Pediatric Anesthesia: A Guide for the Non-Pediatric Anesthesia Provider Part II

Children undergoing anesthesia have many considerations of disease processes that require careful attention to details and addressing specific needs. There are several comorbidities that are frequently encountered in a pediatric setting. A common scenario is a child with an upper respiratory tract infection presenting for elective surgery. We will discuss the criteria to be considered regarding when it is safe to proceed with elective surgery and when the risk is high. Asthma is common among children, and exacerbation can occur during an anesthetic. Anesthetic management of children with these respiratory illnesses is discussed. Children with Down syndrome frequently present for various cardiac and non-cardiac surgical interventions. Anesthetic issues relating to their non-cardiac surgeries will be discussed. Children with sickle cell disease is yet another group of patients frequently admitted to the hospital with sickle cell crisis. They warrant attention to specific details to ensure getting through surgery safely and require optimal pain management. Obstructive sleep apnea is increasingly encountered in children presenting for surgical procedures. Anesthetic challenges and risks they pose will be discussed. 

In this chapter, we will discuss the perioperative anesthetic concerns of children with autism, attention-deficit hyperactivity disorder (ADHD), cystic fibrosis, epidermolysis bullosa, and von Willebrand disease. Children with autism and ADHD are at high risk for having severe anxiety and distress when undergoing anesthesia due to difficulty in adjusting to the changes in daily routine and new environment. Careful planning to limit waiting time in the preoperative area and appropriate premedication are critical to minimizing distress. Children with cystic fibrosis may have involvement of pulmonary, gastrointestinal, and pancreaticobiliary organ systems. Due to the increasing longevity of this population, both children and adults may present for a variety of surgical procedures; pulmonary status is a key concern when formulating an anesthetic plan. Children with epidermolysis bullosa present special challenges when undergoing anesthesia because both airway devices and the equipment used to monitor vital signs can cause serious postoperative complications. Von Willebrand disease is the most common congenital bleeding disorder encountered in pediatric anesthesia. Safe perioperative management requires interdisciplinary coordination to create a plan for prophylaxis prior to surgery, intraoperative and postoperative management to maintain hemostasis.

There have been dramatic improvements in the survival of neonates and children with many diseases and disorders due to advancements in medicine over the past several decades. These advances are attributed to the better understanding of these disease processes, the advent of multidrug combinations, molecularly targeted therapies, critical care and various surgical interventions. In the wake of this rapidly developing wide range of treatment protocols, the anesthesiologist needs to have a clear understanding of these disorders and their comorbidities, and stay abreast of the various treatment modalities, including their safety and toxicity profiles. This review attempts to emphasize some of the clinical conditions unique to these patients and special considerations for the conduct of anesthesia in this population. Some of the disease processes and comorbidities discussed here include anesthetic considerations for ex-premature infants, diabetes mellitus, obesity, childhood cancer, and children with congenital heart disease who present for non-cardiac surgery. The objective of this discussion is to provide an updated and comprehensive review of current perioperative anesthetic management of pediatric patients with these conditions. We also delineate the effects of anesthesia during the perioperative course, including major metabolic changes that may result in increased morbidity. We provide guidelines for any anesthesia provider involved in the care of these vulnerable patients. Special considerations need to be taken to promote the physical and mental wellbeing of these children and their families. Collaborative coordination with all providers involved in care is essential to provide safe and effective anesthesia to this subset of patients.

In comparison to adults, critical illness is relatively uncommon in the pediatric population. Many facilities may be unprepared or ill-equipped for treating these rare conditions. The conventional anesthesiologist may not be familiar with the common pathologies of critical illness in children. These patients may need complex and life-saving urgent procedures to stabilize them. It is logistically challenging to have pediatric trained personnel readily available at all hospitals and care centers. These patients have special perioperative needs that the anesthesiologist should consider. They may potentially present to the operating room, emergency department, or pediatric intensive care unit. Here, we offer a direct and practical approach to managing the care of these younger patients.

Numerous pediatric genetic disorders have anesthetic implications; however, the ones that should be familiar to all anesthesiologists are those associated with malignant hyperthermia, pseudocholinesterase deficiency, and opioid prescribing. Genetic defects leading to ryanodine receptor mutations in malignant hyperthermia require attention in both anesthetic technique and pre-induction preparation. Pseudocholinesterase deficiency associated with rare mutations of pseudocholinesterase may be encountered by all anesthesiologists and has specific operative and postoperative considerations. Finally, opioids are a common modality for pain control throughout surgical care. Awareness of genetic differences in activation and metabolism for different opioids, as illustrated with codeine usage in pediatrics, is necessary for safe pain management prescribing. 

Inadequate pain management in children not only leads to immediate stress and suffering, but it can also influence long-term psychological, physiological, and emotional outcomes for the patient and family. It is well-established that neonates not only feel pain, but also have an exaggerated perception due to immaturity of the descending inhibitory pathways. In addition, various physiologic differences influence the effect and duration of various medications. Despite the existence of many validated tools and pain scales, the assessment of pain in children is challenging. Successful acute pain management targets various elements in the complex system of pain transduction, transmission, modulation, and perception. A multimodal approach targeting multiple steps in the nociceptive pathway is more effective than those involving a single target. Regional analgesic techniques supplemented with opiates and nonopioid medications have proven to be effective in controlling postoperative pain. Traditionally, caudal and epidural analgesia have an established record of safety and are popular regional techniques in children. However, advances in ultrasound-guided techniques have encouraged pediatric anesthesiologists to perform more regional blocks in children. Additionally, application of the enhanced recovery after surgery (ERAS) protocol for major surgeries and concern about the opioid epidemic has led to increasing awareness about the benefits of regional anesthesia. In this chapter, we will discuss the fundamentals of pain perception in children, the assessment of pain and the multimodal approach to manage it, relevant pharmacology, and various regional techniques in routine and complex pediatric surgical patients. 

