Dupuytren's Disease

Author: Brian Weatherford

Topic updated on 03/01/13 11:55am

Introduction

A benign proliferative disorder characterized by fascial nodules and contractures of the hand
Epidemiology & genetics
- autosomal dominant with variable penetrance
- 5-7th decade of life with 2:1 male to female ratio
- highest incidence in caucasian males of northern European descent
Pathophysiology
- myofibroblast is the dominant cell type
- cytokines have been implicated
- ectopic manifestations
  - Ledderhose disease (plantar fascia)
  - Peyronie's disease (dartos fascia of penis)
  - Garrod disease (knuckle pads)
Associated conditions
- HIV, alcoholism, diabetes, antiseizure medications
Prognosis
- long-term recurrence rate after operative management up to 50%

Pathoanatomy

Fascial bands become pathologic cords.
Different named cords include but are not limited to
- central cord
  - pretendinous band
  - not involved with neurovascular bundle
  - cause of MCP contracture
- retrovascular cord
  - usual cause of DIP contracture
- spiral cord
  - clinically the most important
  - cause of PIP contracture
  - involves several structures including
    - pretendinous band
    - spiral band
    - natatory band/ligament
    - lateral digital sheet
    - Grayson's ligament
  - travels under the neurovascular bundle
    - bundle is displaced central and superficial
    - at risk during surgical resection
    - best predictors of displacement are
    - PIP joint flexion contracture (77% positive predictive value)
    - interdigital soft-tissue mass (71% positive predictive value)
Cleland's ligament is the only retaining dermal ligament not involved with Dupuytren's disease

Histopathology

Stages of Dupuytren's (Luck)
Proliferative stage	Hypercellular with a predominance of large myofibroblast Very vascular with many gap junctions Minimal extracellular matrix
Involutional stage	Dense myofibroblast network Increase ratio of type III to type I collagen
Residual stage	Myofibroblast disapear leaving fibrocytes as the predominate cell line

Presentation
Symptoms decreased range of motion affecting activities of daily living nodules may be painful Physical exam nodule in the pretendinous bands of the palmar fascia most commonly involve small or ring finger Hueston's tabletop test ask patient to place palm flat on table look for MCP or PIP contracture look for bilateral involvement and ectopic associations (plantar fascia) indicative of more aggressive form (Dupuytren's diathesis)
Treatment
Nonoperative range of motion exercises, injections indications may be attempted but condition will not spontaneously resolve modalities early efficacy seen with injections of clostridial collagenase into Dupuytren's cords causes lysis and rupture of cords Operative surgical resection/fasciectomy indications MCP flexion contractures > 30° PIP flexion contractures painful nodules are not an indication for surgery
Surgical Techniques
Regional/limited palmar fasciectomy technique removal of all diseased tissue only in involved digits pros most widely used surgical treatment overlying skin is preserved postoperative care early active range of motion (starting postoperative day 5-7) night-time extension brace or splint Total/radical palmar fasciectomy technique release and/or excision of all palmar fascia including portions that do not appear involved in the disease process cons decreased utilization due to recurrence rate and complications Open palm technique (McCash technique) approach transverse skin incision at the distal palmar crease may be left open with or without delayed skin grafting pros useful in older patients who are at risk for stiffness low risk of complication due to lack of hematoma formation
Complications
Hematoma most common surgical complication can lead to flap necrosis Recurrence long term recurrence up to 50% Flare reaction pain syndrome with diffuse swelling, hyperesthesia, redness and stiffness increased risk with concomitant carpal tunnel release Neurovascular injury bundle at risk due to central and superficial displacement from spiral cord identify prior to excising cord

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Qbank (4 Questions)

TAG

(OBQ10.110) Which of the following patients with Dupuytren's contracture would benefit the most from dermatofasciectomy and full-thickness skin grafting opposed to traditional fasciectomy? Topic

Review Topic

1. 70-year-old sedentary male with small finger involvement isolated to the MCP joint
2. 50-year-old male systems analyst with ring and small finger involvement limited to the MCP joints
3. 65-year-old female golfer with ring and small finger involvement including MCP and PIP joints
4. 40-year-old female stenographer with middle, ring, and small finger involvement including MCP and PIP joints with 50 and 55 degree contractures of ring and small finger MCP joints, respectively
5. None of the above as no difference in outcome has been demonstrated between the two procedures

PREFERRED RESPONSE ▶

DISCUSSION: Dermatofasciectomy and full-thickness grafting has not demonstrated superior finger range of motion, recurrence rate, or patient satisfaction in comparison with traditional fasciectomy.

