parallel to the deep flexor tendons and overlie the transverse fibres that are located over the metacarpophalangeal (MCP) joints. As they pass distal to the distal palmar crease, the longitudinal fibres divide into three distinct layers.20 Within layer 1, the most superficial region, fibres insert into the skin between the distal palmar and proximal digital creases. Upon passing from radial to ulnar, the insertion into skin is more distal, thereby explaining why contracture development is more readily apparent in the ulnar digits. Layer 2 passes on either side of the flexor sheath, below the neurovascular bundle, and continues distally as the spiral bands of Gosset to insert on the lateral digital sheath. The deeper longitudinal fibres, layer 3, pass deep to the flexor tendons on each side of the MCP joints. The transverse fibres of the central aponeurosis pass dorsal to the longitudinal cords from the ulnar border of the small finger to the radial side of the index finger. Between the index and thumb, this structure continues as the proximal commissural ligament. The distal part of this ligament is termed the distal commissural ligament and is more often involved in Dupuytren’s contracture compared to its proximal counterpart.18 Located deep to the transverse fibres are vertical septa of the fibres of Legueu and Juvara (Figure 2). They arise from the
Pathophysiology of Dupuytren’s disease Dupuytren’s disease occurs in three main stages, namely proliferative, involutional and residual processes.13 As popularized by Luck,13 the disease is characterized by the development of nodules that occur within specific locations within the longitudinal tension lines in the palmar and digital fascia. The proliferative stage is characterized by an abundance of myofibroblast activity, believed to be the precursor cell for Dupuytren’s disease, into a characteristic whorl like pattern. These cells express a smooth muscle actin which plays a role in cell contractility and synthesize fibronectin which connects the cells together as well as to the extracellular matrix via integrins.14 Several theories exist as to the stimulus for myofibroblast activity. Murrell et al. believed that a hypoxic insult within the tissues resulted in the generation of free radicals. These in turn caused damage to surrounding tissues resulting in fibroblast and myofibroblast proliferation. The resulting increase in collagen production (type III to type I)15 caused further ischaemia thereby causing further free radical formation.16 In addition to free radicals, numerous other factors like platelet derived growth factor, basic fibroblast growth factor and interleukins 1a and b have been linked to myofibroblast stimulation. Badalamente and colleagues investigated the role of transforming growth factor beta (TGFb) isoforms 1 and 2 in the three stages of Dupuytren’s disease.17 Results demonstrated that TGFb1 was active during all three stages of the disease process whereas TGFb2 was most active during the proliferative and involutional stages. Compared to TGFb1, TGFb2 had the most significant proliferative effect. The involutional stage is characterized by the alignment of myofibroblasts along tension lines within the fascia. This is an example of mechanical transduction whereby mechanical stresses have a direct effect at the cellular level, thereby perpetuating the disease process.18 This was demonstrated by Tomasek et al.19 where they noted, in cell culture experiments, that mechanical stress promoted the differentiation of fibroblasts into myofibroblasts. The final phase is the residual process. During this quiescent stage, the tissue is largely devoid of the myofibroblast cells and is predominantly tendon like with only thick bands of collagen tissue remaining.13,19
Figure 1 Palmar fascia arrangement within the hand. RA ¼ radial aponeurosis, UA ¼ ulnar aponeurosis, PA ¼ palmar aponeurosis, NL ¼ natatory ligament, TLPA ¼ transverse ligament of the palmar aponeurosis (Reprinted with permission from Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 1: 190e198).
Anatomy The retinacular system of the hand comprises the transverse carpal ligament, palmar fascia, natatory ligaments, pulley system
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Figure 2 Three-dimensional structure of the central aponeurosis. s ¼ skin, lf ¼ longitudinal fibres, tf ¼ transverse fibres, t ¼ flexor tendon, dtl ¼ deep transverse ligament, slj ¼ septa of the fibres of Legueu and Juvara, m ¼ metacarpal.
