Epidemiology
Dupuytren disease is a fibroproliferative disorder of unclear etiology and pathogenesis.
It is a disease that occurs primarily in men with Celtic ancestry. In Australia, as many as 20% of men older than 60 years are affected. This condition is rare in Asian individuals and very rare in African Americans. The male-to-female ratio is 5:1 and one third of patients have a positive family history. Usually, both hands are involved, with unilateral disease an uncommon variant.
Etiology
A strong correlation exists with Peyronie disease. Dupuytren disease is not an etiologically uniform disease. Abnormal chromosomal patterns (mosaicism) may be the cause of a fundamental abnormality in fibrogenesis. The Murrell hypothesis states that localized fat hypoxia in the palmar tissue results in the release of oxygen free radicals by the reaction of xanthine oxidase and oxygen. Myofibroblasts have been demonstrated to proliferate after stimulation by oxygen free radicals. Treatment with allopurinol is suggested.
Associated conditions include the following:
- Knuckle pads (or Garrod nodes), plantar fibromatosis (Lederhose disease), Peyronie disease: Because these often are observed in association, the term Dupuytren disease is used rather than contracture. Knuckle pads frequently are observed (1% incidence in Dupuytren) and do not result in proximal interphalangeal joint (PIP) contracture. These 3 associated conditions have an approximate incidence of 1% of patients with Dupuytren disease.
- Diabetes: The incidence rises with the duration of diabetes to 80% of patients who have had diabetes for more than 20 years. It is usually mild, with palmar fascial thickening rather than contracture. This may be related to a microangiopathy.
- Epilepsy: Dupuytren disease may be a sequela of long-term phenobarbitone administration. A low incidence is found prior to the use of the drug.
- Alcoholism: Similarly to patients with diabetes, actual contracture is rare.
- Chronic pulmonary disease is associated.
- Trauma: Recurrent microtrauma has been demonstrated to be related, as have unrelated injuries or wounds.
Pathology
The fibromatoses as a group are intermediate between benign fibrous lesions and fibrosarcoma. Dupuytren disease is a quasi-neoplastic proliferative disorder. The cell of Dupuytren is the fibroblast in the early stages, whereas the myofibroblast is the principal cell in the contractile phase. Fibrocytes occasionally are observed in cords of long-standing contracture. Myofibroblasts derive from pericytes following a hypoxic stimulus (luminal occlusion).
In addition to having contractile properties, myofibroblasts are capable of manufacturing collagen and elastin. In Dupuytren disease, types III, V, and type I trimer collagen are increased (as in healing wounds). The collagen types are no different than those found in normal healing wounds. Biochemical changes (ie, increased chondroitin sulfate and dermatan sulfate) are found throughout the palmar fascia, underlying the multifocal nature of the disease. The biochemical changes may be secondary to mechanical loading of the soft tissues, rather than a primary abnormality.
Pathogenesis
The pathognomonic lesion is the nodule, which is usually adjacent to the distal palmar crease. It is the site of the contractile process and may be multiple. Three stages are as follows:
- Early: This stage involves thickening and nodularity of the fascia, prominence of type III collagen, and blanching of palmar skin on finger extension.
- Active: This stage is marked by the initiation of contraction. The first clinical sign of contraction is a palpable cord proximal to a nodule. Grooves and pits demonstrate attachment of palmar skin to fascia, and joint contraction follows. Myofibroblasts are dominant. Types III and V collagen are present, and glycosaminoglycans are increased.
- Advanced: Nodules have disappeared, leaving joint contracture and firm tendonlike cords. Type I collagen is present.
In 1985, Flint noted fibrous replacement of subdermal fat in Dupuytren disease and argued that this was the primary abnormality. Loss of cushioning resulted in damage to the longitudinal fibers of the fascia, and the fibrotic reaction in the palmar fascia represents a reparative response.
Mechanism of contraction
Myofibroblasts contain actin and myosin filaments and strong intercellular bonds that permit a synchronized contraction process. Once the dermis is fixed to the palmar fascia, the normal hand movements result in continuation of the process to a severe contracture.
Anatomy
The only two components of the palmar fascia to become involved are the pretendinous band and the natatory ligament.
The former is the only structure that results in metacarpophalangeal (MP) joint contracture. According to Luck, normal fascial tissue is referred to as bands and diseased tissue is referred to as cords. The pretendinous bands of the palmar aponeurosis run longitudinally down the hand. The band to the index finger frequently ends in the skin on the radial side of the hand; the band to the thumb is inconsistent. The insertion of the pretendinous bands is to the skin distal to the distal palmar crease and by means of a bifurcate insertion into the side of the finger dorsal to the neurovascular bundle. The natatory ligament runs transversely across the hand distal to the MP joints into the first web space, giving fibers that blend with the lateral digital sheet to each digit and attaching to the superficial aspect of the flexor tendon sheath. The superficial transverse ligament lies deep to the pretendinous bands, proximal to the MP joints and the natatory ligament.
