bluuebunny/crossref_metadata_2025 huggingface dataset:
Disclosure after Complication in the Operating Room
Authors: Young David A.
Published: October 2018

Patients and families overwhelmingly want to be informed regarding matters related to poor outcomes and medical errors. After the occurrence of a medical error, most patients and families highly value an honest and transparent disclosure of the details as well as a sincere apology from the parties responsible regardless of the degree of apparent harm. When disclosing a medical error to a parent or patient, an organized approach using a truthful and compassionate discussion as the backbone is the most prudent strategy. Additionally, effective disclosure practices can decrease legal liability. This chapter discusses the differences between medical errors and poor clinical outcomes, the distinctions between near misses and sentinel events, the indications for disclosure of medical errors without apparent harm as well as the appropriate use of apology, and an approach for the effective disclosure of a pediatric medical error to a parent.
Managing Blood Loss in a Jehovah’s Witness Patient
Authors: Dalton Michelle
Published: October 2018

The Jehovah’s Witnesses are a unique group of patients that can pose a challenge to the anesthesiologist given their desire to avoid transfusion of blood products. This religious belief can include refusal of red cells, white cells, plasma, as well as platelets. The perioperative care of such patients can include the use of bloodless techniques in order to respect their beliefs. Given the desire to avoid blood products, it is imperative to utilize strategies that will prevent unnecessary transfusion. It is also important to understand the ethical and or legal ramifications of overriding parental/adolescent requests for no transfusion as well as acceptance of adolescent transfusion refusal.
Organ Donation after Cardiac Death in the Pediatric Patient
Authors: Sutton Caitlin D. , Mann David G.
Published: October 2018

The need for organ transplantation is ever increasing. Currently there are 115,000 people on the waitlist and the number is still growing. Organs that are transplanted may be obtained via a living or deceased donor. The organs may be obtained from a deceased donor after either brain death or after cardiac death. The majority of deceased donor organ transplants occur via deceased donor after brain death; however, deceased donor after cardiac death organ donation is increasing. This concept of organ transplantation can be quite difficult to discuss with families, therefore, the anesthesiologist and the entire care team must be knowledgeable and respectful regarding the patient’s and families wishes. The team should also be familiar with the overall process and organ procurement protocols of the institution. By having respectful, thoughtful, early discussions regarding the potential for organ donation, families will be able to make better informed decisions.
Charge Syndrome
Authors: Mercado Pilar , Eklund Jamey E. , Anderson Jennifer L.
Published: October 2018

The major diagnostic features of CHARGE syndrome include coloboma of the eyes, choanal atresia or stenosis, distinctive external ears, cranial nerve abnormalities, and absent or small semicircular canals. The mnemonic refers to coloboma of the eye, heart defects, atresia of choanae, retardation of growth and development, cenitalia hypoplasia, and ear abnormalities and deafness. There is no defined etiology, though a de novo mutation on the CHD 7 gene located on Chromosome 8 is responsible for more than 50% of CHARGE cases. The incidence of CHARGE is about 1:10,000 live births with an equal distribution between males and females. The anesthetic implications of this syndrome are many and vary with the patient’s phenotype. A potential difficult airway, congenital heart defects, choanal atresia, and cranial nerve abnormalities present the most significant issues for the anesthesiologist. A multidisciplinary team must be established early to properly care for these complex patients.
Ventricular Septal Defect
Authors: Serfass Evan R. , Ramos Justin D.
Published: October 2018

Ventricular septal defect (VSD) is the most common congenital cardiac lesion, and VSDs are found as isolated lesions in up to 20% of children with congenital heart disease. The natural history and pathophysiology of VSD varies by patient age, patient size, anatomic location, and size of the defect. Patients who have large lesions and significant left-to-right shunt resulting in heart failure symptoms, failure to thrive, pulmonary hypertension, or recurrent respiratory infections may be indicated for early surgical repair during infancy. This chapter presents a clinical scenario of a symptomatic infant undergoing primary surgical repair of a VSD to demonstrate principles of the anatomy, pathophysiology, diagnosis, and medical management of patients with VSDs. Anesthetic management is also discussed, considering the effects of left-to-right shunt, pulmonary hypertension, delayed sternal closure, and Eisenmenger’s syndrome.
Tetralogy of Fallot
Authors: Lilien Matthew , Sharma Anshuman
Published: October 2018

[Abstract]
Cardiomyopathy and Heart Failure
Authors: Kamra Komal , Williams Glyn D.
Published: October 2018

Congenital heart disease and cardiomyopathy are common causes of heart failure in children. Among cardiomyopathies, dilated cardiomyopathy is the most common cause of heart transplant in children. These patients with end-stage heart failure secondary to cardiomyopathy present for multiple interventions requiring anesthesia and may be challenging to manage because of their hemodynamic instability. Thoughtful, collaborative planning and execution of perioperative care is recommended to optimize patient outcomes. This includes good understanding of the patient’s clinical status and the relevant cardiac pathophysiology. Also, an appreciation of the implications of heart failure therapies and the invasive procedures for which the patient requires anesthesia care.
Pediatric Heart Transplantation
Authors: Burbano-Vera Nelson , Schure Annette Y.
Published: October 2018

