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Background X‐linked adrenoleukodystrophy is a progressive demyelinating disease that primarily affects males with an incidence of 1:20 000‐30 000. The disease has a wide spectrum of phenotypic expression and may include adrenal insufficiency, cerebral X‐linked adrenoleukodystrophy and adrenomyeloneuropathy. The condition has implications for the administration of anesthesia and reports of anesthetic management in those patients are limited at this point. Aim To review the perioperative care, complications and outcomes of patients diagnosed with X‐linked adrenoleukodystrophy at the University of Minnesota Masonic Children's Hospital. Method After obtaining IRB approval, we performed a retrospective chart review of pediatric patients diagnosed with X‐linked adrenoleukodystrophy who underwent either surgery or diagnostic/therapeutic procedures that included anesthesia services between January 2014 and December 2016. Data included demographics, American Society of Anesthesiologists classification, preoperative diagnosis, history of hematopoietic stem cell transplant, anesthetic approaches, airway management, medications used, intra‐ and postoperative complications, and patient disposition. Results We identified 38 patients who had a total of 166 anesthetic encounters. The majority of patients underwent procedures in the sedation unit (75.9%) and received a total intravenous anesthetic with spontaneous ventilation via a natural airway (86.1%). Preoperative adrenal insufficiency was documented in 87.3% of the encounters. Stress‐dose steroids were administered in 70.5% of the performed anesthetics. A variety of anesthetic agents were successfully used including sevoflurane, isoflurane, propofol, midazolam, ketamine, and dexmedetomidine. There were few perioperative complications noted (6.6%) and the majority were of low severity. No anesthesia‐related mortality was observed. Conclusions With the availability of skilled pediatric anesthesia care, children with X‐linked adrenoleukodystrophy can undergo procedures under anesthesia in sedation units and regular operating rooms with low overall anesthesia risk.

To safely care for newborns in the operating room, anesthesiologists need a thorough understanding of the neonatal physiology at different gestational ages and anticipate how immature organs might respond to surgery and anesthetic agents. There are differences in neonatal, pediatric, and adult physiology. The neonates have transitional circulation during the first few weeks of life, and various factors need to be considered to avoid reversion to fetal circulation. There are major differences in the neonatal and adult airway that require modification in the choice of equipment and intubation technique. It is important to know that the respiration is less efficient in neonates, and they are more prone to hypoxia. Most of the major organs, such as kidneys, liver, and heart, are immature at birth and develop at different rates as the neonate grows. Similarly, metabolism and fluid distribution are very different from adults. This involves consideration with the use of anesthetic agents and management in the operating room. Neonates are especially vulnerable to intraoperative hypothermia. Preoperative evaluation is arguably the most important part of anesthetic care. The purpose of this chapter is to allow anesthesia providers to administer safe care to newborns based on an understanding of developmental physiology.

Commonly performed pediatric ophthalmologic procedures performed under anesthesia include eye examination for tonometry, fundoscopy, and assessment of visual evoked potentials, cryotherapy or laser therapy for retinopathy of prematurity or retinoblastoma, strabismus repair, lens extraction in cataracts, and enucleation, which are predominantly performed in the ambulatory setting. However, special considerations are needed in premature infants with congenital disorders associated with pathology of the eye. An understanding of oculocardiac reflex (OCR) and postoperative nausea and vomiting (PONV) is more pertinent to pediatric ophthalmic surgeries. Differing from adult ophthalmic surgeries, use of regional blocks are not commonly used. Therefore, perioperative pain management needs to be considered, especially with more invasive procedures. This chapter describes the anatomy and physiology of the eye and the perioperative anesthetic management of commonly performed ophthalmic procedures in the pediatric population.

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