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Dupuytren’s Disease

Mark Bagg, MDMarch 24, 2017

Outline

• History

• Basic Science

• Anatomy

• Diagnosis

• Treatment

• Complications

Dupuytren’s Disease

• Unsolved Issues of the 21st Century– Cause

– Cure

History of Dupuytren’s

• Disease of the Vikings

• Felix Plater- 1614

• Cline/ Cooper- 1777

• Baron Dupuytren-1832– “Napoleon of Surgery”

– “First among surgeons, last among men”


Basic Science

• DD is a fibroproliferative disorder affecting the palmar fascia.

• Shortening of the palmar fascia can be attributed to fibroblasts and myofibroblasts present in diseased tissue.

• These cells lead to the formation of a nodule followed by a cord that are the hallmark findings in DD.

Basic ScienceTomasek, et al JBJS-A 69:1987

• Myofibroblast - cell responsible for tissue contraction

• Fibronectin– migration

– differentiation

– adhesion

• TGF-B1

Dupuytren’s Is Progressive

• Palmar lesions• Nodule formation

• Cord formation• Digital contracture begins

• Contracted cords• Flexion deformities

cord formation

contractures

Progression of Dupuytren’s*

nodule

Luck JV. J Bone Joint Surg [Am]. 1959;41:635-664.

cord

Epidemiology

• Northern European descent- “disease of the Vikings”

• prevalence 2- 42%

• Mikkelsen’s study- 9.4% men/ 2.8% women

• Low prevalence in Blacks and Asians

• Autosomal dominant with incomplete penetrance


Epidemiology

• Autosomal dominant with incomplete penetrance

• Gender issues– Men:Women- 6:1

• JHS Volume 32, Issue 9, November 2007, 1423-1428

– Men- peak onset about 10 years before women

– Women- Flare rxn after surgery more common (7-20%)

Etiology (Associations)

• Alcoholism (Noble, JHS-B 17:1992)• Smoking (An, JHS-Am 13:1988, Burge, JBJS-B

79:1997)• Diabetes (Noble JBJS-B 66:1984) • Epilepsy- role of anti-epileptics may play a role

– D/C phenobarbital- regression of cords and knuckle pads

• Injury- little conclusive evidence• Manual Work- conflicting data

– Exposure to vibration- positive correlation

Clinical Presentation

• Skin changes → Nodule formation → Cord formation → Contracture– Reilly, Stern JHS Am 2005 30(5)

– Skin changes can be the earliest manifestation of DD.• Loss of mobility, thickening, dimpling, and skin pits.

– The nodule is key for early diagnosis.• Firm soft tissue mass, well defined, and localized.• Located between distal palmar crease and PIP flexion crease.• Usually painless.

– The nodule often regresses giving way to the cord.• Normal bands are precursors of the cord.• Cord becomes prominent, tendon like, with an abundance of collagen tissue.

– Contracture: RF > SF > Thumb > MF > IF• Small size of pretendinous bands on radial side likely to be reason for this

distribution.

Dupuytren’s Diathesis• Diseased tissue outside the

hands:– Plantar fibromatosis

(Lederhose’s disease)– Penile fascial involvement

(Peyronie’s disease)– Knuckle pad keratosis (Garrod’s

nodes)

• Strong gene expression.• Disease in the 20’s and 30’s.• Very aggressive cord

development.• Higher risk for poor outcome.


Anatomy• Longitudinal fibers:

– Pretendinous bands

– Spiral bands

– Lateral digital sheets

• Transverse fibers– Transverse ligament of PA (Skoog’s

fibers)

– Natatory ligament

– Grayson’s ligament

– Cleland’s ligament

• Vertical fibers– Superficial vertical bands

– Septa of Legueu and Juvara

Anatomy of the Palmar Fascial Complex

• Radial Aponeurosis

• Ulnar Aponeurosis

• Palmar (central) Aponeurosis– Retinaculum for flexor

tendons

– Stabilize the metacarpals

– Supports and anchors palmar skin

Anatomy of the Palmar Fascial Complex

• Pre-tendinous Bands

• Transverse fibers

• Vertical Fibers– superficial connect skin to

fascia

– deep fibers- Septa of Legueu and Juvara which form 7 distinct compartments

Anatomy of the Palmo- Digital FascialComplex

Spiral BandNatatory ligament


Anatomy of the Digital Fascial Complex

• Digital Fascia– Lateral digital sheath

– Cleland’s ligament (dorsal)

– Grayson’s ligament (volar)

– Retrovascular Fascia

Patho-anatomy

• Nodules tend to form between the distal palmar crease and the PIP flexion crease.