transverse ligament of the palmar aponeurosis and insert into a complex comprising of the palmar plate, sagittal band, A1 pulley, interpalmar plate and septa of Legueu and Juvara (Figure 3).14 The septae form fibro-osseous tunnels, four of which contain the flexor tendons and three webspace channels, house the lumbricals and neurovascular bundles (Figures 2 and 3). The radial aponeurosis consists of the fascia over the thenar musculature, a pretendinous band (that may be absent) and the proximal and distal commissural ligaments. Constituents of the ulnar aponeurosis are the hypothenar muscle fascia, pretendinous band to the small finger and abductor digiti minimi fascial strands. The digital fascia comprises the natatory ligaments, lateral digital sheaths, Grayson’s and Cleland’s ligaments (Figure 4). The former span the digital webspaces and lie superficial to the
Figure 4 a Components of normal digital fascia that produce b the spiral cord. 1 ¼ pretendinous fascia, 2 ¼ spiral band, 3 ¼ lateral digital sheet, 4 ¼ Grayson’s ligament. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
transverse metacarpal ligament. They are composed of transverse and curved fibres with the latter joining the lateral digital sheath. The lateral digital sheaths are believed to be thickenings of the superficial digital fascia on either sides of the finger and receive contributions from the spiral bands, natatory and Grayson’s ligaments. Grayson’s ligaments run from the skin to the flexor tendon sheath and prevent bowstringing of the underlying neurovascular bundle upon digital flexion.21 In contrast to Grayson’s ligaments, Cleland’s ligaments lie dorsal to the neurovascular bundles. They arise from either side of the proximal interphalangeal joints of the fingers (interphalangeal joint in the thumb) to the distal interphalangeal joints and insert into the skin.21
Pathological anatomy in Dupuytren’s contracture With a detailed understanding of normal palmar and palmodigital fascia of the hand, the lesions of Dupuytren’s disease can be seen to follow this well defined anatomy. Pathological cords include the pretendinous or vertical cords in the palm, the spiral and natatory cords extending from the palm of the hand into the fingers, and the central retrovascular and lateral cords in the digits (Table 1) (Figure 5). The pretendinous cord is a thickening of the pretendinous band (layer 1) of the palmar aponeurosis and is the most common cord in Dupuytren’s contracture. The cord attaches to the skin within the distal palmar crease causing MCP joint flexion contracture. Occasionally, rather than passing into a single digit, the cord bifurcates into a “y” cord with each limb attaching into an adjacent finger. Owing to its superficial course,
Figure 3 Soft tissue confluence and septa of Legueu and Juvara. IPPL ¼ interpalmar plate ligament, PA ¼ palmar aponeurosis (Reprinted with permission from Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 190e198).
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Origin of diseased cords in Dupuytren’s disease and their clinical significance. (Reprinted with permission from Shaw RB, Chong AKS, Zhang A Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis and treatment. Plast Recon Surgery 2007; 120(3): 44ee54e) Disease structure Palmar cords Pretendinous cord Vertical cord
Palmo-digital cords Spiral cord Natatory cord Digital cords Central cord Retrovascular cord Lateral cord Abductor digiti minimi cord
Thumb and first web Proximal commissural cord Distal commissural cord Thumb pretendinous cord
Origins Pretendinous band
Uncommon, from diseased vertical fibres of McGrouther or septa of Legueu and Juvara Pretendinous band, spiral band, lateral digital sheet and Grayson’s ligament Distal fibres of natatory ligament Extension of pretendinous cord in the finger Retrovascular band of Thomine Lateral digital sheet, often associated with pretendinous and natatory cord Abductor digiti minimi tendon Proximal commissural ligament Distal commissural ligament Pretendinous band
Clinical significance Most common cord in hand; causes MCP flexion contracture; does not displace neurovascular bundle May cause painful triggering Displaces the neurovascular bundle palmarly and to the midline, creating a “spiral nerve” Web space contracture limiting digital abduction Does not usually displace the neurovascular bundle Causes DIPJ contracture and prevents full correction of PIPJ contracture Flexion contracture of PIPJ and DIPJ; displaces neurovascular bundle to midline Flexion contracture of PIPJ First web contracture First web contracture MCP contracture
Table 1
the neurovascular bundles are not usually displaced by this cord. Vertical cords are diseased septa of Legueu and Juvara and are relatively uncommon. They form a small cord emanating from the pretendinous cords and run between the neurovascular bundle and flexor tendon sheaths.3 Disease affecting the natatory ligaments, natatory cords, within the palmo-digital fascia can be seen within the second to
