Preoperative evaluation and comprehensive risk assessment for children with Down syndrome

Abstract

Down syndrome is a common chromosome disorder affecting all body systems. This creates unique physiologic concerns that can affect safety during anesthesia and surgery. Little consensus exists, however, on the best way to evaluate children with Down syndrome in preparation for surgery. We review a number of salient topics affecting these children in the perioperative period, including cervical spine instability, cardiovascular abnormalities, pulmonary hypertension, upper airway obstruction, hematologic disturbances, prematurity, low birth weight, and the use of supplements and alternative therapies. Recommendations include obtaining a complete blood count to detect an increased risk for bleeding or stroke, and cardiology evaluation to identify patients with pulmonary hypertension, as well as undiagnosed or residual heart disease. Pediatric cardiac anesthesiologists and intensivists should be involved as needed. The potential for cervical spine instability should be considered, and the anesthesiologist may wish to have several options available both for the medications and equipment used. The child's family should always be asked if he or she is on any nutritional supplements, as some products marketed to families may have secondary effects such as inhibition of platelet function. Using this evaluation in presurgical planning will allow physicians to better consider the individual circumstances for their patients with Down syndrome. Our goal was to optimize patient safety by choosing the most appropriate setting and perioperative personnel, and to mitigate those risk factors amenable to intervention.

Protocol study for a randomised, controlled, double-blind, clinical trial involving virtual reality and anodal transcranial direct current stimulation for the improvement of upper limb motor function in children with Down syndrome

“…1 The incidence in the USA is one out of every 700 births, and it is estimated that at least 100 thousand individuals in Brazil are diagnosed with the syndrome. [2][3][4] Structural and functional abnormalities are found in the nervous system of children with DS. Diffuse brain damage and peculiar electrical functioning during cognitive development result in poor analysis, synthesis and speech skills.…”

Cervical instability in patients with Trisomy 21: The eternal gamble

“…Patients with Trisomy 21 are particularly at risk of cervical instability due to ligamentous laxity and osseous abnormalities . Over half of Trisomy 21 patients may be affected by atlanto‐axial instability (AAI) or atlanto‐occipital instability (AOI), but only 1%‐2% are symptomatic …”

“…The radiologic assessment of cervical instability is not unanimously considered mandatory. The dynamic cervical spine radiograph is the most common screening tool to rule out cervical spine instability in patients with Trisomy 21 and is often performed before surgery . MRI and CT scans have also been employed less frequently, for the second intention.…”

El niño con Síndrome de Down en la Unidad de Cuidados Intensivos

“…que se presentan frecuentemente, o también por la necesidad de efectuar procedimientos diagnósticos prolongados, el niño con SD debe ser sometido a anestesia o sedación profunda en forma más reiterada que otros individuos, siendo necesario maximizar las condiciones antes, durante y después de la cirugía 58 . De esta manera, generalmente estos niños necesitan ser ingresados a la UCI para su recuperación.…”

“…Se debe colocar especial énfasis en la inestabilidad atlantoaxial 60 , hipertensión pulmonar, patología cardiovascular, obstrucción de la vía aérea superior, alteraciones hematológicas y antecedente de prematurez o bajo peso de nacimiento 58 .…”