bluuebunny/crossref_metadata_2025 huggingface dataset:
Reticuloendothelial System: The Basics
Authors: Qian Jenny X. , Pua Bradley B.
Published: October 2018

The spleen is the largest organ of the reticuloendothelial system and the largest lymphoid organ. Derived from embryologic mesoderm, it is a solid, intraperitoneal organ situated in the left upper abdomen, lateral and slightly posterior to the stomach. Long thought to be a superfluous structure, the spleen is now known to play a major role in immunity, as asplenic patients are predisposed to sepsis from encapsulated organisms. It is also an important reservoir for platelets, monocytes, and erythrocytes, and it performs the vital function of filtering the blood to remove senescent or abnormal cells, as well as circulating debris. Its ability to perform these functions is closely tied to the microarchitecture of both its red- and white-pulp components. In addition, the spleen plays an important role in the development of many diseases, and may be secondarily involved or, more rarely, the primary site of malignant neoplasms such as lymphoma.
Reticuloendothelial System: Vascular, Nonvascular, and Oncologic
Authors: Santos Ernesto , Pinter Joshua , McCluskey Kevin
Published: October 2018

The reticuloendothelial system is the portion of the immune system consisting of phagocytic cells found in reticular connective tissue in the spleen, liver, lungs, bone marrow, and lymph nodes. For the purposes of this chapter, the discussion will be limited to interventions within the spleen and the lymphatic system. Splenic arterial interventions are performed to treat a variety of clinical conditions, including abdominal trauma, hypersplenism, splenic arterial aneurysm/pseudoaneurysm, portal hypertension, and splenic neoplasm, and they provide an alternative to open surgery. Although not commonly performed, percutaneous splenic biopsy and drainage are relatively safe and efficacious procedures. Lymphangiography is a therapeutic option for patients with chylothorax, chylous ascites, and lymphatic fistula. Percutaneous thoracic duct embolization (TDE) is an alternative to surgical ligation of the thoracic duct (TD).
Musculoskeletal Interventions: The Basics
Authors: Maybody Majid
Published: October 2018

Comparable to other trends in medicine toward minimally invasive approaches, there has been a steady increase in demand for image-guided musculoskeletal (MSK) interventions. Interventional radiologists are involved in a variety of MSK interventions, including diagnostic, therapeutic, and palliative procedures. While many of the concepts that apply to other organs also apply to the MSK system, some are unique. This chapter will review MSK anatomy relevant to the safe and effective performance of biopsy, ablation, sclerotherapy, and embolization. It also will review imaging modalities used for MSK intervention and the special instruments that are used to access bony targets with an intact cortex. Finally, optimizing conditions for intervention, including patient position, anesthesia, use of buffers, and intraoperative nerve monitoring, will be discussed.
Diseases of the Skeletal System
Authors: Friedman Tamir , Brown James E. , Quencer Keith B.
Published: October 2018

Musculoskeletal (MSK) interventions are a diverse group of procedures aimed at both therapeutic and diagnostic arenas for patient care and management. As the field evolves, MSK interventions are offering a minimally invasive approach as an alternative for open surgical procedures, such a biopsies of the axial and appendicular skeleton for diagnostic and treatment purposes, treatment of benign and malignant tumors or spinal interventions for pain or oncology therapies. However, the proceduralist must be adept with anatomy, devices and practices of referring specialties. This chapter will delve into the technical aspects of MSK procedures as well as recommendation and pitfalls of various procedures.
Interventional-Related Spine Anatomy
Authors: Ebani Edward Jack , Dean Kathryn , Tsiouris Apostolos John
Published: October 2018

This chapter on interventional-related spine anatomy provides a concise overview of normal spinal anatomy, as well as commonly encountered pathologic conditions, with a particular emphasis on the relevant imaging findings. The introduction outlines potential sources of back pain and their presenting symptomatology. The chapter reviews the main imaging modalities used to evaluate the spine and discusses their specific advantages and disadvantages. The anatomy of the muscles of the vertebral column, the vertebral column itself, and common variations), intervertebral ligaments and discs, vertebral joints, meninges and spinal cord, spinal nerves, and vasculature of the spinal column and spinal cord are reviewed. The discussion includes multiple radiographic, computed tomography (CT), magnetic resonance imaging (MRI), and angiographic images, as well as illustrations to supplement the text.
Median Neuropathy—Carpal Tunnel Syndrome
Authors: Lark Meghan E. , Fujihara Nasa , Chung Kevin C.
Published: October 2018

This chapter presents general treatment strategies for carpal tunnel syndrome using a clinical case example. It discusses assessment and planning, diagnostic pearls, decision-making, surgical procedures (open and endoscopic carpal tunnel release), management pearls, aftercare, complications and their management, and evidence and outcomes. Physical exams, such as Phalen’s test or a Tinel sign over the median nerve, are introduced, whereas steps in the surgical procedure are shown with intraoperative photographs. The chapter provides information on modern practices for comprehensive management of carpal tunnel syndrome from start to finish.
Median Neuropathy—Pronator Teres Syndrome and Anterior Interosseous Neuropathy
Authors: Payne Russell A. , Harbaugh Kimberly S.
Published: October 2018