fourth webspaces. The normal U shape of the webspace is lost, thereby limiting digit abduction. These cords tend to run volar to the neurovascular bundles thereby sparing any displacement. The spiral cord arises from the pretendinous band and follows layer 2 of the palmar fascia to the lateral digital sheath and attaches to the middle phalanx via Grayson’s ligament (Table 1 and Figure 5). From its origin, the spiral cord passes dorsal to
Figure 5 How Dupuytren’s disease affects normal digital fascia. Left: normal digital fascial components. Right: Diseased tissue that results in joint contracture. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
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the neurovascular bundle just distal to the MCP joint. It then passes lateral to the artery and nerve as it enters the digit and finally runs superficial as it attaches into Grayson’s ligament. Owing to this protracted course, with worsening flexion contracture of the proximal interphalangeal (PIP) joint, secondary to cord thickening and shortening, the neurovascular bundle displaces proximal, superficial and towards the midline of the digit (Figure 6). This displacement accounts for the increased susceptibility to injury during surgical contracture releases of this joint. Within the small finger, the spiral cord is known as the abductor digit minimi cord as it arises from this muscle and inserts at the base of either the middle or distal phalanges. Running volar to the neurovascular bundle, this cord can cause displacement of the neurovascular bundle from its usual lateral location. Within the digit, the central cord is an extension of the pretendinous cord of the palm and attaches to the ulnar base of the middle phalanx or flexor tendon sheath. Owing to its midline course, it rarely displaces the neurovascular bundle.22 The lateral cord arises from the lateral digital sheath and attaches via Grayson’s ligament to the palmar skin or flexor tendon sheath, thereby aiding in PIP joint contracture and occasionally distal interphalangeal joint flexion contracture (Figure 5). Often present with the pretendinous and natatory cords, the lateral cord can cause midline displacement of the neurovascular bundle.3 The retrovascular cord is located dorsal to the neurovascular bundle.14 Originating from the diseased retrovascular cord of Thomine, this cord needs to be excised to achieve full PIP joint contracture release. In the thumb, adductor contracture development of the first web space is related to disease of the proximal commissural ligament ( proximal commissural cord ) and distal commissural ligament (distal commissural cord ). Metacarpophalangeal joint contracture is primarily attributed to thumb pretendinous cord
development with a contribution from the thenar cord, a thickening of the thenar fascia.
Clinical presentation and differential diagnosis Dupuytren’s disease typically presents in a 50-year-old Caucasian male who has had palmar fascia contracture for several years that is beginning to affect his activities of daily living or the appearance of the hand. The disease usually starts proximally and progresses distally into the digits, affecting in decreasing order, the ring finger, followed by the small, middle, index fingers and the thumb. On the palmar surface, changes that can be seen include skin pits, nodules and cord formation. The former are related to thickening of the Grapow fibres which connect the dermis to the palmar fascia. The skin loses its mobility as it becomes increasingly tethered to the fascia. Commonly seen in the palm distal to the distal palmar crease, skin pits are the earliest sign of the disease. Dupuytren nodules originate from the superficial palmar or digital fascia and mark the intermediate stage of the disease.14 They are well defined thickenings of the underlying deep fascia and can be symptomatic if they abut upon the underlying flexor tendons inducing a tenosynovitis reaction. Palmar nodules usually arise at the distal palmar crease whereas digital nodules are seen at the base of the digit or the PIP joint.3 As nodules regress, they are typically replaced by pathologic cords (as discussed earlier). This marks the onset of the late phase of the disease, and as they mature, form distinct band like structures within the hand. Contracture development usually progresses from a proximal to distal direction, first affecting the MCP joint of the ring finger followed by ipsilateral PIP joint involvement. This is by no means absolute and can be confined to just the palm or digit. Dupuytren’s disease can manifest with dorsal skin changes such as Garrod’s node (PIP joint nodules) or knuckle pad
Figure 6 Neurovascular bundle displacement proximally, palmarly and towards the midline with increasing PIP joint contracture thereby increasing its susceptibility to injury during surgery. a By a spiral cord, b by spiral and central cords, c by the pretendinous, natatory and lateral cords, d by a communication between Grayson’s ligament and the central cord and e by the spiral and natatory cords. (Reprinted with permission from MacFarlane RM: Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Plast Reconstr Surg 1974; 54: 31e44).
