Guideline for Children With Cancer Receiving General Anesthesia for Procedures and Imaging.

Children with cancer and those undergoing hematopoietic stem cell transplantation frequently require anesthesia for imaging as well as diagnostic and therapeutic procedures from diagnosis through follow-up. Due to their underlying disease and side effects of chemotherapy and radiation, they are at risk for complications during this time, yet no published guideline exists for preanesthesia preparation. A comprehensive literature review served as the basis for discussions among our multidisciplinary panel of oncologists, anesthesiologists, nurse practitioners, clinical pharmacists, pediatric psychologists, surgeons and child life specialists at the Children's Hospital of Philadelphia. Due to limited literature available, this panel created an expert consensus guideline addressing anesthesia preparation for this population.

Management of neonatal difficult airway emergencies in the delivery room.

Neonatal airway emergencies in the delivery room are associated with significant morbidity and mortality. Etiologies vary, but often predispose the neonate to life threatening airway obstruction. With the recent expansion of fetal medicine programs, pediatric anesthesiologists are increasingly being asked to care for these patients. In this review, we discuss common etiologies of difficult airway at delivery, management tools and techniques, and surgical approaches.

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.

Comparison of clinicians to radiologists in assessment of abdominal radiographs for suspected intussusception.

OBJECTIVES: The objectives of this study were to determine agreement of abdominal radiography (AXR) interpretation and to compare test characteristics, between pediatric emergency medicine (PEM) physicians and pediatric radiologists for evaluation of intussusception. METHODS: This was a retrospective cohort study of children aged 3 months to 3 years presenting to a pediatric emergency department (ED) between 2007 and 2009. For inclusion, subjects required an ED presentation for suspected intussusception, performance of a 2-view AXR (supine and upright/lateral decubitus views) and abdominal ultrasound performed during the ED visit, and a subsequent criterion-standard measure for intussusception available (contrast enema, operative report, or clinical follow-up). All AXRs were reviewed by 2 blinded PEM physicians and 2 pediatric radiologists. Interrater agreement (κ) for AXR interpretation and diagnostic test characteristics were calculated for comparison. RESULTS: A total of 286 children were included; intussusception was present in 61 patients (21.3%). Moderate agreement was present between the PEM physicians and radiologists for AXR assessment, with κ = 0.57 (95% confidence interval [CI], 0.47-0.66). Radiologist AXR interpretation had specificity of 86.7% (95% CI, 81.6-90.5), sensitivity of 62.3% (95% CI, 49.8-73.4), positive predictive value of 55.9% (95% CI, 43.3-67.9), and negative predictive value of 89.4% (95% CI, 84.6-93.2). Pediatric emergency medicine physician AXR interpretation had specificity of 68.9% (95% CI, 62.6-74.6), sensitivity of 78.7% (95% CI, 66.9-87.1), positive predictive value of 40.7% (95% CI, 32.2-49.7), and negative predictive value of 92.3% (95% CI, 87.1-95.5). CONCLUSIONS: Agreement between PEM physicians and pediatric radiologists for evaluation of AXR in suspected intussusception is moderate. Sensitivity and negative predictive value of AXR alone are not sufficiently high overall to exclude intussusception; however, PEM physician interpretation of AXR may possess utility in determining need for abdominal ultrasound in low-risk patients given the high negative predictive value.