Pronator teres syndrome results from median nerve compression or irritation at the elbow region. Patients typically note volar forearm pain and median sensory disturbance that includes the palm. Electrodiagnostic studies are helpful in excluding carpal tunnel syndrome and cervical radiculopathy, and findings may be normal in pronator syndrome. A lack of sensory findings and motor loss in flexion of the distal phalanx of the radial three digits suggests anterior interosseous nerve palsy, typically due to neuralgic amyotrophy. When conservative treatment fails, surgical release of all potential points of compression is successful in alleviating symptoms in the majority of patients with pronator syndrome.
Ulnar Neuropathy—Guyon’s Canal Syndrome
Authors: Varma Abhay K. , Cheng Ron Ron
Published: October 2018

The chapter describes ulnar nerve compression at the wrist secondary to a mass lesion. The clinical picture can mimic pathology of the nerve roots or the brachial plexus or ulnar nerve entrapment at the elbow. Meticulous clinical examination and electrodiagnostic study help to differentiate ulnar nerve compression at the wrist from radiculopathy and to localize the site of compression. Imaging of the wrist is essential, as compression by an organic lesion is common. Conservative management is recommended when organic pathology has been ruled out and there is no motor involvement. Surgery is indicated when conservative treatment fails, there is motor weakness, or a mass lesion has been identified.
Nerve Sheath Tumors—Schwannomas and Neurofibromas
Authors: Heinen Christian , Kretschmer Thomas
Published: October 2018

A benign peripheral nerve sheath tumor is illustrated in a case presentation of a painful mass in the medial thigh, with paresthesias radiating along the course of the saphenous nerve. The presenting features, appropriate workup, treatment timing, surgical strategies, follow-up, and results for nerve-associated masses are outlined. Specific imaging findings for peripheral nerve sheath tumors on contrast-enhanced MR imaging and the merits of high-resolution ultrasound are detailed. The typical features of a well-defined and noninvasive peripheral nerve tumor, the principles of exploration, and microsurgical enucleation technique are highlighted. Other nerve tumor entities that should be considered in the differential diagnosis, as well as their respective features, are discussed.
Adult Upper Trunk Brachial Plexus Injury
Authors: Pisapia Jared M. , Ali Zarina S. , Heuer Gregory G. , Zager Eric L.
Published: October 2018

This chapter takes a case-based approach to the diagnosis and management of adult brachial plexus injury involving the upper trunk. The clinical presentation and differential diagnosis associated with this injury pattern are reviewed, as well as the findings of electrodiagnostic and imaging studies. Preoperative considerations include the timing from initial injury and the difference between pre- and postganglionic injury. Options for nerve reconstruction include nerve grafting, nerve transfer, or a combination of both. The options are compared, and a detailed description of each surgical procedure is provided, along with related complications, alternative repair strategies, and outcomes.
Spinal Accessory Nerve Injury
Authors: Chan Kevin , Dihr Rishi , Fox Michael
Published: October 2018

Spinal accessory nerve (SAN) injuries can be idiopathic or iatrogenic. Providers who understand the essential anatomy of the SAN can direct the history, physical exam, and ancillary studies to localize the lesion, while considering the differential diagnosis. The differential diagnosis includes both traumatic and atraumatic causes, including penetrating or blunt trauma to the neck, fracture malunion, glenohumeral instability, brachial neuritis, progressive neuromuscular disease, and cerebrovascular accident. The chapter discusses the timing of, and indications for, operative exploration, with or without nerve repair, as well as the details of the surgical procedure. The authors provide instructive pearls for initial management, establishing patient expectations, and potential complications.
Iatrogenic Peripheral Nerve Injury
Authors: Heinen Christian , Kretschmer Thomas
Published: October 2018

Iatrogenic nerve lesions are frequently neglected. The chapter stresses the importance of adequate assessment, surgical timing, surgical strategies, follow-up, and results. Using the example of a radial nerve lesion in discontinuity due to osteosynthesis after humeral fracture, the authors describe a typical patient history with delayed presentation, as well as the role of physical examination, electrophysiology, and high-resolution ultrasound in demonstrating substantial nerve damage incompatible with spontaneous recovery. Surgical findings are demonstrated, along with a stepwise approach for nerve reconstruction via sural nerve graft. Clinical approach and surgery for traumatic radial nerve lesions are detailed, as well as general information on iatrogenic nerve lesions.
Conceptualizing Moral Resilience
Authors: Rushton Cynda Hylton
Published: October 2018

Moral resilience, the ability of an individual to preserve or restore integrity in response to moral adversity, draws on targeted scholarship of the broader concept of resilience in other contexts. This chapter builds on definitions in the literature and qualitative analysis of clinicians’ definitions of moral resilience in order to outline the key attributes of moral resilience. The foundation of moral resilience is personal and relational integrity. The attributes of self-regulation and self-awareness, such as mindfulness, buoyancy, moral efficacy, and self-stewardship, support the preservation or restoration of integrity. These attributes are defined and illustrated with quotes from clinicians. Taken together, these attributes constitute a conceptual basis for moral resilience.
Cultivating Essential Capacities for Moral Resilience
Authors: Rushton Cynda Hylton , Kaszniak Alfred W. , Halifax Roshi Joan S.
Published: October 2018

