Dupuytrens Disease

Dr Ajit Pati

Overview
Definition Surgical Anatomy Aetiology and pathophysiology Clinical assessment Treatment options Recurrence and treatment

Introduction

BARON GUILLAUME DUPUYTREN (1777-1835)

First among surgeons, Last among men"

Definition
Dupuytrens disease is an abnormal thickening of the fascial tissue in the form of nodule and cords resulting in flexion contractures of palm and fingers.

nodule is the fundamental lesion the site of onset is fibrofatty layer between the skin and deep structure of the palmar surface of the hand

Fascia
Three dimensional system of fine ligamentous structures providing guide channels and retinacular restraint for longitudinally running structure and also act as a system that anchors the skin while allowing it to flex and extend.

Palmar fascia
2 layers
Deep anterior fascia covering the interosseous muscle not involved in DD. Superficial fascia (mid palmar superficial aponeurosis) triangle shaped with apex in continuity with palmaris longus tendon . *

Palmar fascia

superficial fascia - Longitudinal - Transverse - Vertical

Longitudinal fibers
Course superficial to the flexor retinaculum and flexor digitorum tendons. Termed pretendinous bands. In the mid palm (just distal to the distal palmar crease) the longitudinal fibers pass distally in 3 layers.(McFarlane & McGrouther)

 Layer 1 :
most superficial fibers pass into the skin of distal palm midway between the distal palmar & proximal digital crease) insertion is progressively more proximal from ulnar to radial side ( contracture more common on ulnar side)

 Layer 2 :
Spiral fibers pass on either side of flexor tendon deep to the neuro vascular bundle to reach the lateral digital sheet (retrovascular band). Receive fibers from natatory ligament & gives fiber to Cleland & Grayson ligaments.

Layer 3 :
Deep longitudinal fibers pass deeply on either side of flexor tendons & MP joint.( Zancolli)

 The importance of this anatomic arrangement is that different layers of a Dupuytren contracture can be followed to separate layers thus presenting a more logical approach than simply preserving the neurovascular structure and excising all else.

Transverse fibers
Proximal transverse fibers (proximal transverse ligament) :
Deep surface of pretendinous bands, superficial to NV bundles. Approx. 1.5 cm wide with distal border at the level of distal palmar skin crease. On radial side continue as proximal commissural ligament of first web space. Not involved in DD.

 Distal transverse fibers ( Natatory ligament):

Cross the base of proximal phalanges superficially. Extend from lateral border of index finger to ulnar border of little finger where it envelopes abd. Digiti. Minimi m. & ulnar NV bundle. Radially continue to first web space as distal commisural ligament o Grapow.

Vertical fibers
Extend from superficial fascia to deep fascia . Form a series of eight vertical septa on either side of digital flexor apparatus. Delineate the longitudinal compartments that contain either flexor tendon or lumbrical or digital nv bundle.

Digital fascia
Circular fascial covering of the finger made of a volar sheet that lies superficial to the flexor tendon sheath and a dorsal sheet supericial to the extensor apparatus which unite along the medial and lateral aspects of finger forming an elliptical sheath around neurovascular bundle.

 Cleland ligament :
Dorsal to NV bundle. Arising of each side of each ip joint and fanning out to the lateral skin.

Grayson ligament :
Volar to NV bundle. Arise from the volar surface of flexor tendon sheath & run laterally to skin.

Palmodigital fascia and bands

Spiral band and cord

Spiral cord and Nerve

Digital band and cord

Digital cord - Central - Lateral

Pathological anatomy
The lesions of DD follow certain well defined anatomic pathway through the fibrous tissue continuum of hand longitudinal lines of tension. Bands Normal tissue Cords Abnormal tissue

 Palm :
Palmar cords follow the pretendinous bands, often continue into the finger & terminate in the proximal or distal phalanx forming central cord. Flexion contracture of MP joint. Superficial, leaving NV bundles undisturbed.

