DUPUYTREN'S DISEASE

Introduction

Dupuytren’s disease (DD) is a benign, generally painless fibroproliferative disorder of the palmar and digital fascia, whereby a thick collagen cord develops, causing flexion deformity -- Dupuytren's contracture (DC) -- of the affected metacarpophalangeal (MP) or proximal interphalangeal (PIP) joints. The pathogenesis of DD is largely genetic, and the complexities of its underlying cellular and subcellular mechanisms are only starting to be understood. Surgery has been the mainstay of treatment for centuries; however, less invasive approaches have been shown to be effective in more recent clinical trials and in clinical practice.

Related anatomy

Normal anatomy
- Palmar fascia: triangular-shaped structure composed of broad sheets or narrow bands of fibers oriented longitudinally and transversely
- Pretendinous Bands: longitudinal fiber bands runing superficial to the flexor tendons from the level of the superficial arch to the MP joint area distally
- Natatory ligament: oriented transversely and lies just beneath the skin at each commissure; sends fibers distally along the lateral border of each digit to merge with the lateral digital sheet (LDS)
- Lateral Digital Sheet(LDS): Formed by merging fibers of the spiral band and natatory ligament; runs lateral to and along side the neurovascular bundle (NVB)
- Grayson ligament: Palmar to the NVB, passing from the flexor sheath to the skin; in DC, it can become part of a lateral cord when it joins the diseased lateral digital sheath (LDS)
- Cleland ligament: Dorsal to the NVB and arises from the phalanges; relatively uninvolved in DC
Diseased fascia (collagen cords)
- Nodules usually appear before contractile cords
- Central cord: involvement of the pretendinous bands; usually results in metacarpophalangeal joint contracture
- Spiral cord: involvement of pretendinous band, spiral band, LDS, Grayson ligament; generally results in PIP joint contracture
- Natatory cord: develops from the distal fibers of the natatory ligament, resulting in a web-space contracture
- Retrovascular cord: dorsal to NVB; usual cause of a distal interphalangeal (DIP) joint contracture
- Transverse Ligament of Palmar Aponeurosis (TLPA) [superficial intermetacarpal ligaments or Skoog's ligament is an important part of the palmar aponeurosis. Radially, the TLPA forms the proximal commissural ligaments. The deep surface of the TLPA attaches to the septa of Legueu and Juvara. This septa forms confluence with the palmar plate, inter-palmar plate ligament, sagittal band and A1 pulley.

Relevant basic science

Luck's Three phases of Dupuytren's Disease
- proliferative (random proliferation of myofibroblasts)
- involutional (myofibroblasts align along tension lines in the palm)
- residual (acellular disease with collagen-laden tissue)
Normal palmar tissue
- type I collagen
- fibroblasts
Dupuytren’s disease (DD)
- type III collagen predominates
- Myofibroblasts containing bundles of actin microfilaments, conferring contractile properties that fibroblasts do not have.
- Increasing levels of mechanical tension have also been shown to influence fibroblast differentiation into myofibroblasts.
- Adjacent myofibroblasts connect to each other and to collagen via fibronectin
- Transforming growth factor-beta 1 (TGF-β1), TGF- β2, epidermal growth factor, platelet-derived growth factor and connective tissue growth factor(PDGF) have been suggested to play a role in initiating abnormal cellular proliferation. These growth factors found in Dupuytren’s fascia in abnormal amounts.
- Bone morphogenic protein 4 (BMP-4) is expressed by normal palmar fascia but not by the diseased fascia in reverse-transcription polymerase chain reaction studies. Therefore BMP-4 may play a protective role in normal palmar fascia.
- Hypoxanthine is a by-product of ischemia found in higher-than-normal levels in Dupuytren's tissue. This free radical may play role in triggering the change of fibroblasts to myofibroblasts in elderly hands that have ischemia from diabetes or peripheral vascular disease.

Incidence and related conditions

Most prevalent in older men of northern European descent
Global incidence estimates are 1-3% of caucasians and increases with advancing age
Mendelian autosomal dominant inheritance with variable penetrance in 10-30% of cases
The tenascin C (TNC) gene, associated with fibrotic disease and cell migration, is up regulated in DD
Often occurs with other fibroproliferative disorders (Ledderhose's disease, Peyronie’s disease, knuckle pads), which are associated with Dupuytren diathesis.

