here - Dr. Pouria Moradi

Dupuytren’s Disease
• Baron Guillaume Dupuytren, 1831
– Described the condition of palmar fascial
contraction
Epidemiology
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Prevalence – Age, sex, Race, Geographical distribution
Increasing Age Peaks between 40-60
Men > Women 7-15 times
White Caucasians of North European descent
Genetics unclear autosomal dominant, variable penetrance
Associations
Alcohol and liver disease Icelandic cohort study
Smoking
Manual work
Diabetes
Epilepsy
Strong Diathesis
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Strong family history
Young patient
Bilateral disease with radial involvement
Diffuse dermal involvement
Lederhosen – soles of feet
Peyronie’ s disease – penile
Garrod’s knuckle pads – PIP joints
Recurrence and extension
Palmar Fascia Anatomy
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Palmar
Hypothenar
Thenar
Digital Fascia
Aponeurosis
Aponeurosis
Aponeurosis
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
Pretendinous Bands
• McGrouther – three different insertions for the
pretendinous bands
• Superficial layer – terminates into the dermis distal to
the MCP joint midway between the distal palmar and
proximal digital creases
• Intermediate layer – passes deep to the natatory
ligament and the neurovascular bundles, merges with
the lateral digital sheath. Spiral bands of Gosset and
may attach to the retrovascular band
• Deep layer – passes vertically on either side
FTS at the level of the A1 pulley and
terminates in the vicinity of the extensor
tendon
Pretendinous Bands
Palmar Aponeurosis - Vertical Fibers
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Exist throughout
Superficially they connect the PA to the
dermis
• Deep fibers are three types
1. Septa of Legueu and Juvara
2. McGrouther’s Fibers
3. Vertical septa between the lumbricals and
flexor tendons
• Septa of Legueu and Juvara – well developed fibrous
structures arising from the deep surface of PA at the
level of the MC head and neck
• Pass down to the palmar plate and fascia over the
interossei
• Most developed distally where they blend with the
deep transverse intermetacarpal ligament
• They have a sharp proximal border lying 1cm distal
to the superficial palmar arch and approx. 1 cm in
length
• Eight septa, one on either side - four fibro
osseous tunnels
• Each tunnel has three compartments
containing the common neurovascular bundles
and the lumbricals
• The radial nv bundle of index and the ulnar nv
bundle of little are not included
Palmar Aponeurosis – Transverse
Fibers
• Natatory Ligament (NL, Superficial transverse
metacarpal ligament, STML)
• Transverse ligament of the palmar aponeurosis
(TLPA)
The TLPA differs from the deep transverse
intermetacarpal ligament It is a distinct part of
the palmar aponeurosis and gives origin to the
vertical fibers of L&J
Hypothenar Aponeurosis
• Covers the muscles of the hypothenar eminence
• Continuous with the ulnar border of the palmar
aponeurosis
• Merges distally with the tendon of ADM and
continues close to the lateral digital sheath
• Also attached to the palmar plate of the mcp joint,
TLPA, ulnar saggital band while vertical fibers
connect to the dermis
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
• The distal transverse commissural ligament –
NL
• The proximal transverse commissural ligament
- TLPA
Digital Fascia
• The digital fascia holds the skin in
position as the fingers or thumbs are
moved
1. Grayson’s ligament –
palmar
2. Cleland’s ligament –
midaxial, dorsal
midaxial,
thicker,
Digital Fascia
3. Lateral Digital Sheet – superficial fascia
lateral to the nv bundles – NL , Spiral band
4. Retrovascular band – deep to the nv bundles
longitudinal fibers
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
• Palm – Pretendinous cord resulting in MCPJ
flexion Does not affect the nv bundles
- Vertical cords can cause pain and
triggering
Central cord ADM
Natatory & Central Cord
Spiral Band of Gosset
Pretendinous band, its
distal continuation, the
lateral digital sheet and
the Grayson’s ligament
May involve the
retrovascular band
Gradual contraction of
the spiral cord pulls the
nv bundle towards the
midline which may
come to lie transverse
to the long axis
Spiral Band of Gosset
Biology and Biochemistry
• Fibroblast proliferation, collagen deposition
• LUCK, Three Stages
• Proliferative Stage – increased number of cells during
nodule formation
• Involutional Stage – longitudinal bands of collagen
fibers – less biologically active
• Residual Stage – biologically quiescent disappearance
of cells, contracted cords densely packed tough
inelastic fibrotic palmar fascia
Biology and Biochemistry
• Biologically similar to wound repair
• Myofibroblasts – nodules
• Gelberman – myofibroblasts in nodules and
fibroblasts with prominent microtubules in the
fascia
• Murell – compared fibroblasts in both tissues
and found them identical Just more abundant
in Dupuytren's
Biology and Biochemistry
• Increased levels of fibroblastic growth factors in the diseased
fascia
• Macrophages – perivascular cuff early in disease
• Basic Fibroblastic growth factor (bFGF)
bFGF – proliferation of myofibroblasts and endothelial cells
which narrow the lumina of vs
• Platelet derived growth factor (PDGF)
cellular signal for myofibroblast proliferation
increase rate of type III collagen synthesis
• Transforming growth factor (TGF beta)
stimulates growth of mesenchymal cells, fibroblasts
increased deposition of extra cellular matrix