• Cords form along the pathways of normal fascial anatomy.

• Normal bands become pathologic cords.

• The NV bundle becomes intertwined with diseased tissue.

• As the cords contract, the encircling pathway becomes more linear causing the NV bundle to spiral.

Pathoantomy of the Palmar Fascial Complex

• Palmar Cords-– from pretendinous band

• Vertical cord– from vertical fibers of

Legueu/Javara

• Abductor Digiti Minimi Cord

Fascial structures NOT involved in Dupuytren’s disease

– Transverse Ligament of Palmar Aponeurosis (Skoog)

– Cleland’s ligament

– Transverse retinacular ligament

– Oblique retinacular ligament


Treatment Options

• Surgical options– Open (fasciotomy, fasciectomy)

– Closed (needle aponeurotomy)

• Non-surgical options– In February 2010, the FDA approved

XIAFLEX®, anonsurgical treatment option for adults with Dupuytren’s contracture with a palpable cord

Efficacy: MP and PIP Joints

n=133 n=70n=20 n=25n=69 n=34n=11 n=10

Percentage of patients achieving a reduction in contracture to 0°to 5° of normal 30 days after up to 3 injections

Hand Center MPJ – 76 %

Efficacy By Severity of Joint Contracture Before Treatment

MP Joints PIP Joints

(20°-50°) (>50°-100°) (20°-40°) (>40°-80°)

n=81 n=10 n=52 n=10 n=21 n=5 n=49 n=20

Percentage of patients achieving a reduction in contracture to 0°to 5° of normal 30 days after up to 3 injections

n=43 n=7 n=26 n=4 n=9 n=2 n=25 n=8


• When do I use Collagenase now– Isolated single digit MPJ contracture >30 degrees

with a DISTINCT pre-tendinous cord with associated PIP contracture less than 30 degrees.

– Isolated single digit PIPJ contracture between 20-40 degrees with DISTINCT raised cord

Indications for Surgery

• MCPJ contracture > 30°– McFarlane RM, In McCarthy’s:

Plastic Surgery 1990

• PIPJ contracture > 30°– McFarlane RM, In Green’s:

Operative Hand Surgery, 1993

• Hueston’s Table Top Test

Treatment- Fasciotomy

• May be useful in patients with limited life expectancy

• As a prelude to more definitive mgmt

Treatment- Fasciectomy

• Limited (Moerman, Gonzalez)– Disease likely to progress– May not require additional surgery

• Regional (partial)– most commonly done procedure– removes only the diseased tissue

• Extensive (radical)– Removes all palmar fascia– increased complication/ stiffness


Treatment - Incisions

Treatment- Open Palm

• Originally proposed by Dupuytren

• McCash popularized

• Lubahn (JHS, 9A: 1984)– Retrospective comparison of open vs close palm

– Open palm did better

– Better ROM, no hematomas

– Recurrence rate similar for both groups

Treatment - Skin Grafting

• Thought to prevent recurrence of disease – Hueston JT, Br J Plastic Surg 1970

• “Firebreaks”

• Dermofasciectomy with FTSG

• Risks: Hematoma, graft loss, stiffness

• May be indicated in young person with diathesis

Treatment - Joint Contracture

• MPJ- usually corrects with fasciectomy

• PIPJ – Tight structures: skin, flexor sheath, palmar plate

adhesion, assessory collateral ligament, intra-articular changes

– Attenuation of the central slip

– Secondary ctx release less predictable (Weinzweig, JHS, 1996)

– Gentle passive manipulation (Breed, et al. JHS, 1996)


