Comprehensive surgical management of the paralyzed diaphragm
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Comprehensive surgical management of the paralyzed diaphragm

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Comprehensive surgical management of the paralyzed diaphragm Comprehensive surgical management of the paralyzed diaphragm Presentation Transcript

  • Comprehensive Surgical Management of the Paralyzed Diaphragm Matthew R. Kaufman, MD, FACS Plastic & Reconstructive Surgery The Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Surgical Management of the Paralyzed Diaphragm
    • No financial disclosures
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Anatomy
    • Originates from C3-C5
      • Primarily C4
    • Motor innervation to diaphragm
    • Sensory fibers
      • Pleura
      • Pericardium
      • Abdominal components
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Anatomy Accessory Phrenic Nerve
    • Occurs in 15-25% of people
    • A branch of C5 which would otherwise pass to subclavius
    • Begins lateral to the phrenic nerve in the neck
    • Obliquely traverses scalenus anterior
    • Joins the phrenic nerve at the root of the neck
    • Also supplies diaphragm with efferent fibers
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Anatomy Accessory Phrenic Nerve Accessory Phrenic Nerve Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Respiratory Physiology
    • Volitional (day time) breathing controlled by respiratory control center
    • Night breathing regulated by CO2 levels measured in brain
    • Signals go down nerves in the neck, chest to diaphragm
    • Phrenic nerve branches out into the diaphragm muscles
    • The diaphragm muscles contract drawing in air
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Etiology
    • Peripheral
    • (Phrenic Nerve Injury)
      • Iatrogenic
        • Surgery
        • Anesthetic Blocks
        • Chiropractic
      • Trauma
        • Blunt
        • Penetrating
      • Neuropathy / Neuritis
      • Viral
    • Central
      • Spinal Cord Injury
      • Cord Compression
      • Central Hypoventilation Syndrome
        • Ondine’s Curse
      • Tumors
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Phrenic Nerve Injury
    • Demographics
      • Usually unilateral
      • Rarely ventilator or oxygen dependent
      • Severe limitations in physical functioning
      • Association with sleep-disordered breathing
      • PFTs often normal or near-normal
      • Abnormal Sniff Test
      • Told by MD: “…just learn to live with it…”
      • Some are offered diaphragm plication
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Injury Etiology
    • Iatrogenic
      • Neck
        • Interscalene Block
        • Cervical Epidural
        • Neck Dissection
        • Carotid Bypass
        • Chiropractic Manipulation
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Injury Etiology
    • Iatrogenic
      • Mediastinal / Chest
        • Carotid-Subclavian Bypass
        • Aortic Surgery
        • Thymectomy
        • Coronary Bypass
        • Thoracic Outlet Release
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Injury Mediastinal Surgery
    • Thymectomy
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Paralysis
    • Spinal Cord Injury
      • Usually C3 or higher (rarely C4-C5)
      • Ventilator Dependent
      • Tracheostomy Dependent
    • Central Hypoventilation Syndrome
      • Ondine’s curse
      • Oxygen dependent
      • Ventilator/ Bi-Pap at night
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Paralysis
    • Spinal Cord Injury (SCI)
      • 11,000 new cases/year
      • >50% cervical level
      • Interruption of descending bulbospinal respiratory pathways
        • Respiratory paresis or paralysis
      • Primary cause of death after SCI, regardless of level – Respiratory *
    * National Spinal Cord Injury Statistical Center, 2006 Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Spinal Cord Injury
    • Effect of SCI on Respiration
      • Paralysis of respiratory muscles
        • Diaphragm
          • Major inspiratory muscle
          • Innervated by phrenic motoneurons C3-5
        • Accessory respiratory muscles
          • Intercostals
          • Thoracic/Abdominal muscles
      • Reduction in tidal volume
      • Blunted response to hypercapnia
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Spinal Cord Injury
    • SCI is among the most expensive condition among all causes for hospitalization.