In the fingers, only the superficial fascial structures become involved (Cleland and Landsmeer ligaments are spared), as follows:
- Spiral band (runs from pretendinous band to lateral digital sheet dorsal to the neurovascular bundle)
- Lateral digital sheet
- Grayson ligament
- Superficial volar fascia
Knuckle pads develop in the dorsal subcutaneous wrinkle ligaments of McGrouther. The spiral cord originates from the pretendinous band and winds around the neurovascular bundle in disease. The central, lateral, and spiral cords terminate in the tendon sheath and adjacent middle phalanx. One or more may be found in any patient, but seldom on both sides of the finger. The disease process in the finger results in the formation of the following 4 cords:
- Central cord (most common cause of PIP contracture)
- Lateral cord (rarely observed, except on ulnar aspect of little [L] finger)
- Spiral cord (particularly in ring [R] and L fingers)
- Retrovascular cord
Spiral and lateral cords displace the neurovascular bundle toward the mid line, while the central cord encases the bundle and usually is directed toward one or the other side of the mid line of the finger. The spiral cord is made up of (1) the pretendinous band, (2) the spiral band, (3) the lateral digital sheet, and (4) Grayson ligament.
The retrovascular cord of Thomine is an oft-neglected part of the disease and a common cause of postoperative failure. With increasing flexion contracture, the neurovascular bundle comes to lie more superficial and more proximal.
The thumb may be involved by 1 of 3 fascial structures: the natatory ligament, the pretendinous band, or the superficial transverse ligament of the palm.
In short, the cardinal features are the nodule, the cord, and the digital flexion contracture.
Treatment
Incision or excision of the diseased fascia is the only available treatment to correct joint contracture. However, surgery does not cure the disease. Dupuytren disease is usually no more than an inconvenience, thus the need for treatment must be tailored to the individual.
Indications
The presence of the disease does not constitute an indication for operation. This depends on the severity of the contracture and the joint involved. Tenderness is seldom a symptom since it soon resolves. When the tabletop test is positive, begin thinking about surgery. The goals of treatment, in order, are improvement of functional capability, reduction of deformity, and lessened recurrence. Presently, the latter is clearly unattainable although a skin graft prevents local recurrence. The incurability of the disease should be communicated clearly to the patient because it may affect the decision of initial operation.
- MP flexion contracture of 30° is a universally accepted criterion for surgical correction. At this point, the finger starts getting in the way. Correction of MP flexion contractures is never urgent. The expectations of full and final correction are good for flexion contractures of up to 60°.
- PIP flexion contractures need to be addressed early. The patient is best advised to have an operation as soon as the contracture appears. Complete correction is less common and recurrence is likely. Therefore, PIP flexion contracture constitutes an indication for operation.
- Correction of thumb deformities usually can wait for deformities to develop elsewhere in the hand.
Types of surgery
All techniques fall into 1 of 3 groups: fasciotomy, regional fasciectomy, and extensive fasciectomy. Three decisions must be made: (1) type of operation needed, (2) type of incision required, and (3) method of wound closure employed.
Two surgical principles are the release of longitudinal tension and the management of the skin. At the heart of the problem is the question of recurrence since curing Dupuytren disease is impossible. The continued ability to produce Dupuytren tissue within an operated area (ie, recurrence) or without (ie, extension) is dictated by the patient's Dupuytren diathesis. The second factor is ongoing tension on the cord. More often it seems that the patient's disease, not the surgeon, dictates recurrence.
Fasciotomy
- In the closed fasciotomy, the fascia simply is incised via the skin incision. Sir Astley Cooper popularized the technique of closed fasciotomy. This technique is successful for MP contracture since only the pretendinous band needs to be incised. It is of little use in the PIP contracture since more than one cord is involved and the danger of neurovascular damage exists. Closed fasciotomy seldom is performed anymore because of the risk of neurovascular damage. The remaining indication is perhaps in patients who are debilitated to facilitate hand toilet or in the severe contracture to facilitate preparation of the skin prior to definitive release.
- Open fasciotomy is a better option for the finger since the structures can be visualized directly. The cord is divided at a point not underlying the skin incision. Division of the cord may not be sufficient for complete joint release, since the periarticular structures may need release. The procedure is performed under local anesthesia, recovery is rapid, and the patient usually regains full range of motion (ROM) quickly. The recurrence rate is high, thus open fasciotomy usually is reserved for patients who cannot tolerate a more extensive procedure.