Differential diagnosis

For nodules: callus, ganglion, epithelial inclusion cyst
For contractures: chronic locked trigger fingers, unlnar nerve palsy with sign of benediction, limited joint mobility (LJM), posterior interosseous nerve palsy or Vaughan-Jackson Syndrome (ruptured extensor tendons)

Note: Limited Joint Mobility (LJM) is seen in 30-50% of type I diabetics and in 25–30% of type II diabetics. LJM causes painless flexion contractures of the PIP and/or DIP joints with pathologic cords. The ring and small fingers are most often affected. No treatment will reliably eliminate these contractures.

Clinical Presentation Photos and Related Diagrams

Clinical presentation of Dupuytren's Disease (Hover over right edge to see more images)
Guillaume Dupuytren 1777-1835
Dupuytren's Contracture fifth finger caused by classic central cord AP view
Dupuytren's Contracture fifth finger caused by classic central cord AP view
Dupuytren's Contracture ring finger caused by central cord
Dupuytren's Contracture ring and fifth finger caused by two separate central cords
Dupuytren's Contracture fifth finger caused by an Abductor Digiti Minimi Cord
Dupuytren's Contracture with left ring central cord and natatory cord to fifth finger. On right there is a thumb-index commissurral cord, natatory cords to index, long and fifth fingers with central cord to ring.
Dupuytren's knuckle pads on dorsum of PIP joints. Sometimes first clinical sign of Dupuytren's Disease.
Dupuytren's Disease symptoms - Contractures disrupt activities of daily living.
Dupuytren's Disease symptoms - Contractures disrupt activities of daily living.
Dupuytren's Disease symptoms - Contractures disrupt activities of daily living.
Dupuytren's Disease Signs - Classic Positive table top test
Dupuytren's Disease -Clinical signs that supports symptomatic complaints and justify therapeutic intervention.
Dupuytren's Disease -Clinical signs -joint contracture measurements. Note central cords cross two joints therefore the position of one joint influences the measurement of the FC in the other joint.

Basic Science Photos and Related Diagrams

Dupuytren's Disease Basic Science (Hover over right edge to see more images)
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science
Dupuytren's Disease Basic Science

Pathoanatomy Photos and Related Diagrams

The Pathoanatomy of Dupuytren's Disease (Hover over right edge to see more images)
On the left the normal retention pigments (fascial bands) that support the volar surface of the palm and digit. On the right the pathologic cords caused by Dupuytren's disease that originate from the fascia bands.
The normal retention pigments (fascial bands) that support the volar surface of the palm and digit with a cross sectional image of the digital fascial structures.
The pathologic cords of Dupuytren's disease that originate from the fascia bands. Note the cross sectional digital diagram with the pathologic cords.
A Dupuyten's central cord displaced pal marly and "bowstrung" away from the flexor tendon sheath. Average distance from skin to sheath in the palm is 7mm and at the PIP level this distance is 4mm's.
Abductor digiti minimi cord and the appropriate green injection target sites.
Abductor digiti minimi cord with ulnar digital nerve and dorsal sensory nerve.
The ulnar digital nerve and dorsal sensory nerve
Classical central cord with insert showing collagenase targets
Note the thickness of this central cord ( see circle). this may require transverse placement of targets.
Central cord and natatory cord combining to form a "Y" cord and contact the MP joints of two adjacent fingers. targets for collgenase in green.
Central cord and two natatory cords combining to form a "crow's foot" cord and contact the MP joints of three adjacent fingers. Targets for collgenase in green.
Central cord in palm proximal to ring finger and two natatory cords combining to form a "super Y" cord and contact the MP joints of two fingerson either side of the ring finger.. Targets for collgenase in green.
Commissural cord, Adductor cord and radial cord of the thumb which moves the thumb into the palm, contracts the first web and creates a thumb MP flexion contracture.

Symptoms

Flexion contractures of thumb or fingers caused by Dupuytren's cords under the skin

Firm lumps(nodules) under the skin which are usually PAINLESS !

Puckering, dimples or pits in the skin

Difficult shaking hands, washing face or putting hand into pocket

Can't put the hand flat on a table

Can't separate the thumb from the index finger

Positive family history for Dupuytren's disease

Typical History

Patients usually present when contracture becomes severe enough to interfere with their ability to perform activities of daily living, athletic activities, or work-related tasks. They may complain of difficulty putting on gloves in the winter. They are often of northern European descent and have relatives with similar finger contractures. In severe cases, there may be secondary complaints of lumps in the feet; some men will have Peyronie's disease.

Positive Tests, Exams or Signs

Range of Motion: Active

Range of Motion: Passive

Table-Top Test

Flexor Tendon Exam

Work-up Options

Magnetic resonance imaging - MRI without contrast

Sonographic studies - Ultrasound

Images (X-Ray, MRI, etc.)

