Dupuytren's Disease - A Literature Review

Transcription

1 Dupuytren's Disease - A Literature Review Poster No.: C-0415 Congress: ECR 2013 Type: Educational Exhibit Authors: R. M. Lopes, L. H. Domingos ; Mortágua/PT, Azoia de Cima/PT Keywords: Connective tissue disorders, Surgery, Ultrasound, Musculoskeletal soft tissue, Extremities DOI: /ecr2013/C Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 23

2 Learning objectives The aim of this study is to increase knowledge about the Dupuytren's Disease doing a review of recent literature. Background Dupuytren's disease is characterized by a progressive contracture and thickening of the palmar fascia and its fingers extensions, leading to a flexion deformity of the metacarpophalangeal and interphalangeal joints (Fig. 1). The fourth and fifth fingers are most commonly affected, with special focus in dominant hand. Occasionally, the disease will cause thickening on top of the finger knuckles (knuckle pads), or nodules or developing fibrosis in the subcutaneous dorsal ligament (of McGrouther) in the proximal interphalangeal joints or cords within the soles of the feet (plantar fribomatosis). This usually indicates progressive disease. The Dupuytren's disease etiology remains uncertain. Today, the hereditary factors are (2) most accepted. It is believed that this may be associated to a genetic predisposition through dominant chromosome. Men are mostly affected by this disease, preferably in northern regions of Europe, Scandinavian countries and United Kingdom. The ratio between men and woman is 3:1 in (2) Europe and 9,5:1 in Australia. A study conducted in Boston, Massachusetts, showed a ratio of 4:1 in patients less than 54 years, however, this ratio would approach 1:1 up with advancing age. In Australia, 20% of men over 60 years are affected by this disease. It is unusual in young people, however, have been reported cases in patients under 16 years. Dupuytren's disease patients, in 5-10% of cases, may compromise similar lesions in the medial plantar fascia (Lederhosen's disease or plantar fibromatosis). In 10% of (2,3) cases, there is a compromise of the deep penis fascia (Peyronie's disease). In this conditions, Garrod nodules, are often observed. In conclusion it is known that: The 4th finger is most commonly affected; The 5th finger is the second most commonly affected; 33% of cases affected only one finger; Page 2 of 23

3 33% of cases affected two fingers; 33% of cases affected more than two fingers; 45% of cases are bilateral, however, the right side is most affected (4) ; The following table shows the predisposing factors by Dupuytren's disease (Table 1): Table 1: Influent factors to Dupuytren's Disease development (2). References: ARAUJO, D.B Dupuytren's disease was described by McIndoe and Beare into four phases of clinical development. However, Luck, in 1959, proposed three phases. Actually, these is more acceptable (2) (Fig. 2). 1st. -Proliferative phase - when the contraction begins, will have a cord near the nodule and will have predominance of collagen type III and IV; 2nd. - Involution Phase - occurs when disappearance of nodules and will have presence of joint contractures, with formation of fibrous cords and will have a predominance of collagen type I; 3rd. - Residual stage - when there are only contracted fibrous bands (3). DUPUYTREN'S DISEASE CLASSIFICATION Page 3 of 23

4 Tubiana's classification is the most commonly accepted measurement of this contracture (5). This is based on the measurement of total flexion of each digit affected (Table 2). Table 2: Dupuytren's disease stages proposed by Tubiana (5). TFD (Total flexion deformity) - measured angle between the proximal metacarpophalangeal and distal interphalangeal joint. References: HINDOCHA S, et al In Tubiana's classification, the contracture is evaluated by measuring between the proximal metacarpophalangeal joint and distal interphalangeal joint (Fig. 3). Other variables were also considered in Dupuytren's disease. The number of surgical procedures and number of affected fingers were taken into account. The relapse presence after surgical treatment indicates severity disease. This review presents the new stadiums initially proposed by Tubiana and 9 other elements prevalent in patients with Dupuytren's disease (Table 3). Page 4 of 23

5 Table 3: Tubina's classification review (5). References: HINDOCHA S, et al Images for this section: Page 5 of 23

6 Fig. 1: Fig. 1 - Contracture in flexion of the 4th finger of the right hand (1). Fig. 2: Fig. 2 - Representation of clinical states in Dupuytren's disease (2). Page 6 of 23

