Dupuytren's disease: history, diagnosis, and treatment

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe the clinical features of the disease. 2. Describe the pathoanatomical structures in Dupuytren's disease. 3. Outline the various factors associated with Dupuytren's disease. 4. Describe the modalities for surgical and nonsurgical treatment of the condition. 5. Outline recent biomolecular knowledge about the basis of Dupuytren's disease. SUMMARY: Dupuytren's disease is characterized by nodule formation and contracture of the palmar fascia, resulting in flexion deformity of the fingers and loss of hand function. The authors review the historical background, clinical features, and current therapy of Dupuytren's disease; preview treatment innovations; and present molecular data related to Dupuytren's disease. These new findings may improve screening for Dupuytren's disease and provide a better understanding of the disease's pathogenesis.     

Dupuytren's fasciectomies in 60 consecutive digits using lidocaine with epinephrine and no tourniquet

Dupuytren's contracture is a common hand problem. Its treatment, other than percutaneous fasciectomy, is an outpatient operation performed with the patient under regional or general anesthesia. If local anesthetics are used, they are used without epinephrine, and an arm or forearm tourniquet is essential. Multiple reviews have described the safety of local anesthetics with epinephrine in the digits. This study is a retrospective review of operations performed by the same surgeon in the hospital (43 digits) versus the office using local anesthetics with epinephrine and no tourniquet (60 digits). Results and complications were compared and tabulated. The hospital and epinephrine groups were comparable regarding preoperative measurements. Postoperative improvement in extension at the metacarpophalangeal and proximal interphalangeal joints was similar in both the hospital and epinephrine groups. Complications, including digital nerve and artery injuries, infections, and hematomas, were similar between the groups. There were no cases of digital necrosis or gangrene in the epinephrine group, even though one patient sustained a transection of a digital artery at the proximal interphalangeal joint. Because of the similarities in results and complications, there does not seem to be an advantage to performing Dupuytren's excisions with full arm tourniquet and outpatient admission in many cases. Local anesthetics with epinephrine are safe in the treatment of Dupuytren's contracture, although both the use of digital epinephrine and the use of mechanical tourniquets carry the potential for complications. Visibility is similar to that for facial or head and neck surgery and is aided by the use of loupe magnification. The old dogma against its use in the fingers is refuted, as seen by the results in these 60 consecutive Dupuytren's fasciectomies and the results of other authors.     

Matrix metalloproteinases and tissue inhibitors of metalloproteinases in sera and tissue of patients with Dupuytren's disease

Dupuytren's contracture is a fibroproliferative disorder characterized by progressive deposition of mature collagen fibers. In other fibrotic diseases affecting organs such as the liver, lung, heart, and skin, matrix metalloproteinases (MMPs) and their natural inhibitors, the tissue inhibitors of metalloproteinases (TIMPs), play an important role. In this study, serum concentrations of MMP-1, MMP-2, MMP-9, TIMP-1, and TIMP-2 were determined in 22 patients (five women and 17 men; average age, 67 +/- 11 years) with Dupuytren's disease using an enzyme-linked immunosorbent assay. Tissue samples were obtained for standard histological and immunohistochemical analyses. Sera and samples of palmar fascia from 20 patients (13 women and seven men; average age, 60 +/- 15 years) who had undergone hand surgery for carpal tunnel syndrome were used as the control group. Statistical analysis was performed using the Mann-Whitney test. Patients with Dupuytren's contracture presented with a TIMP-1 concentration of 437 +/- 160 ng/ml, a significantly higher TIMP-1 concentration than that seen in the control patients, who had a concentration of 321 +/- 70 ng/ml (p < 0.05). Patients with a proliferative active disease (n = 14) had a significantly higher TIMP-1 concentration (525 +/- 136 ng/ml) than patients (n = 8) with a contracture in the late involutional and residual phase (286 +/- 41 ng/ml; p < 0.05). There were no significant differences in the TIMP-2, MMP-1, MMP-2, and MMP-9 serum concentrations between patients with palmar fibromatosis and the control group. Patients with Dupuytren's disease had a significantly lower MMP-to-TIMP ratio (1.1 +/- 0.3; p < 0.05) than the control group (1.5 +/- 0.35). Patients with an active palmar fibromatosis presented a significantly (p < 0.05) reduced ratio (1 +/- 0.2) compared with those in later phases (1.4 +/- 0.3). TIMP-1 and TIMP-2 could be detected in tissue of patients with Dupuytren's contracture, with an accumulation in proliferative areas. MMPs could be detected locally in Dupuytren's tissue in a few patients, with less positive staining than for TIMPs. In the control group, there was just little or no staining for TIMPs and MMPs. The data indicate that the physiological balance between MMPs and their natural inhibitors is disturbed in patients with a proliferative active Dupuytren's disease. The decrease in the systemic MMP-to-TIMP ratio can cause increased synthesis and deposition of collagen, leading to palmar fibromatosis.     

