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Neural tube defects, also referred to as spinal dysraphism, myelodysplasia, or spina bifida, result from incomplete fusion of midline structures with lack of covering over neural elements. Although introduction of the use of folic acid to potential mothers prior to conception has decreased the risk of having a fetus with a neural tube defect, the incidence is not negligible at about 3000 per year in the United States, or about 1 per 1000 live births (0.1%). Worldwide, there are more than 300,000 babies born each year with neural tube defects. A myelomeningocele involves protrusion of only the meninges through the dysraphic defect and spares the neural contents. However, a myelomeningocele refers to a defect with protrusion of neural elements along with their covering and has implications for the patient and the provider from infancy to adulthood. This chapter focuses on perioperative complications of myelomeningocele as follows: (1) damage to neural placode, (2) hydrocephalus, (3) CSF leak, and (4) latex allergy. A miscellaneous section (5) is also included.

SummaryIntroduction Surgical correction of pediatric scoliosis is associated with significant blood loss. Minimizing estimated blood loss and blood transfusion is beneficial as transfusions have been associated with increased morbidity, including risk of surgical site infections, longer hospitalizations, and increased cost. Although there is evidence that African-American or Black adults are more likely to require intraoperative blood transfusion compared with Caucasian or White adults, the reasons for this difference are unclear. Methods The electronic records for all patients

Introduction Recent reports have suggested that caudal anesthesia may be associated with an increased risk of postoperative surgical complications. We examined our experience with caudal anesthesia in hypospadias repair to evaluate for increased risk of urethrocutaneous fistula or glanular dehiscence. Methods All hypospadias repairs performed by a single surgeon in 2001–2014 were reviewed. Staged or revision surgeries were excluded. Patient age, weight, hypospadias severity, surgery duration, month and year of surgery, caudal anesthesia use, and postoperative complications were recorded. Bivariate and multivariate statistical analyses were performed. Results We identified 395 single-stage primary hypospadias repairs. Mean age was 15.6 months; 326 patients had distal (83%) and 69 had proximal (17%) hypospadias. Caudal anesthetics were used in 230 (58%) cases; 165 patients (42%) underwent local penile block at the discretion of the surgeon and/or anesthesiologist. Complications of urethrocutaneous fistula or glanular deshiscence occurred in 22 patients (5.6%) and were associated with caudal anesthetic use (OR 16.5, 95% CI 2.2–123.8, P = 0.007), proximal hypospadias (OR 8.2, 95% CI 3.3–20.0, P < 0.001), increased surgical duration (OR 1.01, 95% CI 1.01–1.02, P < 0.001), and earlier year of practice (OR 3.0, 95% CI 1.2–7.9, P = 0.03 for trend). After adjusting for confounding variables via multivariable logistic regression, both caudal anesthetic use (OR 13.4, 95% CI 1.8–101.8, P = 0.01) and proximal hypospadias (OR 6.8, 95% CI 2.7–16.9, P < 0.001) remained highly associated with postoperative complications. Conclusions In our experience, caudal anesthesia was associated with an over 13-fold increase in the odds of developing postoperative surgical complications in boys undergoing hypospadias repair even after adjusting for urethral meatus location. Until further investigation occurs, clinicians should carefully consider the use of caudal anesthesia for children undergoing hypospadias repair.

Intraosseous (IO) access is used most frequently for emergency care of critically ill infants and children when IV access cannot be rapidly achieved. Despite its efficacy in such situations, applications outside of the emergency room or resuscitation scenario have been limited. Furthermore, although the technique is emphasized in the teaching of those caring for critically ill infants and children in the emergency room or critical care setting, there is limited emphasis on its potential use in the perioperative setting. When peripheral venous access cannot be achieved in the operating room, alternative means of securing vascular access such as central line placement or surgical cutdown are generally successful; however, these techniques may be time consuming. Anyone providing anesthesia care for infants and children may want to become facile with the use of IO infusions for selected indications. We present the history of IO infusions, review the anatomy of the bone marrow space, discuss the potential role of IO infusions in the perioperative period, and analyze its adverse effect profile.

