Approximately one in every 700 babies in the United States is born with Down syndrome, or 0.14%. Children with Down syndrome have cognitive impairment and congenital malformations necessitating frequent occurrences of general anesthesia and surgery. The thoughtful perioperative care of children with Down syndrome is relevant and acutely complex for the pediatric anesthesiologist. Behavior, sedation, hypotonia, upper airway obstruction, venous access, and bradycardia are omnipresent concerns apart from the surgical pathology. Down syndrome is also associated with autonomic nervous system dysfunction, a comorbidity that is overlooked in discussions of perioperative care and is described thus far in adults. Autonomic nervous system function or dysfunction may explain the phenotypical features of the perioperative challenges listed above. For this reason, understanding the development and measurement of autonomic nervous system function is important for the pediatric anesthesiologist. Definition and quantification of sympathetic and parasympathetic function will be reviewed.
Clinical researchers studying the long-term neurocognitive effects of anesthetic and sedative agents on children continue to struggle with identifying a phenotype for anesthetic neurotoxicity, the window of vulnerability, and the toxicity threshold in terms of concentration and duration. The Sixth Biennial Pediatric Anesthesia Neurodevelopment Assessment (PANDA) symposium at Columbia University included a moderated poster presentation session where 4 investigators presented their latest contributions to the landscape of clinical anesthetic neurotoxicity research. A lack of standardization in the design of clinical studies in terms of age at exposure, duration and type of exposure, and outcome measures assessed were highlighted by all the investigators. Suggestions for the future direction of clinical trials included the implementation of more consistent study parameters and the employment of standardized neurocognitive testing and imaging before and after exposure to general anesthesia. Presentations covered a broad range of topics including the valid translation of preclinical studies to human subjects, the quantification of real-world exposures to anesthetic and sedative medications, and possible alternatives to these exposures.
Cyanotic congenital heart disease (CCHD) is often complex, with multiple lesions present in a single heart. CCHD accounts for 1300 per million live births with congenital heart disease. This review covers the physiology of chronic cyanosis and adaptations and the common cyanotic heart diseases: hypoplastic left heart syndrome (HLHS), d-transposition of great arteries (d-TGA), and tetralogy of Fallot (TOF). Medical and surgical treatments as well as the usual palliative course are reviewed. Additionally, the spectrum of disease in TOF is classified. Late complications of these diseases are mentioned ; they are reviewed in the chapter on chronic congenital heart disease.
Perioperative autonomic nervous system (ANS) measurements are evolving toward increasing import and utility. We present a three-year-old male with Down Syndrome who underwent ambulatory autonomic monitoring during surgery followed by cardiac magnetic resonance (CMR) imaging. Autonomic data from both environments are compared to age-related norms. We are the first to describe a method for acquiring and trending autonomic data from clinically indicated CMR scans in order to monitor autonomic function. These data are proof of concept for the use of routinely collected CMR data as a surrogate for autonomic data in children, noting differences in the autonomic effects of anesthetic techniques.
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