Dupuytren's contractures in a black patient.

We report a case of bilateral Dupuytren's contracture occurring in a black patient, apparently the sixth reported occurrence.     

Epidermal growth factor in Dupuytren's disease

The aim of this article was to show the participation of epidermal growth factor (EGF) in the pathogenesis of Dupuytren's disease (palmar contracture). The concentration of EGF in specimens obtained from 68 patients with Dupuytren's contracture and 14 controls was examined immunochemically with the use of enzyme-linked immunosorbent assay. The determined EGF concentration in pathologic aponeurosis with symptoms of Dupuytren's disease (median, 6.29 ng/g; range, 1.67 to 63.09 ng/g) showed significantly different values (p = 0.036) in comparison with the control group (median, 10.1 ng/g; range, 5.13 to 39.81 ng/g). The changes in EGF concentration were shown in tested groups of pathologic tissues that were formed according to the clinical stage of disease progression. The significantly lower concentration than that seen in the control group characterizes tissues with first and third degrees of palmar contracture progression (p = 0.025 and p = 0.018, respectively). In the group of patients with second-degree disease progression, the EGF level increased transiently. Nevertheless, in comparison with the other groups, the difference was not significant. The group with the fourth degree of the disease showed EGF concentrations that resembled the control values. The authors conclude that significant differences in levels of EGF concentration between contractured and normal fasciae may suggest the participation of this cytokine in the pathogenesis of Dupuytren's disease.     

Dupuytren's disease: physiologic changes in nodule and cord fibroblasts through aging in vitro

The pathogenesis of the fibrotic disease Dupuytren's contracture remains unclear. The disease process includes two structurally distinct fibrotic elements, the nodule and the cord. It has been proposed that as the disease progresses, nodules develop into cords. To corroborate that hypothesis, the authors took advantage of cultured fibroblast differences found between gap junction intercellular communication and fibroblast-populated collagen lattice contraction. Paired fibroblast cell lines of nodules and cords derived from four patients with Dupuytren's disease were maintained in culture for at least eight passages. The presence of gap junction intercellular communication in nodule- and cord-derived fibroblasts was documented and reported as a coupling index. The contraction of free-floating nodule- or cord-derived collagen lattices was also documented and reported. Early passage (passage 4) cord-derived fibroblasts showed a significant increase in coupling index compared with passage 4 nodule-derived fibroblasts (4.0 +/- 0.4 versus 2.5 +/- 0.3, respectively), where p < or = 0.01. However, late passage (passage 8) nodule- and cord-derived fibroblasts were equivalent in their coupling index (4.1 +/- 0.4 versus 4.4 +/- 0.4, respectively). Early passage nodule-derived fibroblast-populated collagen lattices contracted by 64 percent, whereas late passage nodule-derived lattices showed less contraction, at only 40 percent. Early and late passage cord-derived lattices contracted 46 and 37 percent, respectively. All nodule- and cord-derived cell lines were statistically equivalent at lattice contraction by passage 8. These in vitro studies support the hypothesis that fibroblasts derived from Dupuytren's contracture nodules change their phenotype after undergoing repeated cell passage, acquiring a cord-like fibroblast phenotype. Dupuytren's nodules represent the early, active form of fibrosis in which cells are more proliferative, better at fibroblast-populated collagen lattice contraction, and display less gap junction intercellular communication. The speculation is that alterations in gap junction intercellular communication may be involved in the progression of Dupuytren's nodules to cords as the disease progresses.     