Pediatric heart transplantation is a well-established treatment for complex congenital heart defects or severe heart failure in children of all ages. Sophisticated bridging therapies (ventricular assist devices, home inotropic support, etc.) and improved survival rates have increased the likelihood for pediatric anesthesiologists to encounter patients on the waiting list or at various times after heart transplantation, not only in the cardiac catheterization suite or the operating rooms but also for other diagnostic studies or noncardiac surgery. A thorough understanding of indications, altered physiology, and specific challenges is essential for the safe anesthetic management of these patients. This chapter highlights important steps during the preoperative evaluation, typical intraoperative problems, and management strategies for pediatric heart transplant patients.
Transcatheter Closure of Atrial Septal Defects
Authors: Nguyen Khoa , Callahan Patrick
Published: October 2018

The term congenital heart disease encompasses a vast array of lesions that present unique anesthetic challenges. Making up close to 10% of all congenital heart disease, atrial septal defects are some of the more commonly encountered congenital lesions. Atrial chambers in the heart are separated by a septum that forms during embryological development. When the septum does not develop normally, blood communicates between the right and left atria. This alteration in flow has significant effects on both cardiac and pulmonary anatomy and physiology. Cardiothoracic surgery used to be the only way to close defects that did not spontaneously close. Transcatheeter device closure of atrial septal defects in the cardiac catheterization lab has become increasingly common and offers significant advantages over open heart surgery. This chapter highlights the anatomic and physiologic considerations of the different types of atrial septal defects and discusses the details of transcatheter closure including indications, timing, and risks.
Congenital Pulmonary Airway Malformation
Authors: Lazar Alina
Published: October 2018

Respiratory distress in infants may be caused by perinatal events and physiologic changes (e.g., lung immaturity, meconium aspiration, and persistent pulmonary hypertension); infectious processes; cardiovascular, neurologic, and metabolic abnormalities; as well as congenital lung abnormalities. Some of these may coexist, further complicating the diagnosis, clinical course, and management of the affected infant. Sound anesthetic management of congenital lung abnormalities requires a clear understanding of the pathophysiology of lung lesions and, in particular, the consequences of positive-pressure ventilation in patients with cystic and emphysematous lesions. Also critical is an appreciation for the physiologic differences in children undergoing thoracic surgery, indications for one-lung ventilation, age-appropriate lung isolation techniques, potential respiratory and cardiovascular complications that may occur during pediatric thoracic surgery, and the optimal choices for postoperative analgesia.
Congenital Diaphragmatic Hernia
Authors: Khan Sabina A. , Wadhwa Nitin
Published: October 2018

Congenital diaphragmatic hernia (CDH) is characterized by malformation of the diaphragm, allowing for herniation of abdominal contents into the thoracic cavity. The most significant sequelae of this herniation are pulmonary hypoplasia and pulmonary hypertension, both contributing to significant morbidity and mortality. Multiple strategies exist to minimize respiratory compromise and improve outcome in a patient with CDH, including fetal intervention in selective cases, medical and pharmaceutical management, advanced ventilation strategies, extracorporeal membrane oxygenation (ECMO), and complete surgical repair. Veno-arterial ECMO (circuit between the internal jugular vein and the carotid artery) is used in infants who are unstable and require aggressive cardiopulmonary support, and veno-venous ECMO (circuit with a double lumen catheter in the internal jugular vein) is used in infants who only need respiratory support.
Pectus Excavatum
Authors: Dalal Priti G. , Whitley Meghan
Published: October 2018

Pectus excavatum is a funnel-shaped congenital deformity of the chest. Although the deformity can appear minimal at birth, it may be progressive. There may be cardiac or pulmonary compromise in addition to subjective complaints of pain and shortness of breath. Management ranges from breathing exercises to surgical repair with mobilization of the sternum and ribs. This can be performed using an open or thoracoscopic technique. Complications of surgical repair include atelectasis and pneumothorax. Significant pain is associated with the surgical procedures and multimodal analgesic therapy, including thoracic epidural analgesia and intravenous narcotics, are typically used. This chapter discusses the etiology and management of pectus excavatum.
Craniosynostosis and Apert Syndrome
Authors: Meier Petra M. , Erb Thomas O.
Published: October 2018

Apert syndrome is a complex, progressive multisystem condition of the craniosynostosis spectrum originating from a fibroblast growth factor receptor disorder. Multidisciplinary treatment teams may include craniofacial surgery, neurosurgery, otolaryngology, ophthalmology, oro-maxillofacial surgery, and pediatric intensive care. Secondary to midface hypoplasia, children often present with a compromised airway and have a high incidence of sleep disorders. Anesthetic considerations include difficult airway assessment, the presence of obstructive sleep apnea syndrome, and increased intracranial pressure. Extensive remodeling procedures can be associated with massive hemorrhage (e.g., venous sinus bleeding) and venous air embolism. Transfusion-related complications include coagulopathy, metabolic derangements, and primarily noninfectious hazards such as transfusion-related acute lung injury and transfusion-related immunomodulation. Multimodal blood management should focus on a combination of appropriate surgical techniques and blood conservation, along with guidance from point-of-care testing (including coagulation).
Laryngotracheal Reconstruction
Authors: Garcia-Marcinkiewicz Annery , Fiadjoe John E.
Published: October 2018

