dupuytrens contracture
TRANSCRIPT
Introduction• In 1831,Baron Guillaume Dupuytren
described the condition of palmar fascial contraction (Dupuytren disease)
• It is a proliferative fibroplasia of the subcutaneous palmar tissue, occurring in the form of nodules and cords, that may result in secondary progressive and irreversible flexion contractures of the finger joints.
• Other changes include:– thinning of the overlying subcutaneous fat– adhesion to skin– and later pitting or dimpling of the skin.
History• Felix plater (1536-1614) gave the Ist description
of palmar fibromatosis.• Henry Cline (1750-1836) described the anatomy
& recommended surgical release.• Astley cooper (1768-1841) explained the etiology
as repeated trauma and described percutaneous fasciotomy.
• Guillaume Dupuytrene (1834) gave detailed anatomic pathology, C/F, natural history, surgical technique, postop care, response, follow up.
Epidemiology
• Age: Incidence increases with increasing age and peaks between 40-60 years
• Sex: Males > Females (7-15 times)
• Race: White Caucasians
• Geography: North European descent• Genetics is Unclear (Autosomal dominant with
variable penetrance)
Viking’s Disease
• Greatest concentration in Scandinavia and Great Britain (Ireland and Scotland)
• Viking heritage in original gene pool and follows pattern of Viking travel (prevalence decreases as distance increases from Europe)
• High prevalence in Australia due to British population.
Curse of The MacCrimmons
• First known to be prevalent in western isles of Scotland.
• MacCrimmons were musicians and pipers to the chieftains of the clan MacLeod of Skye
• Contractures inhibited playing bagpipes.
• Famous patients include Ronald Reagan, Margaret Thatcher, and creator of Captain Hook (inspiration for his claw hand).
• Associated with:1. Diabetes mellitus2. Cigarette smoking3. Alcoholism and liver disease4. HIV infection5. Epilepsy: Anti-epileptic drug Phenobarbitone6. Trauma7. Manual labor8. Rheumatoid disease9. Previous myocardial infarction10.Plantar fasciitis11.Peyronie disease
Dupuytren’s Diathesis• Strong gene expression causing physical findings.• Present earlier in life (20s and 30s).• Aggressive cord development with high incidence
of multi-digit and bilateral hand involvement.• Knuckles (Garrod’s nodes), plantar fibromatosis
(Lederhose’s disease), penile fascial involvement (Peyronie’s disease).
• High risk for poor surgical outcome due to higher recurrence rates, greater risk of surgical technical complications, and longer post-op care.
Patient Complaints
Fingers get in the way with:Washing faceCombing hairPutting hand in pocketRacquet sportsGolfPutting hand in glove
Symptoms
• First notice tender nodule or progressive palmar cord development.
• May be painless, and may avoid care until joint motion reduced.
• Symptoms may be present bilaterally, with one hand occurring first (not necessarily dominant hand).
• MCP joint affected first and then PIP joint.
• Ring and small finger affected first, after palmar involvement.
Palpable Nodules and Cords
• Firm nodules may be tender to palpation.• Cords proximal to nodules painless.• Atrophic grooves or pits in skin signify adherence to
the underlying fascia.• Tender knuckle pads over dorsal aspect of PIP joints--
indicates aggressive disease.
Positive Table top Test:The distance marked should be zero in a normal hand with a negative table top test.
Dynamic flexion contracture:When MCP joint is at neutral, the PIP joint contracture is more.When MCP joint is flexed, the deformity at PIP is reduced.This is attributed to the Central Cord involvement.
Grading
• Grade I: Thickened nodule & band skin tethering & puckering – full movements.
• Grade II: Pretendinous bands involved extension of fingers limited.
• Grade III: Flexion contracture.