 Finger :
4 types of cord that contract the PIP joint. 1. Central cord follows the layer 1. attaches to the base of middle phalanx on one side and often the tendon sheath. 2. lateral cord from natatory ligament to involve lateral digital fascia. does nt displace NV bundle. does nt usually cause PIP joint contracture bt can cause severe PIP jt flexion of little finger as it attaches to abductor cord overlying the abductor digiti minimi.

 3. spiral cord : arises from the longitudinal pretendinous fibers and follows layer 2 through the spiral band to lateral digital sheet & then attaches to the middle phalanx by Grayson ligament. proximally it is dorsal to the NV bundle then lateral and finally superficial & volar to the bundle .

usually involved in severe PIP contracture as it contracts , displaces the NV bundles medially and superficially.

4. retrovascular cord : arising from retrovascular structures. arises from the periosteoum on the side of proximal phalanx , courses very close to the capsule of PIP joint and attaches to the side of distal phalanx

 In the first web space usually involved structures distal or proximal commissural ligament. DIP joint involvement is not common. DIP joint hyperflexion in the event of severe PIP joint flexion may occur due to tendinous imbalance caused by the latter resulting in overstretching of the volar plate of DIP joint.

Anatomy

Anatomy

Summary

Pathogenesis

Histopathology
Contributions 1941, Meyerding : disease affects not only palmar fascia but also subcutaneous tissue and dermis. 1958, Nezelof & Tubiana : two different histologic type Lamellar Nodular Luck : 3 histologic stages Proliferative nodular tissue with proliferative fibroblasts that dont align themselves with lines of stress. Involutional fibroblasts align themselves along the lines of stress that go through the nodules. Residual disappearance of nodules with acellular scar like tissue.

 Gabbiani & Majno : contraction of palmar fascia & surrounding structure is directly related to the contractile property of myofibroblast. Chiu & McFarlane : 3 stages
Early perivascular fibroblasts. Active myofibroblasts Advanced- paucicellular.

Gelberman : recurrence is related to presence of myofibroblasts in the nodule and fibroblasts with microtubules in the fascia not with age of onset or duration or severity.

Cell biology
Myofibroblasts
Shares morphologic features of both fibroblasts and smooth muscle cell. Intracellular myofibrils, indentatioin & folds of the nuclei, specific basement membrane & hemidesmosomal structure. difference from fibroblasts
Myofibrils bundles of microfilament( actin, myosin) ILel to long axis of cell.

 Difference from smooth muscle cell Well developed golgi apparatus. Dilated RER. Absence of enveloping basal lamina.

Regulation of myofibroblast contraction

Prostaglandins ( E2 & F2) relaxation & contraction respectively. Contraction depends upon specific agonists lysophosphaditic acid. ( inhibitors of the agonist i.e. ca channel blocker, prostaglandin E1& E2 are investigated for their possible use in DD.)

 Differentiation of myofibroblasts.
Growth factors like PDGF, FGF, TGF- ( differentiation of myofibroblasts from palmar fascia fibroblasts) O 2 free radicals stimulate production of abnormal fibroblast. INF- suppress both differentiation of myofibroblast and generation of contractile force.

 Collagen :
Normal palmar fascia

Predominantly type I collagen Lesser extent type III collagen In DD, increase in amount of type III collagen. Collagen tissue are progressively shortened by the contractile forces of the myofibroblasts as they are synthesized.( Brickley- Parsons)

Associated conditions
1. 2. 3. 4. 5. 6. Epilepsy (42%) Alcohol-induced liver disease Diabetes mellitus COAD Hypertension IHD

Influencing factors
Genetics Trauma Ischaemia Alcohol Phenobarbitone MMPs and TIMPs Reduced apoptosis Free radicals Interleukin 1

Similar fibromatosis
Garrods pads Ledderhose disease -5% Peyronies disease -3%

Genetics
Common among Caucasian (Scottish)
Curse of Mac Crimmons

Rare in Africans and Middle Eastern descent Male predominance

Trauma
Micro ruptures in palmar fascia triggers IL-1 Vasomotor disturbance following swelling in hand causing secondary Ischaemia

