Perioperative airway complications in infants and children with Crouzon and Pfeiffer syndromes: A single-center experience.

BACKGROUND: Crouzon and Pfeiffer syndromes are rare genetic disorders characterized by craniosynostosis, exorbitism, and maxillary hypoplasia. Patients with these syndromes frequently require general anesthesia for various diagnostic and surgical procedures and may present a challenge to anesthetists with regard to airway management. AIMS: The primary aim of this study was to determine the incidence, timing, and management of perioperative upper airway obstruction in infants and children with Crouzon and Pfeiffer syndromes. The secondary aim was to determine the degree of difficulty in performing endotracheal intubation. METHODS: A retrospective review of 812 anesthetic encounters in 67 patients was conducted. The following were recorded: timing and management of episodes of perioperative upper airway obstruction, from induction of anesthesia to discharge from recovery, degree of difficulty with laryngoscopy using the Cormack-Lehane grading system and number of intubation attempts required, patient demographics, respiratory comorbidity, surgical procedure, and anesthetic airway management techniques. RESULTS: Upper airway obstruction at induction of anesthesia was very common, with an incidence of 31% (167/542 anesthetic encounters affecting 54 patients). In a quarter of these incidents, bag-valve-mask ventilation was challenging, but a laryngeal mask airway was almost always effective. Upper airway obstruction on emergence from anesthesia was less common, with an incidence of 2.7% (14/515 anesthetic encounters affecting 10 patients). Contributing factors included patient comorbidity (obstructive sleep apnea, nasal stenosis) and the nature of surgery (craniofacial or airway procedures). Intubation was rarely difficult in this cohort, with 85% of laryngoscopies rated Cormack-Lehane grade 1 or 2 (n = 373), and 89% of intubations successful on the first attempt (n = 306). CONCLUSIONS: Upper airway obstruction at induction of anesthesia is common in patients with Crouzon and Pfeiffer syndrome. These patients are likely to present some difficulties with perioperative airway management, especially bag-valve-mask ventilation, but rarely endotracheal intubation.

We Asked the Experts: The WHO Surgical Safety Checklist and the COVID-19 Pandemic: Recommendations for Content and Implementation Adaptations.

BACKGROUND: As surgical systems are forced to adapt and respond to new challenges, so should the patient safety tools within those systems. We sought to determine how the WHO SSC might best be adapted during the COVID-19 pandemic. METHODS: 18 Panelists from five continents and multiple clinical specialties participated in a three-round modified Delphi technique to identify potential recommendations, assess agreement with proposed recommendations and address items not meeting consensus. RESULTS: From an initial 29 recommendations identified in the first round, 12 were identified for inclusion in the second round. After discussion of recommendations without consensus for inclusion or exclusion, four additional recommendations were added for an eventual 16 recommendations. Nine of these recommendations were related to checklist content, while seven recommendations were related to implementation. CONCLUSIONS: This multinational panel has identified 16 recommendations for sites looking to use the surgical safety checklist during the COVID-19 pandemic. These recommendations provide an example of how the SSC can adapt to meet urgent and emerging needs of surgical systems by targeting important processes and encouraging critical discussions.

Consensus Guidelines for Perioperative Care in Neonatal Intestinal Surgery: Enhanced Recovery After Surgery (ERAS®) Society Recommendations.

BACKGROUND: Enhanced Recovery After Surgery (ERAS®) Society guidelines integrate evidence-based practices into multimodal care pathways that have improved outcomes in multiple adult surgical specialties. There are currently no pediatric ERAS® Society guidelines. We created an ERAS® guideline designed to enhance quality of care in neonatal intestinal resection surgery. METHODS: A multidisciplinary guideline generation group defined the scope, population, and guideline topics. Systematic reviews were supplemented by targeted searching and expert identification to identify 3514 publications that were screened to develop and support recommendations. Final recommendations were determined through consensus and were assessed for evidence quality and recommendation strength. Parental input was attained throughout the process. RESULTS: Final recommendations ranged from communication strategies to antibiotic use. Topics with poor-quality and conflicting evidence were eliminated. Several recommendations were combined. The quality of supporting evidence was variable. Seventeen final recommendations are included in the proposed guideline. DISCUSSION: We have developed a comprehensive, evidence-based ERAS guideline for neonates undergoing intestinal resection surgery. This guideline, and its creation process, provides a foundation for future ERAS guideline development and can ultimately lead to improved perioperative care across a variety of pediatric surgical specialties.