This chapter on laryngotracheal reconstruction (LTR) describes and reviews the preoperative, intraoperative, and postoperative course of a virtual case of a patient undergoing double stage laryngotracheal reconstruction (ds-LTR). The discussion section reviews the etiology and pathogenesis of subglottic stenosis (SGS) and describes the Myer-Cotton classification system of SGS. The chapter also reviews the medical management of gastroesophageal reflux disease, endoscopic treatment of granulations, anterior cricoid split, LTR with cartilage augmentation (single and double stage), and cricotracheal resection. The differences in the technique and patient selection of single stage LTR (ss-LTR) and ds-LTR are reviewed. The intraoperative and postoperative complications of LTR and postoperative sedation management are also discussed.
Cystic Fibrosis
Authors: Snell Jamey , Mancuso Thomas J.
Published: October 2018

Cystic fibrosis (CF) is an inherited, autosomal recessive, multisystem disease. Dysfunction of the cystic fibrosis transmembrane conductance regulator protein (CFTR) in epithelial cells is the primary defect in CF. Defects in CFTR are the cause for lung disease, exocrine pancreatic insufficiency and failure, male infertility, and liver disease. CF can present with a variety of respiratory and gastrointestinal signs, including meconium ileus in the newborn period, hypernatremic dehydration, pulmonary insufficiency, nasal polyps, and insulin-dependent diabetes mellitus. As affected children grow, dysfunction in CFTR leads to chronic and progressive lung disease, characterized by suppurative infection and the development of bronchiectasis. CFTR dysfunction also affects exocrine function, leading to pancreatic insufficiency, malabsorption, and growth failure. In the past, history and physical exam with sweat chloride testing were the cornerstones of diagnosis. Diagnosis is now made with the newborn screening test for immunoreactive trypsinogen.
Airway Foreign Body Aspiration
Authors: Nizamuddin Sarah , Aveyard Caitlin
Published: October 2018

Aspiration of a foreign body is a potentially life-threatening problem that often necessitates an anesthetic for removal of the foreign body. Foreign body aspiration is most common among children aged 1 to 4 years old and has a wide variety of symptoms ranging from a mild, nagging cough to complete airway obstruction. Definitive diagnosis and treatment of foreign body aspiration involve flexible or rigid bronchoscopy. The urgency of the procedure depends on the type of object aspirated and the location of the foreign body in the airway. The appropriate anesthetic for removal of the foreign body is dependent upon the surgeon’s plan and involves several steps in decision-making: intravenous versus inhalational induction, airway maintenance (endotracheal tube vs. supraglottic airway vs. mask), spontaneous versus controlled ventilation, maintenance of anesthesia (total intravenous anesthesia vs. volatile agents). Good communication with the surgeon or proceduralist is key to a safe and effective anesthetic.
Neural Tube Defects
Authors: Hoang Jimmy , Yanofsky Samuel David
Published: October 2018

Neural tube defects (NTDs) are congenital abnormalities that arise from the neural tube failing to close. These defects can affect the brain, spine, or spinal cord and generally happen within the first month of pregnancy. Cranial dysraphism is failure of cranial neural tube closure and includes anencephaly and encephalocele. Spinal dysraphism is failure of caudal neuropore closure and includes spina bifida cystica and occulta. Myelomeningocele is a type of spina bifida cystica where the membranous sac containing neural tissue protrudes through an opening in the back. It is the most common NTD and considered a surgical emergency due to the increased risk for infection, further neurologic damage, and dehydration. Advancements in medicine have allowed for the possibility of early diagnosis and even in utero surgical intervention. This chapter focuses more exclusively on the approach and management of myelomeningocele.
Intracranial Tumor
Authors: Isserman Rebecca S. , Lockman Justin L.
Published: October 2018

Intracranial tumors are the most common solid malignancy in pediatrics, with the majority found in the posterior fossa. In these patients, presenting symptoms and signs are frequently related to intracranial hypertension due to obstruction of cerebrospinal fluid flow. Specific cranial nerve palsies and ataxia may also be presenting signs, with or without intracranial hypertension. The anesthesia for surgical resection is nuanced by management of intracranial hypertension, the potential for hemodynamic instability, and postoperative complications resulting from damage to critical brainstem structures. This chapter discusses signs, symptoms, and differential diagnosis; tumor classification; anesthetic induction; and intraoperative and postoperative complications of surgery for a posterior fossa tumor.
Anesthetic Management of Scoliosis Surgery in Children
Authors: Patil Arati , Pestieau Sophie R.
Published: October 2018

Scoliosis is defined as an abnormal curvature of the spine, which, if severe enough, can affect pulmonary or cardiac function and ultimately require surgical repair. There are 3 well-defined types of scoliosis: idiopathic, neuromuscular, and congenital scoliosis. Anesthetic management of children undergoing posterior spinal fusion can be challenging due to patient comorbidities, the need for neuromonitoring, the potential for significant blood loss, and various perioperative complications. In addition, postoperative pain after spinal fusion is known to be severe and often exceeds those reported in other major surgical procedures. To properly care for these patients, it is vital to be knowledgeable about the anesthetic and pain management considerations of patients undergoing posterior spinal fusion.
Moyamoya Disease
Authors: Brown Christina , Rubin Jamie E.
Published: October 2018

