Anesthetic management of a pediatric patient with Loeys-Dietz syndrome: A case report.

Loeys-Dietz syndrome, a rare autosomal dominant disease, is characterized by arterial tortuosity and skeletal manifestations. We report anesthetic management of scoliosis surgery for a pediatric Loeys-Dietz syndrome patient. During surgery, severe hypotension occurred when a rotation-correction was performed; blood pressure returned to the baseline level after the maneuver was abandoned. We decided to not conduct rotation correction after discussing risks and benefits of the maneuver with surgeons. Since severe hemodynamic instability is expected in scoliosis surgery for Loeys-Dietz syndrome patients, we should be prepare for hemodynamic collapse and risks and benefits of the operative procedure should be discussed before undertaking the procedure.