- Segmental aponeurectomy of Moermans (1991) is a variant of open fasciotomy: 1-cm segments of fascia are excised through C-shaped incisions. Moermans claims that the band resolves once the tension across it is diminished. Dupuytren disease is not cancer. A prospective study performed by Andrew demonstrated a recurrence rate no higher than in other techniques, with fewer complications. This is an outpatient procedure.
Many of these procedures end up as regional fasciectomies since minimal dissection results in further exposure and resection of the band. Bear in mind that an interposed skin graft functions as a virtual firebreak, with no possibility of recurrence beneath it (Gonzalez principle).
Regional fasciectomy
In this technique, previously known as limited fasciectomy, only the diseased fascia is excised (in the palm, the pretendinous cords and the natatory ligament; in the fingers, only those cords obviously involved). This procedure is flawed because Dupuytren disease is a multifocal disease and likely to recur in other sites. Nevertheless, good results can be obtained. Limited fasciectomy is the most commonly performed procedure for Dupuytren disease. Gonzalez's limited open fasciectomy plus graft (1985) falls into this category (incision and excision of the diseased tissue, with grafting of the defect). Whether dermofasciectomy has an advantage over this remains to be proven, with this technique preserving more vital palmar skin.
Extensive fasciectomy
In extensive fasciectomy, perform as near complete removal of the fascia as possible. In the palm, the entire palmar aponeurosis and natatory ligament are excised. In the fingers, all cords and bands are excised.
McIndoe and Beare (1958) popularized the extensive palmar fasciectomy but hematoma formation with subsequent swelling and stiffness proved insurmountable. Currently, this is seldom performed. The more aggressive the fasciectomy, the more numerous the complications, and the risk of recurrence does not diminish with more aggressive surgery. McCash (1964) solved the hematoma problem by leaving the wound to close secondarily (open palm technique). A delayed skin graft appears to be a better solution. The extensive technique is used only when the entire palm is involved.
Dermofasciectomy
This is a more radical approach in which the fascia and overlying skin are excised completely and full-thickness skin is grafted. This approach is recommended for recurrence and aggressive primary disease.
Hueston recommends dermofasciectomy for digital disease. McFarlane criticizes this because it does not address the retrovascular cord, the unfavorable flexor sheath is left as the graft bed, and the diseased fascia can be separated from the overlying skin.
Hueston feels the only difference between conservative and radical surgery is in the degree of palmar dissection; the digital dissection remains the same in either.
Proximal interphalangeal joint contracture
If complete extension is not present following fasciectomy, the options are to rely on splintage ( <30°) or to perform a joint release (>30°). In the latter option, the flexor sheath is incised and accessory collateral ligaments and the proximal attachment of the volar plate are released, in that order, as necessary. Most consider capsulotomy meddlesome at the time of the original operation. Amputation is rarely, if ever, necessary, since prophylactic resurfacing with skin grafts has provided permanent local control of the disease. For severe PIP joint contracture, consider a Swanson arthroplasty if the contracture is due to scar and not Dupuytren disease. The concomitant shortening results in improvement of the contracture. PIP arthrodesis is not a good option since it further limits function.
Distal interphalangeal joint hyperextension
Hyperextension of the distal interphalangeal (DIP) joint usually occurs secondary to long-standing PIP joint contracture. No inherent disease is present in the DIP joint. The Landsmeer ligament becomes foreshortened. If passively correctable, it usually corrects with PIP correction. If not, division of Landsmeer ligaments corrects the deformity. DIP may not flex due to dorsal knuckle pads.
Incisions and wound closure
The best location for the skin incision is along the line of greatest dermal involvement (flaps are then thicker at their bases). Incisions are transverse or longitudinal, depending on the type of operation. McFarlane states the following:
- For a single finger a midline volar incision is made and closed with 3 Z-plasties.
- For the thumb a similar approach is used.
- For multiple finger and palmar disease a single transverse incision at the level of the distal palmar crease is made. This is joined to the longitudinal incision if necessary.
Technical points
Use loupe magnification and sharp dissection and change blades frequently. Most wounds are closed by direct suture or Z-plasty. The Z-plasty flaps are not designed until the time of closure so that they are placed in an area where skin viability is guaranteed. A midline scar along the volar aspect of the DIP joint never contracts. Full-thickness skin graft (FTSG) from the inner arm is preferred to split-thickness skin graft (STSG) because of contraction in the latter. Transverse wounds may be left open in the manner of McCash.
Dissection of the fascia off the skin may leave very thin skin flaps, particularly in the digits. The neurovascular bundle is best located distally. A midline digital incision is least likely to damage the neurovascular bundle. Dissecting retrovascular disease is important. Hueston always immobilizes in a plaster and does not release the tourniquet. The lateral stationary line of the finger is the line from which no flexion contracture can develop: grafts should be placed well back to this line.
