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Disease States

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Considerations
  1. Most common form of dwarfism (more than 100 other types). Autosomal dominant inheritance

    Appearance: large head-to-body size difference, prominent forehead, shortened arms and legs, decreased muscle tone

  2. Airway management difficulties

    Craniofacial and spinal abnormalities:

    • limited neck extension
    • foramen magnum stenosis
    • large tongue
    • large mandible
    • atlanto-axial instability

  3. Kyphosis, scoliosis, and spinal stenosis: difficult and unpredictable spread of local anesthetics in epidural and subarachnoid spaces

  4. Comorbidities: central and obstructive sleep apnea, otitis media (childhood), obesity

History
  • Pain/ataxia/incontinence/apnea—due to cervicomedullary/spinal cord compression
Physical Exam
  • Neurologic—hypotonia in infancy
  • Craniofacial features—large head, midface hypoplasia, dental crowding
  • Short stature (normal trunk length)
  • Bow legs (genu varum)
Lab Tests/Imaging

Polysomnography (assess CSA/OSA)

Head/neck CT/MRI (assess craniocervical junction)

Consults
  • Neurology as indicated from history and exam
Conflict(s)
  • Endotracheal intubation and cervical instability
  • CSA/OSA and use of sedatives/analgesics
  • Regional anesthesia and spinal/neurologic abnormalities
Optimize/Goals
  • Minimize movement of cervical spine during ETT placement (consider referral to neurology if new onset/worsening symptoms)
  • Consider adjuvants to opioids for pain management regarding OSA
  • Consider imaging techniques before regional anesthesia to assess anatomy of vertebrae and spinal cord; also consider epidural versus spinal (titratable)
Options
  • General anesthesia, regional anesthesia, or sedation
Preop:
  • Premed
  • Blood: as indicated by surgical procedure
  • ICU/stepdown bed: consider severity of OSA
Room Setup (Special Drugs/Monitors)
  • Difficult airway cart available
  • Body size appropriate airways/laryngoscopes
  • Consider use of alternative analgesics than opioids (dexmedetomidine, low-dose ketamine, regional)
Induction
  • If GETA—consider AFOI as determined from airway/C-spine assessment
  • If regional—use smaller doses of LA; beware high block
Maintenance
  • Maintain neck in neutral position
  • Positioning—consider patient’s body habitus
Emergence
  • If difficult intubation—consider leaving ETT in place or extubating fully awake, use of tube exchanger
Disposition/Pain
  • Recovery/stepdown/ICU as required
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Clinical Pearls

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For the achondroplastic obstetric patient—consider imaging early in pregnancy to assess lumbar anatomy during labor—early placement of epidural catheter to allow for slow titration.

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Reference

Shirley ED, Ain MC. Achondroplasia: manifestations and treatment. J Am Acad Orthop Surg. April 2009;17(4):231-241.

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Considerations
  1. GH hypersecretion.

    Usually from GH secreting pituitary adenoma—may be complicated by headaches, visual field defects, elevated ICP, hypopituitarism (requires replacement with hydrocortisone/thyroxine)

  2. Potentially difficult airway/difficult ventilation: gigantism, facial changes, large tongue, hypertrophy of pharyngeal mucosa, small glottic opening, prominent jaw (prognathism), obstructive sleep apnea (OSA)

  3. Comorbidities: OSA, hypertension, cardiac arrhythmias, diastolic dysfunction (heart failure), coronary artery disease, glucose intolerance, renal failure, arthritis, kyphoscoliosis

  4. Treatments: pituitary surgery (transsphenoidal), radiotherapy, medical (octreotide—suppresses GH secretion)

History
  • Comorbidities: duration, severity, and functional capacity/limitations
  • Respiratory—apnea, snoring, somnolence, PH OSA, use of CPAP, PH of difficult intubation?
  • Cardiac disease—HTN? Angina? Exercise capacity?
  • Therapies: medical, radiotherapy, surgical?
Physical Exam
  • Body habitus/BMI
  • Airway—hypertrophy of facial bones, mandible, tongue
  • Vital signs—hypertension?
  • Heart failure—tachycardia, elevated JVP, S3/S4, hepatomegaly, peripheral/pulmonary edema
Lab Tests/Imaging
  • CBC—anemia? Electrolytes— hyponatremia, ↓K, hyperglycemia?
  • TSH—thyroid function?
  • EKG/echocardiography—LV hypertrophy? Systolic/diastolic dysfunction?
Conflict(s)
  • Potential difficult airway/difficult mask ventilation (requiring AFOI) and ...

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