A retrospective observational cohort study of the anesthetic management and outcomes of pediatric patients with Alexander disease undergoing lumbar puncture or magnetic resonance imaging.

BACKGROUND: Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited. AIMS: Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia-related complications for Alexander disease patients undergoing magnetic resonance imaging and/or lumbar puncture at a quaternary-care children's hospital. METHODS: We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24 h. We performed descriptive statistics as appropriate. RESULTS: Forty patients undergoing 64 procedures met inclusion criteria. Fifty-six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia/MAC group tended to be younger than nonanesthetized patients (median age 6 years [IQR 3.8; 9] vs. 14.5 years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (N = 48; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty-three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care. CONCLUSIONS: In this single-center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia/MAC. Co-morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.

Imaging sedation and anesthesia practice patterns in pediatric radiology departments - a survey of the Society of Chiefs of Radiology at Children's Hospitals (SCORCH).

BACKGROUND: There are few data describing practice patterns related to the use of sedation/anesthesia for diagnostic imaging in pediatric radiology departments. OBJECTIVE: To understand current practice patterns related to imaging with sedation/anesthesia in pediatric radiology departments based on a survey of the Society of Chiefs of Radiology at Children's Hospitals (SCORCH) in conjunction with the American College of Radiology's Pediatric Imaging Sedation and Anesthesia Committee. MATERIALS AND METHODS: A multi-question survey related to imaging with sedation/anesthesia in pediatric radiology departments was distributed to SCORCH member institutions in January 2019. A single reminder email was sent. Descriptive statistical analyses were performed. RESULTS: Of the 84 pediatric radiology departments, 23 (27%) completed the survey. Fifty-seven percent of the respondents self-identified as academic/university-affiliated and 13% as a division/section in an adult radiology department. Imaging sedation (excluding general anesthesia) is commonly performed by pediatric anesthesiologists (76%) and intensive care unit physicians (intensivists, 48%); only 14% of departments expect their pediatric radiologists to supervise imaging sedation. Ninety-six percent of departments use child life specialists for patient preparation. Seventy percent of departments have preparatory resources available on a website, including simulation videos (26%) and audio clips (17%). Nearly half (48%) of the departments have a mock scanner to aid in patient preparation. Imaging sedation/anesthesia is most often scheduled at the request of ordering clinicians (65%), while 57% of departments allow schedulers to place patients into imaging sedation/anesthesia slots based on specified criteria. CONCLUSION: Imaging sedation/anesthesia practice patterns vary among pediatric radiology departments, and understanding current approaches can help with standardization and practice improvement.

Clinical pediatric positron emission tomography/magnetic resonance program: a guide to successful implementation.

Children with malignancies undergo recurrent imaging as part of tumor diagnosis, staging and therapy response assessment. Simultaneous positron emission tomography (PET) and magnetic resonance (MR) allows for decreased radiation exposure and acts as a one-stop shop for disease in which MR imaging is required. Nevertheless, PET/MR is still less readily available than PET/CT across institutions. This article serves as a guide to successful implementation of a clinical pediatric PET/MR program based on our extensive clinical experience. Challenges include making scanners more affordable and increasing patient throughput by decreasing total scan time. With improvements in workflow and robust acquisition protocols, PET/MR imaging is expected to play an increasingly important role in pediatric oncology.

Guidelines and checklists for short-term missions in global pediatric surgery: Recommendations from the American Academy of Pediatrics Delivery of Surgical Care Global Health Subcommittee, American Pediatric Surgical Association Global Pediatric Surgery Committee, Society for Pediatric Anesthesia Committee on International Education and Service, and American Pediatric Surgical Nurses Association, Inc. Global Health Special Interest Group.

