Hand Arm Vibration Syndrome with Concomitant Hypothenar Hammer Syndrome

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Hand Arm Vibration Syndrome with Concomitant
Hypothenar Hammer Syndrome

• Dr. Aaron Thompson
• Rheumatology Rounds
• Tuesday, April 26, 2005

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Lecture Outline

• Anatomy
• Raynaulds Phenomenon
• Seconday Raynaulds - Differential Diagnosis
• Hand-Arm Vibration Syndrome - Definition
• Hand-Arm Vibration Syndrome - Pathophysiology
• Hypothenar Hammer Syndrome - Definition
• Hypothenar Hammer Syndrome - Pathophysiology
• Screening using the Allens test -
  Sensitivity/Specificity
• Case Series
• Conclusions

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Illustrated Encyclopedia of Human Anatomic
Variation Opus II Cardiovascular System
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Raynauds PhenomenonDefinition

• Paroxysmal pallor and coldness of the
  extremities, usually precipitated by cold, and
  followed by cyanosis and arterial hyperaemia.

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Mechanisms contributing to Raynaud's phenomenon
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Wigley F. Raynauds Phenomenon. N Engl J Med 2002
vol. 347 No. 13. Pp 1001-1008
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Secondary Raynauds

• Connective Tissue Disease
• Scleroderma
• Mixed connective tissue disease
• Systemic lupus erythematosus
• Sjögren's syndrome
• Dermatomyositis
• Polymyositis

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Secondary Raynauds

• Drugs and Toxins
• ?-Blockers
• Ergotamines
• Clonidine
• Chemotherapeutic agents
• Polyvinyl chloride
• Cyclosporine
• Interferon
• Estrogen
• Narcotics
• Cocaine
• Nicotine

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Secondary Raynauds

• Large-artery disease
• Vasculitis
• Atherosclerosis
• Thromboangiitis obliterans
• Embolic disease
• Paraproteinemia
• Hyperviscosity state (e.g., Polycythemia vera)
• Cryoglobulinemias

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Secondary Raynauds

• Neuropathy
• Carpal tunnel syndrome
• Thoracic outlet syndrome
• Environmental agents and injury
• Cold Stress (Frostbite)
• Repetitive occupational stress (handarm
  vibration syndrome)
• Hypothenar hammer syndrome

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Secondary Raynauds

• Wrong Diagnosis
• Acrocyanosis
• Central Cyanosis
• Chilblains/Pernio

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Hand-Arm Vibration Syndrome

• A type of secondary Raynauds phenomenon
  resulting from the transfer of vibration from a
  tool to a workers hands and arms.
• Documented vascular effects include digital
  sclerosis, digital organic microangiopathy,
  arterial vasospastic phenomenon, arterial
  thrombosis, vibration neuropathies, carpal tunnel
  syndrome, osteoarticular lesions and hearing
  deficits.

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Hand-Arm Vibration Syndrome History

• 1862 - Primary Raynaud's phenomenon (Raynaud's
  Disease) was identified by Dr Maurice Raynaud.
• 1911 - Secondary Raynaud's phenomenon first
  linked to use of pneumatic tools.
• The use of power tools and machines became more
  widespread with the development of electrical
  power and the internal combustion engine.
• 1975 - Taylor-Pelmear scale published allowing
  consistant assessment.

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Alice Hamilton, the year she graduated from
medical school, 1893The Schlesinger Library,
Radcliffe Institute, Harvard University
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Alice Hamilton at her home, 1957 The Schlesinger
Library, Radcliffe Institute, Harvard University,
Scope Weekly
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History HAVS

• 1987 - the Stockholm workshop revised the
  Taylor-Pelmear scale and also
• divided the condition into two parts - vascular
  and neurological
• looked at each hand separately
• discounted seasonal variations in symptoms
• 1992 - The Supply of Machinery (Safety)
  Regulations were introduced which required that
  risks resulting from vibration emissions should
  be reduced to the lowest level
• 1997 - England - Miners High Court compensation
  award 127,000 to 7 miners for HAVS

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Stage Assessment for Hand-arm Vibration Syndrome
(Tayler-Pelmear classification System)
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Stockholm (Revised) Hand-arm Vibration Syndrome
Classification
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HAVS Pathophysiology

• 1. Neural Dysfunction
• 2. Local Acral vasodysregulation
• 3. Shear stresses
• 4. Blood Viscosity and cell activation

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HAVS Pathophysiology

• Neural Dysfunction
• Autonomic dysfunction
• Receptor dysfunction
• Nerve ending dysfunction

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HAVS Pathophysiology

• Local Acral vasodysregulation
• Endothelial damage
• Endothelial dysregulation

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HAVS Pathophysiology

• Shear stresses
• cause of endothelial damage

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HAVS Pathophysiology

• Blood Viscosity and cell activation
• Erythrocyte activation
• Platelet activation
• Leukocyte activation