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Cost Analysis

Societal standard for cost effective treatment: $50,000/ QALY Open partial palmar fasciectomy- $820,114/ QALY

Percutaneous Needle Aponeurotomy $96,474/QALY

Without anesthesia $49,631/QALY

Collagenase

@ $250.00 $31,856/QALY

@ $945.00 $49,995/QALY

@ $3250.00 $166,268/QALY

Cost Effectiveness of Open Partial Fasciectomy, Needle Aponeurotomy, and Collagenease Injection for Dupuytren Contracture, Chen, et. al. JHS Vol 36 A 1826

Complications• Overall complication rate – 20% McFarlane RM, In

McCarthy’s: Plastic Surgery 1990

– Nerve – spiral nerve– Arterial injury – redo contractures– Hematoma formation– Flap necrosis– Infection– Loss of flexion/ decrease ROM– Reflex sympathetic dystrophy “flare”

• 4% males• 8% females• Rate of 58% noted with simultaneous carpal

tunnel release????– McFarlane RM, Dupuytren’s Disease: Biology and Treatment 1990

– Recurrence• 2% to 74%

– McFarlane RM, Dupuytren’s Disease: Biology and Treatment 1990

Dupuytren’s Recurrence• Surgery- 2% to 60%, with an average of 33% depending

on the type of surgery (fasciectomy, fasciotomy, or needle aponeurotomy)

• Collagenase- 19.3%– Joint contracture to ≥20° in the presence of a palpable

cord in a joint that previously achieved a 0°-5° contractureimprovement or (b) a joint which underwent surgicalcorrection to treat contracture in that joint

• Update of original diathesis factors– Positive family history– Male gender– Early age

(<50 years) of onset– Bilateral involvement– Garrod’s pads

Hindocha S et al. J Hand Surg. 2006;31A:1626-1634.Rayan GM. Dupuytren’s disease: anatomy, pathology, presentation, and treatment. J Bone Joint Surg Am. 2007;89A(1):190-198.Tubiana R et al. Dupuytren’s Disease. London: Martin Dunitz Ltd.; 2000;243.

Predictive risk of recurrence 22% when no factors are present 71% when all 5 factors are present

Rehabilitation

• Begin AROM and AAROM at 3 days

• Extension night time splint beginning 3 days for 6 months

• Scar mgmt

• Edema Control

• MOTION is LOTION!!


RehabilitationSCIENTIFIC ARTICLE

• The Effect of Night Extension Orthoses Following Surgical Release of DupuytrenContracture: A Single-Center, Randomized, Controlled Trial

• Julie Collis, MSc (Hons), Shirley Collocott, BSc, Wayne Hing, PhD, Edel Kelly, MSc (Hons)

• JHS, 38A 2013

– randomized, controlled trial

– No difference at three months • therapy and nighttime splinting

• therapy alone

Questions?

SITE LAYOUT

Basic Science• Theory of local microvessel ischemia which

stimulates proliferation of fibroblasts and related cell types.

• Related growth factors – myofibroblast proliferation:

– TGF-1, TGF-– PDGF– bFGF

• Cytokines responsible for myofibroblast contraction:

– PGF2 alpha– LPA– Angiotensin II– Serotonin

• ratio of type III to type I collagen increased

• IFN-Ca++ Channel blockers counter effect of TGF-1


OITE

• Surgical intervention is first indicated for DD when which of the following findings is present?

– MCPJ contracture > 60° and a PIPJ contracture of any degree

– MCPJ contracture and a PIPJ contracture > 40°

– MCPJ contracture > 30° or PIPJ contracture of any degree

– MCPJ contracture of any degree and a PIPJ contracture > 30°

– Any contracture of the MCPJ or PIPJ

Cellular StructureTomasek, et al JBJS-A 69:1987

• Myofibroblast - cell responsible for tissue contraction

• Fibronectin– migration

– differentiation

– adhesion

• TGF-B1

OITE

• When performing palmar fasciectomy for DD, which of the following should not be performed at the same time?

– Trigger finger release

– Intra-operative digital nerve laceration repair

– Knuckle pad excision

– PIPJ arthrodesis

– Carpal tunnel release