Pathogenesis
• Intrinsic theory- McFarlane 1974
• Diseased cords are as a result of the pathologic
changes in the normal fascia
• Cords arise from defined fascial precursors and
progress along predictable routes determined
by normal fascial anatomy
• Does not offer a rational explanation for the
central cord commonly seen in Dupuytren’s
Pathogenesis
• Extrinsic Theory – Hueston 1985
• The fibrotic process begins with nodules and
progresses to cords
• Nodules arise de novo by metaplastic transformation
of fibrofatty tissue and later spread as cords just
superficial to the palmar aponeurosis
• Rational explanation for the presence of nodules,
recurrence after fascial excision, lower recurrences
after dermofasciectomy
Pathogenesis
• Synthesis Theory - Gosset’s 1985
• Cords and nodules represent different forms rather
than different stages of the disease process
• Cords arise from palmar fascia and nodules de novo
• Anatomic studies Strickland and Leibovic support the
synthesis theory as the best reconciliation between the
intrinsic and extrinsic
Murell’s Free Radical Hypothesis
Localized ischemia
Free Radical
generation
Microvs narrow
lumina thickened
endothelial cells
Endothelial
xanthine oxidase
derived free
radicals
Stimulate
fibroblasts
localized damage
Pericyte damage
collagen deposition
further ischemia
Increased
Hypoxanthine
concentration
Xanthine
oxidase activity
1990
Unifying Pathogenesis Andrew 1991
Clinical Presentation
Anatomical
Distribution
Skin Involvement
Contracture
Digital Allen’s Test
Maturity
General Condition
Anatomical Distribution
Normally Ulnar
One or more digit
Different stages of
involvement
Skin Involvement
Nodules, Cords, Pits, Skin Shortening
Non Operative Management
1. External Fixation and Distraction
• Progressive lengthening of the digital nv bundle
• Easier access to the diseased cords after distraction
in the fingers that are very contracted
• Only useful in end stages
• Long distraction time
• Complications – infection, stiffness, pain, CRPS
type I
• Contractures recur quickly without fasciectomy
Non Operative Management
• Collagenase – achieved full extension in 90%
patients with a single injection and maintained
9 mths after treatment
• Radiotherapy, dimethyl sulfoxide, ultrasound,
steroids, colchicine, alfa interferon None has
shown any significant benefit
Operative Management
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Age
General Health
Motivation
Type of hand – Aesthetic , Workman’s
H/O CRPS
Type of involvement
Deformity and progression
Surgical Management
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Formulation of a plan regarding the
management of the skin, involved fascia,
joints and extensor apparatus
• Management of Skin
1. Incision
Longitudinal
Spiral cord – The nv bundle is pulled towards the
centre and may lie transversely just under the
skin
Skin Management
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 replacement
Surgical Management
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
Skin Replacement
Skin Replacement
Skin Replacement
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Flaps
Cross finger
Seagull
Dorsal transposition flaps
Dorsal Rotation Flaps
Flag Flaps
Contracted Fascia Management
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Fasciotomy
Fasciotomy and grafting Extensive
Dupuytren’s --- Firebreak
Fasciectomy
Segmental
Complete Longitudinal fasciectomy
Radical Palmar Fasciectomy
Dermofasciectomy
Contracted Fascia
Contracted Fascia
Contracted PIP Joint
• Gentle passive manipulation
40% peri articular adhesions which require GPM
• Long standing contractures may produce joint
changes requiring surgical release
Volar plate – check rein ligaments
Accessory collateral ligaments
Flexor tendon sheath release between A2-A4
Vascular compromise
• Extensor apparatus – patients with 60 degree
contracture, 80% will show central slip attenuation
Postoperative Rehabilitation
• Very important
• Commenced after early inflammatory phase
(3-5 days)
• ROM exercises, short periods, repetitive
• Splinting, initial static for 2 wks, MCPJ 10-20
deg. Flexion, PIPJ straight, DIPJ free then PIP
splint at night – 8-10 wks.
• Scar management
Complications
• 17-19 % 0verall
• Intra operative
Nerve Injury
Digital circulation
Skin flap Thinning , Button hole
• Post operative
Haematoma
Skin loss
Infection
Edema
Wound Dehiscence
Complications
• Dupuytren’s Flare – Inflammatory reaction occurring 2-3 wks
after the surgery
More common in women 20 %
Acute carpal tunnel syndrome
Redness, pain, edema, stiffness
Sympathetic blockade, oral steroids, carbamazapine
• Reflex Sympathetic Dystrophy – 5 x more common in women
(5 %)
Pain, edema, stiffness, vasomotor symptoms
Sympathetic blockade, oral steroids, carbamazapine
Recurrence and Extension
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Recurrence is the reappearance of disease in the
area of previous surgery
26-80 %
• Extension is the appearance of new disease in an
area not subjected to surgery
• Common causes of failure
1. Failure to remove all the involved tissues
2. Failure to correct PIP joint contractures at initial
surgery
Recurrence
3. Failure to recognize central slip attenuation at initial
procedure 80 % of patients with a 60 deg flexion
contracture at PIPJ
4. Failure to recognize perivascular fibrosis leading to
ischemia on full extension
5. Poor cooperation with hand therapy After excision
of fascia haematoma and scarring is inevitable
Vigorous hand therapy limits the effect of scarring