Xray and MRI imaging is rarely needed in Dupuytren's disease but in recurrent contractures after fasciectomy it may necessary to distinguish a Dupuytren's cord from a displaced flexor tendon with a ruptured A-2 pulley.

Treatment Options

Conservative:

Splinting has been tried but is not usually helpful
Collagenase (Xiaflex in USA, Xiapex in EU): injectable collagenase Clostridium histolyticum (CCH) affects a chemical enzymatic fasciotomy of the pathologic Dupuytren’s cords by lysis of the collagen. It does this by cleaving the bivalent bonds of the collagen helix. CCH consisted of 2 collagenase peptides in a fixed-ratio mixture of 2 purified collagenolytic enzymes, clostridial Type I collagenase (AUX-I) and clostridial Type II collagenase (AUX-II). These 2 peptides cleave the collagen type III molecule at 2 different locations. The collagen type least affected by collagenase is Type IV, which plays an important role in the basement membranes of digital arteries and nerves. This may explain why neurovascular bundles are not affected by CCH

Operative:

Percutaneous needle aponeurotomy
Fasciotomy open or closed
Fasciectomy, limited/partial
Fasciectomy, radical
Dermofasciectomy
Arthrodesis
Amputation

Treatment Photos and Diagrams - Collagenase

Collagenase Treatment for Dupuytren's Disease (Hover over right edge to see more images)
Central Cord - Injection site located in part of the cord which is most "bowstrung" (displaced away from the flexor tendons.
Initial technique for needle insertion and collagenase injection
Older Injection technique for dividing single dose into aliquots
Current dose and needle positioning technique
Latest injection technique to inject separate aliquots and keep them in the cord
Forearm and wrist position for manipulation (finger extension) procedure
Four part manipulation technique to maximize cord disruption and minimize skin tears

Treatment Photos and Diagrams - Needle Apponeurotomy

Needle Aponeurotomy
Needle Aponeurotomy in progress
Needle Aponeurotomy completed. Note proximity of digital nerves.

Treatment Photos and Diagrams - Open Surgery

Surgical treatment of Dupuytren's Disease (Hover over right edge to see more images)
Dupuytren's Incisions - First two common patterns out of many choices
Dupuytren's Incisions - Second two common patterns out of many choices
Cross sectional anatomy at Mp joint level and at the proximal extent of the dissection of Dupuytren's cords. Note relationship of the pathologic cords to the tendons and neuromuscular structures.
Central cords exposed with zig-zag incisions in two adjacent affect fingers.
Spiral cord moving the neuromuscular bundle proximally, superficially, and centrally.
Abductor Digiti Minimi Cord and ulnar digital nerve and dorsal ulnar sensory nerve,
"Y" Cord (Combination of a central cord and a natatory cord) to the ring and little fingers.
Standard double zig-Zag incisions with a distally based web flap.
Central cord and digital nerve
Central cord with both digital nerves exposed and flexor tendon visible proximally.
Zig-Zag incisions with distal web flap with retention sutures in place.
Central cords to long and ring fingers exposed
Close up view of central cords, Skoog's pigments and location of neuromuscular bundles.
Close up view of Skoog's ligaments(superficial inter metacarpal ligaments)
Delegate handling of the flaps without excessive undermining.
Closure of simple zig-zag incision without drain.
Knuckle pads caused by Dupuytren's Disease which sometimes appear before palmar cords or nodules.
Excision of painful enlarging knuckle pad
Excised knuckle pad. Note the extensor tendon is immediately underneath and attached to the base of the knuckle pad.
Segmental fasciectomies of a Dupuytren's central cord

Complications

More invasive procedures are usually associated with more short-term complications
Risk factors for recurrence: early age of onset, Dupuytren’s diathesis, multifocal disease, PIP contracture, little finger contracture
Conservative treatment complications: parasthesia, puritis, erythema, edema, infection, wound dehiscence, delayed wound healing hematoma, stiffness
Operative Complications: nerve injury, vascular injury, deep infection, loss of grip, digit loss
Complex Regional Pain Syndrome (CRPS): formerly called Reflex Sympathetic Distrophy (RSD) can occur after a fasciectomy. Postoperative patients who are doing well may suddenly have increasing pain and swelling and decreased function without infection. This “ Flare Reaction” (CRPS-like syndrome) can be treated with anti-inflammatory medications such as a Medrol dose pack and hand therapy, including progressive ROM exercises.