7 Fig. 3: Fig. 3 - Schematic of the measurement of different angles at different stages (5). Page 7 of 23

8 Imaging findings OR Procedure details The diagnosis of Dupuytren's disease is essentially through of the history and clinical examination. As this disease is not typically painful, even in advanced stages, the only symptoms can be numbness and morning stiffness. Usually, the first sign is the appearance of a subcutaneous nodule in the palm of the hand. The palmar observation and palpation identifies nodules and different thickness in the palm of the hand, near the 4th or the 5th finger (Fig. 4). The table test, it is a test to evaluate the type of contracture, that consists in to put the patients palm of the hand on the table (Fig. 5). Clinical examination should follow the following summary table (Table 4): Table 4: Following evaluating in the clinical examination (7). References: McRAE, R. et al Besides the clinical evaluation, in some cases, it is necessary using imaging techniques, such as ultrasonography, conventional radiography and Magnetic Resonance Imaging, that help in the differential diagnosis and planning of a surgery. ULTRASONOGRAPHY Ultrasound is a useful imaging technique for differential diagnosis of pathologies involving the palmar surface of the hand, demonstrating the thickness of the palmar fascia as (3) well the presence of a nodule (Fig 6). This disease is characterized by hypoechoic bands adhering to the marging of the flexor tendons and deep surface of the dermis. Others ultrasound characteristics are the superficial palmar aponeurosis appears as a thin echogenic lamellar structure overlying the flexor tendons, early nodules are Page 8 of 23

9 hypoechoic and typically hypervascular whereas older nodules are iso- to-hyperechoic, without hypervascular Doppler signal. CONVENTIONAL RADIOLOGY Although not being a choice exam, radiography allows viewing of flexion deformities of the metacarpophalangeal and interphalangeal joints and also shows subluxations in the joints (9) (Fig. 7 and 8). MAGNETIC RESONANCE IMAGING MRI has the ability to correlate the MRI signal intensity and the degree of cellularity of the lesions and to evaluate of the palmar involvement. For this reason, this technique is useful for distinguishing this disease from other soft-tissue disorders, such as neurofibroma and giant -cell tumor of tendon sheath and for surgery planning. Usually, hypercellular lesions tend to have higher rates of recurrence after surgery than the hipocellular lesions. If the lesion is hypercellular, it is necessary delayed the surgery until the increased collagen content is confirmed (9). The appearance in MRI image is the cords and tendons have uniformly low signal intensity on both T1- and T2-weighted images, in intermediate and late disease. Nodules and muscle have intermediate signal intensity with focal areas of lower signal intensity, on both T1- and T2- weighted images. The next image shows low signal intensity corresponding with fibrous tissue and thickness palmar aponeurosis, consistent in Dupuytren's disease (Fig. 9). DIFFERENTIAL DIAGNOSIS The differential diagnosis of Dupuytren's disease should be: Diabetic cheiropathy (diabetic syndrome in the hand); Epithelioid sarcoma; Fibroma; Giant cell tumor; Intrinsic joint disease; Page 9 of 23

10 Lipoma; Neurofibroma; Palmar fibromatosis; Palmar tendinitis; Tendon nodule of stenosing tenosynovitis; Tophi; Traumatic scars (4). Images for this section: Fig. 4: Nodules scheme (2). Page 10 of 23

11 Fig. 5: Table Test (6). Page 11 of 23

12 Fig. 6: Ultrasonography of the fourth finger of the right hand. Legend: flexor tendon (green arrow), deformity of the skin (yellow arrow), nodules (red arrow), thickened palmar fascia (blue arrow) (3) Page 12 of 23

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14 Fig. 7: Lateral radiographic view of the right hand demonstrates flexion deformities in the ring finger. (Image courtesy of ACES Northeast - Portugal) Page 14 of 23

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16 Fig. 8: Lateral radiographic view of the postoperatively right hand (Image courtesy of ACES Northeast - Portugal) Fig. 9: MRI (T2 fat sat) demonstrates low signal intensity fibrous band in the superficial subcutaneous tissue in the ring finger, which running parallel the flexor tendon (9). Page 16 of 23

17 Conclusion TREATMENT The specific treatment of Dupuytren's disease will be determined taking into account the following factors: Age of the patient; Clinical history; Disease stage; Tolerance to certain medications, procedures or therapies; Expectations regarding the disease; Opinion and patient preferences. After evaluating all these factors, the clinician should adopt the best therapy for each case. CONSERVATIVE TREATMENT The injection of fibrinolytic substances, radiotherapy and physiotherapy exercises predestined to hyperextend the fingers are the bests methods for this pathology. SURGICAL TREATMENT The indications for surgery depend the hand function, age of the patient, the severity of contracture and involved joints (Fig. 10). Surgery have indications such as: Phalangeal flexion contracture of 30 degrees or more; The proximal interphalangeal flexion contracture of any degree is an indication for surgery; The neurovascular compromise; Page 17 of 23