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(PIPJ fibroma like lesions) formation (Figure 7).23 They tend to be seen in patients with bilateral disease and in patients with similar lesions like on the feet or genitalia. Other conditions may mimic Dupuytren’s disease, especially early in the disease process. These include hyperkeratosis, callus formation, inclusion cysts, rheumatoid nodules, palmar fibromatosis, ganglions, soft tissue giant cell tumours (pigmented villonodular synovitis) and epithelioid sarcomas.
Non-operative treatment of Dupuytren’s disease Observation is indicated for patients whose Dupuytren’s disease is relatively inactive with mild contracture or limited functional compromise. In this case, patients need to be monitored since the disease is one of gradual progression at various rates.25 Other modalities such as the use of intermittent splinting, radiation and vitamin E are limited in terms of their applicability and lack data demonstrating efficacy. More recently, there has been a resurgent interest in the use of intralesional injection therapy for early and late disease. Ketchum and Donahue26 evaluated the efficacy of steroid injections to soften and flatten Dupuytren nodules. Using triamcinolone acetonide intralesional injections, the authors reported after an average of 3.2 injections per nodule, 97% of the hands showed regression of disease. This effect appeared to be temporary, however, as half of the patients developed reactivation of the disease in the nodules 1e3 years after the last injection. Given the excessive collagen deposition seen within this condition, efforts have been made to use targeted enzymatic collagen degradation as a potential treatment modality. This was popularized by Badalamente and Hurst27 who tested the safety and clinical efficacy of intralesional clostridial collagenase injections as a non-surgical treatment of Dupuytren’s disease. Twenty-nine patients received collagenase injections (10 000 units) into contractures of 34 MCP joints, nine PIP joints and one thumb. Results showed that 82% of MCP and 44% of PIP joint contractures corrected to full extension within 2 weeks of injection. In a recent prospective randomized double blind controlled trial,28 23 patients with flexion deformities of the MCP and/or PIP joints of 20 or more were randomized to receiving placebo or collagenase injections. Clinical success, as defined as joint contracture being within 5 of full extension, was achieved in 70% of patients receiving one injection and in 91% patients receiving three injections. None of the placebo treated patients achieved joint correction. Currently, the Food and Drug Administration is evaluating the potential of collagenase for widespread clinical use as concerns exist regarding potential complications such as local extravasation, haematoma, swelling and toxicity to adjacent tendons, neurovascular structures and skin. Percutaneous needle aponeurotomy can be used as a one stage or part of a staged procedure (with weekly intralesional steroid injections) for cord disruption within the office setting. Popularized by French rheumatologists, this procedure is gaining increasing demand due to perceived limited invasiveness, good outcomes, fast recovery and overall patient satisfaction. This was further highlighted by Van Rijssen and co-workers who conducted a randomized controlled trial comparing needle aponeurotomy (88 digits) to limited fasciectomy (78 digits).29 At 6 weeks post treatment, patient satisfaction between both treatments was similar but function, as demonstrated by improved DASH scores, and complication rates were better in the percutaneous needle fasciotomy group. At longer follow-up, recurrence of deformity was more common after percutaneous treatment. This was further demonstrated by van Rijssen and Werker30 who reported immediate contracture improvement in over 75% of patients who underwent 74 percutaneous needle releases. By 32 months, however, recurrence was noted in 65% of patients demonstrating that this procedure has good short-term effects and may be suitable
Treatment options of hand contracture in patients with Dupuytren’s disease Numerous treatment options exist in the management of this disease ranging from simple observation to radical palmar fasciectomy. When balancing the advantages and disadvantages of any treatment option, it is important to consider several factors including the fingers’ deformity, loss of function, rate of disease progression and patient factors like occupation and comorbidities.18 The deformity of the fingers may be cosmetic only or could be significant enough to affect activities of daily living. Though surgical correction has been widely advocated for a flexion contracture of more than 30 at the MCP joint and/or any contracture at the PIP,24 treatment principles are better guided on the patient’s functional difficulty and the rate of deformity progression. A patient with a quiescent contracture may be more amenable to non-operative treatment compared to one with clear deterioration.
