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BACKGROUND: Beckwith-Wiedemann Syndrome is the most common congenital overgrowth disorder with an incidence of approximately 1 in 10,000 live births. The condition is characterized by lateralized overgrowth, abdominal wall defects, macroglossia, and predisposition to malignancy. Historically, children with Beckwith-Wiedemann Syndrome have been presumed to be difficult airways; however, most of the evidence to support this has been anecdotal and derived from case reports. Our study aimed to determine the prevalence of difficult airway in patients with Beckwith-Wiedemann Syndrome. We hypothesized that most patients with Beckwith-Wiedemann Syndrome would not have difficult airways. METHODS: We retrospectively reviewed the electronic medical records of patients enrolled in our institution’s Beckwith-Wiedemann Syndrome registry. Patients with a molecular diagnosis of Beckwith-Wiedemann Syndrome who were anesthetized between January 2012 and July 2019 were included for analysis. The primary outcome was the presence of difficult airway, defined as difficult facemask ventilation, difficult intubation or both. We defined difficult intubation as the need for 3 or more tracheal intubation attempts, the need for advanced airway techniques (non-direct laryngoscopy) to perform tracheal intubation or a Cormack and Lehane grade ≥ 3 during direct laryngoscopy. Secondary objectives were to define predictors of difficult intubation and difficult facemask ventilation, and the prevalence of adverse airway events. Generalized linear-mixed effect models were used to account for multiple anesthesia events per patients. RESULTS: Of 201 Beckwith-Wiedemann Syndrome patients enrolled in the registry, 60% (n=122) had one or more documented anesthetics for a total of 310 anesthetics. A pre-existing airway was present in 22 anesthetics. The prevalence of difficult airway was 5.3% (95% confidence interval [CI] 3.0%-9.3%,18/288) of the cases. The prevalence of difficult intubation was 5.2% (95%CI 2.9%-9.4%,12/226). The prevalence of difficult facemask ventilation was 2.9% (95%CI 1.4%-6.2%, 12/277) and facemask ventilation was not attempted in 42 anesthetics. Age < 1 year, macroglossia, lower weight, endocrine comorbidities, plastics/craniofacial surgery, tongue reduction surgery, and obstructive sleep apnea were associated with difficult airways in cases without a pre-existing airway. 83.8% (95%CI 77.6%-88.5%) of the cases were intubated with a single attempt. Hypoxemia was the most common adverse event. CONCLUSION: The prevalence of difficult tracheal intubation and difficult facemask ventilation in children with Beckwith-Wiedemann Syndrome was 5.2% and 2.9% respectively. We identified factors associated with difficult airway which included age < 1 year, macroglossia, endocrine abnormalities, plastics/craniofacial surgery, tongue reduction surgery and obstructive sleep apnea. Clinicians should anticipate difficult airways in patients with these factors.

BACKGROUND Ventilation is critical in airway management, and failure can be fatal. The optimal ventilation approach for endotracheal intubation in children with difficult airways remains controversial. The Pediatric Difficult Intubation (PeDI) Registry is an international multicenter registry that collects intubation data in difficult to intubate children. The registry captures the initial (at induction) and final ventilation technique (at intubation), the use of neuromuscular blocking drugs (NMBDs), airway reactivity during intubation, and complications. We analyzed data in the PeDI Registry to determine the frequency of use of various ventilation techniques and associated complications. Because spontaneously breathing patients ventilate throughout intubation, we hypothesized that spontaneous ventilation would be associated with fewer complications than other approaches. METHODS We queried the PeDI Registry for cases entered between September 2012 and February 2016, from 16 children's hospitals. We categorized the attending anesthesiologist's ventilation plan into 3 groups: spontaneous ventilation, controlled ventilation after administering an NMBD, and controlled ventilation without administering an NMBD. Generalized Estimating Equation (GEE) model, with a binomial family distribution and logit link, was used to determine the association between ventilation technique and the risk of complications, as well as to account for within-site clustering. Propensity score matching was further applied to balance pretreatment characteristics of ventilation groups. RESULTS Of 1289 anticipated difficult intubations, 507 (39%) were managed with spontaneous ventilation, 453 (35%) controlled ventilation with an NMBD, and 329 (26%) controlled ventilation without an NMBD. Complications occurred in 242 (18.8%; 95% confidence interval [CI], 16.6%-20.9%) patients. Of these, 218 (16.9%) were nonsevere, and 24 (1.9%) were severe. The spontaneous ventilation group had 114 (22.5%, standardized residual [Std.Res] = 4.29) nonsevere complications, which was higher than the controlled ventilation with an NMBD 60 (13.3%, Std.Res = -2.58), and controlled ventilation without an NMBD 44 (13.4%, Std.Res = -1.98), P < .001. Nearest neighbor matching with caliper width equal to 0.2 of the standard deviation (SD) of the logit of the propensity score also demonstrated that patients with spontaneous ventilation had greater odds of complications compared to controlled ventilation techniques: odds ratio (OR) = 2.07 (95% CI, 1.36-3.15; P = .001). CONCLUSIONS Spontaneous ventilation is associated with more nonsevere complications, such as hypoxemia and laryngospasm, than controlled ventilation techniques during intubation of children with difficult airways. Inadequate anesthetic depth may contribute to increased complications.

BACKGROUND In infants and young children, anesthetic dosing is based on population pharmacokinetics and patient hemodynamics not on patient-specific brain activity. Electroencephalography (EEG) provides insight into brain activity during anesthesia. The primary goal of this prospective observational pilot study was to assess the prevalence of isoelectric EEG events-a sign of deep anesthesia-in infants and young children undergoing general anesthesia using sevoflurane or propofol infusion for maintenance. METHODS Children 0-37 months of age requiring general anesthesia for surgery excluding cardiac, intracranial, and emergency cases were enrolled by age: 0-3, 4-6, 7-12, 13-18, and 19-37 months. Anesthesia was maintained with sevoflurane or propofol infusion. EEG was recorded from induction to extubation. Isoelectric EEG events (amplitude