Moyamoya is a rare cerebrovascular disorder characterized by progressive stenosis of the large cerebral arteries. Cerebral ischemia is the most common manifestation in the pediatric population and may present with symptoms of headache, hypertension, blindness, and developmental delay. The gold standard for diagnosis of Moyamoya is digital subtraction angiography but less invasive imaging modalities such as magnetic resonance imaging/angiogram or perfusion magnetic resonance imaging are typically used. Progressive disease is usually treated with surgical revascularization. Indirect revascularization procedures are preferred to direct revascularization in children due to small caliber of vessels, but regardless of the surgical procedure performed, patients often have some degree of long-term neurological impairment after surgery. Maintenance of cerebral perfusion pressure is critical during the perioperative period, and the patient must be monitored closely for signs of cerebral ischemia.
Myotonic Dystrophy
Authors: Johnson Andrea
Published: October 2018

Myotonic dystrophy (DM) is a multisystemic autosomal dominant disorder. Individuals may present with symptoms at any age, but pediatric patients typically will present before 10 years of age. The clinical features of DM differ depending on the type of dystrophy and include skeletal muscle weakness, myotonia, sleep apnea, decreased gastrointestinal motility, insulin hypersecretion, cardiac conduction abnormalities, and occasionally cognitive impairment. Anesthetic management of the patient with DM should begin in the preoperative arena and should take into account the postoperative considerations and concerns for the patient with DM. This chapter will help the clinician develop an appropriate anesthetic plan and implement a safe and effective perioperative experience.
Duchenne’s Muscular Dystrophy
Authors: Sutherland Tori N. , Lalwani Kirk
Published: October 2018

Duchenne’s muscular dystrophy (DMD) is a progressive X-linked recessive disorder that affects boys and female carriers. It is the most common dystrophy with onset in childhood in the United States. It is associated with severe, progressive proximal muscle weakening due to absence of dystrophin, which is found in skeletal and cardiac muscles This chapter presents a review of anesthetic considerations for patients with DMD in the context of the disease’s natural history with special consideration for cardiomyopathy evaluation and management, restrictive lung disease evaluation, and management and postoperative ventilation. The chapter covers an overview of the disease; etiology and pathogenesis; cognitive, neuromuscular, cardiac, and pulmonary clinical presentation; diagnosis and management; and special anesthetic considerations.
Pediatric Liver Transplantation
Authors: Nelson Jonathon , Cladis Franklyn P.
Published: October 2018

Liver transplantation has become a standard surgical treatment for pediatric patients with hepatic failure, tumors, and metabolic derangements. Liver transplantation in the pediatric population can be extremely challenging for the anesthesiologist due to multiple perioperative considerations. The first successful liver transplant was performed in a pediatric patient in the 1960s, and since then, there have been significant advances in immunosuppressant medications and preservation solutions which have led to improved survival. Nevertheless, the number of liver transplants continues to be limited by organ availability, although the pediatric donor pool has been increased by living related donors and split livers. The most common pediatric pathology that results in hepatic failure and transplantation is biliary atresia. This chapter covers the perioperative care of a pediatric patient undergoing a liver transplant, from the preoperative preparation to the intraoperative management, and discusses postoperative challenges which may be encountered while in the intensive care unit.
Bladder Exstrophy
Authors: Bryant Jason
Published: October 2018

Bladder exstrophy is a rare condition in which there is an error in fetal development leaving the bladder and pelvic structure malformed. The bladder, pelvic girdle, and external genitalia are often split, requiring repair. The goals of this repair are to improve quality of life in terms of urinary continence, aesthetics, pelvic stabilization, and sexual function. This repair often requires prolonged traction and external fixation to adequately fuse the pelvic girdle. To aid in the healing and tolerance of this, a prolonged epidural catheter is often used. The pharmokinetics and risk of infection are major considerations during the prolonged use of a caudal catheter.
Wilms Tumor
Authors: Argote-Romero Graciela
Published: October 2018

Wilms tumor, known as well as nephroblastoma, is the most common primary malignant renal tumor in children. Over 95% of all renal tumors in patients under the age of 15 are Wilms tumors. The mean age at the time of diagnosis is 3.5 years. Wilms tumors are usually an incidental finding, a large abdominal mass discovered by a family member or pediatrician. Hematuria and hypertension can be present at the time of initial diagnosis. Up to 8% of the patients will have von Willebrand disease; therefore, all patients should have baseline coagulation studies. All patients should have either computed tomography of the abdomen and pelvis with oral and intravenous contrast or magnetic resonance imaging of the abdomen and pelvis with gadolinium. Treatment includes radical nephrectomy, chemotherapy, and, in some cases, radiotherapy. Emergency surgery is rarely. The disease-free survival rate is 86% for favorable-histology tumors and 64% for anaplastic tumors.
Pediatric Renal Transplantation
Authors: Chenault Kristin
Published: October 2018