Developing the capacities that help clinicians recognize moral adversity and suffering in their daily work and efforts to support them to design and practice strategies that protect their integrity at the heart of clinical practice. These capacities include knowing fundamental values, cultivating mindful awareness and self-attunement, cultivating reflection and insight, developing moral and ethical efficacy, engaging in activities that support self-stewardship, and engaging in ongoing, transformational learning. Each of these capacities must be intentionally cultivated and practiced. Clinicians can begin wherever they are to explore each of them in a synergistic manner. They are enabled by a culture that encourages clinicians to regularly do the right thing for the right reason without fear of reprisal.
Afterword
Authors: Rushton Cynda Hylton
Published: October 2018

A new paradigm for addressing the consequences of moral adversity, especially when it produces moral suffering, is an urgent priority for the healthcare system. The time is now for clinicians, leaders, healthcare organizations, and communities to align their values and commitments and to design for sustainable and meaningful results. More empirical and scholarly work is needed to expand upon the concepts offered in this book. We must foster innovation that sources our inner capacity, shifts patterns that no longer serve a useful purpose, and creates new methods and programs to cultivate moral resilience and a robust and lasting culture that supports ethical practice. Together we hold the keys to transforming moral suffering in healthcare.
A History of the National Cancer Institute’s Support for Implementation Science Across the Cancer Control Continuum
Authors: Kerner Jon , Glasgow Russell E. , Vinson Cynthia A.
Published: October 2018

After a 20-year investment in growing cancer prevention and control intervention research, implementation science activities at the National Cancer Institute (NCI) were initiated from a recognition that most scientific and health intervention research findings were not being translated into programs and policies benefitting their intended audiences. Initial efforts focused on enhancing the dissemination and diffusion of evidence-based programs and policies combined with small investments in supplementing intervention research grants with dissemination and diffusion awards to build intervention researcher experience with implementation science. The focus later shifted to a greater emphasis supporting the implementation science. Throughout its history, the NCI implementation science approach has involved strategic partnerships and multilevel perspectives, along with strong stakeholder engagement. NCI has been and continues to be an important leader in and funder of implementation science, and current and future efforts should build upon this important foundation and infrastructure.
Evidence-Based Cancer Practices, Programs, and Interventions
Authors: Fernandez Maria E. , Mullen Patricia Dolan , Leeman Jennifer , Walker Timothy J. , Escoffery Cam
Published: October 2018

There are many evidence-based interventions, cancer control practices, programs, treatments, and clinical practice guidelines across cancer control and prevention topic areas that have great potential for decreasing the cancer burden. Nevertheless, challenges in identifying evidence-based interventions (EBIs) that match the needs of community and practice settings, adapting EBIs for new populations and contexts, and implementing EBIs in real-world settings limit the public health impact of cancer control research and its products. This chapter provides an introduction to existing EBIs for cancer control and provides examples of different types of EBIs across the cancer continuum. It highlights issues related to the identification of EBIs, including the evaluation of EBI resources. It also describes processes that can be used to enhance the development, adaptation, and implementation of evidence-based cancer control interventions.
Cancer Prevention and Public Health Promotion
Authors: Klesges Lisa M.
Published: October 2018

In this chapter, four case studies offer practical examples of implementation approaches that can accelerate evidence-based cancer prevention. The applied knowledge from the cases adds to the understanding that culture, context, politics, and partnership are key elements in driving health improvement, and although not always well understood or easily measured, they are a reminder that cancer prevention and care delivery exist within a complex system. In considering transformations in health care, moving from a linear and deconstructed model of delivery to consider complex adaptive models that could drive better outcomes was key to improved outcomes. Similarly, understanding context and complexity is a key consideration for implementing cancer prevention and control interventions into existing multisector social systems, be they health care, community, or statewide systems.
Cancer Prevention Through Scaling-Up the Process of Evidence-Based Decision-Making in a State Health Department
Authors: Allen Peg M. , Ahrendt Linda J. , Hump Kiley A. , Brownson Ross C.
Published: October 2018

This case study provides an example of a collaboration between a university and a public health agency to build organizational capacity to spread data-driven decision-making, implementation, and evaluation of evidence-based cancer prevention strategies. The Office of Chronic Disease Prevention and Health Promotion at the South Dakota Department of Health provided the key management practices for scale-up of evidence-based decision-making (EBDM): leadership support, training, a supportive organizational climate and culture, inclusion of partners, and outcomes-based contracting with partnering organizations. A pre–post survey showed increased use of research evidence for several job tasks, including selection of interventions and evaluation. Perceived work unit access to skills in prioritization and adapting interventions also increased. The 16 staff and partners interviewed perceived leadership support, federal funding requirements, and an initial multi-day training as the key facilitators for spreading EBDM.
Cancer Detection and Screening
Authors: Coronado Gloria D.
Published: October 2018