The main reference from Ullah et al conducted a prospective randomized study of 84 Dupuytren's cases treated with fasciectomy alone or dermatofasciectomy with full-thickness skin grafting. The question was whether the overlying skin needed to be excised. No difference in clinical outcome or recurrence rate was discovered.

Roy et al reviewed 79 cases of advanced Dupuytren's treated with radical fasciectomy (but preservation of the overlying skin) and then adding full-thickness skin grafting to the open areas once the fingers were extended. They found their results of fasciectomy to be similar to those published for dermofasciectomy.

REFERENCES:

1. Ullah AS, Dias JJ, Bhowal B. Does a 'firebreak' full-thickness skin graft prevent recurrence after surgery for Dupuytren's contracture?: a prospective, randomised trial. J Bone Joint Surg Br. 2009 Mar;91(3):374-8. PMID:19258615 (Link to Abstract)

2. Roy N, Sharma D, Mirza AH, Fahmy N. Fasciectomy and conservative fullthickness skin grafting in Dupuytren's contracture. The fish technique. ActaOrthop Belg. 2006 Dec;72(6):678-82. PMID:17260604 (Link to Abstract)

Question Authors:

John Badylak MD, Sanj Kakar MD, Ben Taylor MD,

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(OBQ04.133) All of the following have been implicated in the pathogenesis of Duputryen's contracture EXCEPT? Topic

Review Topic

1. Fibroblast growth factor (FGF)
2. Transforming growth factor- beta (TGF-beta)
3. Myofibroblasts
4. Platelet-derived growth facture (PDGF)
5. CBFA-1

PREFERRED RESPONSE ▶

DISCUSSION: Of the answers listed, only CBFA-1 has NO known role in the pathogenesis of Duputryen's contracture.

Dupuytren contracture, a disease of the palmar fascia, results in the thickening and shortening of fibrous bands in the hands and fingers. The offending cells are thought to be myofibroblasts and fibroblasts. Growth factors such as basic fibroblast growth factor (FGF), platelet-derived growth factor (PDGF), and transforming growth factor-beta (TGF-beta) may signal the overproduction of the myofibroblasts and/or myofibroblastic activity of the fibroblasts. In addition, high levels of TGF-Beta may hinder apoptosis of the active myofibroblasts, unlike normal tissue healing.

McGrouther discusses how the pathophysiology of Dupuytren's is related to the anatomy of the palmar ligaments. The article discusses the proposed etiology of Dupuytren's with a loss of normal motion between palmar fascial ligaments causes stress concentrations which stimulate fibrous tissue deposition and contracture.

Baird et al performed a tissue analysis of 12 patients with Dupuytren's contracture compared to 12 control patients. They found that Dupuytren's expressed a higher percentage of peptide regulatory factors including interleukin-1 alpha, interleukin-1 beta, transforming growth factor beta, and basic fibroblast growth factor.