Optimizing the correction of severe postburn hand deformities by using aggressive contracture releases and fasciocutaneous free-tissue transfers

Severe postburn hand deformities were classified into three major patterns: hyperextension deformity of the metacarpophalangeal joint of the fingers with dorsal contracture of the hand, adduction contracture of the thumb with hyperextension deformity of the interphalangeal joint, and flexion contracture of the palm. Over the past 6 years, 18 cases of severe postburn hand deformities were corrected with extensor tenotomy, joint capsulotomy, and release of volar plate and collateral ligament. The soft-tissue defects were reconstructed with various fasciocutaneous free flaps, including the arterialized venous flap (n = 4), dorsalis pedis flap (n = 3), posterior interosseous flap (n = 3), first web space free flap (n = 3), and radial forearm flap (n = 1). Early active physical therapy was applied. All flaps survived. Functional return of pinch and grip strength was possible in 16 cases. In 11 cases of reconstruction of the dorsum of the hand, the total active range of motion in all joints of the fingers averaged 140 degrees. The mean grip strength was 16.5 kg and key pinch was 3.5 kg. In palm reconstruction, the wider contact area facilitated the grasping of larger objects. In thumb reconstruction, key-pinch increased to 5.5 kg and the angle of the first web space increased to 45 degrees. Jebsen's hand function test was not possible before surgery; postoperatively, it showed more functional recovery in gross motion and in the dominant hand. Aggressive contracture release of the bone,joints, tendons, and soft tissue is required for optimal results in the correction of severe postburn hand deformities. Various fasciocutaneous free flaps used to reconstruct the defect provide early motion, appropriate thinness, and excellent cosmesis of the hand.     

Dermatan sulfate remodeling associated with advanced Dupuytren's contracture

Dermatan sulfate (DS) widespread as a component of extracellular matrix proteoglycans, is characterized by great bio-reactivity and remarkable structural heterogeneity due to distinct degrees of sulfation and glucuronosyl epimerization and different polymerization degrees. However, DS metabolism under various biological conditions is poorly known. Dupuytren's contracture is a benign fibromatosis leading to complex remodeling of the palmar fascia structure and properties. However, it remains unclear whether the disease affects the structure of DS, which is the major tissue glycosaminoglycan. Thus the aim of the study was to examine the structure of the total DS in Dupuytren's fascia. DS chains were extracted from 5 samples of normal fascia and 7 specimens of Dupuytren's tissue by papain digestion followed by fractionation with cetylpyridinium chloride. Then, DS structure analysis was performed comprising the evaluation of its molecular masses and sensitivity to hyaluronidase and chondroitinase B. Dupuytren's contracture is associated with significant remodeling of DS chain structure revealed by (1) a distinct profile of chain molecular masses characterized by the appearance of long size components as well as the increase in the content of small size chains; (2) a different glucuronosyl epimerization pattern connected with the enhanced content of glucuronate disaccharide blocks; (3) chain oversulfation. These structural alterations in total DS may modify the GAG interactions especially affecting collagen fibrillogenesis and growth factor availability. Thus, Dupuytren's contracture associated DS remodeling may promote the phenomena typical for advanced disease: apoptosis and reduction in cell number as well as the appearance of dense pseudotendinous collagen matrix.     

Characterization of implants from Dupuytren's contracture tissue into the nude (athymic) mouse

Dupuytren's contracture tissues were obtained from six patients as excess surgical material. Pieces of these tissues (a total of 38 implants) were placed into subcutaneous pockets in the suprascapular area of nude (athymic) mice. The objective was to determine whether the implant tissues would be maintained in the mouse with the characteristics of Dupuytren's tissue. The implants were removed for study at 14-179 days after implantation. Microvascular anastomosis between implant and host skin was established within the first 14 days. Histologic character and electron microscopic structure of the implants did not change during the course of the study. The implants became reduced in size with time. However, neither the spatial pattern of collagen nor the appearance of fibroblast cells changed. The original high levels of chondroitin-4-sulfate were significantly decreased in the 66- to 179-day postimplantation group, but were not significantly different from the values for normal fascial bands. The hyaluronic acid of the implants increased significantly with time of implantation, but never reached the level found in the normal fascial bands. The use of implants into nude mice may be useful for further experimental studies of Dupuytren's contracture.     