      • Yet a 20 year old tetraplegic on MV has their life expectancy drop by ~18 yrs vs. non-depenadent 1
    National Spinal Cord Injury Statistical Center, Birmingham, Alabama January 2008 Treatment of Pulmonary Disease Following Cervical Spinal Cord Injury, Evidence Report/Technology Assessment, Number 27 Center for Treatment of Paralysis and Reconstructive Nerve Surgery Severity of Injury Avg Yearly Expenses 1 st Yr Avg. Yearly Expenses Subsequent Estimated Lifetime Costs 25 yrs old Estimated Lifetime Cost 50 yrs old High (C1-C4) Tetraplegia $775,567 $138,923 $3,059,184 $1,800,958 Low (C5-C8) Tetraplegia $500,829 $56,905 $1,729,754 $1,095,411
  • Ventilator Dependency Impact On the Diaphragm
    • Compared 14 brain dead donors on PPV to 8 controls
    • 18 hours of PPV causes marked atrophy
    • 57% decrease Type 1 slow twitch
    • Active muscles atrophy faster
    • Inactivity leads to oxidative stress
    • Increased proteolysis
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Paralysis
    • A CNS problem with no clinical CNS solution
    • Peripheral nerve solution(s)
      • Electrical stimulation
      • Nerve transfer
      • Electrical stimulation + Nerve transfer
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Spinal Cord Injury
    • Above level of injury
      • Functioning nerves
    • At level of injury
      • Inability to propagate a nerve stimulus
      • Cannot respond to externally applied electrical stimulus
    • Below level of injury
      • Inability to propagate a nerve stimulus
      • May respond to externally applied electrical stimulus
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Congenital Central Hypoventilation Syndrome (Ondine’s Curse)
    • Demographics
      • Rare disorder (1 in 200,000/U.S.)
      • No known cure
    • Pathophysiology
      • Problem integrating chemoreceptor input to central ventilatory controllers
      • Genetic mutation identified
      • Association with Hirschprung’s disease
    • Characteristics
      • Breathe reasonably well while awake
      • Apneic during sleep - require ventilatory support
      • A third require ventilatory support 24h/day
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Pacing
    • Purpose
      • Electrically pulse phrenic nerves (or nerve/muscle interface), causing diaphragmatic excursion
    • Goal
      • Reduce or eliminate ventilator dependency
    • Experience
      • Avery
        • 25 years
        • 82% rate of permanent diaphragmatically-paced breathing
      • Synapse
        • Recently FDA approved
        • SCI trial (100% paced with intact nerves)
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Pacing
    • Criteria
      • Chronic respiratory failure
      • Central neurological disorder or High Spinal injury
      • Preserved phrenic nerve integrity
      • Acceptable pulmonary function
      • Normal level of consciousness
      • Appropriate care and support
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Pacing
    • Procedure (Avery)
      • Small incisions
      • Intraoperative neurophysiological testing
      • Implantation of electrodes and receiver
    • Diaphragm conditioning
    • Long-term pacing
    Case Western University Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker Avery Biolabs Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • NeuRx Diaphragm Pacing System™
    • External Pulse Generator “Paces” Diaphragm
    • Home based conditioning replaces Mechanical Ventilation
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Pacemaker
    • NeuRx Diaphragm Pacing Device (Synapse)
      • Laparoscopic placement
      • Electrodes (4) applied to nerve/muscle junction
      • Avoid nerve manipulation
      • Application for neurodegnerative conditions (ALS)
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Pacing
    • Benefits
      • Lower rate of pulmonary complications
      • Improved venous return
      • Normal breathing and speech
      • Ease of eating and drinking
      • Increased patient mobility
      • Cost-effective
    • Risks
      • Phrenic nerve injury
      • Wire displacement
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Diaphragm Pacing
    • Limitations
      • Lack of voluntary control of breathing
      • Only inspiration is triggered, thus cough may be weak
      • Inability to time spontaneous inspiratory efforts
      • No benefit for unilateral injuries*
      • C3-5 injuries may not be candidates due to Wallerian degeneration*
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Neurotization
    • Nerve transfers (Neurotization)
      • Definition
        • Transferring intact, functioning nerves to phrenic nerves in order to restore function to the diaphragm
      • Advantages
        • Tremendous experience with this technique for brachial plexus, extremities, facial nerve
        • A reconstructive option for patients not candidates for pacing
        • Or, perhaps to enhance results of pacing