Approximately 700 pediatric kidney transplants were performed in 2014, with roughly one-third of those being living-related kidney transplantations. There are distinct differences between renal disease and transplantation in children compared to adults. Overall, end-stage renal disease (ESRD) is less common in the pediatric population than in adults. While the most common etiology for ESRD in adults is diabetes mellitus, the most common etiologies of ESRD in the pediatric population are congenital, such as dysplastic kidney or obstructive uropathy. Surgical technique can also vary depending on the age and size of the recipient, as well as the donor kidney size.
Pheochromocytoma
Authors: Beltran Ralph J.
Published: October 2018

Pheochromocytoma is a rare type of neoplasm diagnosed in children. It originates in the adrenal gland and is different from paragangliomas which arise outside the adrenals. Both types of tumors arise from neural crest cells and lead to signs and symptoms related to hypersecretion of catecholamines. Related symptoms include hypertension, tachycardia, episodic headache, sweating, and abdominal pain. These tumors may be associated with multiple endocrine type 2 syndrome, multiple endocrine neoplasia, and von Hippel-Lindau disease, among other hereditary conditions. Pheochromocytomas can be malignant in nature but may be indistinguishable from benign disease histologically and functionally. When malignant, they present with regional invasion or distant metastasis. Conditions presenting as sympathetic overactivity in pediatric patients can resemble pheochromocytoma (i.e., panic disorder, amphetamine consumption).
Hemophilias
Authors: Elsey Nicole M.
Published: October 2018

Hemophilia A, B, C, and Acquired Hemophilia A make up a rare group of bleeding disorders that can be challenging to manage in the perioperative setting. The management of hemophilia patients within the surgical setting can require additional planning and extensive health care resources. This chapter will provide a brief overview of the hemophilias, including the etiology and clinical characteristics of the hemophilias, relevant diagnostic methods and laboratory tests utilized in treating a patient with hemophilia, and the guidelines for drug therapy and factor replacement in the perioperative setting. Common comorbidities, chronic pain concerns, psychosocial impacts, prophylactic factor management, and alternative treatment methods affecting hemophiliac patients are also discussed
Sickle Cell Disease
Authors: Ferrari Lynne R.
Published: October 2018

Sickle cell anemia is a disease that combines molecular biology, clinical features, biochemistry, pathology, natural selection, population genetics, gene expression, and genomics and is the world’s most common life-threatening monogenic disorder. Clinical features include anemia; painful crisis especially in fingers, chest, and long bones; hemolysis; splenic infarction resulting in functional asplenia; and microinfarction leading to neurologic and renal impairment. The maintenance of adequate body temperature with active warming devices and warmed intravenous fluids, monitoring hydration and urine output, providing supplemental oxygen, and limiting surgical and anesthesia times to reduce pulmonary complications constitute the best management for patients with sickle cell disease.
Pediatric Complex Regional Pain Syndrome Type 1
Authors: Riley Bobbie , Sethna Navil
Published: October 2018

Complex regional pain syndrome type 1 (CRPS-1) is a condition that affects adolescents and children under the age of 7. It usually follows minor injury and rarely occurs spontaneously. The pain is usually out of proportion to the inciting injury. Pain, allodynia, and/or hyperalgesia are severe enough to inhibit use of the affected limb. Delay in diagnosis and self and/or iatrogenic immobilization of the affected limb may lead to worsening pain, skin hypersensitivity and discoloration, swelling, and vasomotor and dystrophic abnormalities. The diagnosis of CRPS-1 and 2 is based on symptoms. There are no diagnostic tests that can confirm the presence or absence of CRPS-1. CRPS-2 diagnosis is established by nerve conduction test and electromyography. Clinical practice neuropathic guidelines are most effective for CRPS-2 treatment. Pharmacological and interventional treatment options for CRPS-1 are limited and usually ineffective because the underlying mechanism(s) are yet to be determined.
Psychological Preparation of the Pediatric Patient for Surgery
Authors: Saadat Haleh , Kain Zeev N.
Published: October 2018

Each year, about six million children undergo surgical procedures in the United States. More than 80% of all pediatric procedures in the United States are on an outpatient basis without hospital preoperative or postoperative admission. More than 60% of these children manifest emotional distress prior to the procedures and at the induction of anesthesia. The intense distress may lead to negative postoperative physiological and psychological outcomes in children and affect parental satisfaction. Preoperative stress can also exaggerate negative memories, which can exaggerate distress at subsequent procedures. Studies have looked into the incorporation of pharmacological agents as well as nonpharmacological strategies such as parental presence, and behavioral techniques such as imagery, relaxation, and hypnosis in the management of preoperative anxiety. The goal of this chapter is to examine the effects of preoperative anxiety and the effectiveness of these strategies in preventing preoperative psychological stress in children.
Von Willebrand Disease
Authors: Downey Laura A. , Guzzetta Nina A.
Published: October 2018