Lung and colorectal cancers account for high numbers of preventable deaths. Because of this, scaling up effective interventions to increase routine screening and lower tobacco use is critically important. Screening programs for these diseases vary greatly in their anticipated outcomes. Colorectal cancer screening can both prevent colorectal cancer and identify it in early, treatable stages. Screening for lung cancer, on the other hand, cannot prevent most lung cancer-related deaths, and up to 80% of deaths could be averted from smoking cessation. In this complicated environment for ongoing refinement of screening programs, the two case studies presented in this chapter showcase promising interventions for addressing the troubling high rates of mortality from lung and colorectal cancers. They underscore the value of designing experiments considering long-term implementation, aligning the intervention with existing clinic workflows and processes and incorporating end user feedback.
Implementation of an Evidence-Based Tobacco Use Treatment Intervention in the Context of Lung Cancer Screening
Authors: Ostroff Jamie S. , Shelley Donna
Published: October 2018

Lung cancer screening using low-dose helical computed tomography is now recommended for early detection of lung cancer. This case study provides an overview of a study that is testing the effectiveness of tobacco treatment interventions for high-risk smokers seeking lung cancer screening and examining factors that may influence implementation process and sustainability for delivering effective models of smoking cessation treatment in lung cancer screening settings. The focus of the case study is a description of how and why two implementation frameworks were applied to evaluate the implementation outcomes and additional multilevel factors (i.e., organization and intervention characteristics) that may influence effective implementation of evidence-based tobacco use treatment interventions in the context of lung cancer screening. Ultimately, implementation of high-quality tobacco treatment in lung cancer screening settings is likely to further reduce tobacco-related cancer morbidity and mortality.
Provider-Level Factors Influencing Implementation
Authors: Krist Alex H. , Jiang Vivian
Published: October 2018

Cancer treatment is increasingly complex. The tools for diagnosis, staging, and predicting prognosis are rapidly evolving, as are the therapies, treatment modalities, and treatment protocols. The complexity of care, the need for a multidisciplinary team across settings, and patient-level factors all present providers with a unique set of challenges. The three case studies presented in this chapter explore strategies that help providers by (1) ensuring low-income patients with breast cancer receive care consistent with guidelines through patient engagement and navigation, (2) promoting and incorporating the routine use of shared decision-making in determining prostate cancer treatment, and (3) supporting the adoption of concurrent palliative care for patients with advanced cancer. The specific challenges and needs for future implementation science are highlighted throughout each case.
Integrating Patient-Reported Outcomes into Routine Cancer Care Delivery
Authors: Stover Angela M.
Published: October 2018

This case study describes a cancer hospital’s experience with implementing symptom questionnaires into routine care delivery. Implementing symptom questionnaires (patient-reported outcome measures [PROs]) into cancer care delivery is a useful case study for implementation science because there is ample evidence for effectiveness in improving care but stalled implementation throughout most US health care systems. No “turnkey” PRO implementation strategies exist; thus, each clinic or health care system has to make many complex implementation decisions on its own. The Organizational Model of Innovation Implementation and the Theoretical Domains Framework are used to illustrate the implementation planning process and barriers and facilitators encountered.
Patient Navigation and Cancer Care Delivery
Authors: Freeman Harold P. , Simon Melissa A.
Published: October 2018

Although the US health care system offers the very best care to many, the poor and uninsured typically face challenges in accessing timely health care, even when faced with a life-threatening disease such as cancer. Spurred by unmet patient needs and the growing complexity of health care delivery systems, patient navigation seeks to diminish social, economic, cultural, and medical system barriers to timely quality care. This case study discusses the emergence of patient navigation as a strategy for improving cancer outcomes, especially among vulnerable populations. It explores challenges and opportunities related to advancing successful implementation of patient navigation across the cancer care continuum. It seeks to harness and apply the power and energy of patient navigators with the goal of guiding individuals across the health care continuum—from the communities where they live all the way through screening, diagnosis, and treatment at clinical care sites.
Implementing Lynch Syndrome Screening in the Veterans Health Administration
Authors: Scheuner Maren T. , Russell Marcia , Peredo Jane , Hamilton Alison B. , Yano Elizabeth M.
Published: October 2018

Lynch syndrome (LS) is the most common hereditary colorectal cancer (CRC) syndrome. Diagnosis of LS has important clinical implications for CRC patients and their family members. LS screening in tumor tissue is possible, and screen-positive cases are referred for diagnostic testing. This case study describes how implementation science informed a population-based LS screening program in the Veterans Health Administration (VHA), the largest integrated health care delivery system in the United States. Successful implementation strategies relied on the organizational structures and processes characteristic of integrated health care systems, including data warehousing methods that leverage the electronic health record, case management, and centralized technical assistance. Challenges to sustainability of the population-based program include low prevalence of LS among veterans, limited expertise, organizational changes, and the rapidly evolving field of precision oncology. LS screening is an exemplar case study for implementation science in integrated health care delivery systems.
Sustainability of Cancer Practices and Programs
Authors: Stirman Shannon Wiltsey , Dearing James W.
Published: October 2018