a and b Photograph demonstrating knuckle pads on the dorsum of the hand. Figure 7
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for patients who desire a minimally invasive approach or can be used to delay formal surgical fasciectomy. Of note, two patients experienced evidence of partial digital nerve injury but did elect to undergo any further intervention. Given these encouraging results, caution needs to be given as to which cords are amenable to this technique. Complications such as digital nerve injury, flexor tendon damage, infection, recurrence, complex regional pain syndrome (CRPS) and skin breakdown mean that this procedure may be best suited for isolated palmar or well defined central digital cords as opposed to the digits containing spiral cords.
covered with full thickness grafts. This technique is best indicated for recurrent and severe primary disease when the skin is adherent to Dupuytren tissue. Multiple options exist for skin incisions and can be categorized into the longitudinal and transverse types. The longitudinal incisions can be divided into a straight line incision with multiple Z plasties, Brunner type incisions, multiple V-Y advancement flaps and Moerman’s small curved incisions.18 They have the advantage over transverse incisions as they provide extensile exposure and address the skin shortage secondary to contracture.18,31 When raising the skin flaps, caution should be followed to ensure that the flaps are not too thin for fear of skin necrosis. At the palmo-digital junction, great care should be practised to try and identify the neurovascular bundles before cutting down deep onto the pathological cords. Dissection should be carried out from proximal and distal directions, thereby allowing an easier and safer way to identify the neurovascular bundles and prevent their inadvertent injury. Transverse incisions across the palm and/or digits permit access to the diseased tissue without providing a pathway for secondary scar contracture. Commonly known as the open palm technique, these wounds are either left open and allowed to heal via secondary intention or closed with a full thickness skin graft. This technique is primarily indicated for recurrence associated with considerable contracture and induration of the palm rather than for primary disease. Several different options exist for management of the fascia (Table 2). These range from simple fasciotomy to more radical fasciectomy. Fasciotomy is aimed at releasing the longitudinal fascia tension lines without removal of diseased tissue. Advised for patients with isolated palmar longitudinal central cords or in whom anaesthesia is best avoided, this procedure is associated with a high recurrence rate (up to 43%), injury to nerves and complex regional pain syndrome.14,35 Limited fasciectomy is a procedure whereby a portion of the diseased cord is excised. As reported by Rodrigo et al.35, postoperative stiffness and haematoma formation were common as well as the high recurrence rate. Sixty-three percent of patients reported recurrent deformity of which 15% required additional treatment. In light of these high recurrence rates, the most widely used procedure is regional fasciectomy. Through a longitudinal incision, full thickness skin flaps are raised from the palm to the digit. The longitudinal central cord is dissected as far proximally as needed, divided and
Surgical treatment of Dupuytren’s disease When surgical correction is planned, the patient must have clear expectations and understanding of the nature of the disease and the postoperative and rehabilitative course involved. There is no such surgery that cures the disease, but the main purpose of the surgery is to correct the deformity whenever possible. The postoperative rehabilitation may take at least 3 months before patients regain their hand function depending on the severity of the contracture before surgery and type of patients. For instance, patients with comorbidities such as cerebrovascular accident or Parkinson’s disease may lack compliance towards their hands’ rehabilitation compared to other patients. A very common postoperative complication that all patients should also be informed about is the recurrence rate after surgery which can be as high as 50% after 10 years.18 Once the decision has been made to proceed to surgery, the plan must target three fundamental areas, namely management of the skin, the fascia and the joints.18,31 In terms of the first, the surgeon needs to decide whether the type of skin incision will allow adequate exposure of the diseased tissue or whether the contracture is so far advanced that skin excision is preferable. Skin excision or dermofasciectomy is a technique whereby the diseased fascia and overlying skin are excised en bloc and combined with skin grafting. Popularized by Hueston,32,33 this technique serves three purposes: replace myofibroblast populated dermis with normal skin, replace the skin shortage and also interpose a piece of healthy skin between diseased tissue, thereby disrupting lines of longitudinal tension (so called “firebreak”). Ketchum and Hixson34 reported on their 15-year experience of this technique and noted no recurrence of disease in the palms and digits that were