RelevantAnatomy
The Palmar Aponeurosis• Thick triangular fascial
layer that covers the lumbrical and flexor tunnels between the thenar and hypothenar eminences
• Proximally: palmaris longus
• Distally: Longitudinal bands, called Pretendinous Bands
• Bifurcates distally to pass on either side of the tendons
Vertical Fibers
• Superficially they connect the aponeurosis to the dermis
• Deep fibers are of three types:1. Septa of Legueu and Juvara2. McGrouther’s Fibers3. Vertical septa between the lumbricals and flexor
tendons
• Septa of Legueu and Juvara are well developed fibrous structures arising from the deep surface of the aponeurosis at the level of the Metacarpal head and neck
• Pass down to the palmar plate and fascia over the interossei
• Eight septa, one on either side - four fibro osseous tunnels • Each tunnel has three compartments containing the
common neurovascular bundles and the lumbricals
Transverse Fibers • Natatory Ligament (NL, Superficial transverse
metacarpal ligament, STML)• Transverse ligament of the palmar aponeurosis (TLPA):
It is a distinct part of the palmar aponeurosis and gives origin to the vertical fibers of Legueu and Juvara
Natatory & Central Cord
Pretendinous BandsThree different insertions for the pretendinous bands:• Superficial layer: terminates into
the dermis distal to the MCP joint• Intermediate layer: passes deep to
the natatory ligament and the neurovascular bundles, merges with the lateral digital sheath, Spiral bands and may attach to the retrovascular band
• Deep layer: passes vertically down at the level of the A1 pulley and terminates in the vicinity of the extensor tendon
Hypothenar Aponeurosis
• Covers the muscles of the hypothenar eminence• Continuous with the ulnar border of
the palmar aponeurosis• Merges distally with the tendon of
Abductor Digiti Minimi and continues close to the lateral digital sheath
Thenar Aponeurosis
• Radial continuation of the palmar aponeurosis, much thinner
• Skin over thenar aponeurosis more mobile because there are a few vertical fibers connecting it to the dermis
Digital Fascia• It holds the skin in
position as the fingers or thumb move
1. Grayson’s ligament: Midaxial, Palmar
2. Cleland’s ligament: Thicker, Midaxial, Dorsal
3. Lateral Digital Sheet: Superficial fascia lateral to the Neurovascular bundles
4. Retrovascular band: Deep to the Neurovascular bundles, longitudinal fibers
Spiral Band of Gosset:
Pretendinous band, the lateral digital sheet and the Grayson’s ligament may involve the retrovascular band
• Gradual contraction of the spiral cord pulls the neurovascular bundle towards the midline which may come to lie transverse to the long axis
Spiral Band of Gosset
Pathologic Anatomy• Normal fascial structures in the hand and
digits are referred to as BANDS • Diseased fascial structures in Dupuytren’s
are referred to as CORDS • In Palm:
Pretendinous cords are involved resulting in MCP Joint flexion. Does not affect the neurovascular bundles and are painless.
Involvement of Vertical cords can cause pain and triggering.
Basic Pathology• Myofibroblasts are the histologic
hallmark of Dupuytren’s contracture• Increase in:–Type III collagen–Total collagen–Lysyl oxidase–Glycosoaminoglycans
• Increase in cellularity (fibroblasts).
Pathogenesis
• Local ischemia at the microvascular level increase in fibroblast & related cell types
• Fibroblasts then organize themselves along line of stress cords deformity
• Ischemia free radicals increased cells (fibroblasts)• Smoking, HIV, alcohol promote
free radicals• Increase Fibroblast
Vasoconstriction Ischemia (self perpetuating cycle)
Role Of Protein Factors
• PDGF, FGF, TGF-B increased collagen production• Myofibroblasts are more sensitive
Nodules & Cords:Major forms of diseased tissuesTwo distinct histological tissues
Nodules• Dense cellular collections of myofibroblasts: indicates
centers of high metaplastic activity.
• LUCK described 3 stages of progression of nodule:1. Proliferative: Young nodules with non-stress
aligned fibroblasts, grows & fuses to skin2. Involutional: Growth stops, Stress alignment of
fibroblasts, More collagen Fascial hypertrophy Nodule cord units
3. Residual: Size reduces, Acelullar fibrous cords
Nodules, Pits, Skin Contractures
Cords• No myofibroblasts• Highly organised collagen structure similar
to tendon• Nodules produce the contraction by
pulling the cords which expand across the joints
Myofibroblasts found in dermal & epidermal tissue cause recurrence
Treatment
Non Operative Management
• Collagenase Studies show good results in 90% patients with a single injection and maintained 9 months after treatment
• Radiotherapy, Dimethyl sulfoxide, Ultrasound, Steroids, Colchicine, Alfa interferon: None has shown any significant benefit
Operative Management• Indications:–A Positive Table Top Test: correlates with
MCP contracture of > 30-40°–MCP joint contracture ≥ 40°– Treatment of other digits on the same hand
should be considered when their MCP contracture are 20-30° or more.–PIP joint release if PIP joint contracture > 30°
• Important to distinguish true PIP joint contracture from apparent contracture (due to spiral cord)
• MCP joint contracture is measured with PIP joint held in extension
• PIP joint contracture is measured with MCP joint in flexion
Management Of Palmar Fascia
• Treatment options include:–Radical vs. Selective vs. Segmental
Fasciectomy–Fasciotomy–Amputation–Joint resection and arthrodesis
Surgical Fasciectomy• Radical Fasciectomy: Mostly abandoned– All palmar fascia removed– High amounts of wound complications, and
recurrence• Selective Fasciectomy: Most commonly used– Removal of all diseased fascia in palm/finger– Indicated when only ulnar one or two fingers involved– Rate of recurrence is 50%– Need for another surgery: 15%– Recurrence due to undetectable diseased fascia
remaining
• Segmental Fasciectomy–Removal of one or more segments of
diseased fascia through multiple small incisions in palms and fingers or through transverse/longitudinal plasties, with skin grafts
Incision (Basic Principles)• No incision should cross a flexion crease at
right angles on wound closure
• Thin potentially avascular flap should be avoided.