Ischaemia
Ischaemia
Adenosine Triphosphate (ATP) Xanthine dehydrogenase

Hypoxanthine

Xanthine Oxidase

Free Radicals
Xanthine & Uric Acid

Ischaemia
Increase in free radicals Decrease in antioxidant enzyme activity Microangiopathy with narrow vessels are found in dupuytren tissue

Alcohol
Conversion of Xanthine dehydrogenase to Xanthine oxidase
Increases in free radicals

Increase in Lysophospatidic acid (LPA)

Increases intracellular calcium aiding contracture

Phenobarbitone
Increase in Lysophospatidic acid (LPA)
Increases intracellular calcium aiding contracture

MMPs and TIMPs

Normal levels of MMPs Increased levels of TIMPs-1 Abnormally low MMP : TIMPs ratio
External fixator to improve contraction prior to surgery is said to increase the level of MMPs

Reduced Apoptosis
IL 1 and TGF beta reduces the apoptosis of damaged and inflamed cells

Free radicals
Fibroblast proliferation
6 fold increase in cords 40 fold increase in nodule

Increased production of IL-1 Indirect increase in collagen III

IL-1
Fibroblast proliferation Stimulates platelets and macrophages to produce various growth factors (TGF beta) Reduces apoptosis Stimulates langerhans cells of the epidermis

Pathogenesis
Mechanisms
Intrinsic
TGF beta induces the differentiation of fibroblasts into myofibroblasts

Extrinsic
Langerhans cells pathway

Mechanism

Intrinsic

Extrinsic ( Migration to dermoepidermal junction initiates events contractures)

Final contraction
Myosin triggered by LPA

Contraction of Intracellular actin microfilaments

Dupuytren contracture

Dupuytren diathesis
Diathesis is a body condition , constitution or morbid habit that predisposes to a particular disease. According to Hueston, predisposing actors to DD
Positive family history B/L Ectopic lesions( plantar, knuckle pads)

Stages
Proliferative
large myofibroblasts very vascular

Involution
Dense network of myofibroblasts Increased ratio of type III to type I collagen

Residual
Myofibroblasts disappear Predominantly fibrocytes

Clinical prsentation
Age group Primarily volar aspect of palm and fingers. Early stage
Restricted to palm. Nodule :
firm, painless subcutaneous mass on the superficial aspect of longitudinal bands of palmar fascia. Usually near or at the distal palmar crease.

 skin pit : Usually earliest sign , may disappear later. Due to adhesion bw longitudinal and vertical fibers of fascia. distortion of the palmar crease : Deepening or widening of crease ( Hugh Johnson sign)

 Late stage Longitudinal cords appear usually over the ulnar part of the hand. Circular ,soft, pulpy appearance to either side of the cord at the level of MP joint suggest that adjacent NV bundle is raised superficially and medially ( Short Watson sign)

 Joint contracture 1st affected joint MP joint. Early phase limitation of MP joint hyperextension MP joint contracture is rarely > 60 degree and usually cause minimal functional impairment. PIP joint is involved later bt more disabling. DIP joint is rarely involved and is usually hyperextended & fixed

Surgical management
There is no perfect operation for DD. Outcome of treatment depends upon
Case selection Timing of surgery. Patients expectations modified by informed consent. Operation technique Post operative rehabilitation programme.

Aims of treatment
Being a genetic disease it is wrong to contemplate a lasting cure. Careful decision is advised before planning an operative treatment as it is a double edged sword for this condition. Key aim
Alteration in biomechanics of the hand to shield any residual DD tissue from tensile forces .

 Limitations
Impossible to define where lesion stops. Margin of clearance is not possible. Residual tissue seem to capable of amounting the same response of nodule , cord , contracture. Though an operation has ability to benefit the patient in the form of releasing joint contracture, removing Dupuytren tissue & interposing healthy tissue to prevent future linking up but has the risk of inadvertent division of digital nerves and vessels, creating a PIP jt hyperextension, problems of wound healing, stiffness of hand & reflex sympathetic dystrophy.

Operative indications
Loss of function Rate of progression
It is important to rely on above criteria than amount of contracture when deciding on surgery.( MP joint contracture can be corrected no matter how long lasting but PIP joint may not be.)