Sustainability is the continued use of program components and activities for the continued achievement of desirable program and population outcomes. Research on sustainability suggests that for some interventions and programs, the potential for long-term sustainment is fairly high, whereas for those that are complex and multifaceted, sustainability is more precarious and may always be at risk. This chapter considers broad issues of sustainability outcomes, frameworks for conceptualizing sustainability, and the depth or meaningfulness of what is sustained. It reviews research on the sustainability of cancer practices and programs in both public health and health care settings. Without careful planning and attention to relevant drivers of sustainability, practices and programs may not be continued over the long term. Still, the broader literature does provide guidance for those who seek to implement cancer programs and interventions with an eye toward sustainment. This guidance is discussed at the conclusion of the chapter.
Cost-Effectiveness Analysis in Implementation Science
Authors: Gold Heather Taffet
Published: October 2018

Cost-effectiveness analysis is a tool used to systematically and quantitatively compare trade-offs between health outcomes and costs of alternative health care interventions with standards set for the United States. Many recommendations, however, may not coalesce with implementation science methods. There is a lack of consensus for economic evaluation in implementation science that has resulted in conflicting norms and conventions, which in turn make analyses difficult to compare, raise quality concerns, and may put the relevance of research into question. This chapter suggests new standards as areas for future research to improve the quality, rigor, transparency, and ultimately comparability of cost-effectiveness analyses in implementation science.
Upper Respiratory Infection
Authors: Wayman Kenneth , Samol Nancy B. , Wittkugel Eric
Published: October 2018

The child with an upper respiratory tract infection presenting for surgery is probably the most common dilemma that faces the pediatric anesthesiologist. While cancellation of such a child’s operation had been a common practice in the past, nowadays, an anesthesiologist will more than likely proceed with the anesthetic management of a child with a mild common cold. Research has shown that while perioperative respiratory adverse events are likely to occur in a child with a mild cold, these events are very easily managed. In addition, the use of a laryngeal mask airway which prevents instrumentation of a child’s airway drastically decreases the incidence of perioperative adverse events in this patient population. Planned airway surgery, history of prematurity, reactive airway disease, and passive smoking in the home are factors that increase the incidence of perioperative adverse respiratory events in a child with an active cold.
Malignant Hyperthermia
Authors: Williams Erin S.
Published: October 2018

Malignant hyperthermia (MH) is one of the most feared syndromes in anesthesiology. Due to its rarity and adequate precautions instituted for susceptible patients, most anesthesiologist have never been involved in a case of MH. However, the lack of familiarity with the early signs of MH and quick and effective treatment can increase the potential for increased morbidity and mortality. MH involves a derangement at the molecular level at the ryanodine R1 receptor. This leads to abnormally high levels of intracellular calcium and extracellular potassium. These biochemical mechanisms lead to a hypermetabolic syndrome that if not treated early will prove deadly.
Anaphylaxis
Authors: Gordon Diane , Sjoblom Matthew D. , Aronson Lori A.
Published: October 2018

Anaphylaxis is an acute multisystem process resulting from allergic or nonallergic reactions to a foreign substance in the body. Regardless of trigger or pathophysiology, the mainstay of treatment is epinephrine. Common triggers of anaphylaxis in children undergoing anesthesia are latex, neuromuscular blocking agents, and antibiotics. Risk factors for latex sensitivity include frequent catheterization of the urogenital tract, atopic conditions, and mastocytosis. Presentation of anaphylaxis during anesthesia can be subtle or sudden, and cutaneous signs may be obscured by surgical drapes. A high index of suspicion facilitates prompt treatment with epinephrine, volume replacement, and airway management. Histamine and tryptase blood tests collected during the event can confirm the diagnosis acutely. Allergy consultation and skin testing may determine the causative agent.
Foreign Body in the Airway
Authors: Chidambaran Vidya , Sadhasivam Senthilkumar
Published: October 2018

Anesthetic management of suspected foreign body aspiration in the airway can be challenging. It is critical to develop a coordinated plan with the surgeon. Removal of a foreign body may necessitate laryngoscopy, bronchoscopy (commonly used), thoracoscopy, thoracotomy, or even a tracheotomy. Anesthesia could be induced using inhalation or intravenous anesthetics, while maintaining spontaneous ventilation. However, there is no consensus as to whether controlled or spontaneous ventilation is more advantageous. Maintaining deep planes of anesthesia, with minimal airway reflexes, during bronchoscopy is essential. In the event that total airway obstruction due to a tracheal foreign body occurs, a potentially life-saving technique is to push the object deeper into one of the main bronchi, for temporary relief. Postoperatively, steroids, racemic epinephrine, and intubation/ventilation may be necessary for airway edema. A chest x-ray may be indicated to rule out postobstructive pulmonary edema, pneumothorax, and pneumonia.
Laryngospasm
Authors: Schakett Brent , Chen Kathleen
Published: October 2018