Outcomes

Regardless of treatment approach, immediate post-interventional improvements in contractures are generally significant. Over time, however, these tend to diminish and/or lead to recurrence
Outcomes are generally better for MP joints, for thin cords, older individuals and those with more limited disease
During fasciectomy, an associated PIP joint release has not been shown to affect long-term outcome

Video

Collagenase Injection & and finger manipulation( finger extension procedure)

Key Educational Points

Regardless of the type of treatment, DD can not be cured
In DD, there are increased amounts of collagen type III in the nodule, cord and surrounding fat. Total collagen increases, and the ratio of type III to type I also increases
The primary pathologic cell type associated with DD disease is the myofibroblast
In DD, the "spiral cord" displaces the neurovascular bundle volarly, superficially, and proximally; therefore, between the distal palmar and the first finger crease, the neurovascular bundle may be found between the skin and the cord.
Retrovascular cords volar to Cleland’s ligament cause DIP joint contractures
PIP contractures may not resolve after fasciectomy or CCH treatment because of an attenuation of the central slip, tight collateral ligaments, volar plate or secondary to arthritic PIP joint changes
Dupuytren’s diathesis, defined as patients with a positive family history, bilateral disease, male gender, onset of disease before age 50 years, and the presence of dorsal knuckle pads or other sites of fibromatosis, are prone to higher recurrence rates than are patients without a Dupuytren’s diathesis.
In DD, the transverse ligaments of the palmar aponeurosis (superficial intrametacarpal ligaments of the Skoog) are not typically involved into the joint disease. In the finger, the dorsal Cleland’s ligaments are also not involved.
Simultaneous surgery for carpal tunnel syndrome and DD is not associated with increased complications

Practice and CME

References

New articles

Gajendran VK, Hentz V, Kenney D, Curtin CM. Multiple collagenase injections are safe for treatment of Dupuytren's contractures. Orthopedics 2014;37(7):e657-60.
Rizzo M, Stern PJ, Benhaim P, Hurst LC. Contemporary management of dupuytren contracture. Instr Course Lect 2014;63:131-42.
Shih B, D Wijeratne, Armstrong DJ, Lindau T, Day P, Bayat A. Identification of Biomarkers in Dupuytren’s Disease by Comparative Analysis of Fibroblasts Versus Tissue Biopsies in Disease-Specific Phenotypes. J Hand Surg;34A:124:2009.
Lilly SI, Stern PJ. Simultaneous carpal tunnel release and Dupuytren’s fasciectomy. J Hand Surg 2010;35A:754-757.

Reviews

Eaton C. Evidence-based medicine: Dupuytren contracture. Plast Reconstr Surg 2014;133(5):1241-51.
Ball C, Pratt AL, Nanchahal J. Optimal functional outcome measures for assessing treatment for Dupuytren's disease: a systematic review and recommendations for future practice. BMC Musculoskelet Disord 2013;14:131.
Hurst L.Dupuytren’scontracture. In:Wolfe SW, Hotchkiss RN, Pederson WC, Kozin SH (eds) Green’s Operative Hand Surgery 6th edition. Philadelphia: Elsevier 2010;141-158.
Bulstrode NW, Jenec B, Smith PJ. The complications of Dupuytren’s contracture surgery. J Hand Surg 2005; 30A(5):1021-
Beyermann K, Prommersberger KJ, Jacobs C, Lanz UB. Severe contracture of the proximal interphalangeal joint in Dupuytren’s disease: does capsuloligamentous release improve outcome? J Hand Surg [Br]. 2004 Jun;29(3):240-3
Shin SS, Liu C, Chang EY, Carslson CS, Di Cesare PE. Expression of bone morphogenic proteins by Dupuytren’s fibroblasts. J Hand Surg Am 2004;29:809–814.
Bilderback K, Rayan G. The Septa of Legueu and Juvara: an anatomic study. JHS 2004;29A:494–499.
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;21:210-215.

Classics

Hueston JT. Recurrent Dupuytren's contracture. Plast Reconstr Surg 1963;31:66-9.
Tubiana R. Prognosis and treatment of Dupuytren's contracture. J Bone Joint Surg Am 1955;37-A(6):1155-68.
Hurst LC, Badalamente MA, Hentz VR, Hotchkiss RN, Kaplan FT, Meals RA, et al. Injectable collagenase clostridium histolyticum for Dupuytren’s contracture. N Engl J Med 2009;361:968–979
McFarlane RM. On the origin and spread of Dupuytren’s disease. J Hand Surg Am. 2002; 27(3):385-390.
Chammas M, Bousquet P, Renard E, Poirier J-L, Jaffiol C, Allieu Y: Dupuytren’s disease, carpal tunnel syn- drome, trigger finger, and diabetes mellitus. J Hand Surg 20A:109-114, 1995.