Osteogenesis Imperfecta (OI)

Handle as if everything can fracture - Fractures occur from blood-pressure cuff inflation, tourniquets, manual positioning, and even succinylcholine fasciculations. Use minimal or manual cuff pressure (or an arterial line), pad everything, move the patient gently, and avoid depolarizing-induced fasciculations.

Airway - Fragile teeth, a brittle mandible, and cervical fragility demand gentle laryngoscopy and minimal neck manipulation; video laryngoscopy helps, and protect the dentition.

Hyperthermia tendency - Monitor temperature and treat any rise; the hypermetabolic response resembles malignant hyperthermia but does not require dantrolene, though keep MH in the differential.

Bleeding - A platelet-related bleeding tendency can increase surgical blood loss; anticipate it.

Cardiopulmonary - Screen for valvular regurgitation and the restrictive lung disease of kyphoscoliosis.

Regional - Regional techniques are reasonable, but the positioning required to perform them is itself the hazard - move deliberately.

An inherited disorder of type I collagen that makes bone brittle, causing recurrent fractures from minimal force. Associated features include blue sclerae, hearing loss, ligamentous laxity, dentinogenesis imperfecta with fragile teeth, kyphoscoliosis with restrictive lung disease, cardiac valve disease (aortic and mitral regurgitation), a mild bleeding tendency from platelet dysfunction, and a hypermetabolic tendency to intraoperative hyperthermia that resembles - but is not - malignant hyperthermia. Nearly every anesthetic touchpoint carries a fracture or fragility risk.