Creation of an Enhanced Recovery After Surgery (ERAS) Guideline for neonatal intestinal surgery patients: a knowledge synthesis and consensus generation approach and protocol study.

INTRODUCTION: Enhanced Recovery After Surgery (ERAS) guidelines integrate evidence-based practices into multimodal care pathways designed to optimise patient recovery following surgery. The objective of this project is to create an ERAS protocol for neonatal abdominal surgery. The protocol will identify and attempt to bridge the gaps between current practices and best evidence. Our study is the first paediatric ERAS protocol endorsed by the International ERAS Society. METHODS: A research team consisting of international clinical and family stakeholders as well as methodological experts have iteratively defined the scope of the protocol in addition to individual topic areas. A modified Delphi method was used to reach consensus. The second phase will include a series of knowledge syntheses involving a rapid review coupled with expert opinion. Potential protocol elements supported by synthesised evidence will be identified. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system will be used to determine strength of recommendations and the quality of evidence. The third phase will involve creation of the protocol using a modified RAND/UCLA Appropriateness Method. Group consensus will be used to rate each element in relation to the quality of evidence supporting the recommendation and the appropriateness for guideline inclusion. This protocol will form the basis of a future implementation study. ETHICS AND DISSEMINATION: This study has been registered with the ERAS Society. Human ethics approval (REB 18-0579) is in place to engage patient families within protocol development. This research is to be published in peer-reviewed journals and will form the care standard for neonatal intestinal surgery.

Ten years of simulation-based training in pediatric anesthesia: The inception, evolution, and dissemination of the Managing Emergencies in Pediatric Anesthesia (MEPA) course.

2016 marked the 10-year anniversary of the inception of the Managing Emergencies in Paediatric Anaesthesia (MEPA) course. This simulation-based program was originally created to allow trainees in pediatric anesthesia to experience operating room emergencies which although infrequent, would be considered key competencies for any practicing anesthetist with responsibility for providing care to children. Since its original manifestation, the course has evolved in content, scope, and worldwide availability, such that it is now available at over 60 locations on five continents. The content has been modified for different learner groups and translated into several languages. This article describes the history, evolution, and dissemination of the MEPA course to share lessons learnt with educators considering the launch of similar initiatives in their field.

The child with facial abnormalities.

PURPOSE OF REVIEW: The aim of this review is to outline the priorities in the anaesthetic management of the child with facial abnormalities. It presents a practical approach to this, based on the anatomical site of the deformity and degree of mouth opening. RECENT FINDINGS: The literature reviewed primarily consists of case reports and series describing anaesthesia in children with relevant syndromes. Also scrutinized is the literature examining the role and effectiveness of recently developed airway management equipment. SUMMARY: This is a challenging area of anaesthetic practice but the use of a structured approach, combined with supraglottic airway devices and fibre-optic and indirect laryngoscopic equipment, has allowed the safe administration of anaesthesia to almost all children with conditions resulting in facial abnormality.

Metabolic changes during major craniofacial surgery.

OBJECTIVES: The purpose of this study was to document the degree and duration of perioperative metabolic disturbance during major craniofacial surgery in children. AIM: The aim was to quantify the degree and duration of perioperative metabolic disturbance and to determine the relationship between the metabolic changes and the duration of surgery and total volume of blood and colloid given during surgery. BACKGROUND: These patients have the potential for massive blood loss and significant metabolic acidosis. Routine perioperative monitoring includes the serial measurement of base deficit (BD) as a marker of metabolic disturbance. METHODS/MATERIALS: All patients undergoing elective major craniofacial surgery were prospectively studied over a 10-month period. BD from arterial blood gas analysis was measured at standardized intervals during the perioperative period. The duration of surgery and total volume of blood and colloid given intraoperatively were used as covariates in a multiple regression analysis. RESULTS: Maximum recorded BD ranged from -3 to -20 (median -9). Median time taken to return to normal was 9.25 h (range 0-18 h). Median duration of significant BD was 3.8 h (range 0-20 h). CONCLUSIONS: Children undergoing major craniofacial surgery develop a varying degree of perioperative metabolic acidosis persisting for several hours. The maximum BD appears to be related to the amount of intraoperative blood loss and replacement rather than duration of surgery. As it is difficult to predict the extent and duration of metabolic acidosis for an individual patient, this study confirmed our current practice that all patients should be admitted to a neurosurgical high-dependency unit postoperatively for overnight monitoring.

