dupuytren’s disease

definition

·      deforming fibrotic condition of the palmar aponeurosis

epidemiology

·      incidence is 4-6% (in Caucasians)

·      more common in elderly males

·      M:F = 2:1 to 10:1 (greater sex difference in younger patients)

·      in 17% of males > 65 yrs

·      bilateral in 50%

·      virtually confined to Caucasians

·      significant racial differences

·      most common in Scandinavia and British Isles (Celtic)

·      rare in Mediterranean

·      almost unseen in blacks and Asians

Aetiology
Heredity

·      there is a hereditary component

·      probably autosomal dominant with incomplete penetrance

·      may be Dupuytren’s diathesis with similar cellular changes in

·      hands (palmar and digital fibromatosis)

·      dorsal knuckle pads

·      feet (plantar fibromatosis - Lederhose’s disease)

·      penis (Peyronie’s disease)

Alcohol

·      increased incidence in alcoholism

Diabetes

·      30% higher prevalence in diabetics

Epilepsy

·      Dupuytren’s seen in 40% of institutionalised male epileptics

·      may be related to phenobarbitone administration

Occupation

·      association suggested in past

·      weight of evidence against it

·      not more common in manual workers

·      not related to handedness

·      not seen in dark-skinned races who use hands

Smoking

·      increased incidence

·      may cause microvascular occlusion

Regional injury

·      increased incidence found after Colles fracture

·      specific regional injury may accelerate appearance

pathogenesis
Myofibroblast (Gabiani)

·      active contractile cell

·      causes fascial contraction

·      origin is perivascular fibroblast

·      conversion to myofibroblast may be triggered by localised hypoxia in response to microvascular occulsion

Collagen

·      presence of type III collagen in diseased fascia

·      all type I in normal fascia

·      type III found in scar tissue

·      role controversial

·      may be response to injury

Free radicals (Murrell)

·      initiating event is microvascular ischaemia

·      leads to conversion of

·      ATP to hypoxanthine

·      endothelial xanthine hydrogenase to xanthine oxidase

·      xanthine oxidase converts hypoxanthine to uric acid

·      releases free radicals

·      free radicals stimulate fibroblast proilferation

pathology
Palm

Palmar aponeurosis

·      almost always involved

Anatomy

·      starts proximally

·      continuous with palmaris longus if present

·      fans out to pretendinous bands to each digit

·      bands

·      attach to palmar skin just beyond MCP jt (accounts for DPC)

·      bifurcate at level of MCP jt and enter finger deep to NV bundle

Nodule

·      nodule is pathognomonic of Dupuytren’s

·      most frequently appears in palm near DPC

·      in pretendinous band of ring and little fingers

Cord

·      pretendinous bands become cords

·      bands are normal tissue

·      cords are diseased tissue

·      nodule in band induces formation of cord proximal and distal

·      cause MCP contracture

·      do not alter anatomy of NV bundles

·      can be excised safely proximal to DPC

Superficial transverse metacarpal ligament

·      syn. superficial transverse palmar ligament

·      lies deep to palmar aponeurosis

·      passes across MCP joints

·      not involved in disease process (?except in thumb)

Natatory ligament

Anatomy

·      passes across palm at level of web spaces

·      gives structure to web

·      attaches to each fibrous flexor sheath

·      contributes to lateral digital fascia

·      on ulnar side

·      blends with hypothenar fascia at base of little finger

·      on radial side

·      continues to thumb web

·      terminates in skin at PIP jt of thumb

Pathology

·      commonly involved

·      causes contracture of

·      web space

·      finger joint

·      narrows web from U to V

·      results in inability to separate fingers

·      contributes to PIP jt contracture

·      by contribution to lateral digital fascia

·      contributes to thumb web contracture

Hypothenar fascia

·      condensation of fascia along ulnar border of palm

·      may become involved

Thumb web

Anatomy

·      fascia comes from

·      pretendinous bands

·      superficial transverse metacarpal ligament

·      natatory ligament

Pathology

·      pretendinous bands of thumb and index finger rarely involved

·      thumb web contracture may occur from contracture of

·      superficial transverse metacarpal ligament (?)

