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Anesthesia & Airway Management

Considerations for Physicians

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Children with CdLS tend to have a variety of procedures performed during their lifetime. Depending on the procedure being performed, different anaesthetic techniques may be chosen. These recommendations are based on responses to a survey on the airway

and anesthetic management of children with CdLS. The survey primarily dealt with response to medications, intubation, aspiration, ventilation, oxygenation, and emergence.

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Preoperative

• It is important to discuss the medications that the child is already taking, if any.

• Certain medications, such as benzodiazepines (Midazolam), and/or psychiatric medications, can cause excitation and/or aggression, so beware of paradoxical responses.

• The responses to medications are unpredictable among children with CdLS. Certain patients may be sensitive to narcotics.

• The cognitive function of children with CdLS ranges from age-appropriate to almost complete cognitive dysfunction. Therefore, expect a spectrum of behavioural issues in spite of their age.

• Be careful with the limbs of the child due to deformities of the upper and lower extremities leading to difficult IV access.

• Be careful of aspiration due to the high risk of Gastroesophageal Reflux Disease (GERD).

• Due to the high incidence of a high-arched palate, small mouth opening, and micrognathia expect difficult intubation.

• Use a smaller sized endotracheal tube or supraglottic airway device.

• Expect difficulty with the insertion of the airway device.

• There is a high risk of problems with conventional devices; therefore, consider using an alternate device, such as a flexible fiberoptic bronchoscopy (FFB), or supraglottic airway device.

 

Intraoperative

• A choice of intravenous or inhalational anesthetics is possible.

• Beware of increasing CPAP during bag mask ventilation due to an increase in the incidence of GERD.

• Consider using flexible fiberoptic bronchoscopy (FFB), with or without a supraglottic airway, due to the greater maneuverability leading to an easier insertion of the endotracheal tube.

• Be careful of the risk of aspiration.

• Consider using alternate supraglottic airway devices for a better seal.

• There is a higher risk of desaturation which may lead to bradycardia or cardiac arrest.

• Standard anesthetic agents and medications may be used; however, expect variability in the response to the medication.

• If multiple procedures are being done, it is important to decide whether all procedures should be done under one anesthetic or if different anesthetics should be given for each procedure. There is a lack of evidence for there being one safest anesthetic technique.

 

Postoperative

• There is variability in how children with CdLS emerge from sedation and/or general anesthesia.

• Delayed emergence can occur and may last up to one week.

• There is sometimes a change in the diet patterns of the child.

• Amnesia has been known to occur after sedation and/or anesthesia in some children with CdLS.

• Watch for self-mutilating behaviour in response to being sedated and/or put under anesthesia.

• Beware of breathing problems postoperatively as there is a higher incidence of lost airway/reintubation and subsequent cardiac arrest in this population

• Reintubation and/or postoperative ventilation is sometimes required.

• Consider bridging the extubation with an airway exchange catheter.

• Most of the postoperative problems occur shortly after the procedure; therefore,

overnight stay is not always necessary.

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