Summary Background: Patients with infantile-onset Pompe disease suffer from marked hypertrophic cardiomyopathy and an increased risk of arrhythmia. A noncompliant left ventricle predisposes these infants to diastolic heart failure with elevated left ventricular enddiastolic pressure (LVEDP); these patients also commonly develop systolic heart failure. Given this baseline cardiac physiology, coronary perfusion pressure becomes highly sensitive to abrupt changes in diastolic blood pressure (DBP). Methods: We retrospectively reviewed the experiences of 139 patients enrolled in clinical trials investigating the treatment of infantile-onset Pompe disease with recombinant human acid α-glucosidase (rhGAA). Adverse events were screened for those involving anesthesia. Results: Nine patients (6%) with infantile-onset Pompe disease experienced an arrhythmia or cardiopulmonary arrest soon after the induction of general anesthesia. Of these events, propofol was involved in four arrhythmias; sevoflurane without propofol was associated with an additional two. Deaths resulting from arrhythmia appeared to correlate with left ventricular mass indices >350 g·m−2. Conclusions: With the advent of enzyme replacement therapy (ERT) using rhGAA, and increased survivability, more infantile Pompe patients will likely present for surgical procedures. Additional care in maximizing coronary perfusion pressure and minimizing arrhythmia risk must be given. For these reasons, it is recommended that anesthesia for infantile Pompe patients specifically avoid propofol or high concentrations of sevoflurane and, instead, use an agent such as ketamine as the cornerstone for induction in order to better support coronary perfusion pressure and to avoid decreasing DBP with vasodilatory agents.

Paravertebral nerve block is a very effective method of postoperative pain control that is ideal for certain unilateral operative procedures in which bilateral blockade is not necessary. The quality of the block may be superior to that seen with epidural analgesia. Paravertebral nerve blockade in children has primarily involved the use of postoperative infusions of local anesthetic via indwelling catheters for renal and thoracic surgery. Its use has thus been mostly limited to the inpatient population. Recently, bolus injections into the paravertebral space have been shown to be effective in adults undergoing outpatient surgery, yet experience with this technique in children is limited. This article briefly reviews the current use of paravertebral blockade in children and using case illustration, specifically outlines the use of paravertebral blockade for outpatient inguinal surgery in children. It also includes a description of the many benefits of the technique in this population. Copyright 2002, Elsevier Science (USA). All rights reserved.

Regional anaesthetic techniques are useful for providing post-operative pain control for ambulatory surgery in children. This chapter focuses on unique features of paediatric anatomy and physiology that allow successful performance of these techniques. An overview of the effective use of long-acting local anaesthetics in children is presented. Combinations of local anaesthetics and adjunct medications that prolong caudal blockade, the most commonly performed paediatric block, are reviewed, with a goal of providing prolonged analgesia with minimal side-effects. An overview of the use of peripheral nerve blockade and specific recommendations for the use of these blocks in children is also presented.

PhD§*Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina; †Departments ofAnesthesiology and Pediatrics, University of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh, Pittsburgh,Pennsylvania; ‡Department of Anesthesiology, Harvard Medical School, and Boston Children’s Hospital, Boston,Massachusetts; and §Glaxo Wellcome, Inc., Research Triangle Park, North CarolinaRemifentanil hydrochloride is an ultra-short-actingopioid that undergoes rapid metabolism by tissue andplasma esterases. We aimed to characterize the phar-macokinetics and determine the hemodynamic profileof remifentanil after a single-bolus dose in childrenaged 0 to 18 yr. Forty-two children undergoing electivesurgical procedures received remifentanil 5 g/kg in-fusedover1min.Patientsweredividedintoagegroupsasfollows:younginfants(2mo),olderinfants(2moto 2 yr), young children (2 to 7 yr), older children (7to13yr),adolescents(13to 16yr),andyoungadults(16 to 18 yr). Arterial blood samples were collectedand analyzed by mass spectroscopy to determineremifentanil pharmacokinetic profiles. Hemodynamicmeasurementsforremifentanil’seffectweremadeafterthe infusion. Methods of statistical analysis includedanalysis of variance and linear regression, with signifi-cance at

Progress has been made in the prevention of accidents in children; however, pediatric trauma continues to be the number one cause of death in the United States in children who are older than 1 year of age, accounting for 15,000 deaths per year. 29,101,118 For every child who dies because of trauma, an estimated 1000 more present to the emergency department for treatment. 54 Most of these traumatic injuries are caused by motor vehicle and bicycle accidents in children with a mean age of 8 years. 29 In younger children, a significant percentage of trauma in infants is caused by child abuse, whereas trauma in toddlers most often is caused by falls from heights. 101,117 Anesthesiologists often are called to the emergency department after the primary survey has been performed and intervention is required. It is important that they become familiar with the immediate evaluation and resuscitative efforts of children in the emergency department who have suffered trauma and that they continue this intensive care into the operative and postoperative arenas.