·      natatory ligament

·      starts with nodule at proximal crease of thumb

·      may develop into cord in web space

Palmar digital area

Anatomy

·      pretendinous bands

·      bifurcate at MCP jt

·      pass deep to NV bundle

·      lie on each side of finger

·      called spiral bands

·      natatory ligament

·      sends fibres down each side of finger

·      pass superficial to NV bundle

Pathology

·      spiral bands become spiral cords

Fingers

Anatomy

·      volar fascia consists of

·      superficial fascia like tube around finger

·      condensations deep in finger

·      superficial fascia

·      forms lateral condensation called lateral digital sheet

·      is fibrofatty on volar surface

·      deep fascia consists of

·      Grayson’s ligament

·      Cleland’s ligament

·      both pass from tendon sheath to lateral digital sheath

·      Grayson’s ligament is

·      volar

·      superficial to NV bundle

·      Cleland’s ligament is

·      midlateral

·      deep to NV bundle

Cords

·      four cords contract PIP jt

1.   central cord

·      direct extension of pretendinous cord

·      arises from superficial fascia of volar surface of finger

·      passes distally between NV bundles

·      adherent to skin

·      attaches to flexor sheath and bone of P2

·      fibres of Grayson’s ligament become part of cord at attachment

·      common cause of PIP jt contracture

·      found in little and ring fingers

2.   spiral cord

·      continuation of pretendinous cord

·      passes deep to NV bundle distal to MCP jt

·      joins lateral digital sheet

·      becomes continuous with Grayson’s ligament

·      fibres then pass superficial to NV bundle

·      attach to flexor sheath at P2

·      fibres therefore spiral around NV bundle

3.   lateral cord

·      disease of lateral digital sheet

·      may extend distally

·      may cause DIP jt contracture

4.   retrovascular cord

·      diseased fascia deep (dorsal) to NV bundle

·      separate to Cleland’s ligament (not involved)

Displacement of NV bundle

·      normal parts of fascia that produce spiral cord are

·      pretendinous band

·      spiral band

·      lateral digital sheath

·      Grayson’s ligament

·      as this spiral structure contracts it straightens

·      causes NV bundle to spiral around it

·      as PIP jt contracts, spiral cord lies more volar

·      NV bundle becomes subcutaneous

·      danger zone is between

·      distal palmar crease

·      proximal crease of finger

·      should divide pretendinous cord to allow finger to be extended

Flexion deformities

·      MCP jt flexion always correctable

·      collateral ligaments tightest in flexion

·      flexion deformity does not lead to collateral shortening

·      resection of central cord restores extension

·      PIP jt flexion not always correctable

·      collateral ligaments tightest in extension

·      flexion deformity leads to collateral shortening

·      contracture of volar plate and flexor sheath may contribute to fixed deformity

clinical features
Symptoms

·      may present with thickening in palm

·      usually complain of problems related to flexion defomities of fingers

Hand

Nodule

·      starts with nodular thickening in ulnar palm

·      along line of little or ring finger

·      just proximal to distal palmar crease

·      skin becomes dimpled and adherent (pitting)

Band

·      extends distally to involve little or ring finger

·      if extend to fingers cause

·      flexion deformities of MCP jts and PIP jts

·      lack of finger abduction

·      PIP flexion may lead to secondary DIP hyperextension

Severity

·      table-top test of Hueston

·      positive when hand cannot be placed fully flat on table

·      indicates deformity severe enough to warrant surgical intervention

·      PIP flexion deformity

·      assess PIP extension with MCP jt extended maximally

·      then maximally flex MCP jt to eliminate ‘tenodesis’ effect of central cord

·      allows assessment of intrinsic PIP jt contracture

Dorsum

·      may be thickening of dorsal knuckle pads (Garrod’s pads)