Von Willebrand disease (vWD) is the most common bleeding disorder in humans. It is the result of an abnormality in the amount, structure, or function of von Willebrand factor (vWF), a glycoprotein important in maintaining normal hemostasis.. In children with vWD, the most frequent presentation is easy bruising and epistaxis. Other symptoms include hematomas, menorrhagia, and bleeding from minor wounds. Although intraarticular bleeding may occur, especially in certain subtypes, it is much more commonly seen with hemophilia. There are several subtypes of vWD based on the underlying defect in vWF, but, in general, they may be categorized as quantitative (types 1 and 3) or qualitative (all types 2). If vWD is suspected, consultation with a hematologist to establish the correct diagnosis and perioperative approach to hemostasis is essential. Avoidance of medications that interfere with coagulation, anticipation of intraoperative and postoperative bleeding, and an appropriate hemostatic treatment plan should be addressed.
Trauma
Authors: Young David A.
Published: October 2018

Traumatic injuries are the most common cause of death within the United States for children above one year of age Most traumatic injuries in children are a result of motor vehicle accidents, child abuse (or nonaccidental trauma), drowning, thermal injury, or falls. Motor vehicle accidents are the leading cause of death for children above the age of one year. Strategies of Advanced Trauma Life Support (ATLS) utilize a standardized approach to promptly identify and manage life-threatening traumatic injuries. These strategies include recognition of cardiopulmonary disorders, volume resuscitation, and prevention of additional injuries including to the cervical spine. A management plan for severe and uncontrolled bleeding is critically important to have established when caring for children with traumatic injuries. Many strategies have been developed to manage severe and uncontrolled bleeding including the use of massive transfusion protocols, damage control surgery, and hemostatic agents.
Nonaccidental Trauma
Authors: Padover Alyssa , Lee Jennifer K.
Published: October 2018

Nonaccidental trauma from child abuse presents unique challenges to the anesthesiologist. Diagnosing abuse is difficult because children may present with nonspecific symptoms and vague clinical histories. Multiple organ systems may be involved, but the greatest risk of death stems from abusive head trauma. Anesthesiologists must know the pediatric traumatic brain injury treatment guidelines and be prepared to treat the complex disease processes of child abuse and abusive head trauma. This chapter discusses anesthesia for nonaccidental pediatric trauma, including abusive head trauma. Topics covered include cervical instability, intracranial hypertension, seizures, and anesthetic agents. Debriefing after a poor outcome is also covered.
Anesthesia for the Child with Autism Spectrum Disorder
Authors: Vlassakova Bistra G.
Published: October 2018

Autism spectrum disorder (ASD) is the fastest growing neurodevelopmental disorder. Approximately 1% of the world’s population is affected. Patients with ASD have impairment in social interactions and communication and exhibit repetitive and restrictive behaviors and interests. The exact etiology of the condition is unknown, and it is believed to be multifactorial in origin—genetic predisposition and environmental factors have been implicated. Patients with ASD can be highly intelligent or severely developmentally delayed. The wide variety of clinical presentations poses serious challenges to the anesthesiologist. Common clinical manifestations include difficulties with changing daily routines and stereotyped behavior as well as hypersensitivity to light, noise, and unfamiliar people. Patients with ASD are therefore susceptible to behavioral outbursts during the perioperative period. Assuring a smooth perioperative course for this patient population can be achieved by a multidisciplinary team approach and good communication between caregivers and team members.
Anesthesia for Adolescent Bariatric Surgery
Authors: Starker Elizabeth Q. , Allen Staci N. , Chatterjee Debnath
Published: October 2018

The growing epidemic of childhood obesity has resulted in an increasing number of obese children presenting for a wide range of surgical procedures. Bariatric surgery is gaining popularity as a valid treatment option in severely obese adolescents in whom life style interventions are not successful. The perioperative management of these patients pose several anesthetic challenges. The role of the anesthesiologist in the multidisciplinary model is outlined in this chapter. A case-based approach focuses on the preoperative evaluation, intraoperative anesthetic considerations, appropriate drug dosing, and recommended strategies for postoperative monitoring and analgesia. Common complications encountered during these procedures are also discussed.
High-Grade Gliomas
Authors: Huntoon Kristin , Elder J. Bradley
Published: October 2018

Glioblastoma is the most common primary malignant brain tumor. This chapter discusses the clinical presentation and initial workup for a patient with a suspected glioblastoma, as well as the optimal treatment strategy and prognosis. Diagnosis is typically made using magnetic resonance imaging. Optimal treatment involves maximal safe surgical resection followed by adjuvant chemotherapy and radiation therapy. Surgical adjuncts including intraoperative imaging modalities and brain mapping techniques help improve neurologic morbidity associated with surgery. Despite maximal treatment, virtually all patients with glioblastoma will experience recurrence of their tumor and may be considered for clinical trials or second-line therapy. This chapter highlights important pearls associated with management of patients with glioblastoma and written for those who are interested in neuro-oncology, neurosurgery, and the field of brain tumors.
Unusual Gliomas
Authors: Eseonu Chikezie I. , Rincon-Torroella Jordina , Quiñones-Hinojosa Alfredo
Published: October 2018

Patients with intra-axial brain tumors often present with neurologic symptoms based on the anatomic location of their tumor. Workup for a brain tumor includes cranial imaging such as magnetic resonance imaging and computed tomography, as well as systemic imaging to assess for primary tumor if metastasis is suspected. Maximal safe resection optimizes outcomes including overall survival. Surgical decisions are based on variables such as medical comorbidities and anatomic location of the tumor. Gliomas in eloquent areas may require intraoperative cortical and subcortical mapping of motor and/or language areas to optimize safety and help maximize resection. Adjuvant chemotherapy and radiation lead to a median survival of 14.6 months for patients with glioblastoma. Rapidly recurring glioblastoma after surgery has a poor prognosis.
Choroid Plexus Tumors
Authors: Sribnick Eric , Leonard Jeffrey
Published: October 2018

