Down syndrome and the autonomic nervous system, an educational review for the anesthesiologist

Abstract

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.

Airway Management and General Anesthesia in Pediatric Patients with Disabilities Undergoing Dental Surgery: A Retrospective Study

“…In addition, a noncooperative pediatric patient may present with peculiarities directly related to the underlying pathology: for those with autism spectrum disorder difficulties in cooperation and communication and possible drug interactions between home therapy and drugs administered for anesthetic premedication and anesthesia [12,13]; for those with Down's syndrome the presence of congenital heart defects, peculiar craniofacial anatomical features, atlantoaxial instability, and obesity [13][14][15]; for those with infantile cerebral palsy or spastic tetraparesis sialorrhea, scoliosis, gastroesophageal reflux, and low subcutaneous fat [16,17]; finally, for those with epilepsy there may be drug interactions between antiepileptic therapy and drugs administered as anesthetic premedication or as anesthetics, with an increased risk of seizures [18].…”

Autonomic Nervous System Monitoring: Cardiac Magnetic Resonance Imaging Data as a Surrogate for Autonomic Data in Children

“…Adults with Down Syndrome are known to have sympathetic failure, which would be manifested as a lengthening of PEP compared to typically developed adults. At what age this occurs and to what degree have yet to be characterized [ 2 ]. For these reasons, precision medicine, using each patient as his own control and trending autonomic values over time, may document heart disease trajectories (albeit with impact due to factors other than heart disease).…”

“…When available, it was often too invasive for routine monitoring. However, technological and research advances have increased the availability and utility of autonomic function measurement [ 1 , 2 ]. Age-related normative values for ANS measurements during maturation have become available [ 3 ].…”

Ocular Cyclopentolate: A Mini Review Concerning Its Benefits and Risks

“…Special care must be taken during cyclopentolate use in patients with certain conditions due to the possibility of severe ADRs, 74,78,93,94 ie children (tachycardia, language problems, feeding intolerance, seizures, toxicity in CNS, delirium, ataxia, decreased motility, necrotizing enterocolitis, paralytic ileus), 21,68,78,[83][84][85][86]90,94 geriatrics (toxicity in CNS, delirium, dementia). 68,74,86 Patients with brain damage (psychotic reactions, seizures, memory loss, ataxia, disorientation, language problems), 74,78,[83][84][85]102 patient with low blood level of pseudocholinesterase (seizures), 83,92,103 narrow anterior chamber or glaucoma patients (increased of intraocular pressure, angle-closure glaucoma), 1,68,78,104,105 and users of substances with CNS effects (amantadine: confusion, hallucinations; belladonna: excessive sedation, dry mouth, constipation, reduced urination; fluvoxamine: acute psychotic reactions). 68,80…”

“…Premature 21,68,76,82,84,92 Cardiovascular, nervous, digestive, metabolic and excretory system immaturity Lower blood volume Increase brain-blood permeability Geriatrics 68,92 Impaired nervous system Epilepsy Brain damage Down's syndrome 68,76,83,84,86,94,102 Disjointed stimulation of the nervous system…”