Fascial treatment options. (Reprinted with permission from Shaw RB, Chong AKS, Zhang A Hentz VR, Chang J. Dupuytren’s disease: history, diagnosis and treatment. Plast Recon Surgery 2007; 120(3): 44ee54e) Surgical technique Fasciotomy Local fasciectomy Regional fasciectomy Radical fasciectomy Open palm technique (McCash) Dermo-fasciectomy
Description Division of diseased cords without excision; it can be performed percutaneously Removal of some diseased tissue More extensive dissection and removal of involved tissues Removal of all the palmar fascia with extension into the involved fingers; the extensive dissection and skin flaps required increases the risks of complications Transverse skin incision and division of the aponeurosis, leaving the palm wound open; requires prolonged postoperative splintage Removal of the skin and fascia followed by coverage with skin grafts
Table 2
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elevated in a proximal to distal direction. Knowledge of the three layers of the palmar fascia becomes crucial to ensure all the diseased tissue is excised and the cord raised in a safe manner, with care taken to identify and protect the displaced neurovascular bundles. Within the digit, the cord is followed to its distal attachment and divided. If the joint contracture is released, surgery is discontinued. If contracture persists, however, additional cords need to be excised and consideration given to joint release. Radical fasciectomy is the process whereby all diseased fascia as well as uninvolved tissue is excised to prevent disease recurrence. The aim in the palm is to remove all of longitudinal fibres to the digits. This entails the raising of large skin flaps with the subsequent risk of increased wound healing complications and postoperative stiffness. Because of these reported risks, this procedure is advocated in patients who have extensive disease or an increased diathesis. Residual joint contracture should be addressed if the digit remained contracted after the skin is released and the fascia excised. Interestingly, almost all MCP joint contractures tend to be corrected after the fascial release procedure as this joint can tolerate prolonged immobilization in flexion due to the CAM effect of the collateral ligaments. For this reason, MCP flexion contracture is not an urgent indication for surgery. The PIP joint, however, is very intolerant to prolonged flexion and simple fascial excision may not be sufficient to resolve this contracture. MacFarlane conceptualized PIP joint involvement in Dupuytren’s disease as either primary or secondary flexion contractures.36 Primary contractures were directly attributed to fascial disease whereas secondary flexion contractures were due to either periarticular adhesions, contracture of the flexor sheath, shortening of the palmar skin or flexor muscles, contracture of the palmar plate, checkrein ligaments, collateral or accessory collateral ligaments.36,37 In patients with greater than 60 PIP joint contracture, Smith and Breed38 reported that this may be due to central slip attenuation. If the joint remains contracted after fasciectomy, splinting of the digit in full extension may help to restore the tone of the central slip. Draviaraj and Chakrabarti39 reported on 30 patients who underwent MCP and PIP joint corrective surgery over a 1 year time frame. The average preoperative MCP and PIP joint deformity was 31 and 35 respectively. Postoperatively, this corrected to 19 at the MCP and 16 at the PIP joint. Hand function tests demonstrated improved function between the total correction, PIP joint correction, and hand function up to 1 year, but not with MCP joint correction. Despite the theoretical causes of PIP joint flexion contracture, Weinzweig et al.40 failed to show any advantage to capsuloligamentous release in addition to fasciectomy in treating severe PIP joint contracture (greater than 60 ). Eighteen patients underwent fasciectomy alone and ten patients underwent capsulotomy in addition to fasciectomy. In the non-capsulotomy group, preoperative contracture averaged 78 and improved to 36 postoperatively. In the capsulotomy group, preoperative measurements averaged 82 and averaged 36 after surgery. No statistically significant difference was seen in the percentage of contracture correction in the capsulotomy group compared with the non-capsulotomy group at final follow up. In addition, the complication rate was higher in the capsulotomy group. Interestingly, the authors reported that the degree of correction
