Catherine A. Gruffi’s research while affiliated with Westchester Medical Center and other places

Propofol infusion syndrome was first reported in the literature by Bray in 1998. He described a series of fatal outcomes after a presenting constellation of symptoms observed in pediatric patients who had received prolonged propofol infusions. Profound metabolic acidosis and bradycardia are the disease's hallmark features, which can further develop expeditiously to rhabdomyolysis, renal failure, and heart failure. It has been subsequently theorized that a triggering mechanism or a precipitating factor sets up the progressive physiologic spiral which can ensue. The name of the disease was expanded to Propofol Related Infusion Syndrome (PRIS), as propofol alone was no longer considered the culprit. The disease process is rare and can present with an insidious onset in some cases, causing much speculation of whether there is a proper grasp of the disease entity in its entirety as currently reported. The case discussed in this article depicts an adverse neurologic outcome following a craniotomy for temporal lobectomy in a child with lesional epilepsy. Since there was no obvious causative factor for these findings, PRIS became a diagnosis that was robustly discussed among the involved services.

Harlequin ichthyosis is a severe and often fatal form of congenital ichthyosis caused by defective lipid transport which results in a dysfunctional skin barrier. Patients who survive the neonatal period are predisposed to skin infections, sepsis, impaired thermoregulation, and dehydration. The unique skin characteristics can present significant anesthetic challenges. We highlight the relevant anesthetic considerations in a 3-year-old presenting for syndactyly release of the right second and fourth digits. We describe the steps to ensure protection of the fragile skin barrier during establishment of intravenous access and airway management, therefore providing guidance for care of this vulnerable patient population.

Otolaryngologic procedures in the pediatric population can present unique challenges to the anesthesiologist. Pediatric patients should not be considered “little adults” because they display differences in physical characteristics, physiology, pharmacology, coping strategies, emotional needs, and care coordination compared with the adult population. This chapter focuses on patient optimization, risk evaluation and mitigation, anesthetic concerns for specific pediatric conditions, and the perioperative management of children undergoing otolaryngologic procedures.