This chapter presents the case of an 11-month-old male with a history of macrocephaly, anorexia, and right upper extremity tremors. His family history was significant for a father with anaplastic astrocytoma. Physical exam was notable for left asymmetric macrocephaly and a full/tense fontanelle. The chapter reviews the differential diagnoses for macrocephaly. Imaging, including magnetic resonance spectroscopy, revealed a highly vascular intraventricular mass with some areas of likely necrosis, suggesting choroid plexus carcinoma. The authors review the preoperative planning and surgical management of this case, including potential complications and their management. Finally, choroid plexus carcinomas are reviewed in light of evidence-based medicine and clinical outcomes.
Dysembryoplastic Neuroepithelial Tumors
Authors: Haldeman Clayton L. , Kuo John S.
Published: October 2018

Dysembryoplastic neuroepithelial tumors (DNETs) are World Health Organization grade I tumors and have a low potential for malignant transformation. However, DNETs often affect a patient’s quality of life and can cause medically intractable seizures. The estimated prevalence of DNET is 0.6% to .0.8%; however, they are found in approximately 20% of patients undergoing surgery for epilepsy. Greater than 90% of patients with DNET have onset of epilepsy prior to the age of 20. The most common presentations of DNET are complex partial seizures and simple partial seizures. Generalized tonic–clonic seizures are present in about 50% of patients. Patients are often seizure-free after surgical resection.
Medulloblastoma
Authors: North Robert , Rao Ganesh
Published: October 2018

Medulloblastoma is the most common brain malignancy in children. This chapter details the current diagnostic criteria, pathological classification, and treatment paradigms. Recent molecular characterization of medulloblastoma has revealed significant variations in clinical behavior of different tumor types. While the treatments for medulloblastoma are generally quite successful, with 5 year survival rates approaching 80%, the responsiveness of each subtype to treatment varies. The authors cover common clinical scenarios along with management pearls and key references.
Hemangioblastoma
Authors: Hatef Jeffrey , Lonser Russell R.
Published: October 2018

Hemangioblastomas are benign central nervous system tumors that are found primarily (99%) in the cerebellum, brainstem, and spinal cord. They can occur sporadically (67% of cases) or in the context of the familial neoplasia syndrome, von Hippel-Lindau disease (VHL; 33%). These lesions often remain quiescent or grow in a saltatory pattern. When these tumors cause signs or symptoms, the signs or symptoms are often associated with peritumoral cyst formation. Whether the tumor occurs sporadically or in the context of VHL, complete resection is the treatment of choice when necessary. This chapter describes the clinical, imaging, and treatment features of this neoplasm.
Primary CNS Lymphoma
Authors: Diaz Roberto Jose , Basil Gregory W. , Komotar Ricardo J.
Published: October 2018

Central nervous system (CNS) lymphoma must be considered in the differential diagnosis of any immunocompromised patient with a solid brain lesion. In such patients, diagnosis can be made via a careful review of important signs, symptoms, and classic radiologic findings. While there is no single physical exam finding classic for lymphoma, the clinician must carefully evaluate patients for the presence or absence of findings that may suggest an alternative diagnosis. Such findings include the stigmata of endocarditis, symptoms suggestive of pneumonia, or additional non-CNS mass lesions. Additionally, several imaging modalities including magnetic resonance imaging, diffusion-weighted magnetic resonance imaging, susceptibility weighted imaging, and dynamic contrast-enhanced imaging can be useful in identifying this condition. While steroids can be helpful in reducing the disease burden and decreasing edema, they may also hinder diagnosis. Surgery may be indicated for either diagnostic or decompressive purposes; however, the mainstay of treatment is chemotherapeutic and immunotherapeutic agents with radiation reserved for refractory cases.
Chordoma and Chondrosarcoma
Authors: Mohyeldin Ahmed , Carrau Ricardo L. , Prevedello Daniel M.
Published: October 2018

Chordomas and chondrosarcomas are rare tumors that arise from the axial skeleton and skull base. They are often difficult to manage given their locally destructive behavior and predilection to grow near vascular and critical neural structures. They have an indolent clinical course that results in the late clinical presentation of patients. Unfortunately, these factors make complete surgical resection of these tumors difficult to perform without causing significant morbidity, although advancements in surgical and high-dose radiation techniques for treatment of these lesions continues to be developed. In a case-based approach, this chapter reviews the nuances in the treatment and management of these very challenging pathologies.
Endocrine Active Pituitary Tumor
Authors: Raheja Amol , Couldwell William T.
Published: October 2018