Managing pain in children is a complex area that requires an understanding of the unique challenges offered by this young population. Regional anesthesia is emerging as an adjunct to pain management in children, particularly in the perioperative arena.The following article will assist the practitioner with the management of pain in children through regional techniques. Prior to the presentation of the regional techniques, an overview is presented to address the neurodevelopment of the child, the pharmacologic concerns in the younger patients, and the assessment of pain in the different age groups. Several of the commonly performed regional blocks with appropriate dosing guidelines are reviewed, with an emphasis on blocks that are more unique to the pediatric population.

To determine if 450 micrograms/kg (1.5 times the ED95) of rocuronium would result in a comparable onset with a shorter duration of action when compared with 600 micrograms/kg (2 times the ED95).Randomized, single-blind study.Teaching hospital.85 ASA physical status I and II children ages 2 through 12, undergoing elective surgery with an inhalation induction using halothane.Group 1 received 600 micrograms/kg rocuronium, and Group 2 received 450 micrograms/kg rocuronium.The two groups were compared using a Student's t-test, with p0.05 significant. The time of onset, or time to 95% suppression of neuromuscular twitch with standard errors, was 140 +/- 13 seconds (range 46 to 365 sec) in Group 1 and 148 +/- 13 seconds (range 82 to 345 sec) in Group 2 (NS = not significant). The times to 25% return of twitch from baseline (T25) in Groups 1 and 2 were 28 +/- 1.5 minutes (range 14 to 45 min) and 26 +/- 1.6 minutes (range 10 to 55 min), respectively (NS). The differences between these two doses in onset of, and recovery from, block were not found to be statistically significant. The results, however, excluded 5% of the higher dose group and 31% of the lower dose group who did not achieve 95% suppression of twitch. Time to maximal suppression of neuromuscular blockade, however, was not statistically significant for the 85 patients with a time of 270 +/- 28 seconds (range 91 to 605 sec) with a mean maximal suppression of 98.7% in Group 1 and 313 +/- 25 seconds (range 91 to 899 sec) with a mean maximal suppression of 93.1% in Group 2.The two doses of rocuronium did not differ statistically in onset or duration. Rocuronium at 600 micrograms/kg offers more reliability than 450 micrograms/kg in achieving adequate muscle relaxation, and the lower dose may result in a significantly large number of patients who may have inadequate intubating conditions.

Background/Purpose : Surgery in patients with sickle hemoglobinopathies can be problematic because of the potential for sickling events in the perioperative and postoperative period. The authors and others have previously reported successful surgical outcomes using an aggressive erythrocyte transfusion regimen, designed to alleviate anemia and to reduce the percentage of sickle hemoglobin to below 30%. Recently, a randomized trial compared this aggressive regimen with a more conservative transfusion regimen and found no differences in perioperative complications. The incidence of complications, however, was very high in each group (31% to 35%). Methods : The authors therefore analyzed retrospectively their surgical experience in children with sickle hemoglobinopathies over the past 10 years to determine the efficacy of an aggressive transfusion regimen and skilled perioperative care in their patient population. Results : A total of 130 surgical procedures were performed on 92 children including 54 cholecystectomies (42%), 23 splenectomies (18%), 12 ENT procedures (9%), 11 central line placements and removals (8%), 7 herniorrhaphies (5%), 7 appendectomies (5%), and 16 miscellaneous operations (13%). The mean age of the children was 10 years (range, 1 to 22 years), and the mean weight was 32.1 kg (range, 9.9 to 76.8 kg). The average hemoglobin (mean ± 1 SD) at the time of surgery was 11.2 ± 1.3 g/dL, and the average percent hemoglobin S was 21 ± 11%. Conclusions : Relatively few transfusions were required to achieve these endpoints, and the complications resulting from transfusions were minimal. Similarly, the number of perioperative and postoperative events was very low.

In a randomized controlled trial at Duke University and University of North Carolina Hospitals, 143 two to six year old children undergoing elective ambulatory surgery were randomized into a control group who received routine preoperative teaching or an intervention group who received an interactive teaching book. Changes in behaviour were measured by a Vernon behavioural questionnaire preoperatively and again two weeks postoperatively. A Global Mood Score was assessed in the preoperative holding area, at arrival into the operating room, and at the time of mask induction. The children in the control group were significantly more aggressive postoperatively than the intervention group (P < 0.05). Children who received the interactive teaching book exhibited higher levels of anxiety on the day of surgery, but fewer behavioural changes two weeks following surgery. Parents in the intervention group reported that preoperative teaching helped their child (87%) and themselves (83%).