18 The secondary involvement of periarticular structures may also require correction. Surgical techniques for Dupuytren's disease include closed and open fasciotomy, regional or limited palmar fasciectomy, and extended or total fasciectomy. In this technique, only the diseased parts of the superficial fascia aponeurosis are excised. Although Dupuytren's disease may reoccur or progress by extension in the nonoperated áreas of the hand, good results have been obtained with a acceptable complication rates. The regional palmar fasciectomy is the most commonly performed procedure for Dupuytren's disease (3). The following scheme is a summary table of treatment of Dupuytren's disease (Table 5). Page 18 of 23

19 Table 5: Scheme summary of Dupuytren's disease (10). References: TOWNLEY, W.L. et al COMPLICATIONS In 20% of cases with Dupuytren's disease, was reported complications (3). IMMEDIATE COMPLICATIONS Hematoma; Skin loss; Page 19 of 23

20 Infection; Division of the digital nerve or artery; Tendon injuries; Joint injuries. LATER COMPLICATIONS Loss of flexion range; Reflex sympathetic dystrophy (It is at least 5 times more common in women than in men with Dupuytren's disease); Local hyperalgesia; Sudek Atrophy; Recurrence (26% and 80%) (3). FOLOW-UP Contractures of the metacarpophalangeal joint are easily corrected with surgery (4) (80-96%), but may have recurrences in about 11% of surgeries. Contractures of the proximal interphalangeal joint are corrected in 25 to 56% of cases. Images for this section: Page 20 of 23

21 Fig. 10: Visible cord characteristics of Dupuytren's disease with planned markings for surgical release (3). Page 21 of 23

22 References (1) DIB, C. C. et al. Dupuytren's disease: our conduct. Brazilian Journal of Plastic Surgery, 2008, 23 (4) :290-3; (2) ARAUJO, D. B. Hand fibromatous disease (Dupuytren's disease), Rheumatology Clinical Issues - Vol.8, No. 3, September 2007; (3) WONG, W. W. Hand, Dupuytren's Disease. Department of Plastic Surgery, Loma Linda University School of Medicine, 2010, accessed May 2011, available at: emedicine.medscape.com/article/ overview; (4) MATHEW, S.D. Dupuytren Contracture. Department of Rheumatology, San Antonio Uniformed Services Health Education Consortium, 2011, accessed May 2011, available at: (5) HINDOCHA, S. et al. Revised Tubiana's Staging System for Assessment of Disease Severity in Dupuytren's Disease Clinical Preliminary Findings. American Association for Hand Surgery, Hand 2008, 3:80-86; (6) BALLESTA, A. J. et al. Utilizacíon silicone implants en la enfermedad Dupuytren. Ibero-Latin American Plastic Surgery, vol.32, No. 2, April-Mayo-Junio 2006, Pag ; (7) McRAE, R. et al. Orthopedics and Traumatology. Novartis, 1999, Madrid; (8) DAVID, L. P. Orthopedic principles - A Resident's Guide. Springer, Berlin Heidelberg 2005; (9) UZOR, R. B. et al. Dupuytren Contracture for Imaging - MRI is valuable in planning surgical treatment. Albert Einstein College of Medicine, New York, 2011; (10) TOWNLEY, W.L. et al. Dupuytren's contracture unfolded. BMJ, 332 Volume 18 February 2006; (11) PF EARLY Populations Dupuytren's Contracture Study in. Department of Orthopaedic Surgery, Manchester Royal Infirmary, The journal of bone and joint surgery, vol. 44 b, no. 3, August 1962; (12) FREITAS, D. A. et al. Dupuytren's contracture: treatment by open palm technique. Brazilian Journal of Orthopaedics, vol.32, No. 4, April 1997; (13) SILVA, J. B. et al. The open palm technique in Dupuytren's contracture writes, Brazilian Journal of Orthopaedics, Vol 34, No. 1, Jnaeiro 1999; Page 22 of 23

23 (14) BONTRAGER, Kenneth L.; Treaty of Technical and Radiological Anatomical Basis, 6th Edition, Guanabara Koogan, 2005; (15) GREENSPAN A. - Orthopedic Imaging: A Practical Approach - 4th Edition, Lippincott Williams & Wilkins, Personal Information Page 23 of 23