This chapter presents an illustrative case demonstrating the principles of diagnosis and management in endocrine active pituitary tumor. The index case involves a 30-year-old male patient who presented with phenotypic markers of acromegaly. On radiological and endocrinological evaluation, growth hormone–secreting pituitary macroadenoma was identified. The philosophy of decision-making and management paradigm is discussed to demonstrate the pros and cons of medical, radiation, and surgical options. Technical nuances of the surgical procedure and its complication management are stressed. A brief review of literature is included to elaborate on the current evidence, including outcomes for the various management options for such tumors, with special emphasis on multimodality management.
Endocrine Silent Pituitary Tumors
Authors: Singh Harminder , Salam Smeer , Schwartz Theodore H.
Published: October 2018

Pituitary adenomas are the most common intracranial neoplasms in adults, with a prevalence of 7% to 17%. Clinically, they can be divided into 2 categories based on whether they secrete pituitary hormones: functional (secretory) and nonfunctional (nonsecretory or endocrine silent) adenomas. The biologic latency of nonfunctional (endocrine silent) adenomas makes them usually diagnosed at the stage of macro (>1 cm) and giant (>4 cm) adenomas. Because these tumors are nonfunctioning, their primary symptoms are due to mass effect, particularly on the optic chiasm and normal pituitary gland and stalk superiorly, and the cavernous sinus laterally. Visual field disturbance is the most common presenting complaint, followed by pituitary dysfunction and headaches. Surgical outcomes, therefore, are aimed at determining visual outcome in addition to rates of gross total resection, recurrence, and postoperative pituitary dysfunction. Several recent case series have documented the increased success of the endonasal endoscopic transsphenoidal approach for resecting nonfunctioning pituitary adenomas, particularly in relation to the classic open cranial and microsurgical transsphenoidal techniques.
Rathke’s Cleft Cysts
Authors: Buchanan Ian A. , Zada Gabriel
Published: October 2018

Cystic lesions involving the sella have a distinct differential diagnosis including Rathke cleft cyst, cystic pituitary adenoma, craniopharyngioma, arachnoid cyst, and epidermoid among other entities. Workup includes not only cranial imaging but also endocrine evaluation for pituitary dysfunction and ophthalmologic evaluation to assess for visual deficits that may not be immediately apparent to the patient at presentation. Rathke cleft cysts are common and may be found in 20% of autopsy specimens. However, symptomatic Rathke cleft cysts are rare, and surgical decisions should be made judiciously based on preoperative symptoms and workup. Endonasal transphenoidal approach for cyst fenestration is a common surgical management technique for symptomatic lesions. Complete resection of the Rathke cleft cyst wall is not required.
Esthesioneuroblastoma and Carcinomas of the Nasal Cavity
Authors: Alkhalili Kenan , Raza Shaan M. , DeMonte Franco
Published: October 2018

Sinonasal malignancies are rare, pathologically diverse, and biologically unpredictable tumors. They tend to have minimal or nonspecific symptoms that mimic benign (inflammatory) disease until there is invasion of adjacent structures. Most patients present with advanced disease. Computed tomography and magnetic resonance imaging are complimentary studies but magnetic resonance imaging optimally defines the tumor’s extent and dictates the need for neurosurgical attention. Advanced endoscopic techniques allow for the resection of some well-selected tumors as part of a multimodal treatment plan. The intimate relationships with the orbit and brain make surgical management challenging. Optimal patient outcomes can only be achieved with carefully constructed, multidisciplinary, multimodal management paradigms.
Extramedullary Spinal Cord Tumors
Authors: Elder J. Bradley , Mohyeldin Ahmed
Published: October 2018

Tumors of the spine that occur within the dura but outside of the spinal cord are characterized as intradural but extramedullary spine tumors. The vast majority of these tumors are benign. This chapter uses a case presentation to present diagnostic and management pearls for this anatomic category of central nervous system neoplasms. These tumors are typically diagnosed due to presenting symptoms including pain or neurologic symptoms that localize the tumor to a specific area of the spine. Management is typically surgical. Some types of tumors are associated with syndromes such as neurofibromatosis. This chapter also discusses potential pitfalls and complications and their management.
Skull Tumors
Authors: Altshuler David , Heth Jason A. , Szerlip Nicholas J.
Published: October 2018

The diagnostic and treatment approach for patients with skull lesions begins with a thorough history and physical and careful attention to anatomic localization. The patient’s history and exam findings can inform a preliminary differential diagnosis, which may be broadly divided into benign and malignant processes. Based on a preliminary assessment, appropriate neuro-imaging involving magnetic resonance, computed tomograph, and/or vascular modalities may be pursued. Characteristic image findings may further refine a differential. While conservative management may be indicated for the most assuredly benign lesions, surgery is appropriate for cases involving compression of neural structures, deformity, pain or when a tissue diagnosis is required.
Stereotatic Radiosurgery and Microsurgery for Brain Metastases
Authors: Cohen-Inbar Or , Trifiletti Daniel M. , Sheehan Jason P.
Published: October 2018

This chapter describes the case of a patient with brain metastases due to metastatic breast cancer. MRI is the best imaging modality for visualizing brain metastases, and advanced techniques such as perfusion imaging and diffusion weighted imaging may provide important additional information beyond standard anatomic imaging. Patients with brain metastases due to systemic cancer may benefit from targeted therapies such as surgery and stereotactic radiosurgery. Understanding the differences between radiation modalities such as stereotactic radiosurgery and whole brain radiotherapy is important for counseling patients.