Background This study determined the accuracy of previously defined adult fentanyl pharmacokinetics in children having surgery; from this population, the pharmacokinetics of fentanyl were characterized in children when administered via a computerized assisted continuous-infusion device. Methods Twenty children between the ages of 2.7 and 11 y scheduled to undergo elective noncardiac surgery were studied. After induction, anesthesia was maintained with 60% nitrous oxide in oxygen supplemented with fentanyl (n = 10) or fentanyl plus isoflurane (n = 10). Fentanyl was administered via computerized assisted continuous-infusion to target concentrations determined by clinical requirements. Plasma fentanyl concentrations were measured and used to evaluate the performance of the fentanyl pharmacokinetics and then to determine a new set of pharmacokinetic parameters and the variance in the context-sensitive half-times simulated for these patients. Results The original adult fentanyl pharmacokinetics resulted in a positive bias (10.4%), indicating that measured concentrations were mostly greater than predicted. A two-compartment model with age and weight as covariates provided the optimal pharmacokinetic parameters. These resulted in a residual performance error of -1.1% and a median absolute performance error of 17.4%. The context-sensitive times determined from this pediatric population were considerably shorter than the context-sensitive times previously published for adults. Conclusions The pharmacokinetics of fentanyl administered by computerized assisted continuous-infusion differ between adults and children. The newly derived parameters are probably more suitable to determine infusion schemes of up to 4 h in children between the ages of 2 and 11 y.

Neural tube defects, also referred to as spinal dysraphism, myelodysplasia, or spina bifida, result from incomplete fusion of midline structures with lack of covering over neural elements. Although introduction of the use of folic acid to potential mothers prior to conception has decreased the risk of having a fetus with an open neural tube defect, the incidence is not negligible at 0.5–1 per 1,000 live births. A meningocele involves protrusion of only the meninges through the dysraphic defect and spares the neural contents. However, a myelomeningocele refers to a defect with protrusion of neural elements along with their covering and has implications for the patient and the provider from infancy to adulthood. This chapter focuses on perioperative complications of myelomeningocele as follows: (1) damage to neural placode, (2) hydrocephalus, (3) CSF leak, and (4) latex allergy. A miscellaneous section (5) is also included.

Hydroxyurea has enhanced the treatment for children with sickle cell anemia. The objectives of this study were to compare perioperative transfusions and outcomes for children taking hydroxyurea versus those not taking hydroxyurea. We retrospectively reviewed perioperative management and outcomes for 51 children with sickle cell anemia (HbSS genotype) who underwent surgery in our center between January 2003 and April 2008. Of the 51 patients, 30 (59%) were taking hydroxyurea and 21 (41%) were not taking hydroxyurea. Eight of 30 (27%) in the hydroxyurea group were not transfused preoperatively, 12 of 30 (40%) received a single transfusion and 10 of 30 (33%) received serial transfusions, compared with 1 of 21 (5%) children in the nonhydroxyurea group who was not transfused, 2 of 21 (10%) who received a single transfusion and 18 of 21 (85%) who received serial transfusions or pheresis (P=0.004; for comparison across groups). One patient not taking hydroxyurea developed a delayed hyperhemolytic transfusion reaction, and 2 children taking hydroxyurea developed acute chest syndrome. Overall, children taking hydroxyurea had substantially fewer transfusions compared with children not taking hydroxyurea. Both groups of children had a low complication rate. Further research should be done to optimize perioperative management of children taking hydroxyurea.

The need for consent to regional anesthetic procedures varies considerably between countries. It is likely that legislation and professional guidance will tighten consent procedures, and in several countries detailed written consent is required for regional blockade. This article discusses aspects of consent to regional anesthesia in children.

In a patient with a known difficult airway, history and examination may be of limited use in formulating a management plan for subsequent tracheal intubation. Further detailed and descriptive review of the airway is necessary. Virtual imaging is a recent advance in radiology that offers noninvasive airway assessment. It creates a movie clip image of the upper airway akin to the view obtained through a fiberscope. We present a patient with Goldenhar syndrome in whom virtual imaging was used to identify the cause of a previous failed tracheal intubation.