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Hand-Arm Vibration Syndrome PathophysiologyStoy
neva et al. Current pathophysiological views on
vibration-induced Raynauds phenomenon.
Cardiovascular Research. 2003. 57, 615-624
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Hand-Arm Vibration Syndrome Local
pathophysiological MechanismsStoyneva et al.
Current pathophysiological views on
vibration-induced Raynauds phenomenon.
Cardiovascular Research. 2003. 57, 615-624
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HAVS Pathophysiology

• Microvascular changes including capillary
  tortuosity, dropout, elongation and
  disarrangement (suggesting a small vessel
  vasculitis)

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Nailfold capillaries from (a) a healthy control
subject and (b) a patient with SSc showing
abnormal, widened capillary loops.
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Hand-Arm Vibration SyndromeDiagnosis

• Physical exam
• Grip strength, Phalens test, Tinels test and
  Allens test.
• Objective tests
• cold provocation testing (thermometry), Doppler
  examination of the upper extremities,
  electromyography, digital plethysmography and
  current perception threshold studies (CPT).
• Blood work
• CBC, serum electrolytes, creatinine, urea,
  urinalysis, glucose, ESR, TSH, uric acid,
  rheumatoid factor, antinuclear antibody,
  cryoglobulins, serum protein electrophoresis.

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Hypothenar Hammer Syndrome Pathophysiology

• Results from repetitive blunt trauma to the palm
  of the hand.
• Repeated trauma over the hypothenar eminence may
  result from gripping a piece of equipment that is
  intrinsically associated with vibration.
• The position of the ulnar artery in the
  hypothenar eminence crossing the hamate bone
  makes it vulnerable to repetitive trauma
• May result in aneurysm formation or ulnar artery
  thrombosis
• The aneurysm occasionally serves as a source for
  digital emboli.

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Hypothenar Hammer SyndromeEpidemiology

• Normal subjects 21 cases of HHS in 1300
  prospectively enrolled subjects, an incidence of
  1.6 (Ferris).
• Raynauds 1.7 in patients with Raynauds
  phenomenon (Mehlhoff).
• Referred to Cath lab positive Allens test (cut
  off of 9 seconds) in 6.4 of 1010 consecutive
  patients referred (Barbeau et al).
• Vibration exposed workers Incidence of HHS 7.3
  in a sample of 330 vibration-exposed workers. 293
  had had Raynaulds phenomenon (Kaji et al.).
• Mechanics 14 in a population of automotive
  mechanics who used their hand as a hammer more
  than once a day (Little).

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Cook RA. Hypothenar Hammer syndrome a discrete
syndrome to be distinguished from hand-arm
vibration syndrome. Occupational Medicine
200353320-324
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Proximity of the ulnar artery to the hamate bone
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Hypothenar Hammer Syndrome Presentation

• Hypothenar pain and pallor
• Hypothenar paresthesias
• Affected digits are cool
• Localized tenderness at hypothenar prominence
• Abnormal Allens Test

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Hypothenar Hammer SyndromeDiagnosis

• Allens test as an initial screening tool
• cut-off of 5 seconds for maximal diagnostic
  accuracy.
• If positive Allens test
• Doppler ultrasound studies
• Combined pulse oximetry and plethysmography
• Note in the future, colour doppler studies may
  be used as a primary means to further investigate
  patients with a positive Allens test.
• If colour Doppler studies are positive
• arteriography

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The Allens Test

• The diagnosis of HHS has traditionally used the
  Allens test as a screening tool.
• Procedure
• patient makes a tightly clenched fist so as to
  exsanguinate the vessels of the hand.
• The examiner compresses the ulnar and radial
  arteries
• patient opens the hand (without hyperextending
  the wrist or fingers).
• The examiner releases the ulnar artery and
  measures the capillary return time.

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The Allens Test

• The usefulness of the Allens test has been
  called into question
• Inter examiner variability in determining when
  complete filling of the vessels of the hand has
  taken place.
• No clear consensus as to what refilling time
  constitutes an abnormal test.

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The Allens Test

• Barbeau et al
• Upper time limit varies between 5 and 15 seconds.
  
• lt 9 seconds - 6.3 more false positives than
  combined PL and OX
• Stead
• phothoplethysmography
• 7-14 seconds as borderline.
• gt 15 seconds clearly positive.
• Jarvis et al
• Doppler U/S
• 5 seconds has maximal diagnostic accuracy (79.6)
• 3 second cut-off required to abolish false
  negatives
• (3 second cut-off only provides a 52
  diagnostic accuracy)

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Alternative Sceening Tools

• Digital Oximetry
• Digital Plethesmography
• Doppler U/S
• Gold Standard for Diagnosis
• Digital Angiography
• High-res contrast enhanced MR angiography

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Treatment of Hypothenar Hammer Syndrome

• Smoking cessation
• Low-lipid diet
• IV heparin
• IV prostaglandin E1

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Treatment of Hypothenar Hammer Syndrome

• Repeated venesection to reduce polycythaemia
• Recanalization (usually dx too late for to this
  to be a viable option
• Resection followed by interposition vein graft
  (84 patency 2 years Ferris 2000).
• Ligation of the ulnar artery to stop blood flow
  to the aneurysm and thereby prevent further
  embolization
• Amputative debridement may be required.
• Cervical sympathectomy stellate ganglion blockade
  and thrombolysis have been suggested.

