- Full assessment: Get as many details from the patient and parents as possible about the affect/severity on the body’s systems, medical regimen, and baseline behaviors/responses. Check for recent cardiac evals, EKG, ECHO, etc.
- Mental Disabilities: A significant challenge in managing anesthesia for patients with mental disabilities includes their limited cooperation and potential for aggressive behavior, complicating the delivery of proper anesthetic care. The combined oral administration of ketamine and midazolam has been proven effective in children and patients who are uncooperative, leveraging their amnesic, sedative, and anxiolytic effects. Additionally, using these drugs together mitigates the psychotomimetic and cardiovascular side effects commonly associated with ketamine alone. Funk and colleagues have shown that this drug combination results in fewer adverse effects compared to administering each drug separately. For combative patients, oral dosing of these medications is not only convenient but also effective and safe.
- Subjective Pain Assessments: The challenge of identifying pain and the limited ability of individuals with DS to express themselves render self-reporting tools for pain assessment less suitable. While research has primarily focused on pediatric patients with DS, the COMFORT-Behavior scale stands out as the sole validated instrument for pain assessment within this specific clinical group.
- Airway Management: The presence of physical traits such as a smaller oropharynx and trachea, enlarged tongue, and laxity of neck tissues can complicate bag-masking and intubation. Awake intubation is typically excluded because of poor patient cooperation. Recommendations include ensuring the availability of a smaller endotracheal tube and oral airway. Perform intubation with a video laryngoscope or have one in the room. Extubate “awake” rather than “deep”, carefully titrate and minimize long acting opioids
- Line Placements: In individuals with DS, achieving vascular access can pose challenges, potentially necessitating the use of ultrasound guidance. Furthermore, when considering the placement of an arterial line, it's important to note that some patients with DS may have hypoplastic or anomalous radial arteries.
- Drug considerations: Children with DS experience altered sensory responses that can significantly affect their perioperative management during general anesthesia, sedation, and pain control. Their reactions to the anesthesia induction agent sevoflurane are notably different from those of children without DS. Children with DS exhibit a higher rate and severity of bradycardia, as well as more significant reductions in heart rate from their baseline. The response of children with DS to medications such as midazolam and dexmedetomidine also varies. Dexmedetomidine, a central alpha-2 agonist, effectively lowers sympathetic nervous system activity. After surgery, these children face a heightened risk of complications like bradycardia and hypotension when given dexmedetomidine. Increased parasympathetic function, as measured by the Newborn Infant Parasympathetic Evaluation (NIPE™) has also been observed. These patients typically have a hypersensitivity to atropine. Use an opioid-sparing analgesic technique or use short-acting opioids to avoid long wake-ups and/or airway obstruction. Less muscle relaxant may be necessary due to generalized hypotonia. There haven’t been any reported significant altered responses to anesthesia drugs.
- Atlantoaxial Instability: A careful assessment and minimization of neck movement during anesthesia are critical.
- Monitoring for Complications: Given the elevated risk of cardiac, respiratory, and endocrine complications, vigilant perioperative, thorough preoperative assessments and monitoring perioperatively is essential to prevent, promptly identify, and manage potential issues.
- Warm the Patient: Patients with DS may be unable to regulate their own body temperature, a problem of which will be exacerbated by anesthetic drugs.
- Consolidate Surgical Procedures: Where possible group surgical procedures for individuals with DS due to the inherent risks and challenges associated with the condition.
| Domain | Issue | Approach |
|---|---|---|
| Airway | Smaller oropharynx and trachea, enlarged tongue, and laxity of neck tissues complicate bag-masking and intubation; awake intubation is typically excluded because of poor cooperation | Have a smaller endotracheal tube and oral airway available; intubate with a video laryngoscope or have one in the room; extubate "awake" rather than "deep". |
| Cervical spine | Roughly 20 percent have excessive movement between the first two cervical vertebrae, raising the risk of spinal subluxation and neurological harm | Careful assessment and minimization of neck movement during anesthesia. |
| Premedication | Limited cooperation and potential for aggressive behavior complicate delivery of anesthetic care | Oral ketamine combined with midazolam is effective in children and uncooperative patients, and the combination mitigates ketamine's psychotomimetic and cardiovascular side effects. |
| Induction agent | In children with Down syndrome, a higher rate and severity of bradycardia with sevoflurane, and more significant reductions in heart rate from baseline | Anticipate the exaggerated bradycardic response. |
| Atropine | Patients typically have a hypersensitivity to atropine | Account for the exaggerated response. |
| Dexmedetomidine | In children with Down syndrome, heightened postoperative risk of bradycardia and hypotension, alongside increased parasympathetic function | Use with awareness of the altered autonomic response. |
| Opioids and relaxants | Long wake-ups and airway obstruction; generalized hypotonia | Use an opioid-sparing technique or short-acting opioids; less muscle relaxant may be necessary. |
| Vascular access | Access can be difficult, and some patients have hypoplastic or anomalous radial arteries | Consider ultrasound guidance, including before arterial line placement. |
| Pain assessment | Difficulty identifying pain and limited ability to self-report make self-reporting tools less suitable | The COMFORT-Behavior scale is the sole validated instrument in this group, studied mainly in pediatric patients. |
| Temperature | Patients may be unable to regulate their own body temperature, a problem exacerbated by anesthetic drugs | Warm the patient. |