Autism is a pervasive neurodevelopmental disorder. Because of the deficits associated with the condition, sedation of children with autism has been considered more challenging than sedation of other children. Objective: To test this hypothesis, we compared children with autism against clinical controls to determine differences in requirements for moderate sedation for MRI. Materials and methods: Children ages 18–36 months with autism (group 1, n = 41) and children with no autistic behavior (group 2, n = 42) were sedated with a combination of pentobarbital and fentanyl per sedation service protocol. The sedation nurse was consistent for all patients, and all were sedated to achieve a Modified Ramsay Score of 4. Demographics and doses of sedatives were recorded and compared. Results: There were no sedation failures in either group. Children in group 1 (autism) were significantly older than group 2 (32.02±3.6 months vs 28.16±6.7 months) and weighed significantly more (14.87±2.1 kg vs 13.42±2.2 kg). When compared on a per-kilogram basis, however, group 1 had a significantly lower fentanyl requirement than group 2 (1.25±0.55 mcg/kg vs 1.57±0.81 mcg/kg), but no significant difference was found in pentobarbital dosing between groups 1 and 2, respectively (4.92±0.92 mg/kg vs 5.21±1.6 mg/kg). Conclusion: Autistic children in this age range are not more difficult to sedate and do not require higher doses of sedative agents for noninvasive imaging studies.

Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anaesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anaesthesia.

The use of office-based surgery and anesthesia will continue to grow. The anesthesia community has embraced the opportunity to become a driving force of office-based surgery and has organized into rapidly growing groups that promote safe practice in the office setting. The Society for Office-Based Anesthesia was developed to continuously improve patient safety and outcomes in office surgery. This group has an active Web site (www.soba.org) that allows for online discussions and widespread participation in working toward the society's stated goal. This Web site may be used as a reference for physicians in the process of considering the move to office-based anesthesia. The advantages of office-based anesthesia are numerous. The financial incentives are tremendous and the convenience to the patient and surgeon is important. For office anesthesia to be successful in children, patient safety, proof of improved outcomes, and family and surgeon satisfaction must be the goals. Anesthesia providers must continue to take active roles in organizing the office environment to ensure that safety is paramount. As the field grows, additional ways to study and improve the overall care children receive in the office should be sought. In the near future, office practice for surgery and anesthesia for children undergoing minor procedures should be a safe and effective alternative to current practices.

Context While the neuroanatomical basis of autism is not yet known, evidence suggests that brain enlargement may be characteristic of this disorder. Inferences about the timing of brain enlargement have recently come from studies of head circumference (HC). Objectives To examine brain volume and HC in individuals with autism as compared with control individuals. Design A cross-sectional study of brain volume was conducted at the first time point in an ongoing longitudinal magnetic resonance imaging study of brain development in autism. Retrospective longitudinal HC measurements were gathered from medical records on a larger sample of individuals with autism and local control individuals. Setting Clinical research center. Participants The magnetic resonance imaging study included 51 children with autism and 25 control children between 18 and 35 months of age (the latter included both developmentally delayed and typically developing children). Retrospective, longitudinal HC data were examined from birth to age 3 years in 113 children with autism and 189 local control children. Main Outcome Measures Cerebral cortical (including cortical lobes) and cerebellar gray and white matter magnetic resonance imaging brain volumes as well as retrospective HC data from medical records were studied. Results Significant enlargement was detected in cerebral cortical volumes but not cerebellar volumes in individuals with autism. Enlargement was present in both white and gray matter, and it was generalized throughout the cerebral cortex. Head circumference appears normal at birth, with a significantly increased rate of HC growth appearing to begin around 12 months of age. Conclusions Generalized enlargement of gray and white matter cerebral volumes, but not cerebellar volumes, are present at 2 years of age in autism. Indirect evidence suggests that this increased rate of brain growth in autism may have its onset postnatally in the latter part of the first year of life.

We attempted to evaluate a course on muscle and joint problems for general practitioners using a practice log diary and a factual test paper, which were completed both before and after the course by the doctors attending. A significant improvement was shown in the group score in the test paper taken after the course (p less than 0.001). Scores in the tests before and after had a negative correlation with age (p less than 0.001). In comparing the data in the second log diary with those in the first there was a significant reduction in the prescribing of drugs (p less than 0.001), use of heat treatment (p less than 0.001), and in "other" forms of treatment (p less than 0.01). No appreciable change in investigations or number of hospital referrals was shown. Those doctors who had low scores in the first factual test referred appreciably more patients to hospital and requested more x ray examinations (p less than 0.01).