Laryngospasm is a complication that all pediatric anesthesia providers must be able to successfully diagnosis and treat. The risk factors include but are not limited to recent upper respiratory infection, history of asthma, preschool-age child, airway surgery, and light anesthesia. Laryngospasm can be defined as either partial laryngospasm with residual opening of the glottis or complete laryngospasm where there is no air movement. Prevention is obtained by limiting risk factors; waiting 6 to 8 weeks after upper respiratory infection symptoms have resolved if possible, smoking cessation, suctioning of residual secretions, and maintaining an adequate depth of anesthesia during crucial times like intubation and extubation. Treatment includes jaw thrust with positive pressure and 100% oxygen, followed by a subhypnotic dose of propofol if the laryngospasm does not break, then finally succinylcholine if all other methods have failed. With treatments that depress respiratory drive, delirium can result and must be diagnosed correctly.
Massive Transfusion in a Child
Authors: Chapman Rachel , Sabato Stefano
Published: October 2018

Massive transfusion in a child is likely to occur in cases of trauma or during surgeries that are at risk for severe blood loss such as liver transplantation and craniofacial procedures. It may also occur when least expected, if inadvertent injury to a vascular structure occurs during surgery. Ability to enlist assistance with administration of the various blood products required and also with checking frequent laboratory results will facilitate the process. Knowledge of the different factors that rapidly become depleted as well as lab values that need to be closely monitored is necessary to avoid further complications during massive blood transfusion.
Hemophilia
Authors: McIntyre Rebecca
Published: October 2018

Patients with hemophilia can be safely managed for elective surgery such as adenotonsillectomy as long as adequate planning and consultation with the hematology department occurs. Adequate factor replacement, antifibrinolytics, and careful monitoring are the mainstays of care. The need for frequent venous access can make cannulation of veins difficult, and this can be stressful for the patient and his or her family. Careful planning of any cannulation attempts can alleviate stress, increase success rate, and reduce trauma to veins. If the patient has a long-term central venous line, all precautions to reduce the risk of infection and thrombosis in the line must be adhered to. Development of inhibitory antibodies to factor VIII, or more rarely to factor IX, is currently the most difficult complication to manage in hemophilia patients.
Congenital Diaphragmatic Hernia Repair
Authors: Mavi Jagroop , Boat Anne C. , Sadhasivam Senthilkumar , Seipel Catherine P.
Published: October 2018

Congenital diaphragmatic hernia is an embryologic defect in diaphragm formation that allows abdominal contents to enter into the fetal pleural cavity, resulting in ipsilateral lung compression, pulmonary hypoplasia, and abnormal pulmonary vasculature. Though diagnosis is frequently made on prenatal imaging, the diagnosis should be considered in any newborn with respiratory distress. Prenatal predictors of defect severity include evaluation of observed-to-expected lung volumes on fetal magnetic resonance imaging and lung-to-head ratio on fetal ultrasound. Treatment focuses on medical stabilization, including optimization of oxygenation and ventilation, followed by surgical repair. Anesthetic considerations for these patients include management of coexisting cardiac disease and ventilatory parameters, in addition to standard neonatal anesthetic considerations.
Mucopolysaccharidoses
Authors: Seipel Catherine P. , Aina Titilopemi A. O.
Published: October 2018

Mucopolysaccharidoses are progressive disease processes characterized by deficiencies in lysosomal enzymes required for catabolism of glycosaminoglycans. This leads to the accumulation of glycosaminoglycans (GAGs) in multiple organs and tissue. In particular, the deposition of GAGs in soft tissue, the central nervous system, and the cervical spine have implications for the anesthetic management of these patients. A detailed history and examination, with a focus on cardiopulmonary status and past airway management, is required pre-operatively. Enzyme replacement therapy and, in select cases, hematopoietic stem cell transplantation may modify disease progression. This chapter illustrates the perioperative considerations necessary to care for patients with this uncommon disease.
Epidermolysis Bullosa
Authors: Nguyen Kim-Phuong
Published: October 2018

The anesthetic management of children with epidermolysis bullosa (EB) presents unique challenges to the pediatric anesthesiologist. Preoperative planning includes anticipation of a potentially difficult airway, focused protection of fragile skin and mucous membranes, and special consideration in the placement of standard monitors and intravenous access. Additionally, this chapter highlights the natural history and common procedures that may be performed in a child with EB; this will help prepare the anesthesiologist and the patient for a smooth anesthetic course. This chapter presents the case study of a 10-year-old girl with a medical history of recessive dystrophic EB, esophageal strictures, and poor nutritional status, who presents for esophageal dilation and percutaneous endoscopic gastrostomy tube placement.
Omphalocele and Beckwith-Wiedemann Syndrome
Authors: Dachsangvorn Pikulkaew
Published: October 2018

