Thorough Preop Eval – 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. Check head/neck range of motion.

Difficult airways – Anticipate and prepare for airway obstruction. Down syndrome (DS) patients have a reduced ability to maintain their airway due to generalized hypotonia. Other anatomical characteristics such as an enlarged tongue difficult airway culprits.

Endotracheal Tube (ETT) considerations – Use a smaller ETT (1 size smaller than usual). Make sure at the beginning of the case to inflate the ETT cuff only as much as you need to (ideally have a confirmed leak at 20 cm H2O). Awake extubation is preferred due to possibilities of airway obstruction / difficult airway.

Strict aseptic technique – these patients are very often immunocompromised. Be aware of this when accessing/placing anything invasive.

Video Laryngoscope / Fiberoptic Intubation – It’s best to prepare for a difficult airway, which is common in these patients.

Avoid increases in pulmonary vascular resistance (PVR) in patients with acyanotic congenital heart defects (CHD)

Common intraoperative complications to be aware of – Severe bradycardia, airway obstruction, and bronchospasm

Drugs – 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.

Avoid Head/Neck Manipulation – Cervical instability makes these patients especially susceptible to injury if the head and neck are manipulated during intubation.

Keep normothermic – hypothermia is common due to hypothyroidism

High risk for post-extubation stridor and croup – this may lead to prolonged desaturation, apnea, or airway obstruction. Treatment includes humidified O2, inhaled racemic epinephrine, and IV steroids.

Parent/Caregiver Presence – Caregiver presence can significantly reduce agitation. Points where this is especially helpful is preoperatively during the IV start and postoperatively at wakeup.

Post-operative agitation – if possible, have the parent/caregiver present. Thorough management of pain will also go a long way.

From the Pros – It has been suggested that having some sedation meds in the IV line prior to IV start can help to calm the patient immediately after the IV has been started. Nasal midazolam is also suggested as a good way to sedate prior to IV placement.

DS is the most common congenital chromosomal abnormality. Approximately 1:800 births worldwide. This accounts for 6000 births per year in the US. Prevalence is linked to advanced maternal age.

Symptomology by System:

Neurologic: Moderate to severe mental retardation, developmental delay, attention deficit and autism in childhood, depression and OCD in adulthood, seizures, vision impairments from nystagmus and cataract formations, hearing loss from chronic otitis media. Childhood milestones (siting, crawling, walking, and first words) are reached between 5 – 13 months behind children without DS.

Cardiovascular: Congenital heart defects are present in 40-50% of Down Syndrome patients. Of those suffering from defects, 55% will have patent ductus arteriosus, 50% will have atrioventricular septal defects, 35% will have ventricular septal defects, and 10% will have atrial septal defects. Life expectancy and quality of life for DS patients is generally determined by how severe the underlying heart anomalies are.

Musculoskeletal: Generalized hypotonia (reduced ability to maintain the airway). Increased joint laxity. This puts these patients at greater risk for cervical spine instability, subluxation, and spinal cord compression. Laxity in the atlantoaxial and occipitoatlantal joint can cause permanent neurologic injury when manipulating the position of the head and neck. Temporomandibular joint laxity may also be present. Large tongues in these patients make intubation difficult.

GI: Duodenal atresia (which is a culprit for GERD), tracheoesophageal fistula, or omphalocele may be present.

Urinary: Renal hypoplasia

Endocrine: Hypothyroidism, diabetes, and reduced SNS activity and circulating catecholamines

Blood: Anemia, polycythemia, immune system deficiency, and heightened risk for leukemia