Assessment of lesion
Out of the several ways , scoring system developed by Tubiana & Michon is most reasonable system to assess the disease. Scoring system of Tubiana & Michon(1961)
Hand is divided into 5 rays. Total contracture (MP+PIP+DIP) is measured & allocated a number depending on the amount of deformity by increments of 45. N stages with no deformity bt nodule.

 0 no lesion. N palmar / digital nodule with out flexion deformity. 1 . total flexion deformity bw 0 45. 2 . ------do------------------- bw 45 90. 3 . ----------do--------------- bw 90 135. 4. ------------do-------------- > 135. palmar lesion are recorded by letter P , digital lesion by D.

Things to be considered prior to surgery

Patient
General condition of patient too frail. Comorbid condition limiting possible rehabilitation parkinsonism / stroke. Unrealistic expectation or unwilling for rehabilitation or criticizing last surgeon. Family history to condition patients expectation. Profession of the patient.

 The disease
Aggressiveness of the disease.
Age , duration and speed of the disease.

Associated diabetes may suggest a milder course, alcoholism - difficulty in compliance with post op rehabilitation. Presence of disease in other sites ( knuckle pads, plantar fibromatosis, Peyronies disease)
More aggressive disease.

Preoperative information to patient

Unknown cause, possibly genetic underlying disease tendency cant be treated. Purpose of operation is to straighten the hand bt correction of flexion contracture may be incomplete. Healing time is surprisingly long ( dressing for minm 3 4 wks or upto 6 wks with a skin graft). Full recovery takes atleast 3 month.

 Possible complication of nerve injury ( sensory loss, tingling) , definite risk of stiffness & risk of digital arterial injury in cases of recurrence. Possibility of recurrence and extension is high ( upto 50% in most of the series) & the possibility depends not only upon the operation but also on the rehabilitation programme and patient diathesis.

Operative technique
It must be recognised that a desire for wide exposure of vital structure to avoid damage will conflict with the aim of limiting tissue trauma and its subsequent inflammatory response in the hope of minimizing pain, stiffness, swelling and scarring. It is necessary to consider three aspects of surgical plan
Management of skin Management of fascia Management of joints - PIP jt.

Management of skin
Skin incision
Longitudinal
Adv : progressive extensile exposure. Principles Scar should nt cross concavities. Zig-zag scar allow better exposure. Shortened skin can be lengthened by either Z -plasty or multiple Y V advancement.

1. T shaped incision exposing all three cords that contract the thumb &1st web space. 2. Lazy s incision not recommended. 3. Brunner incision 4. Multiple Y V incision with Z plasty 5. Midline longitudinal incision closed with Z plasty.

Straight line incision subsequently broken up by Z plasties.

Most popular technique

Adv :
Allows exposure of NV strs progressively. Disadv :
Creating a longitudinal deep wound as a pathway for later recurrence.

Better to keep all the fats on the skin flap in palm to avoid skin necrosis. At the end of fascial dissection , Z plasties are designed and fashioned by taking into account the areas of thinning, buttonholing.

 Brunner type Zigzag incision

Disadv
Does not allow lengthening

Multiple Y V advancement flaps

Can be raised as a zigzag incision. Considerable flexibilty Flaps are safe as no need for rotation of flaps.

 Moermans small curved incision Made along the cord with the aim of excising intermittent length of cord and leaving alternate lengths of fascia intact. 1. Segmental fasciectomy 2. Fasciotomy 3. Limited fasciectomy

 Transverse
Adv : less likely to be a pathway for subsequent scar contracture. Dupuytren used transverse incision dividing the aponeurosis and leaving the wound open followed by splintage in the healing period. Subsequently revised by McCash. Presently the term open palm technique is used which is basically a wound management technique that avoids post operative hematoma. McGregor technique :
palm is divided across transversely so that all fascial cords retract followed by a split thickness grafting . Very useful in managing recurrence associated with much shortening and induration of the palm

 For 2 adjacent rays it is usual to make an incision along the distal palmar crease with a proximal extension and then make distal longitudinal incisions for each involved digit.

