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
Airway management difficulties
Craniofacial and spinal abnormalities:
- limited neck extension
- foramen magnum stenosis
- large tongue
- large mandible
- atlanto-axial instability
Kyphosis, scoliosis, and spinal stenosis: difficult and unpredictable spread of local anesthetics in epidural and subarachnoid spaces
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)Consults
- 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
Room Setup (Special Drugs/Monitors)
- 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
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.
Shirley ED, Ain MC. Achondroplasia: manifestations and treatment. J Am Acad Orthop Surg. April 2009;17(4):231-241.
Usually from GH secreting pituitary adenoma—may be complicated by headaches, visual field defects, elevated ICP, hypopituitarism (requires replacement with hydrocortisone/thyroxine)
Potentially difficult airway/difficult ventilation: gigantism, facial changes, large tongue, hypertrophy of pharyngeal mucosa, small glottic opening, prominent jaw (prognathism), obstructive sleep apnea (OSA)
Comorbidities: OSA, hypertension, cardiac arrhythmias, diastolic dysfunction (heart failure), coronary artery disease, glucose intolerance, renal failure, arthritis, kyphoscoliosis
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 ...