Anesthetic Management of an Infant With Kleefstra Syndrome During Direct Laryngoscopy and Rigid Bronchoscopy: A Case Report.

Kleefstra syndrome (KS) is an autosomal dominant disorder caused by a chromosomal deletion at 9q34.3 resulting in pathogenic variants of the gene that codes for the enzyme, euchromatin histone methyltransferase 1 (EHMT1). KS is a rare, yet clinically relevant congenital disorder for anesthesiologists because of its potential for cardiac and craniofacial involvement. We present a 3-month-old patient with KS who required anesthetic care for diagnostic laryngoscopy and rigid bronchoscopy. The end-organ effects of KS are reviewed and our anesthetic care presented.

The otolaryngologist's and anesthesiologist's collaborative role in a pandemic: A large quaternary pediatric center's experience with COVID-19 preparation and simulation.

There has been a rapid global spread of a novel coronavirus, the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which originated in Wuhan China in late 2019. A serious threat of nosocomial spread exists and as such, there is a critical necessity for well-planned and rehearsed processes during the care of the COVID-19 positive and suspected patient to minimize transmission and risk to healthcare providers and other patients. Because of the aerosolization inherent in airway management, the pediatric otolaryngologist and anesthesiologist should be intimately familiar with strategies to mitigate the high-risk periods of viral contamination that are posed to the environment and healthcare personnel during tracheal intubation and extubation procedures. Since both the pediatric otolaryngologist and anesthesiologist are directly involved in emergency airway interventions, both specialties impact the safety of caring for COVID-19 patients and are a part of overall hospital pandemic preparedness. We describe our institutional approach to COVID-19 perioperative pandemic planning at a large quaternary pediatric hospital including operating room management and remote airway management. We outline our processes for the safe and effective care of these patients with emphasis on simulation and pathways necessary to protect healthcare workers and other personnel from exposure while still providing safe, effective, and rapid care.

Adjustable Pressure Limit Valve Failure During Intraoperative Care: A Case Report.

One of the many safety features of modern day anesthesia machines is the adjustable pressure limiting (APL) valve. This device regulates pressure within the anesthesia circuit during manual ventilation with the anesthesia bag. We report an unusual case where a crack in the APL valve allowed release of pressure from within the circuit resulting in ineffective bag-valve-mask ventilation of an infant. The appropriate steps to prevent such issues are reviewed, and an algorithm to quickly identify such intraoperative problems is presented.

Correction to: Propofol: A Review of its Role in Pediatric Anesthesia and Sedation.

The original article can be found online.

Sugammadex to reverse neuromuscular blockade and provide optimal conditions for motor-evoked potential monitoring.

Sugammadex is a novel pharmacologic agent, which reverses neuromuscular blockade (NMB) via a mechanism that differs completely from acetylcholinesterase inhibitors. By encapsulating rocuronium, sugammadex can provide recovery of neuromuscular function even when there is a profound degree of NMB. We report anecdotal experience with the use of sugammadex to reverse NMB to facilitate intraoperative neurophysiological monitoring (motor evoked potentials) in an adolescent with scoliosis during posterior spinal fusion. Its potential application in this unique clinical scenario is discussed, and potential dosing schemes are reviewed.

Propofol: a review of its role in pediatric anesthesia and sedation.

Propofol is an intravenous agent used commonly for the induction and maintenance of anesthesia, procedural, and critical care sedation in children. The mechanisms of action on the central nervous system involve interactions at various neurotransmitter receptors, especially the gamma-aminobutyric acid A receptor. Approved for use in the USA by the Food and Drug Administration in 1989, its use for induction of anesthesia in children less than 3 years of age still remains off-label. Despite its wide use in pediatric anesthesia, there is conflicting literature about its safety and serious adverse effects in particular subsets of children. Particularly as children are not "little adults", in this review, we emphasize the maturational aspects of propofol pharmacokinetics. Despite the myriad of propofol pharmacokinetic-pharmacodynamic studies and the ability to use allometrical scaling to smooth out differences due to size and age, there is no optimal model that can be used in target controlled infusion pumps for providing closed loop total intravenous anesthesia in children. As the commercial formulation of propofol is a nutrient-rich emulsion, the risk for bacterial contamination exists despite the Food and Drug Administration mandating addition of antimicrobial preservative, calling for manufacturers' directions to discard open vials after 6 h. While propofol has advantages over inhalation anesthesia such as less postoperative nausea and emergence delirium in children, pain on injection remains a problem even with newer formulations. Propofol is known to depress mitochondrial function by its action as an uncoupling agent in oxidative phosphorylation. This has implications for children with mitochondrial diseases and the occurrence of propofol-related infusion syndrome, a rare but seriously life-threatening complication of propofol. At the time of this review, there is no direct evidence in humans for propofol-induced neurotoxicity to the infant brain; however, current concerns of neuroapoptosis in developing brains induced by propofol persist and continue to be a focus of research.

Epidural hematoma with atypical presentation.

We present a case of massive spinal epidural hematoma with an atypical presentation characterized by unilateral, isolated motor deficit in the right lower extremity on postoperative day 2 after Collis-Nissen fundoplication and a T7-8 epidural for postoperative pain. The epidural had been placed in the preoperative theater before surgery. Subcutaneous unfractionated heparin was initiated 18 hours later on postoperative day 1 with 3 times daily dosing. The patient also received 3 doses of ketorolac starting 10 hours after epidural placement. Symptoms were first reported on postoperative day 2, 37 hours after epidural placement. Thoracic magnetic resonance imaging showed an epidural hematoma extending from T2 through T12, requiring emergent decompression and evacuation. The patient made a complete recovery without any resultant neurologic deficit.