 Skin excision ( dermofasciectomy)

Wide excision of the involved palmar and digital skin combined with skin grafting. Popularised by Hueston for 3 reasons To replace skin shortage. To produce a firebreak( biologically new concept gaining much acceptance) To replace dermis infiltrated with myofibroblast. Adv : lower recurrence rate.

Management of fascia
Standard Limited fasciectomy (Hueston)
Longitudinally oriented incision from palm to digit extending proximally upto proximal palmar crease. Longitudinal cord is divided by pressure with sharp scalpel and elevated from proximal to distal. In the digit, removal of involved cords rather than a prophylactic fasciectomy. Important points
Skin flap raised with preserving perforators Bipolar hemostasis dissection of cords should be superficial to Transverse fibers of palmar aponeurosis to avoid damage to NV strs.

 Radical fasciectomy
Involves extensive removal of fascia in the palm with extension into the involved fingers only. Dissection is approached distally ( easier identification of NV bundles) all the fascia removed enblock in between the NV bundles Lost its flavor as there is no clear evidence to suggest the advantage of extensive palmar dissection.

Segmental fasciectomy ( Moermans)

Discontinuous removal of the longitudinal cords through a series of short curved incision and leaving the intermediate section of fascia undisturbed.

 Fasciotomy
Subcutaneous fasciotomy
Longitudinal skin stab incision . No. 11 blade / tenotome / fasciotome (luck) for fasciotomy. Blade introduced in line with the cord and turned perpendicular with firm pressure to force the blade into the cord. Use is restricted to palm ( unpredictable relationship of NV strs with cord at the level of fingers) Complication
Skin tear Nerve dysthesia Incomplete division of cord rapid recurrence.

 Open Fasciotomy
Cord is divide under direct vision through a longitudinal skin incision of size approx 2 cm along one side of the cord

Advantages of fasciotomy
Simple & safe when limited to palmar level. Quick with limited post op handicap.

Indication
Elderly patient or patient with health problems presenting with well defined cord with limited skin involvement at the palmar level.

 Non operative methods

Needle fasciotomy ( de Seze, french rheumatologist)
Corticosteroid injection in and around the cord then dividing the cord subcutaneously with a needle then rupturing it with a forceful extension of the finger. Several sittings with an average of 6 wks is necessary to obtain full extension. Drawbacks
Risk of traumatic division of the NV bundles and flexor tendon. Skin breakage, transient dysesthesia, local infection.

Can be recommended in the early stages of disease, women ( high risk of reflex sympathetic dystrophy with fasciectomy), and in elderly or unhealthy patients.

 Enzymatic fasciotomy
Trypsin + hyaluronidase injected locally followed by forceful extension of the involved finger.
Disadv rapid recurrence.

Phase 3 trial of injecting clostridial collagenase to lyse and rupture cords is undergoing.

 Physiotherapy Steroid injection

Reduced rate of fibroblast proliferation and increased rate of apoptosis of fibroblasts and inflammatory cells. Complication
Dermal atrophy Skin depigmentation Flexor tendon injury.

Management of joint contracture

MP joint tolerate flexion contracture for prolonged period but not PIP joint. MP joint can be released by simple fascial procedure . PIP joint release require sequential release of other structures in addition to fascial surgery.

 PIP joint release

Checkrein ligament release 2 ligamentous cords lying anterolaterally and running from the proximal swallowtail extension of volar plate to the neck of proximal phalanx. Accessory collateral ligament release Extend from the condyle on the head of the proximal phalanx to lateral edges of volar plate. Finally Gentle manipulation to separate vincula.

 However when fasciectomy + joint release is compared with fasciectomy alone , no clear benefit is noted and the management of PIP joint contracture remains controversial.

Choice of operation
Moderate contracture of MP or PIP joint in one or two ray in patient older than 45 yrs without risk factors open fasciectomy. Well defined cord with only MP joint contracture in elderly patient fasciotomy. Diffuse palmar lesion with severe skin adhesion & involvement of multiple rays McCash technique.

