Anesthesia Considerations for Patients with Down Syndrome

Down syndrome is the most common chromosomal disorder, occurring in around 1 out of every 800 live births [1]. The condition occurs when chromosome 21 fails to separate properly during cell division, resulting in patients bearing three copies of the chromosome rather than two [2]. Down syndrome affects a variety of symptoms and body systems in a manner relevant to anesthesiologists, and doctors may need to pay particular attention to cardiac and upper respiratory issues when administering anesthesia to these patients.  

A retrospective review of Down syndrome and non-Down syndrome patients undergoing non-cardiac surgeries found that the Down syndrome group was significantly likelier to suffer complications from bradycardia (at a rate of 3.66%), airway obstruction, (at a rate of 1.83%), and post-intubation croup (also 1.83%) [3]. Another study found that post-intubation stridor and bronchospasm were the most common complications following bradycardia and airway obstruction, at rates of 1.8% and 0.4% respectively [4].  

Roughly 50% of pediatric Down syndrome patients have congenital heart defects, heightening their risk of cardiac complications. Meanwhile, the difficulty of inserting an IV catheter into Down syndrome patients has led to inhaled anesthetics as a common alternative. However, the inhalation of sevoflurane can have cardiac-related effects, particularly bradycardia and hypotension [5]. Kraemer et al. retrospectively analyzed the anesthesia records of child patients, with and without Down syndrome, who were administered sevoflurane. They found that children with Down syndrome experienced bradycardia and hypotension at an incidence of 57%, compared to only 12% of those in the control group. Another retrospective analysis by Bai et al., also focused on pediatric patients, similarly found significantly higher rates of these two cardiac effects in Down syndrome patients. The authors note that reduced amounts of sevoflurane helped to lower incidences of both effects [6]. 

Down syndrome causes a variety of abnormalities in the upper respiratory tract, leading to a high likelihood of related complications as well as difficulty intubating. Patients commonly have macroglossia and micrognathia, as well as tonsillar and adenoidal hypertrophy, among other conditions. Airway obstruction and breathing-related sleep disorders such as sleep apnea are common, affecting between 50% and 100% of children with Down syndrome, and nearly 100% of adults [7]. In order to keep resulting complications to a minimum, anesthesiologists often choose to conduct an especially thorough review of past respiratory infections and complications prior to intubation [4]. Because subglottic stenosis occurs frequently, anesthesiologists may choose to intubate with a narrower tube. Furthermore, because those with Down syndrome are prone to infections, including pneumonia, hospitalization after typically uncomplicated procedures is not uncommon. Patients with a history of croup may be more suited to methods of inducing anesthesia that do not involve endotracheal intubation [4]. 

When administering anesthesia to patients with Down syndrome, doctors may elect to avoid intubation altogether, although inhaled anesthesia can introduce cardiac concerns. Therefore, anesthesiologists must pay close attention to the patient’s individual respiratory and cardiac histories, as well as to elements of their airway structure that might impede or complicate intubation, prior to the procedure. 

References 

[1] Oliveira, Mariana Brito, and Humberto S Machado. “Perioperative Management of Patients with Down syndrome: A Review.” Journal of Anesthesia & Clinical Research, vol. 09, no. 04, 2018, doi:10.4172/2155-6148.1000816.  

[2] Underwood, Emily. “Can Down Syndrome Be Treated?” Science, vol. 343, no. 6174, 2014, pp. 964–967. JSTOR, www.jstor.org/stable/24743351. 

[3] Borland, Lawrence M., et al. “Frequency of Anesthesia-Related Complications in Children with Down Syndrome under General Anesthesia for Noncardiac Procedures.” Pediatric Anesthesia, vol. 14, no. 9, 2004, pp. 733–738., doi:10.1111/j.1460-9592.2004.01329.x.  

[4] Steward, David J. “Anesthesia Considerations in Children with Down Syndrome.” Seminars in Anesthesia, Perioperative Medicine and Pain, vol. 25, no. 3, 2006, pp. 136–141., doi:10.1053/j.sane.2006.05.001.  

[5] Kraemer, F. Wickham, et al. “Bradycardia During Induction of Anesthesia with Sevoflurane in Children with Down Syndrome.” Anesthesia & Analgesia, vol. 111, no. 5, 2010, pp. 1259–1263., doi:10.1213/ane.0b013e3181f2eacf.  

[6] Bai, Wenyu, et al. “Hemodynamic Changes in Children with Down Syndrome during and Following Inhalation Induction of Anesthesia with Sevoflurane.” Journal of Clinical Anesthesia, vol. 22, no. 8, 2010, pp. 592–597., doi:10.1016/j.jclinane.2010.05.002. 

[7] Simpson, Ryne et al. “Obstructive sleep apnea in patients with Down syndrome: current perspectives.” Nature and Science of Sleep vol. 10 287-293. 13 Sep. 2018, doi:10.2147/NSS.S154723.