Disease States

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

• Pain/ataxia/incontinence/apnea—due to cervicomedullary/spinal cord compression

• Neurologic—hypotonia in infancy
• Craniofacial features—large head, midface hypoplasia, dental crowding
• Short stature (normal trunk length)
• Bow legs (genu varum)

Polysomnography (assess CSA/OSA)

Head/neck CT/MRI (assess craniocervical junction)

• Neurology as indicated from history and exam

• Endotracheal intubation and cervical instability
• CSA/OSA and use of sedatives/analgesics
• Regional anesthesia and spinal/neurologic abnormalities

• 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)

• General anesthesia, regional anesthesia, or sedation

• Premed
• Blood: as indicated by surgical procedure
• ICU/stepdown bed: consider severity of OSA

• Difficult airway cart available
• Body size appropriate airways/laryngoscopes
• Consider use of alternative analgesics than opioids (dexmedetomidine, low-dose ketamine, regional)

• If GETA—consider AFOI as determined from airway/C-spine assessment
• If regional—use smaller doses of LA; beware high block

• Maintain neck in neutral position
• Positioning—consider patient’s body habitus

• If difficult intubation—consider leaving ETT in place or extubating fully awake, use of tube exchanger

• Recovery/stepdown/ICU as required

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)

• 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?

• Body habitus/BMI
• Airway—hypertrophy of facial bones, mandible, tongue
• Vital signs—hypertension?
• Heart failure—tachycardia, elevated JVP, S3/S4, hepatomegaly, peripheral/pulmonary edema

• CBC—anemia? Electrolytes— hyponatremia, ↓K, hyperglycemia?
• TSH—thyroid function?
• EKG/echocardiography—LV hypertrophy? Systolic/diastolic dysfunction?

• Potential difficult airway/difficult mask ventilation (requiring AFOI) and potential cardiac ...