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Neurotization
    • Krieger & Krieger (2000)
      • 6 patients
      • C3-5 injuries
      • Intercostal to phrenic + pacemaker
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Neurotization Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Neurotization Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Spinal Accessory Neurotization
    • Potential Advantages
      • Tremendous experience in brachial plexus reconstruction
      • Higher axonal density in the donor nerve
        • Intercostal: 1093
        • Spinal Accessory : 2145
        • [Phrenic: 800]
      • Functionally linked to phrenic
      • Performed through neck incisions
      • Lower morbidity (no thoracotomy)
    • Disadvantage
        • Longer distance for axonal regrowth
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Spinal Accessory Nerve Brainstem Spinal Cord Dual Origins Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-innervation Spinal Accessory Neurotization
    • Goal
      • To provide ventilator-dependent patients the ability for voluntary respiratory control +/- a diaphragm pacemaker
    • Techniques
      • Transfer a branch of functioning Cr XI to nonfunctioning phrenic bilaterally
    • Status
      • Ongoing protocol
      • IRB Approval
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Spinal Accessory Neurotization
    • Phrenic Nerve
    • Spinal Accessory Nerve
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Paralysis Spinal Accessory Neurotization
    • Anticipated outcomes
      • Spontaneous diaphragm function
      • Improve pacemaker results
      • Low morbidity
      • Another treatment for diaphragm paralysis
      • REDUCED COST
        • Medicare/Medicaid reimbursed?
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Ventilator Dependency Summary of Potential Treatment Options
    • Diaphragm Pacemaker
      • C-spine (C3 or higher) or Central abnormality
    • Neurotization
      • C3 or higher, OR Central
      • Functioning donors
      • Poor pacemaker candidate
    • Neurotization +/- Pacemaker
      • C3-5
      • Extensive direct injury to nerve
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Unilateral Diaphragm Paralysis Phrenic Nerve Injury Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Grafting Nerve Interposition Graft or Nerve “Bypass” Neck Chest Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Grafting Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Phrenic Nerve Grafting Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation
    • “ Re-Innervation of the Paralyzed Diaphragm: Application of Nerve Surgery Techniques following Unilateral Phrenic Nerve Injury”. Kaufman et al.*
      • N=12
      • Unilateral, symptomatic diaphragm paralysis
      • Minimum 6 months
      • No spontaneous improvement
    *Submitted for publication to CHEST journal 10/10 Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation
    • Evaluations
      • Sniff Test
      • Phrenic Nerve Conduction Study
      • Diaphragm EMG
      • PFTs
      • Standardized Quality of Life Survey
        • Assess Physical Functioning
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation
    • Results
      • 8 males, 4 females
      • Mean age 54 (range 40-68)
      • Etiology
        • Anesthetic Blocks (2)
        • Surgery (6)
        • Chiropractic Manipulation (2)
        • Traumatic Event (2)
      • Right (3), Left (9)
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation
    • Results
      • Treatment offered
        • Neurolysis (12)
        • Interposition nerve graft (7)
        • Neurotization (2)
      • Operative time
        • Mean= 165 minutes (range 50-250)
      • Hospital stay
        • Mean= 2 days (range 1 to 4)
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation Quality of Life Improvements Normal function = 100
  • Diaphragm Re-Innervation Improvements in PFTs
  • Diaphragm Re-Innervation Phrenic Nerve Grafting Return of Diaphragmatic Function 10 months following grafting Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation
    • Sniff test
      • 8/9 improved
    • Incomplete assessments in 4/12
      • (2) too early to be tested
      • (1) expired from unrelated cardiac event 8 months later
      • (1) no improvement at 8 months*
    • Complications
      • (1) infection at sural nerve harvest site
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Diaphragm Re-Innervation Conclusions
    • Documentation of diaphragm re-innervation following nerve grafting
      • 67% objective improvements in diaphragm function
    • A problem with almost no other treatment options
      • Diaphragm plication
        • Restoring physiologic function always superior to “piggybacking” dead muscle
    • Additional investigation
      • Standard of care for diaphragm paralysis?
    Center for Treatment of Paralysis and Reconstructive Nerve Surgery
  • Thank You! Center for Treatment of Paralysis and Reconstructive Nerve Surgery