Periostin differentially induces proliferation, contraction and apoptosis of primary Dupuytren's disease and adjacent palmar fascia cells

Dupuytren's disease, (DD), is a fibroproliferative condition of the palmar fascia in the hand, typically resulting in permanent contracture of one or more fingers. This fibromatosis is similar to scarring and other fibroses in displaying excess collagen secretion and contractile myofibroblast differentiation. In this report we expand on previous data demonstrating that POSTN mRNA, which encodes the extra-cellular matrix protein periostin, is up-regulated in Dupuytren's disease cord tissue relative to phenotypically normal palmar fascia. We demonstrate that the protein product of POSTN, periostin, is abundant in Dupuytren's disease cord tissue while little or no periostin immunoreactivity is evident in patient-matched control tissues. The relevance of periostin up-regulation in DD was assessed in primary cultures of cells derived from diseased and phenotypically unaffected palmar fascia from the same patients. These cells were grown in type-1 collagen-enriched culture conditions with or without periostin addition to more closely replicate the in vivo environment. Periostin was found to differentially regulate the apoptosis, proliferation, α smooth muscle actin expression and stressed Fibroblast Populated Collagen Lattice contraction of these cell types. We hypothesize that periostin, secreted by disease cord myofibroblasts into the extra-cellular matrix, promotes the transition of resident fibroblasts in the palmar fascia toward a myofibroblast phenotype, thereby promoting disease progression.     

The surgical treatment of Dupuytren's contracture: a synthesis of techniques

Dupuytren's disease is an affliction of the palmar fascia. Selective fasciectomy is recommended once contracture has occurred. Alternatives for wound closure include tissue rearrangement, the open palm technique, and full-thickness skin grafting. In this prospective study, a new "synthesis" technique was used to treat a cohort of patients with advanced Dupuytren's disease. The results were then compared with those of a second cohort of patients who underwent the open palm technique. Thirty consecutive patients were selected. Ten patients (nine men and one woman; average age, 67 years) underwent the open palm technique, and 20 patients (18 men and two women; average age, 70 years) underwent the synthesis method. Follow-up was 3.5 years for the open palm group and 2.7 years for the synthesis group. All patients in both groups improved with respect to motion, function, appearance, and satisfaction. Objectively, for the open palm technique, metacarpophalangeal joint contracture decreased from 50 degrees to 0 degrees, and proximal interphalangeal joint contracture decreased from 40 degrees to 6 degrees. Using the synthesis method, metacarpophalangeal joint contracture decreased from 57 degrees to 0 degrees, and proximal interphalangeal joint contracture decreased from 58 degrees to 10 degrees. The Disabilities of the Arm, Shoulder, and Hand Test scores decreased from 37 to 30 in both groups. There were no significant differences between groups in these parameters. The two significant intergroup differences were healing time (40 days for the open palm technique versus 28 days for the synthesis method) and recurrence rate (50 percent for open palm versus 0 percent for synthesis). The synthesis technique combines with success the best features of current methods for the surgical treatment of advanced Dupuytren's disease.     

Surgical strategy for improving forearm and hand function in late obstetric brachial plexus palsy