The use of filters with small infants.

The use of breathing system filters may be particularly beneficial in small infants, compared with older children and adults, because of their greater need for warming and humidification of inspired gases as well as their increased susceptibility to lower respiratory tract contamination. The only evidence available regarding the safety and efficacy of breathing system filters in small infants comes from a few small studies conducted on intensive care patients, however. These studies have suggested that the use of HME filters may be effective in preserving body temperature and airway humidity while decreasing fluid build-up in the breathing system and therefore reducing breathing system contamination. Nonetheless, the use of filters has not been shown to decrease the incidence of VAP in small infants. In contrast,their use in adult intensive care patients, particularly those requiring prolonged ventilation, has been associated with a decrease in the infection rate. The use of breathing system filters is not associated with a statistically significant increase in the rate of complications, despite the potentially greater hazards associated with their use in small infants compared with older children and adults. In practice the use of breathing system filters, even in small infants, rarely causes any major clinical problems that cannot be prevented with a high degree of vigilance and appropriate monitoring. This vigilance is particularly important to prevent the serious morbidity and even mortality that may result from filter occlusion; when subjected to excessive loading, smaller filters are more prone to obstruction than are their larger counterparts. The increased resistance provided by smaller filters should not translate into a clinically significant increase in the work of breathing during general anesthesia, because it is common practice to ventilate small infants for all but the shortest of surgical procedures. An increase in the work of breathing may, however, become more significant when spontaneous ventilation is established at the end of a surgical case. It remains unclear whether the use of filters allows the safe reuse of breathing systems in small infants. None of the breathing system filters tested by the MHRA had a zero-percent penetrance to sodium chloride particles, and pediatric filters generally had a higher penetrance than their adult counterparts. This finding suggests that there is a potential, albeit small, risk of cross-contamination. The exact risk depends on the type of filter used and on the particular patient undergoing anesthesia or ventilation in the ICU. Although no evidence has been published showing cross-infection occurring when any filter has been used in the anesthesia breathing system for adults or small infants, the level of filtration performance required to allow the safe reuse of anesthesia breathing systems in small infants remains unanswered. Because the incidence of lower respiratory tract colonization is low in unselected small infants, a study with sufficient power to answer accurately the questions regarding the safety of breathing system reuse in small infants would be very difficult to conduct. The effect of filters on post operative infection rates may in fact be of less significance than the adoption of adequate standards of hygiene (eg, hand washing and the use of gloves).Further research is needed to determine if the variations in filtration efficiency demonstrated by the MHRA have any effects on patient outcome. This research might allow setting an effective minimal level of filtration performance for breathing system filters for use in small infants. On a practical note, the publication of the MHRA assessments of breathing system filters provides a useful tool for objective comparison of the different filters available for use in small infants, even though the relevance of the flow used to test pediatric filters has been criticized. Individual institutions will need to formulate policies for the use of breathing system filters for clinical reasons as well as for cost containment or logistical reasons. These policies should be within the frameworks set out by their regulatory agencies. Any problems arising from policies that are in breach of these frame works will remain the responsibility of the individual clinicians caring for these small infants.

Double trouble: prolapsing epiglottis and unexpected dual pathology in an infant.

A 3-week-old full-term female neonate was admitted with a 4-day history of episodic stridor, desaturations and difficult feeding. Initial assessment using fluoroscopy suggested distal tracheomalacia. Inhalational induction for examination under anaesthesia of the upper airway at 4 weeks of age caused almost complete airway obstruction due to severe anterior, or epiglottic, laryngomalacia. This airway obstruction was unresponsive to continuous positive airway pressure, the use of an oropharyngeal airway and hand ventilation and required urgent tracheal intubation using suxamethonium. Epiglottopexy, a relatively unknown procedure, was performed uneventfully 2 days later, with complete relief of the respiratory compromise. However, the infant remained desaturated postoperatively. A ventilation perfusion scan subsequently revealed multiple pulmonary arteriovenous malformations, unsuitable for embolization and requiring nocturnal home oxygen therapy. Review at 3 months of age found a thriving infant with no airway obstruction and good epiglottic positioning on examination under anaesthesia. Although the patient's oxygen requirements had diminished, the long-term outcome remains uncertain.