This chapter discusses omphalocele, which is a neonatal abdominal wall defect through which abdominal visceral content herniates. Although omphalocele is similar in gross appearance to gastroschisis, it is more frequently associated with chromosomal abnormalities such as trisomies 13, 15, 18, and 21, and syndromes such as Beckwith-Wiedemann, Rieger’s, and prune belly syndrome. It is also more frequently associated with anomalies in other organ systems. Management of infants with Beckwith-Wiedemann syndrome and omphalocele can be challenging due to the concurrent risk of difficult airway and aspiration. Associated comorbidities also need to be addressed to avoid secondary insults in the perioperative period.
Hypoplastic Left Heart Syndrome
Authors: Navaratnam M. , Ramamoorthy C.
Published: October 2018

Approximately 960 babies are born per year in the United States with hypoplastic left heart syndrome. Over the last 20 years, advances in surgical techniques, perioperative care, cardiopulmonary bypass, and intensive care unit management have converted this previously fatal condition to one with a neonatal survival rate of 90% to 92% for standard risk patients. Understanding the factors affecting the balance of pulmonary blood flow and systemic blood flow and ensuring adequate cardiac output and end-organ perfusion is critical to successful outcomes. Extracorporeal membrane oxygenation remains an important support modality following stage I palliation. This chapter discusses this syndrome and describes treatment options.
Neuroblastoma
Authors: Brown Christina , Kato Meredith
Published: October 2018

Neuroblastoma is a solid tumor that arises from neural crest cells. It is a common cancer of childhood, accounting for 7.8% of all childhood malignancy. It affects primarily infants and toddlers with older children carrying a worse prognosis. While surgery is a primary treatment modality, these tumors are not well encapsulated and tend to invade adjacent structures making resection more difficult. As such, patients often come to the operating room after neoadjuvant chemotherapy and the surgeries can be long with large blood losses. Anesthesia for the resection for neuroblastoma must take into account the effect of ongoing treatment on the patients as well as the surgical challenges inherent in this disease.
Anesthetic Management for Patients with Cerebral Palsy
Authors: Koh J. , Aliason I.
Published: October 2018

Cerebral palsy is a disorder of muscle tone and often of movement, most commonly due to prenatal damage of the immature brain. It is a diagnosis that encompasses a wide range of symptoms, many of which require surgical intervention. Anesthesiologists regularly care for patients with cerebral palsy as they undergo a variety of orthopedic, dental, neuro, and general surgeries. To provide the best possible care for patients with cerebral palsy, anesthesiologists need to understand the commonly associated comorbidities, be able to assess each individual patient’s cognitive and communication abilities, and understand the challenges of perioperative care in this special population
Anesthetic Management of Pediatric Craniopharyngioma
Authors: King Adele , McKee Christopher
Published: October 2018

The challenging perioperative management of craniopharyngiomas requires a multidisciplinary team approach. Though histologically benign, craniopharyngiomas are aggressive with local invasion and frequent recurrences. Significant morbidity maybe there at presentation. Treatment can worsen. Common perioperative concerns include hypothalamic dysfunction, tumor size and its effects on intracranial pressure, as well as the close proximity to nearby vessels and neural structures. Optimal anesthetic management includes thorough preoperative evaluation and planning with detailed knowledge of the tumor extent and symptomology. Management of hypothalamo-pituitary dysfunction plays a major role in achieving a successful outcome and should feature collaborative input from the endocrinology service.
Perioperative Management of Diabetes Mellitus Type 1 and 2
Authors: Raman Vidya T.
Published: October 2018

Diabetes management offers unique challenges in children and adolescents versus adults especially in the perioperative environment. The obvious challenges of monitoring dietary intake plus possible communication barriers with increased risk of diabetic ketoacidosis and hypoglycemia. Adding the catabolic stressors from surgery also add challenges to the perioperative physician managing the patient’s glycemic control. It is important to work with endocrinology in order to manage their diabetes. Lengthier procedures also complicate glycemic control. It involves sometimes close monitoring of not only glucose but electrolytes and blood and urine ketones.
Mitochondrial Disease
Authors: D’Mello Ajay
Published: October 2018

Mitochondrial disease is now considered to be an important cause for a diverse range of neurological, muscular, cardiac and endocrine disorders. Initially thought to be a rare group of disorders, it is now increasingly common for children with mitochondrial disease to present for a surgical procedure. While the mitochondrial respiratory chain is the essential finally common pathway for aerobic metabolism, mitochondria also play a role in a several important cellular processes. A variety of anesthetic techniques have been successfully used for this group of patients. However, the possibility of complications due to inhibition of mitochondrial function by anesthetic agents and surgical stress is a worry for the physician managing these patients. Anesthetic management focuses on disease symptoms at presentation, maintaining normoglycemia, while preventing further metabolic stress and complications that worsen lactic acidosis.
Mucopolysaccharidoses
Authors: Smith Ashley
Published: October 2018