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obtained at surgery using either method was not maintained during follow up. This was similarly reported by Donaldson and co-workers.41 Fifty-two patients were followed prospectively for 18 months after undergoing fasciectomy for Dupuytren’s disease. Of the 42 MCP joints, full intraoperative correction was obtained in 41. Of these, 37 had full correction at final follow up. Of the 58 PIP joints treated, full intraoperative correction was obtained in 35 cases. After 18 months, less than 38% maintained their correction. The authors concluded that the degree of preoperative deformity and intraoperative correction, especially for the PIP joint, were significant predictors of loss of surgical correction and recurrence of the deformity after surgery. Postoperative rehabilitation The postoperative rehabilitation program is an integral and essential part of the management of Dupuytren’s disease after surgical correction. It is directed towards restoring hand function and preventing complications after surgery. In the immediate postoperative period, the hand is immobilized with the MCP and PIP joints fully extended or in the position of function with the MCP joints flexed and the PIP joints extended. After 3e5 days, once the early inflammatory process has subsided, active motion is begun with night splinting to preserve extension. Early involvement of a hand therapist is advised to aid with range of motion exercises, oedema control, scar massage and early treatment of CRPS. When PIP joint correction has been incomplete, dynamic extension splintage can be introduced. Rives et al.42 treated 23 joints (contracture greater than 45 ) with surgical correction and 6 months of splinting. After 2 years, a 44% improvement was noted in PIP joint contracture suggesting that the soft tissues are able to remodel with time. Postoperative complications Despite the meticulous technique that is usually applied in Dupuytren’s surgery, the reported complication rates are still as high as 17%.18 They are more likely to be encountered in patients with severe or recurrent disease and include intraoperative complications like neurovascular injury and buttonholing of the skin or postoperative complications like haematoma, skin necrosis, infection, dehiscence and CRPS. The incidence of neurovascular injury is approximately 3% and is increased in patients where the bundle is displaced due to a spiral cord or in revision surgery due to abundant scar formation.14,18 This risk is decreased if one approaches the diseased tissue from proximal to distal and distal to proximal directions to ensure the neurovascular bundle can be identified and isolated. Loss of digital artery circulation can result from spasm secondary to stretch, intimal haemorrhage, direct injury or vessel rupture due to overzealous joint contracture correction. When such a compromise is due to spasm or intimal damage, the digit is repositioned to its original resting attitude and the artery bathed in warm saline and a topical vasodilator such as papaverine. However, if direct arterial damage is identified, then a primary repair or use of an interpositional vein graft, should be performed. Postoperative haematoma formation can be addressed by deflating the tourniquet and use of bipolar cautery for haemostatsis. The use of adjuncts such as thrombin spray and drains may lessen their formation. If a haematoma develops, urgent evacuation is recommended to decrease the risk of skin necrosis or infection.
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13 Luck JV. Dupuytren’s contracture; a new concept of the pathogenesis correlated with surgical management. J Bone Joint Surg Am 1959 Jun; 41-A: 635e64. 14 Rayan GM. Dupuytren disease: anatomy, pathology, presentation and treatment. J Bone J Surg Am 2007; 89A: 190e8. 15 Badalamente MA, Hurst LC. The biochemistry of Dupuytren’s disease. Hand Clin 1999; 15: 35. 16 Murrell GA, Francis MJ, Bromley L. Modulation of fibroblast proliferation by oxygen free radicals. Biochem J 1990 Feb 1; 265: 659e65. 17 Badalamente MA, Sampson SP, Hurst LC, Dowd A, Miyasaka K. The role of transforming growth factor beta in Dupuytren’s disease. J Hand Surg Am 1996; 21(2): 210e5. 18 McGrouther DA. Dupuytren’s contracture. In: Green DP, Hotchkiss RN, Pederson WC, Wolfe SW, eds. Green’s operative hand surgery. New York: Churchill Livingstone, 2005: 159e85. 19 Tomasek JJ, Vaughan MB, Haaksma CJ. Cellular structure and biology of Dupuytren’s disease. Hand Clin 1999; 15: 21e34. 20 McGrouther DA. The microanatomy of Dupuytren’s contracture. Hand 1992; 14: 215e36. 