 Young (<45 yrs), strong diathesis, rapidly progressing dermofasciectomy. Recurrence dermofasciectomy. Nodular stage no surgery. Severe flexion deformity pre operative skeletal traction.

Post operative care

Mild compressive bulky dressing Adjacent finger is usually included in the dressing. In case of graft, hand immobilised in dorsal splint with wrist extended, MP joint flexed and IP joint slight extended.

 Hand therapy
In cases of PIP contracture. Ist goal is to recover full finger flexion. Extension is restored progressively. Early dynamic splint is recommended . However if the central slip tenodesis test is positive involved PIP joint is immobilised in passive extension splint for 3 wks. K wire if used should be removed at 10 days then joint mobilised.

Complication
Peri operative
Injury to NV structure. Buttonholing of skin.

Post operative ( approx 17%)

Hematoma is the most common complication which if not recognised or overlooked will lead to infection graft loss + wound dehiscence & joint stiffness. Skin necrosis Reflex sympathetic dystrophy( sympathetic mediated pain) usual incidence of 4.2% ,more common in women.

 Other factors with negative influence

Early age at onset. Presence of associated disease ( diabetes) Isolated involvement of little finger. PIP joint contracture. Proliferative stage

Recurrence
Defn : reappearance of dupuytrens tissue in an area previously operated on & cleared of abnormal fascia at that time. Incidence 63 % to 71% in different series. Appearance of new lesion in a new area extension. Distiguished from scar contracture as scar tissue is thinner, less prominent and decrease with finger flexion.

 Predisposing factors
It is the disease process rather than the operation that determines the recurrence rate , thus a strong dupuytren diathesis, young age, aggressive course are strong risk factors.

Important points to be appreciated

Standard anatomy cant be relied upon nerve & vessels can be any where. Dissection will be much more difficult- all strs. Likely to be encased in hard scar tissue & it may be impossible to dissect out NV str potential damage should be explained. NV strs may be damaged in the first surgery.

 PIP jt may have fixed flexion deformity salvage procedure may be indicated. Patient expectation of long period of remission If a PIP joint release has short lasting benefit its better to go for arthrodesis rather than attempt at another release. Dermofasciectomy is the preferred operative technique. Risk of all complication is higher. Finger amputation may be a possibility.

Critical points
Surgery under loupe with torniquet control. Relief of joint contracture should be guiding principle of surgery with no attempt to completely excise Dupuytrens tissue. Adequate exposure, raising of well vascularised skin flaps( preserving perforaters), allowing skin lengthening in the form of Z plasty or Y-V advancement when necessary.
It should be kept in mind that skin of volar aspect of palm and digit is very weak and skin tears readily ; stiff and doesnt tolerate tension and rotation.

 Hemostasis is vital for final outcome. Bipolar coagulation to coagulate all bleeding point after removal of the torniquet at the end operation is the very imp step. Proximal limit of fasciectomy is Kaplans cardinal line drawn from ulnar border of fully abducted thumb and crossing palm transversly. Dissection at digital palmar junction requires indepth anatomical knowledge & proper identification of natatory ligament, spiral cord, displaced digital NV bundle.

 For removal of diseased tissue at finger level , lateral part of web space is useful starting point. ( regardless of their course in the proximal phalanx, NV bundles found laterally at the PIP level on each side of joint) Little finger contracture is more difficult because of severity of contracture, small size of structures and coexistence of different types of cord. ulnar retrovascular cord, usually deeper and more ulnar than the NV bundle , often confused with tendon of abductor digiti minimi.

 In cases of Significant 1st web space contracture , web space is approached through a standard two flap Z plasty. Identification of NV bundles of thumb & index finger( ulnar bundle is easy to find but radial is difficult) division of distal and proximal transverse commisural cord

 Additional procedures may be required in cases of long standing contracture

Proximal tendon sheath contracture transverse incision at the level of proximal phalanx and PIP joint. PIP jt contracture transverse incision of checkrein ligaments just proximal to arterial br. to vinculum longum.

Future
1. BMP 2. Gene therapy 3. Nitric oxide

Thank you