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DiseaseDisease characteristicsAnesthetic considerations
  • Autosomal dominant
  • Most common cause of dwarfism
  • Odontoid hypoplasia, atlantoaxial instability, bulging discs, and severe cervical kyphosis
  • Central sleep apnea and OSA may be a feature
  • Pulmonary HTN
  • Cor pulmonale
  • Hydrocephalus
  • Proportionately smaller airways
  • Potential airway difficulties, cervical spine instability, and the potential for spinal cord trauma with neck extension; obtain cervical spine x-rays. Consider FOB intubation
  • Consider PFTs
  • ETT size should be weight based
  • Relative contraindication to neuraxial blocks
Alport syndrome (hereditary nephritis)
  • Ocular abnormalities and hearing loss
  • Disease culminates in systemic HTN and renal failure
  • Intraglomerular pressure may be lowered by ACEI
  • Same anesthetic considerations as in renal failure and HTN
Ankylosing spondylitis
  • Chronic, progressive, inflammatory disease spine articulations and adjacent soft tissues
  • Occurs more commonly in men
  • Spine exam reveals skeletal muscle spasm, loss of lordosis, and decreased mobility
  • Weight loss, fatigue, low-grade fever, conjunctivitis, and uveitis are systemic signs
  • Pulmonary involvement may reveal apical cavitary lesions with fibrosis and pleural thickening with ↓ pulmonary compliance and vital capacity secondary to arthritis; restrictive syndrome (30–45% of patients after 15 years)
  • Associated psoriasis
  • TMJ involvement with limitation of mouth opening
  • Cardiovascular: AI (up to 10% after 30 years), dysrhythmias, conduction abnormalities
  • Renal insufficiency: rare
  • May need awake FOB intubation if cervical spine is involved (major kyphosis)
  • Restrictive lung disease may result in higher airway pressures
  • Spinal deformity and instability: careful positioning and padding
  • Neuraxial anesthesia is acceptable but may be difficult
  • Neurologic monitoring should be considered for corrective spine surgery
  • One type, autosomal recessive, is a myopathy
  • Other type: polymalformative syndrome
  • Multiple extremity contractures due to periarticular fibrosis with muscular atrophy
  • Possible TMJ and spine involvement (cervical spine ⇒ intubation issues; thoracic spine ⇒ restrictive syndrome
  • Plan for difficult intubation
  • Careful positioning
  • Sensitive to sedatives/anesthetics
  • Myopathy: avoid succinylcholine; monitor NMB
  • Possible MH
  • Monitor postoperatively for possible respiratory failure
Bartter syndrome
  • Autosomal recessive renal disease
  • Hypokalemic, hypochloremic metabolic alkalosis
  • Normal blood pressure, no edema
  • Elevated plasma renin and aldosterone
  • Treatment aimed at prostaglandin synthetase inhibition with indomethacin
  • Patients may be treated with indomethacin, β-blockers, and spironolactone
  • Monitoring of urine output (compensate for polyuria), arterial blood pressure, and CVP is indicated
  • Patients are resistant to effects of exogenous angiotensin and norepinephrine
  • Watch for hypokalemia and hypotonia
Bullous dermatitis (Lyell, Stevens–Johnson, pemphigus, bullous pemphigoid, etc.)
  • Separation of epidermis
  • Skin and mucous membrane bullous lesions may lacerate
  • Most severe form is Stevens–Johnson syndrome
  • May be associated with viral infection, streptococci, cancer, autoimmunity, collagen vascular diseases or drug-induced
  • Tachycardia, high fever, and tachypnea may be features
  • Treatment may involve long-term corticosteroids
  • Associated diseases include porphyria, amyloidosis, multiple myeloma, DM, and hypercoagulable states
  • Malnutrition, anemia, electrolyte derangements, and hypoalbuminemia are common
  • Upper airway and trachea may have lesions → airway management should be approached with caution
  • Regional anesthesia may be given if skin is intact
  • Pulmonary blebs may be present → ↑ risk of PTX
  • Avoid NO if blebs are suspected
  • Steroid supplementation ...

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