Osteogenesis Imperfecta

 

 

Background

 

  • Rare, autosomal dominant, inherited disease of connective tissue that affects bones, the sclera, & the inner ear, bones are extremely brittle

 

 

Considerations

 

  • Difficult airway: ↓ C-spine mobility, fragile C-spine with fracture risk, mandibular fractures, large head, short neck, brittle teeth

  • Kyphoscoliosis & pectus excavatum 

  • Possible restrictive lung disease, pulmonary hypertension, & RV dysfunction 

  • Cardiac involvement (aortic regurgitation & mitral regurgitation)

  • ↑ risk of fractures: 

    • BP cuffs may be hazardous

    • Careful positioning & padding essential 

    • Succinylcholine fasciculations may cause fractures 

  • Bleeding tendency secondary to ↓ platelet function possible 

  • Hypermetabolic: prone to hyperthermia but not malignant hyperthermia (MH) risk 

 

 

Conflicts

 

  • Full stomach/RSI vs. difficult airway  

  • Regional vs. bleeding tendency 

  • Monitoring/positioning vs. brittle bones 

 

 

Pregnancy Considerations

 

  • ↑ obstetrical risks:

    • High incidence cephalo-pelvic-disproportion → mandatory cesarean section 

    • Higher incidence intra-partum & post partum hemorrhage 

  • ↑ risk uterine rupture/pelvic fracture 

  • Even more difficult airway

  • Succinylcholine can cause bone fractures: give defasciculating NdMR or use rocuronium 

  • Potential contraindication to regional due to platelet dysfunction: if patient history reassuring along with platelet count/INR/PTT, go ahead with regional 

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