Other

·      Lederhose’s disease

·      plantar fibromatosis

·      Peyronie’s disease

·      fibrosis of corpus cavernosum

treatment
Nonoperative

Indications

·      absence of digital flexion deformity

·      negative table-top test

Methods

Observation

·      spontaneous regression may occasionally occur

Drugs

·      allopurinol, trypsin and steroid have been tried

·      no consistent results

Traction

·      continuous passive skeletal traction

·      feeling of release after 4-5 days

·      near full extension achieved at 2 weeks

·      used preoperatively to make surgery easier

Operative

Indications

·      digital flexion deformity

·      positive table-top test

·      contracture in thumb web space

·      premature surgery may hasten progress of disease

·      equivalent to injury of hand

Fasciotomy

·      limited palmar subcutaneous fasciotomy

·      should never be performed distal to DPC

·      temporising solution

·      indicated for

·      elderly patients

·      patients unsuitable for anaesthesia

·      facilitation of access to more definitive procedures

Total fasciectomy

·      total palmar fasciectomy

·      done in hope of eliminating possibility of recurrence

·      should not be performed because of significant postoperative morbidity

Partial fasciectomy

·      confined to rays involved

·      current procedure of choice

Dermatofasciectomy

·      excision of fascia and overlying dermis

·      defect covered by full-thickness graft

·      from medial arm or antecubital fossa

·      role controversial

·      may eliminate recurrence

·      may be indicated when local recurrence expected

·      usually reserved for digits

Amputation

·      indicated in

·      elderly patient

·      severe PIP deformity unlikely to be correctable

Technique of partial fasciectomy

Principles

·      dissection of digital nerves

Equipment

·      loupes or operating microscope

·      tourniquet and Esmarch bandage

Incision

Longitudinal

·      linear midpalmar and middigital incision

·      most commonly used

·      crossing flexion creases at right angles

·      DPC, base of finger and crease of PIP jt

·      can be multiple

·      closure with three z-plasties

·      so that horizontal limbs fall over flexion creases

Transverse

·      of McCash

·      for severe involvement

·      proximal and parallel to DPC

·      separate middigital incisions for fingers

·      leaves gap of 3-4 cm after correction

·      heals spontaneously in 4-6 weeks

·      advantages are

·      allows early mobilisation

·      avoids haematoma, pain and oedema

Fasciectomy

·      skin flaps elevated from diseased fascia

·      as much subcutaneous tissue left on flaps

·      pretendinous cord divided just distal to superficial palmar arch

·      NV bundle safe as deep to palmar fascia

·      NV bundle exposed and dissected out

·      followed carefully distally to distal phalanx

·      may be superficial to fascia distal to DPC

·      diseased tissue removed

PIP jt contracture

·      can usually correct 50% of flexion deformity by excision of diseased tissue

·      should perform further procedures if flexion > 90o

·      may address residual contracture by following procedures

·      closed manipulation

·      open flexor sheath distal to A2 pulley

·      release volar plate

·      release collateral ligaments

·      better to accept mild residual deformity than cause postoperative scarring

Closure

·      release tourniquet

·      obtain haemostasis

·      insert small drain

·      close skin

·      z-plasties as above

·      bulky dressing with fingers in extension

·      may use plaster slab

Postoperative

·      hand elevated

·      drain removed at 24-48 hrs without disturbing dressing

·      if severe pain, inspect wound for haematoma

·      sutures removed in 10 days

·      active flexion started

·      extension splint worn for 3 weeks

·      for severe deformity, night splint for further 12 weeks

Complications

·      reflex sympathetic dystrophy

·      more common in women

·      presposed to by postoperative pain, haematoma and oedema

·      division of digital nerves and arteries

·      repair at time of surgery

·      stiff PIP jt

·      with subsequent loss of grip strength

·      with overzealous PIP correction

Prognosis
Poor prognostic factors

General

·      young age

·      female

·      positive family history

·      epileptic / diabetic

·      Dupuytren’s diathesis

Local

·      rapidity of appearance and progress of nodules

Further disease following surgery

·      surgery not curative

·      further disease may be

·      recurrence (new disease in area that has been operated on previously)

·      extension (disease in area that has not been operated on)

·      incidence depends on

·      age of patient

·      severity of preoperative disease

·      duration of followup

·      incidence is 50%

·      requirement for repeat surgery is 15%

·      most common in 1st few postoperative months

·      may occur after many years