21 Milford LW. Retaining ligaments of the hand. Philadelphia: WB Saunders, 1968. 22 Barton. Dupuytren’s disease arising from the abductor digiti minimi. J Hand Surg Br 1984; 9: 265e70. 23 Garrod AE. On an unusual form of nodule upon the joints of the fingers. St Bartolomews Hosp Rep 1893; 29: 157e61. 24 Smith AC. Diagnosis and indications for surgical treatment. Hand Clin 1991 Nov; 7: 635e42. 25 Rayan GM. Nonoperative treatment of Dupuytren’s disease. J Hand Surg Am 2008; 33: 1208e10. 26 Ketchum LD, Donahue TK. The injection of nodules of Dupuytren’s disease with triamcinolone acetonide. J Hand Surg Am 2000; 25: 1157e62. 27 Badalamente MA, Hurst LC. Enzyme injection as nonsurgical treatment of Dupuytren’s disease. J Hand Surg Am 2000 Jul; 25: 629e36. 28 Badalamente MA, Hurst LC. Efficacy and safety of injectable mixed collagenase subtypes in the treatment of Dupuytren’s contracture. J Hand Surg Am 2007; 32: 767e74. 29 van Rijssen AL, Gerbrandy FS, Ter Linden H, Klip H, Werker PM. A comparison of the direct outcomes of percutaneous needle fasciotomy and limited fasciectomy for Dupuytren’s disease: a 6-week follow-up study. J Hand Surg Am 2006; 31: 717e25. 30 van Rijssen AL, Werker PM. Percutaneous needle fasciotomy in Dupuytren’s disease. J Hand Surg Br 2006; 31: 498e501. 31 Saar JD, Grothaus PC. Dupuytren’s disease: an overview. Plast Reconstr Surg 2000; 106: 125e34. 32 Hueston JT. Dermofasciectomy for Dupuytren’s disease. Bull Hosp Jt Dis Orthop Inst 1984; 44: 224e32. 33 Hueston JT. ‘Firebreak’ grafts in Dupuytren’s contracture. Aust N Z J Surg 1984; 54: 277e81. 34 Ketchum LD, Hixson FP. Dermofasciectomy and full-thickness grafts in the treatment of Dupuytren’s contracture. J Hand Surg Am 1987; 12: 659e64. 35 Rodrigo JJ, Niebauer JJ, Brown RL, Doyle JR. Treatment of Dupuytren’s contracture. Long-term results after fasciotomy and fascial excision. J Bone Joint Surg Am 1976; 58: 380e7. 36 MacFarlane RM. Patterns of the diseased fascia in the fingers in Dupuytren’s contracture. Displacement of the neurovascular bundle. Plast Reconstr Surg 1974; 54: 31e44. 37 Crowley B, Tonkin MA. The proximal interphalangeal joint in Dupuytren’s disease. Hand Clin 1999; 15: 137e47.
Complex regional pain syndrome (CRPS) has been reported as high as 5% and is more common in woman and in patients undergoing simultaneous carpal tunnel release. The aetiology is uncertain and may be due to overzealous handling of the neurovascular bundles during surgery. Once diagnosed, treatment is directed to manage the CRPS with hand therapy, oral medications like steroids or carbamazepine, or sympathetic blockade.14,31,43 Recurrence of contracture is by far the most common complication with ranges from 2% to 60%.43 It is more commonly seen in patients with PIP joint contracture, multiple digit involvement, small finger involvement, advanced disease stage, comorbidities such as diabetes and in patients with Dupuytren diathesis.14,31,43
Conclusion Management of hand contracture in patients with Dupuytren’s disease still poses numerous challenges to hand surgeons, despite advances in medical science and surgical techniques. Surgical correction remains the mainstay of treatment and can result in excellent improvement in hand function. Less invasive techniques like needle aponeurotomy is an alternative to surgery and to date is largely patient driven because of quicker recovery times. Collagenase treatment offers promise for the future, either as a stand alone treatment or as an adjunct to other techniques. A
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38 Smith P, Breed C. Central slip attenuation in Dupuytren’s contracture: a cause of persistent flexion of the proximal interphalangeal joint. J Hand Surg Am 1994; 19: 840e3. 39 Draviaraj KP, Chakrabarti I. Functional outcome after surgery for Dupuytren’s contracture: a prospective study. J Hand Surg Am 2004; 29: 804e8. 40 Weinzweig N, Culver JE, Fleegler EJ. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease: combined fasciectomy with capsuloligamentous release versus fasciectomy alone. Plast Reconstr Surg 1996; 97: 560e6.
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41 Donaldson OW, Pearson D, Reynolds R, Bhatia RK. The association between intraoperative correction of Dupuytren’s disease and residual postoperative contracture. J Hand Surg Eur Vol 2010; 35: 220e3. 42 Rives K, Gelberman R, Smith B, Carney K. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease: results of a prospective trial of operative correction and dynamic extension splinting. J Hand Surg Am 1992; 17: 1153e9. 43 Boyer MI, Gelberman RH. Complications of the operative treatment of Dupuytren’s disease. Hand Clin 1999; 15: 161e6.
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