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Case Series

• Case 1
• 45 M assembly worker at an automotive plant.
• Exposed to vibration x 19 yrs (pneumatic and
  electric guns)
• Cold provoked recurrent numbness, tingling and
  blanching of the 3rd and 4th fingers from the
  tips of the fingers extending to the proximal
  interphalengeal joints of the left hand.
• Smoker w 10-pack year history.
• Allens test L ulnar artery/Cold provocation
  digital thermometry - delayed rewarming all
  digits except the thumb/ Digital plethysmography
  3rd and 4th digits L hand/ EMG/NCS revealed a
  moderate hypoesthetic condition bilaterally of
  the median nerve and a mild hypoesthetic
  condition of the right ulnar nerve.
• Arteriogram - local stenosis radial side digital
  branch of the ring finger, short segment
  occlusions of both digital branches and absent
  perfusion to the tip of the middle finger, and
  occlusion of the ulnar aspect palmer digital
  branch of the index finger.
• Treatment required partial amputation of left
  middle finger following an episode of gangrene.

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Case Series

• Case 2
• 54 M pipe fitter vibration exposure x 33 yrs
  (hammer drills, pneumatic grinders and chippers)
• Presented with 21-year history cold provoked pain
  and blanching of the fingers.
• Ex-smoker 13-pack yrs
• PMHx SLE, RA
• Physical exam - revealed a trophic ulcer on the
  right index finger and a positive Allens test
  over the ulnar arteries bilaterally.
• Blood work ? ESR and ANA.
• Cold provocation digital thermometry - delayed
  rewarming all digits bilat.
• Electromylographic and nerve conduction studies -
  reduced median nerve sensation with normal median
  nerve latency bilaterally
• Digital plethysmography revealed no signal
  waveform present in the 2nd digit of the left
  hand, and 3rd digit of the right. All other
  digits had a reduced signal.
• Angiogram was positive for thrombosis of two
  vessels in the right hand.

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Case Series

• Case 3
• 32 M machinist in the automotive and construction
  industries
• Vibration exposure x 13yrs (impact guns and
  jackhammers).
• Presented with cold provoked blanching, numbness
  and tingling of all digits with sparing of the
  thumbs for approximately 1½ years.
• PMHx smoker 7 ½-pack-year
• Physical exam - hypothenar atrophy bilaterally
  with no trophic changes. Tinels test was
  abnormal bilaterally. Allens test was positive
  for the ulnar artery bilaterally.
• Cold provocation digital thermometry and digital
  plethysmography .
• CPT revealed hypoesthetic ulnar and median nerves
  bilaterally
• Blood work normal.
• Angiogram revealed left distal ulnar artery
  occlusion several centimeters proximal to the
  wrist joint along with diffuse narrowing and
  occlusions in the index, long and ring fingers.

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Case Series

• Case 4
• 38 M water well driller vibration exposure x
  13yrs grinding tools
• The patient also endorsed a history of using the
  hypothenar side of his hand as a hammer.
• He presented with a 2-month history of numbness,
  tingling, pain, and blanching of the 3rd, 4th and
  5th digits of the left hand.
• PMHs smoker with 20-pack years
• Physical exam - palpable mass in the right
  hypothenar eminence and an early ulcer crater on
  the volar tip of the right 5th digit. Allens
  test was positive for the right ulnar artery.
• Cold provocation digital thermometry - delayed
  rewarming during cold provocation digital
  thermometry.
• Angiogram revealed an abnormal ulnar artery with
  an aneurysm in the region of the hook of the
  hamate. There was proximal ulnar artery
  thrombosis and occlusion of the medial and
  lateral digital arteries of the 5th digit, as
  well as occlusion of the medial digital artery of
  3rd and 4th digits.
• Txed surgically with removal of the damaged
  artery and venous grafting from the right forearm
  with excellent result.

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Case Series
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Case Series

• 2-year period
• 139 consecutive patients referred for
  investigation of HAVS
• 134 men, 5 women.
• All patients had a history of vibration exposure,
  and presented with symptoms of cold intolerance
  consistent with HAVS.
• Screening using the Allens test was done by one
  of two physicians in the clinic.
• A positive Allens test was found in 16 workers
  (11.5)

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Case Series
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Conclusions

• HAVS and HHS present in a similar fashion
• Our study finds a prevalence of 11.5 of abnormal
  Allens tests in patients with a coincident
  diagnosis of HAVS.
• Must consider HHS as an alternative or
  contributory cause of Raynauds phenomenon in
  vibration exposed workers.
• Need to include screening for HHS in the HAVS
  work-up
• Suggest Allens test as an initial screening tool
  with a cut-off of 5 seconds for maximal
  diagnostic accuracy

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Conclusions

• A positive Allens test using these criteria
  warrants further investigation.
• Doppler ultrasound studies
• Combined pulse oximetry and plethysmography
• Colour doppler studies
• If above positive, arteriography should be
  performed.
• Importance - different treatment options

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