In recurrent disease, if the digital nerve has been severed by clinical testing, assume that the artery also has been severed and ensure that the contralateral artery is not damaged. A dermofasciectomy is the treatment of choice for recurrent Dupuytren disease. Begin the operation by dividing the natatory ligament of the thumb contracture and releasing by Z-plasty, lest this be forgotten later. Brachial block is preferred.
Postoperative Management
Postoperative management can be performed on an outpatient basis, although one must be sure that the hand is going to be elevated. Swelling, stiffness, and reflex sympathetic dystrophy (RSD) are unlikely to occur if the hand is elevated for the first 48 hours. A static splint maintains the surgical gains during the healing phase. It is removed for active and passive ROM exercises. Splintage also can be used to improve on the ROM gained at surgery. At least 3 months' splintage is necessary.
Postoperative regimen
- First 2 days - Bulky dressing with backslab in full extension or bulky dressing with no backslab
- Two days to 2 weeks - Light dressing, with patient encouraged to regain full flexion
Complications and Results
Overall, 20% of patients develop a complication.
Early complications
These are surgeon dependent and include the following:
- Hematoma: Tourniquet release is mandatory prior to wound closure.
- Skin loss: This occurs for the same reasons as failure of skin flaps but commonly is due to hematoma. The skin may be very thin after dissection of the overlying fascia; in this situation a skin graft may be a better option. The excised skin may be used as a free graft.
- Infection: Infection is usually a sequela of hematoma and skin loss. If contracture prevents adequate skin preparation, a fasciotomy may be required as a preliminary measure to definitive fasciectomy.
- Division of the digital nerve or artery: The operation should be regarded as a neurolysis and the neurovascular bundle should be dissected free along the entire course of the wound. The area of greatest risk is in the web space. Magnification is essential. Division of the pretendinous cord may facilitate dissection by facilitating finger extension and abduction. Divided nerves and arteries should be repaired.
Late complications
These generally reflect the Dupuytren diathesis and include the following:
- Loss of flexion: This is the most common complication. Full flexion must be recorded preoperatively. This often is neglected as one usually is attempting to regain full extension. Schneider reports a 41% loss of flexion using the open palm technique.
- RSD: Every patient operated on for Dupuytren disease is a candidate for RSD. It is 5 times more common in women than in men after Dupuytren release, although few women have sufficiently severe disease to warrant operation. Kleinert described a high incidence of RSD following operation for Dupuytren disease and carpel tunnel release.
- Recurrence: Recurrence (ie, Dupuytren tissue in the area of resection) and recurrence of flexion deformity and extension (ie, Dupuytren tissue outside the area of resection) are separate entities. Recurrence is more likely in a young patient with a strong family history. No operation can change the inherited diathesis.
Results
The patient and surgeon should judge results subjectively, with function and joint ROM evaluated. MP contracture invariably is corrected (86% excellent), while good results for PIP contracture are less frequent (40% excellent result in middle, R fingers; only 20% in L finger). DIP has approximately 50% excellent results. The L finger is the most difficult to correct.
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Keywords
dupuytren, Dupuytren contracture, fibroproliferative disorder, joint contracture, proximal interphalangeal joint contracture, PIP joint contracture, metacarpophalangeal joint contracture, MP joint contracture, knuckle pads, Garrod nodes, plantar fibromatosis, Lederhose disease, Peyronie disease
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References
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Gonzalez RI. The use of skin grafts in the treatment of Dupuytren''s contracture. Hand Clin. Nov 1985;1(4):641-7. [Medline].
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McFarlane RM, Jamieson WG. Dupuytren''s contracture. The management of one hundred patients. J Bone Joint Surg Am. Sep 1966;48(6):1095-105. [Medline].
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Noble J, Harrison DH. Open palm technique for Dupuytren''s contracture. Hand. Oct 1976;8(3):272-8. [Medline].
Schneider LH, Hankin FM, Eisenberg T. Surgery of Dupuytren''s disease: a review of the open palm method. J Hand Surg [Am]. Jan 1986;11(1):23-7. [Medline].
Watson HK, Fong D. Dystrophy, recurrence, and salvage procedures in Dupuytren''s contracture. Hand Clin. Nov 1991;7(4):745-55; discussion 757-8. [Medline].
Further Reading
Keywords
dupuytren, Dupuytren contracture, fibroproliferative disorder, joint contracture, proximal interphalangeal joint contracture, PIP joint contracture, metacarpophalangeal joint contracture, MP joint contracture, knuckle pads, Garrod nodes, plantar fibromatosis, Lederhose disease, Peyronie disease