Pediatric surgeons, anesthesia providers, and nurses from North America and other high-income countries are increasingly engaged in resource-limited areas, with short-term missions as the most common form of involvement. However, consensus recommendations currently do not exist for short-term missions in pediatric general surgery and associated perioperative care. The American Academy of Pediatrics (AAP) Delivery of Surgical Care Subcommittee and American Pediatric Surgical Association (APSA) Global Pediatric Surgery Committee, with the American Pediatric Surgical Nurses Association, Inc. (APSNA) Global Health Special Interest Group, and the Society for Pediatric Anesthesia (SPA) Committee on International Education and Service generated consensus recommendations for short-term missions based on extensive experience with short-term missions. Three distinct, but related areas were identified: (i) Broad goals of surgical partnerships between high-income countries and low- and middle-income countries. A previous set of guidelines published by the Global Paediatric Surgery Network Collaborative (GPSN) was endorsed by all groups; (ii) Guidelines for the conduct of short-term missions were developed, including planning, in-country perioperative patient care, post-trip follow-up, and sustainability; and (iii) travel and safety considerations critical to short-term mission success were enumerated. A diverse group of stakeholders developed these guidelines for short-term missions in low- and middle-income countries. These guidelines may be a useful tool to ensure safe, responsible, and ethical short-term missions given increasing engagement of high-income country providers in this work.

A multidisciplinary approach to dosing nusinersen for spinal muscular atrophy.

BACKGROUND: In December 2016, nusinersen gained FDA approval as the first pharmacologic treatment for spinal muscular atrophy (SMA), a disorder of motor neurons and the leading genetic cause of infant mortality. Nusinersen's intrathecal delivery requirement, strict dosage protocol, and accelerated FDA approval presented a challenge to health care centers hoping to implement treatment of patients with SMA. Scheduling logistics, combined with the specific ventilatory, anesthetic, and spinal access needs of this patient population, requires extensive coordination of care. This complexity, in addition to the high cost of treatment, may lead to overburdening of an institution's dosing resources, causing delays in treatment initiation and limiting patients' access to therapy and may result in barriers to coverage. METHODS: We initiated a comprehensive stepwise protocol to maximize patient inclusion, as well as safety and efficiency outcome measures. This retrospective cohort study reviews the dosing process. RESULTS: As a result of immense collaborative efforts involving care coordination of patients and families, in addition to health providers in the divisions of neurology, anesthesiology, pulmonology, orthopedics, interventional radiology, physical therapy, and neurosurgery, we have successfully dosed 62 SMA patients. Throughout this process, we have improved anesthetic techniques, as well as minimized procedural complications and missed scheduled doses. CONCLUSION: We present here recommendations for safe and effective implementation of nusinersen utilizing a multidisciplinary approach, based on our 1 and a half year experience at a tertiary care children's hospital.

Difficult airway management in children and young adults with arthrogryposis.

OBJECTIVE: To review current evidence and experience with anesthesia and airway management issues in children and young adults with arthrogryposis. DATA SOURCES: Review of existing world literature and description of personal experience at a center for children's orthopedic surgery and rehabilitation over 2 decades. METHODS: Description of common problems and their solutions in this unusual and diverse group of patients. RESULTS: Arthrogryposis multiplex congenital includes more than 400 conditions that lead to congenital joint contractures affecting more than one body area. Among the many causes of arthrogryposis, 50%-65% fall into two large categories - amyoplasia and distal arthrogryposis. There is general agreement that best function in children with arthrogryposis is achieved through early mobilization of joint contractures. Children with arthrogryposis average >5 operative procedures during childhood. Anesthesia for these procedures may be complicated by limited jaw mobility and mouth opening, restricted lung development, positioning difficulties, difficult venous access and concerns about increased risk for malignant hyperthermia. 75% of arthrogryposis patients do not have a difficult airway. For those with a history of airway problems or those meeting criteria for a difficult airway, careful advanced planning helps to assure safe and successful surgery. We describe several specialized techniques for endotracheal intubation of children with arthrogryposis. CONCLUSIONS: Children and young adults with arthrogryposis are a diverse group. Many pose unique challenges for airway and surgical management. Review of individual anesthesia records and careful advanced planning by a coordinated, experienced airway team can lead to best outcomes from arthrogryposis surgery.

