From the Guidelines
Preoperative preparation for a pediatric patient with Down syndrome should include a thorough assessment of common comorbidities, particularly cardiac abnormalities, atlantoaxial instability, airway challenges, and pulmonary issues, to optimize perioperative planning and minimize risks. When evaluating pediatric patients with Down syndrome preoperatively, it is essential to consider the high risk of sleep-disordered breathing (SDB) and obstructive sleep apnea (OSA), as highlighted in a clinical practice guideline on polysomnography for SDB prior to tonsillectomy in children 1. This guideline emphasizes the importance of obtaining polysomnography (PSG) prior to tonsillectomy in children with conditions such as Down syndrome, as history and physical exam alone are poor predictors of OSA severity or risk of postoperative complication. Key considerations in the preoperative assessment include:
- Cardiac abnormalities, which are present in 40-60% of patients and may require echocardiography and cardiology clearance before surgery
- Atlantoaxial instability, affecting 10-30% of Down syndrome patients, which necessitates careful neck positioning during anesthesia
- Airway challenges, including macroglossia, midface hypoplasia, and subglottic stenosis, which may complicate intubation
- Pulmonary issues, such as obstructive sleep apnea and increased risk of respiratory infections
- Thyroid function, as hypothyroidism is common in Down syndrome patients Premedication with midazolam (0.5 mg/kg orally, maximum 20 mg) may help with anxiety, but use cautiously due to potential airway obstruction 1. Allowing parents or caregivers to accompany the child to the operating room when possible can help reduce anxiety. Clear communication with the anesthesia team about all identified concerns, particularly cardiac and airway issues, is crucial for safe perioperative care. Postoperatively, close monitoring for respiratory complications and appropriate pain management are essential, recognizing that patients with Down syndrome may have altered pain expression.
From the Research
Preoperative Evaluation for Down Syndrome Patients
- A comprehensive risk assessment is necessary for children with Down syndrome undergoing surgery, considering their unique physiologic concerns that can affect safety during anesthesia and surgery 2.
- The evaluation should include 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 2.
Anesthesia and Sedation Considerations
- The pharmacokinetics and pharmacodynamics of IV midazolam after cardiac surgery do not differ significantly between children with and without Down syndrome, suggesting that midazolam dosing does not need to be adjusted for children with Down syndrome 3.
- However, 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 2.
Hematologic Considerations
- Children with Down syndrome are more likely to have hematologic and immunologic abnormalities compared to their typically developing peers, and normal ranges for complete blood counts (CBCs) have been defined for this population 4.
- Iron deficiency and iron deficiency anemia are common in children with Down syndrome, and a complete blood count and ferritin should be obtained routinely for children over 1 year old with Down syndrome for detection of iron deficiency 5.
Perioperative Care
- The life expectancy of people with Down syndrome has increased, and perioperative nurses should be aware of the challenges associated with caring for patients with Down syndrome, including patient assessment, communication, and patient safety 6.
- A thorough understanding of the pathophysiology of Down syndrome and its associated comorbidities is essential for providing optimal perioperative care for patients with Down syndrome 6.