REFERENCES:

1. Trumble TE (ed): Hand Surgery Update 3: Hand, elbow, & shoulder. Rosemont, IL, American Society for Surgery of the Hand, 2003, pp 393-401

2. McGrouther DA: The microanatomy of Dupuytren's contracture. Hand 1982,14:215-236 PMID:7152372 (Link to Abstract)

3. Baird KS, Crossan JF, Ralston SH. Abnormal growth factor and cytokine expression in Dupuytren's contracture. J Clin Pathol. 1993 May;46(5):425-8. PMID:8320323 (Link to Abstract)

Question Authors:

Michael Hughes MD, Michael Hughes MD, Ben Taylor MD,

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(OBQ04.267) What is the name of the pathologic structure, identified by the white arrow in Figure A, that displaces the digital neurovascular bundle and places it at risk during during surgical treatment of Dupuytren's disease? Topic

Review Topic

FIGURES: A

1. Pretendinous cord
2. Pretendinous band
3. Spiral cord
4. Spiral band
5. Natatory cord

PREFERRED RESPONSE ▶

DISCUSSION: The spiral cord, shown by the white arrow in Figure A, can displace the neurvascular bundle (blue arrow) and places it at risk during surgical resection. Dupuytren's contracture is a rare and progressive condition characterized by contractures of the fascia of the hand as seen in Illustration A. The fascial components involved in the disease include the pretendinous bands, spiral bands, natatory bands, lateral digital sheets, and Grayson's ligament. The offending cell is the myofibroblast which causes the normal structures to become fibrosed. Once these normal bands become pathologically involved in the disease process, they are termed cords. An easy way to remember this is that bands are normal, and cords are abnormal. The spiral cord travels dorsal to the NVB and displaces it volarly, placing it at risk during surgical resection. Example is shown in Illustrations B. Of note, Cleland's ligament is not involved in this disease process.

Illustrations: A

REFERENCES:

1. Trumble TE (ed): Hand Surgery Update 3: Hand, Elbow, & Shoulder. Rosemont, IL, American Society of Surgery of the Hand, 2003, pp 393-401.

2. McGrouther DA: Dupuytren’s Contracture, in Green DP, Hotchkiss RN, Pederson WC (eds): Green’s Operative Hand Surgery, ed 4. Philadelphia, PA, Churchill Livingstone, 1999, pp 563-591.

Question Authors:

David Abbasi MD, John Badylak MD, Sanj Kakar MD, Bobby Chhabra M.D.,

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Dupuytren's Open Fasciectomy

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Does a 'firebreak' full-thickness skin graft prevent recurrence after surgery for Dupuytren's contracture?: a prospective, randomised trial. Includes Poll

Ullah AS, Dias JJ, Bhowal B
J Bone Joint Surg Br. 2009 Mar;91(3):374-8. PMID: 19258615 (Link to Pubmed)

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Evidence & References Show References

Level of Evidence 1 (Randomized Control Trial)

Ullah AS, Dias JJ, Bhowal B. Does a 'firebreak' full-thickness skin graft prevent recurrence after surgery for Dupuytren's contracture?: a prospective, randomised trial. J Bone Joint Surg Br. 2009 Mar;91(3):374-8. PMID:19258615 (Link to Abstract)

Level of Evidence 3 (Retrospective Cohort Study or Case-Control Study)

Baird KS, Crossan JF, Ralston SH. Abnormal growth factor and cytokine expression in Dupuytren's contracture. J Clin Pathol. 1993 May;46(5):425-8. PMID:8320323 (Link to Abstract)

Level of Evidence 4 (Case Series)

Roy N, Sharma D, Mirza AH, Fahmy N. Fasciectomy and conservative fullthickness skin grafting in Dupuytren's contracture. The fish technique. ActaOrthop Belg. 2006 Dec;72(6):678-82. PMID:17260604 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

McGrouther DA: The microanatomy of Dupuytren's contracture. Hand 1982,14:215-236 PMID:7152372 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Trumble TE (ed): Hand Surgery Update 3: Hand, Elbow, & Shoulder. Rosemont, IL, American Society of Surgery of the Hand, 2003, pp 393-401.
McGrouther DA: Dupuytren’s Contracture, in Green DP, Hotchkiss RN, Pederson WC (eds): Green’s Operative Hand Surgery, ed 4. Philadelphia, PA, Churchill Livingstone, 1999, pp 563-591.

Undefined

Trumble TE (ed): Hand Surgery Update 3: Hand, elbow, & shoulder. Rosemont, IL, American Society for Surgery of the Hand, 2003, pp 393-401

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Dupuytren's Disease

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