Osteogenesis Imperfecta
Background
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Rare, autosomal dominant, inherited disease of connective tissue that affects bones, the sclera, & the inner ear, bones are extremely brittle
Considerations
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Difficult airway: ↓ C-spine mobility, fragile C-spine with fracture risk, mandibular fractures, large head, short neck, brittle teeth
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Kyphoscoliosis & pectus excavatum
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Possible restrictive lung disease, pulmonary hypertension, & RV dysfunction
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Cardiac involvement (aortic regurgitation & mitral regurgitation)
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↑ risk of fractures:
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BP cuffs may be hazardous
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Careful positioning & padding essential
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Succinylcholine fasciculations may cause fractures
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Bleeding tendency secondary to ↓ platelet function possible
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Hypermetabolic: prone to hyperthermia but not malignant hyperthermia (MH) risk
Conflicts
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Full stomach/RSI vs. difficult airway
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Regional vs. bleeding tendency
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Monitoring/positioning vs. brittle bones
Pregnancy Considerations
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↑ obstetrical risks:
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High incidence cephalo-pelvic-disproportion → mandatory cesarean section
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Higher incidence intra-partum & post partum hemorrhage
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↑ risk uterine rupture/pelvic fracture
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Even more difficult airway
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Succinylcholine can cause bone fractures: give defasciculating NdMR or use rocuronium
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Potential contraindication to regional due to platelet dysfunction: if patient history reassuring along with platelet count/INR/PTT, go ahead with regional