• Dissection start in normal anatomy and proceed distally.
• Start cord release in palm and identify Neuro Vascular Bundle>> then palmar-digital skin >>then digital.
Skin Management• Digital Skin Shortening can be
corrected by:–Release of skin corrugations by division
of the vertical fibers running up to the dermis–Multiple Z plasties–Open palm technique–Skin grafting
Skin Replacement• Skin shortage due to dermal contracture • Prophylactic firebreak to separate the ends of
contracted fascia• Recurrent disease • Electively excised as Hueston’s
dermofasciectomy • Skin graft • Flap
Management of Volar Skin
• Three types:–Direct closure–Full-thickness skin grafting–Open technique with wound
contraction
• Direct closure:–Primary wound healing–No need for skin grafts–Simple post-op management–Increased incidence of Hematoma and
Skin flap necrosis
• Full thickness skin grafting:Pros:• Less recurrence where full thickness graft used,
modulating effect on underlying fasciaCons:• Recurrence still possible beyond areas of graft• Graft loss• Hematoma formation• Immobilization may cause stiffness• Altered sensation on graft
• Open wound technique:– Transverse incision in palm at level of midpalmar
crease and extensions in fingers– Transverese incision is left open and covered with
non-adherent dressing– Daily dry dressing changes, healing in weeks– No granulation or epithelialization, instead
transverse wound contracts to pre-contracture length
– Less hematoma, wound edge necrosis, and infection– Inconvenience during 3-5 weeks for closure
Fasciotomy
• Diseased tissue incised but not removed• Used mainly in elderly patients or severe
disease when unable to comply with post-operative rehabilitation protocol
Joint Resection- Arthrodesis
• Severely contracted PIP joint• Avoids the potential for recurrent
PIP joint contracture and potential amputation neuroma
Amputation
• Rare• May be indicated:– In Flexion contracture of PIP joint, especially
little finger, when cannot be corrected enough to make finger useful–Or in case of vascular compromise
Newer TreatmentModalities
Collagenase
• Enzymatic percutaneous fasciotomy of residual stage disease
• Collagenase diluted in calcium chloride • Currently treatment only available at
stony brook medical center, under FDA “orphan drug status” in phase III trials
• Injected straight into nodule
• Minimal side effects: tenderness at injection site, hematoma, edema.
• Preliminary results by Badalamente and Hurst show results of more than 90% correction of MCP joint, 66% correction of the PIP joint, and minimal recurrence rates.
• Although collagenase is showing promise in clinical trials, surgery is still considered the standard of care
Needle Aponeurotomy• Fascia contractures sectioned
percutaneously with sharp-edged bevel of local anesthetic needle.
• The treatment is only performed in Europe, primarily France.
• Outpatient, $150 for 20 minute session and requires no physical therapy.
• Temporary treatment, not cure.
Gamma Interferon
• Gamma-interferon is a cytokine produced by t-helper lymphocytes.
• Shown to decrease fibroblast replication, alpha-smooth-muscle actin expression, and collagen production.
• Fails to have long term disease free effect
Postoperative Rehabilitation• Commenced after early inflammatory phase (3-
5 days)• ROM exercises for short periods, repetitive• Splinting:– Initially static for 2 weeks with MCP in 10-20°
Flexion, PIP straight and DIP joint free– After 2 weeks PIP splint at night for 8-10 weeks
• Scar management
Complications• Intra-operative:– Digital nerve division.– Hematoma formation.– Wound healing difficulties (flaps).– Vascular compromise of a digit.
• Post-operative:– Patient compliance.– Reflex sympathetic dystrophy (flare reaction).(1-8% prevalence, 2x more common in women)
• Recurrence up to 63%.
In Case Of Intra Operative Arterial Insufficiency
Due to-direct trauma, traction and vasospasm Flex the finger Warm the finger with warm irrigant solution Apply topical papavarine (30 mg/mL) / lignocaine Be patient. Allow the relaxation, warming, and
antivasospasm interventions time to work. The artery may require up to 10 minutes for the restoration of perfusion
If arterial insufficiency persists beyond 10 minutes, explore the digital artery throughout the extent of dissection. Repair of a partial or complete laceration should be performed under the operating microscope. A vein graft may be necessary if undue tension is present
Recurrence
• Presence of diseased tissue in surgically treated field.
• Cure at genome level: Surgical excision improves hand function.
• Recurrence more common at young ages and in Dupuytren’s diathesis.
• Most commonly diseased tissue from untreated areas extends into treated areas.
• Recurrence rates are more in presence of residual tissue incompletely excised, leaving behind myofibroblasts in skin.
• Full skin grafts rarely recur, due to complete removal of all nodular area in dermis and epidermis.
Summary
• Dupuytren’s contracture is a genetic disease.• Patients must understand that surgery is not a
cure, and has potential side effects.• Future treatment more medical and less
surgical, with eventual cure to be at genomic level.
Thank You