For the purpose of treatment, obstetric brachial plexus palsy can be subdivided into two distinct phases: initial obstetric brachial plexus palsy, and late obstetric brachial plexus palsy. In the latter, nerve surgery is no longer practical, and treatment often requires palliative surgery to improve function of the shoulder, elbow, forearm, and hand. Late obstetric brachial plexus palsy in the forearm and hand includes weakness or absence of wrist or metacarpophalangeal or interphalangeal joint extension; weakness or absence of finger flexion; forearm supination, or less commonly pronation contracture; ulnar deviation of the wrist; dislocation of the radial or ulnar head; thumb instability; or sensory disturbance of the hand. Palliative reconstruction for these forearm and hand manifestations is more difficult than for the shoulder or elbow because of the lack of powerful regional muscles for transfer. This report reviews the authors' experience performing more than 100 surgical procedures in 54 patients over a 9-year period (between 1988 and 1997) with a minimum of 2 years' follow-up. Surgical treatment is highly individualized, but the optimal age for forearm and hand reconstruction is usually later than for shoulder and elbow reconstruction because of the requirement for a preoperative exercise program. Multiple procedures for forearm and hand function were often performed on any given patient. Frequently, these were done simultaneously with reconstructive procedures for improving shoulder and/or elbow function. Traditional tendon transfer techniques do not provide satisfactory reconstruction for those deformities. Many of the authors' patients required more complex techniques such as nerve transfer and functioning free-muscle transplantation to augment traditional techniques of tendon and/or bone management. Sensory disturbance of the forearm and hand in late obstetric brachial plexus palsy seems a minor problem and further sensory reconstruction is unnecessary.     

Design of splints based on the NiTi alloy for the correction of joint deformities in the fingers

Background  

The proximal interphalange joint (PIP) is fundamental for the functional nature of the hand. The contracture in flexion of the PIP, secondary to traumatisms or illnesses leads to an important functional loss. The use of correcting splints is the common procedure for treating this problem. Its functioning is based on the application of a small load and a prolonged stress which can be dynamic, static progressive or static serial.     

A histological and anatomical profile of pacinian corpuscles from Dupuytren's contracture and the expression of nerve growth factor receptor

The etiology of Dupuytren's disease is unknown. The causes of the fibroplastic response of nodules, fibrosis of cords, and prominence of pacinian corpuscles are not evident. Histological and immunohistology differences in pacinian corpuscles from the hands of five patients with Dupuytren's disease compared with 17 Dupuytren's-free patients are presented. Histological sections of pacinian corpuscle specimens were stained with hematoxylin and eosin and immunostained for nerve growth factor receptor. The length and width of intact pacinian corpuscles were measured, and the number of layers within each corpuscle was counted and recorded. Grossly, the pacinian corpuscles from Dupuytren's patients were larger and more numerous compared with those from unaffected patients. When measured microscopically, the pacinian corpuscles from Dupuytren's diseased fascia were significantly larger (2.0 x 1.1 mm) compared with controls (1.5 x 0.78 mm). The pacinian corpuscles from Dupuytren's-affected patients had significantly more layers (64 +/- 14) compared with those from control patients (40 +/- 9). Nerve growth factor receptor staining of pacinian corpuscles from patients affected with Dupuytren's disease showed greater intensity and more area stained compared with unaffected controls. It is suggested that nerve growth factor may be involved in the increased size of pacinian corpuscles in Dupuytren's-affected fascia. It is proposed that the cellular outgrowth from pacinian corpuscles may generate the cells that develop into Dupuytren's nodules.     

Breast augmentation: compression--a very important factor in preventing capsular contracture

The incidence of capsular contracture is reduced when smooth-surfaced, saline-filled implants are placed subpectorally, avoiding contamination and the presence of blood. In addition, since the authors began using compression, they have not had one single capsular contracture requiring surgery. The authors' technique, their preoperative and postoperative regimens, an explanation of how they use compression, and the scientific and mathematical explanation of why compression could be the most important factor in preventing capsular compression are described. Of more than 1700 augmentations performed in our private clinic, only 830 could be included in the study. The patients were followed clinically for 1 year, after which time they completed a questionnaire, the results of which showed the authors that capsular contracture had not developed.     

