Dupuytren’s Disease

• Baron Guillaume Dupuytren, 1831– Described the condition of palmar fascial

contraction

Epidemiology

• 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 1. Alcohol and liver disease Icelandic cohort study2. Smoking3. Manual work4. Diabetes5. Epilepsy

Strong Diathesis

• 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

• Palmar Aponeurosis

• Hypothenar Aponeurosis

• Thenar Aponeurosis

• Digital Fascia

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

• 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 – midaxial, palmar

2. Cleland’s ligament – thicker, midaxial, dorsal

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, fibroblastsincreased 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 HypothesisLocalized 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

• Age

• General Health

• Motivation

• Type of hand – Aesthetic , Workman’s

• H/O CRPS

• Type of involvement

• Deformity and progression

Surgical Management

• 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

• Flaps

1. Cross finger

2. Seagull

3. Dorsal transposition flaps

4. Dorsal Rotation Flaps

5. Flag Flaps

Contracted Fascia Management

• Fasciotomy• Fasciotomy and grafting Extensive

Dupuytren’s --- Firebreak • Fasciectomy 1. Segmental2. Complete Longitudinal fasciectomy3. Radical Palmar Fasciectomy4. 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-A4Vascular 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 circulationSkin flap Thinning , Button hole

• Post operative Haematoma Skin lossInfection Edema Wound Dehiscence

Complications

• Dupuytren’s Flare – Inflammatory reaction occurring 2-3 wks after the surgeryMore common in women 20 % Acute carpal tunnel syndrome Redness, pain, edema, stiffnessSympathetic blockade, oral steroids, carbamazapine

• Reflex Sympathetic Dystrophy – 5 x more common in women (5 %)Pain, edema, stiffness, vasomotor symptomsSympathetic blockade, oral steroids, carbamazapine

Recurrence and Extension

• 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