Abstract
Features of Noonan syndrome include a distinctive facial appearance, short stature, a broad or webbed neck, congenital heart disease, bleeding problems, skeletal malformations, and developmental delay. Although pulmonary stenosis is most commonly reported, up to 20% of patients have hypertrophic cardiomyopathy (HCM). We report the use of a combined spinal-caudal epidural anesthesia technique during urologic surgery (pyeloplasty) in an 8-week-old infant with Noonan syndrome and HCM. A spinal-caudal epidural technique provides favorable hemodynamic conditions, avoids the need for airway instrumentation, and may serve as an alternative to general anesthesia in these high risk patients.
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