CHARGE Syndrome Anaesthesia
Children with CHARGE syndrome often require multiple surgeries throughout their lifetime to manage structural and functional anatomical abnormalities. It is important therefore, to consider any risks caused by anaesthesia. These risks tend to vary based on the individual child’s CHARGE syndrome characteristics.
Airway problems are common in CHARGE syndrome. Figure 1 shows the normal anatomy of the airway, as well as associated cranial nerves. Most children with CHARGE syndrome have cranial nerve abnormalities. Pertinent problems are those with the glossopharyngeal and vagus nerves, which are responsible for innervating the pharynx and larynx and allowing swallowing to occur (Blake et al. 2008).
Problems dealing with oral secretions can be a result of cranial nerve problems (Blake et al. 2009a). A study by Morgan et al. (1993) examining 50 patients
with CHARGE syndrome found that 86% had upper airway abnormalities, 56% had posterior choanal abnormalities and 42% had retrognathia leading
to intubation difficulties.
A study done by Naito et al. (2007) looked at 10 consecutive patients with CHARGE syndrome who underwent fiberoptic laryngoscopy. All patients had upper airway obstruction at the larynx level, with a hypotonic larynx and hypertrophic arytenoids. They also had uncoordinated movement of the supraglottis, which made it difficult to maintain airway patency. Other problems included glossoptosis, oesophageal fistula and clefts.
Securing an Airway
There are reports of cancelled surgery and aborted anaesthesia from failure to secure airway control in children with CHARGE syndrome (Hara et al. 2009). An early study showed greater difficulty with tracheal intubation that increased with age (Stack and Wyse, 1991). Laryngomalacia may contribute to upper airway collapse during light anaesthesia and may lead to a requirement of CPAP during induction.
Since the pharynx and larynx of CHARGE syndrome patients may be much smaller then expected from their physique, a smaller sized laryngeal mask airway (LMA) should be used first (Hara et al. 2009). A wide range of equipment sizes should be on-hand, including that to perform a tracheotomy (Stack and Wyse, 1991). It is suggested that a paediatric
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