Detection of subclinical infection in significant breast implant capsules

The pathogenesis of fibrous capsular contracture after augmentation mammaplasty is still debated. One hypothesis implicates low-grade bacterial infections as a cause. The presence of a staphylococcal biofilm in a patient with recurrent capsular contracture was previously reported. A comparative, prospective, blinded, clinical study of implants and capsules removed from patients with or without significant capsular contracture was conducted to investigate the association of biofilm contamination, breast implants, and capsular contracture.Capsule and implant samples obtained during explantation were tested by routine microbiological culture, sensitive broth culture (after maceration and sonication), and scanning electron microscopy. Clinical parameters were correlated with microbiological findings. A total of 48 implant and/or capsule samples were obtained from 27 breasts during a 22-month period. Of the 27 breasts, 19 exhibited significant contracture (Baker grade III/IV). The mean duration of implantation was 9.2 years (range, 0.4 to 26.0 years). Routine swab cultures obtained at the time of explantation were negative for bacterial growth for all samples. The sensitive broth culture technique yielded 24 positive samples (50 percent, n = 48). An analysis of capsules demonstrated that 17 of 19 samples obtained from patients with significant contracture were positive, compared with only one of eight samples obtained from patients with minimal or no contracture (p = 0.0006). Fourteen of the 17 positive cultures from significantly contracted breasts yielded coagulase-negative staphylococci, mainly, species of the Staphylococcus epidermidis group. The presence of coagulase-negative staphylococci was also significantly associated with capsular contracture (p = 0.01). There was no significant difference in the frequency of culture positivity for saline versus silicone implants (p = 0.885). Scanning electron microscopy confirmed the presence of extensive biofilm on implants and within capsules.Biofilm, in particular, S. epidermidis biofilm, was detected for a significant proportion of patients with capsular contracture. This implicates biofilm disease in the pathogenesis of contracture, and strategies for its prevention should be explored.     

Procaine in Malignant Hyperpyrexia

The caffeine contracture of normal human muscle, which has been used as a model for malignant hyperpyrexia, is greatly potentiated by halothane. Prior administration of procaine markedly reduces the halothane-potentiated caffeine contracture, and procaine given at the height of the contracture induces relaxation. Lignocaine, on the other hand, produces a variable response and sometimes increases the contracture.The muscle from a patient with an inherited susceptibility to malignant hyperpyrexia contracted spontaneously with halothane alone, and this contracture was reversed by procaine.These experiments support the therapeutic use of procaine in malignant hyperpyrexia.     

Characteristics and lateral dominance of hand grip and elbow flexion powers in young male adults

This study aimed to clarify the characteristics and the lateral dominance of hand grip power and elbow flexion power. The subjects were 15 healthy young males (mean age 22.1+/-0.7 yr, mean height 171.3+/-3.4 cm, mean mass 64.5+/-4.1 kg). All subjects were right-handed. Peak power was measured by both hands with 6 different loads of 20%-70% of maximum voluntary contraction. The maximum voluntary contraction of hand grip movement and elbow flexion movement was significantly larger in the dominant hand. Peak power of the dominant hand was larger in all loads in hand grip movement and in loads of 20% and 30% of maximum voluntary contraction in elbow flexion movement. In short, lateral dominance was confirmed. Peak power was significantly larger in hand grip movement than in elbow flexion movement in both hands. Peak velocity decreased with increasing loads in both movements, but peak power increased until about 50% of maximum voluntary contraction and then decreased. The peak power ratio of the dominant hand to the nondominant hand was significantly larger in hand grip movement than in elbow flexion movement in all loads and the peak power ratio in elbow flexion movement was more marked in light loads. In conclusion, both powers showed lateral dominance. Lateral dominance is more marked in hand grip power.     

Cruciate coupling and screw-home mechanism in passive knee joint during extension--flexion

The screw-home mechanism and coupling between forces in cruciate ligaments during passive knee joint flexion were investigated for various boundary conditions, flexion axis alignments and posterior cruciate ligaments (PCL)/anterior cruciate ligament (ACL) conditions. A developed non-linear 3D finite element model was used to perform detailed elasto-static response analyses of the human tibiofemoral joint as a function of flexion angle varying from 10 degrees hyper-extension to 90 degrees flexion. The tibia rotated internally as the femur flexed and externally as the femur extended. The re-alignment of the flexion axis by +/-5 degrees rotation about the axial (distal-proximal) axis, transection of the ACL and changes in cruciate ligament initial strains substantially influenced the 'screw-home' motion. On the other hand, restraint on this coupled rotation diminished ACL forces in flexion. A remarkable coupling was predicted between ACL and PCL forces in flexion; forces in both cruciate ligaments increased as the initial strain or pretension in one of them increased whereas they both diminished as one of them was cut or became slack. This has important consequences in joint functional biomechanics following a ligament injury or replacement surgery and, hence, in the proper management of joint disorders.