Guidelines and checklists for short-term missions in global pediatric surgery: Recommendations from the American Academy of Pediatrics Delivery of Surgical Care Global Health Subcommittee, American Pediatric Surgical Association Global Pediatric Surgery Committee, Society for Pediatric Anesthesia Committee on International Education and Service, and American Pediatric Surgical Nurses Association, Inc. Global Health Special Interest Group.

INTRODUCTION: Pediatric surgeons, anesthesia providers, and nurses from North America and other high-income countries (HICs) are increasingly engaged in resource-limited areas, with short-term missions (STMs) as the most common form of involvement. However, consensus recommendations currently do not exist for STMs in pediatric general surgery and associated perioperative care. METHODS: The American Academy of Pediatrics (AAP) Delivery of Surgical Care Subcommittee and American Pediatric Surgical Association (APSA) Global Pediatric Surgery Committee, with the American Pediatric Surgical Nurses Association, Inc. (APSNA) Global Health Special Interest Group, and the Society for Pediatric Anesthesia (SPA) Committee on International Education and Service generated consensus recommendations for STMs based on extensive experience with STMs. RESULTS: Three distinct, but related areas were identified: 1) Broad goals of surgical partnerships between HICs- and low and middle-income countries (LMICs). A previous set of guidelines published by the Global Paediatric Surgery Network Collaborative (GPSN), was endorsed by all groups; 2) Guidelines for the conduct of STMs were developed, including planning, in-country perioperative patient care, post-trip follow-up, and sustainability; 3) travel and safety considerations critical to STM success were enumerated. CONCLUSION: A diverse group of stakeholders developed these guidelines for STMs in LMICs. These guidelines may be a useful tool to ensure safe, responsible, and ethical STMs given increasing engagement of HIC providers in this work. LEVEL OF EVIDENCE: 5.

The use of somatosensory evoked potentials to determine the relationship between patient positioning and impending upper extremity nerve injury during spine surgery: a retrospective analysis.

Somatosensory evoked potential (SSEP) monitoring is used to prevent nerve damage in spine surgery and to detect changes in upper extremity nerve function. Upper extremity SSEP conduction changes may indicate impending nerve injury. We investigated the effect of operative positioning on upper extremity nerve function retrospectively in 1000 consecutive spine surgeries that used SSEP monitoring. The vast majority (92%) of upper extremity SSEP changes were reversed by modifying the arm position and were therefore classified as position-related. The incidence of position-related upper extremity SSEP changes was calculated and compared for five different surgical positions: supine arms out, supine arms tucked, lateral decubitus position, prone arms tucked, and the prone "superman" position. The overall incidence of position-related upper extremity SSEP changes was 6.1%. The lateral decubitus position (7.5%) and prone superman position (7.0%) had a significantly more frequent incidence of position-related upper extremity SSEP changes (P < 0.0001, Z-test for Poisson counts) compared with other positions (1.8%-3.2%). No patient with a reversible SSEP change developed a new postoperative deficit in the affected extremity. SSEP monitoring is of value in identifying and reversing impending upper extremity peripheral nerve injury.

Surgical missions to developing countries: Ethical conflicts.

Each year scores of American physicians and nurses travel overseas, usually at their own expense, aiming to improve the lot of desperate patients in developing countries. Our journals are filled with images of smiling children who have benefited from these gifts of care. Still, practicing medicine, and especially surgery, in a sporadic fashion in distant lands can lead to poor outcomes. It does little to improve public health or advance medical education. We address some of the ethical dilemmas intrinsic to international surgical missions and discuss how we might redirect our resources to provide better care to more people.

Anesthesia in a patient with chromosome 11;22 translocation: a case report and literature review.

Chromosome 11;22 translocation is a rare genetic condition, which results in characteristic features some of which may present problems when these children require surgery and anesthesia. We describe a child with this chromosomal variant who presented for surgery and anesthesia. The case report and review of the literature is presented here.