In the United States and globally, governmental organizations have initiated programs to stimulate clinical research in the pediatric population. The most important reason for these programs is a lack of high-quality evidence to guide effective and safe treatment in children. In pediatric anesthesiology, physicians and scientists need to evaluate anesthetic drugs, medical equipment, and devices. Under improved global regulations, current and new anesthetic strategies will undergo a scientific evaluation of safety and effectiveness. Along these lines, the number of pediatric patients participating in anesthesiology research is expected to increase. Federal regulations deem minors to lack the required decision-making capacity to consent to participate in studies. In line with the Declaration of Helsinki, enrolling children in research should encompass legal guardian permission and assent of the child. Proxy consent and assent procedures should enable the legal representative to safeguard the child's interest while allowing the child to give meaningful agreement. Assent has been defined in detail by ethical scholars but is generally poorly defined legally. A specific challenge to appropriate proxy consent and a child's assent in pediatric anesthesiology research is the brief treatment interaction the pediatric anesthesiologist can have with patients. This challenge may require contacting potential research subjects well before admission to the hospital, depending on the risk and complexity associated with the study at hand. 

The delivery of high-quality, safe patient care is fundamental to a successful outcome for pediatric patients undergoing anesthesia. Organizations rooted in a transparent culture where employees are encouraged to speak up for safety will often engage their staff and patients to design systems of care that are meaningful and effective. Successful healthcare organizations continuously analyze data and refine processes in search of opportunities for improvement. This chapter will discuss the core principles of quality and patient safety and review some ongoing national and international collaborative efforts to improve anesthesia care for children. Practical strategies for establishing a quality improvement program are explored, including incorporation of simulation for performance improvement.

In December 2019, patients clustered in Wuhan, China, were identified to have a novel virus now known as COVID-19, a virus from the coronavirus family. This virus has spread all across the world, with close to 8 million confirmed cases and over 432,000 confirmed COVID-19 deaths worldwide. This pandemic has rattled the modern era and presented with several new considerations that the anesthesiologist must be aware of. This chapter will cover several anesthetic concerns that involve this virus and all the precautions that one should take during this pandemic. It should be noted that there is new research emerging rapidly about this novel virus, and one can always depend on World Health Organization, the American Society of Anesthesiologists, Anesthesia Patient Safety Foundation, and Centers for Disease Control and Prevention for updated COVID-19 resources for anesthesiologists. This chapter was written in September 2020 and is the first textbook chapter that we know that is available at this time. 

 Children with neuromuscular diseases have a broad range of presentation and anesthesia considerations. Muscle strength is reduced by degenerative nerve supply and affected neuromuscular junctions or by weakening of muscle fibers directly. Muscular dystrophies are an inherited group of disorders characterized by progressive muscle weakness. These children pose specific challenges related to anesthetic care due to skeletal muscle, pulmonary, and cardiac involvement. This chapter discusses the perioperative management of children with Duchenne, Becker, limb-girdle, EmeryDreifuss, and myotonic muscular dystrophies. Also discussed are mitochondrial myopathies, a group of clinical conditions common to the pediatric population. Cerebral palsy (CP) is a nonspecific, descriptive term that encompasses a constellation of symptoms due to a neurologic lesion resulting from the insult to the developing brain sustained early in life. Although the neurologic lesion in CP is non-progressive, the motor dysfunction due to spasticity may be progressive, leading to spinal deformities, joint contractures, and dislocations requiring medical and surgical interventions. Anesthetic care for children with the above stated neuromuscular disorders requires understanding of their disease process and careful attention to all aspects of perioperative care. Thoughtful planning should include thorough preoperative assessment, attention to co-morbidities, management of chronic medications, and meticulous intraoperative care for these patients. Postoperative assessment of pain and its management are essential to facilitate recovery and uneventful perioperative course.

Several uncommon pediatric diseases encountered by non-pediatric anesthesiologists bear discussion because a failure to recognize unique issues relating to them could lead to significant morbidity and mortality. Anterior mediastinal masses share the same space as major pulmonary and cardiovascular structures, which, if compressed, can compromise the airway, heart and great vessels. Awareness of the potential for major physiologic derangement and a multidisciplinary approach to their diagnosis and management will ensure a safe perioperative course. Congenital pulmonary airway malformation is one of the most frequent pulmonary malformations in children that requires surgical intervention by thoracoscopic approach in the first year of life. Their anesthetic management is often challenging due to their small size, need for lung isolation and maintaining adequate oxygenation during surgery. Understanding lung isolation technique in infants is necessary for the safe administration of anesthetics in these children. Arthrogryposis multiplex congenita syndrome is a rare, non-progressive, and congenital heterogeneous group of disorders characterized by congenital joint contractures. These children require frequent surgeries during childhood to address various musculoskeletal abnormalities. Their anesthetic management is often challenging due to comorbidities related to musculoskeletal deformities, neurologic, cardiovascular and respiratory systems involvement. Understanding the disease with thorough evaluation and preparation prior to anesthetic management is essential for a good perioperative outcome. Pheochromocytoma is a rare neuroendocrine tumor in children that may present unexpectedly for the management by anesthesiologists. It secretes catecholamines which can cause lifethreatening perioperative hemodynamic instability. The meticulous preoperative pharmacotherapy, intraoperative anesthetic management and postoperative monitoring are vital for a safe outcome.