Achondroplasia

 

 

Considerations

 

  • Potential difficult airway:

    • Difficult bag mask ventilation, subglottic stenosis, facial anomalies, cervical spine instability (odontoid hypoplasia)

    • Possible atlantoaxial instability (AAI) 

  • Pulmonary complications: 

    • Kyphoscoliosis, obstructive & central sleep apnea, restrictive lung disease, possible cor pulmonale

  • Neurologic complications:

    • Spinal stenosis, hydrocephalus (cervical kyphoscoliosis) +/- VP shunt 

  • Difficult neuraxial anesthesia: kyphoscoliosis, narrow epidural space, prolapsed discs, deformed vertebral bodies 

  • Difficult IV access, regional/neuraxial, monitoring & positioning 

 

 

Goals

 

  • Safe establishment of airway 

  • Avoid neck hyperextension (brainstem compression)

  • Identification & optimization of cardiorespiratory complications 

  • Goals for pulmonary hypertension if applicable 

 

 

Pregnancy Considerations 

 

  • Cesarean section required due to fetal:pelvic disproportion

  • Titrated epidural (spinal spread is unpredictable) is best, but epidural may also be difficult