Mucopolysaccharidoses (MPS) are a group of genetic diseases that affect connective tissues via lack of key lysosomal enzymes. This deficiency leads to storage of partially degraded glycosaminoglycans that build up in multiple organ systems. There are many types of MPS disorders and each has varying features, expected lifespan, enzyme affected, and resulting clinical effect. All MPS are autosomal recessive except Hunter syndrome, which is X-linked. Because each type has a different enzyme deficiency, each has different comorbidities and unique treatment modalities. These patients require multiple anesthetics for diagnostic and surgical interventions. This chapter discusses treatment ad complication issues regarding MPS.
Anesthetic Management of Anterior Mediastinal Masses in Children
Authors: Trifa Mehdi , Burrier Candice
Published: October 2018

The management of children presenting with an anterior mediastinal mass (AMM) is challenging for anesthesiologists. AMMs are a heterogeneous collection of primary or secondary, benign or malignant tumors. Severe and life-threatening complications related to airway obstruction and/or cardiovascular compression can occur in a patient with an AMM during anesthesia, even in an asymptomatic patient. It is important for the anesthesia provider to understand the pathophysiology of symptoms and complications and the current evidence regarding perioperative management of children with AMM. This chapter explores the pathophysiology of AMM symptoms and of AAM and anesthesia. Perioperative management of AMM patients, including preoperative evaluation and induction and maintenance of anesthesia, is also discussed.
Goldenhar Syndrome
Authors: Soleta Amy , Karlik Joelle
Published: October 2018

Goldenhar syndrome (also known as oculo-auriculo-vertebral spectrum, facio-auriculo-vertebral syndrome, and Goldenhar-Gorlin syndrome) is caused by fetal growth disturbances of the first two brachial clefts. Diagnostic criteria include eye, ear, mandibular, and/or vertebral anomalies. These patients may also have cardiac and renal malformations with varying degrees of severity. Airway management for Goldenhar patients may include difficult ventilation and intubation, which may become increasingly difficult with age. Vertebral anomalies including fused cervical vertebrae and/or cervical instability further complicate airway management. Pulmonary complications occur due to congenital malformations and scoliosis, which can lead to thoracic insufficiency syndrome. This chapter discusses genetics, presentation, and management of Goldenhar syndrome.
Perioperative Care of Patients with Epidermolysis Bullosa
Authors: Burgart Alyssa M. , Furukawa Louise K.
Published: October 2018

Inherited epidermolysis bullosa comprises a group of rare, serious conditions affecting multiple organ systems. While generally referred to as a dermatologic condition, any organ with an epithelial surface is affected, leading to serious extracutaneous manifestations. These have wide-ranging implications for anesthetic management, including airway management, vascular access, skin protection, and effective pain control. A successful and safe patient experience requires thoughtful evaluation, communication with a multidisciplinary team, anesthetic planning, intraoperative management, and postoperative care. The scenario in this chapter takes readers through a series of questions to enhance their understanding of the disease and how to address common anesthetic problems. Particular attention is paid to suggested monitor modification.
Ethics in Pediatric Anesthesiology
Authors: Shah Monica , Waisel David
Published: October 2018

Ethical principles affect daily decision-making in pediatric anesthesiology. These medical decisions are interlaced with the ethical components of informed consent and obligations to the child and family. Informed consent in pediatrics includes the concepts of best interest, in which the parents or other surrogate decision-makers choose acceptable treatment for the child, and assent, which enables children to participate in decision-making to the best of their ability. Of equal significance to informed consent, the process of informed refusal requires anesthesiologists to more fully inform children and their guardians about risks and benefits while respecting refusal of assent and avoiding coercion. Pediatric considerations regarding end-of-life therapy are slightly different than adult considerations. To help resolve these ethical dilemmas, ethics committees are available for consultations to assist the medical team, family members, and patients in order to make the best decision for the child.
Pineal Tumors
Authors: Felbaum Daniel R. , Sherman Jonathan H. , Jean Walter C.
Published: October 2018

Pineal region tumors can include a variety of histologies including pineal parenchymal tumor, germ cell tumor, glial tumor, metastasis and meningioma. The workup for pineal region tumors includes standard magnetic resonance imaging for anatomic imaging, as well as cerebrospinal fluid markers to assess for certain germ cell tumors. Cerebrospinal fluid diversion may be necessary if patients present with hydrocephalus. If surgical resection is indicated based on the suspected diagnosis, magnetic resonance venogram is an important study that influences the surgical trajectory. This chapter reviews common pineal region tumors in the setting of a case presentation. Management strategies and surgical approaches are also discussed in this chapter. Pearls for how to select the surgical approach and complication avoidance are also presented.
Ventricular Tumors
Authors: Majmundar Neil , Liu James K.
Published: October 2018

Central neurocytomas are rare benign tumors that are typically located in the lateral ventricles. Because they are typically intraventricular, these tumors tend to present clinically with hydrocephalus. Currently, surgical removal with a gross-total resection is the treatment of choice. Various radiotherapy techniques, including both conventional radiotherapy and stereotactic radiosurgery, have been shown to be useful in cases of residual tumor after subtotal resection and tumor recurrence. This chapter presents a clinical case of central neurocytoma that demonstrates the typical clinical and radiological findings, as well as the diagnostic workup and surgical management of these tumors.