Surgical Procedures and Anesthesia
There are several literature reports of individuals with AS undergoing general anesthesia without any difficulties. Also, the experience reported by many parents on the web and from parent meetings is generally favorable regarding successful general anesthesia and other aspects of surgical intervention. Some scientific reports mention concern for those who have the deletion mechanism (present in 70% of those with AS) because these individuals also have a deletion of GABA receptor genes which are known to be targets of certain anesthetic agents such as benzodiazepines and halogenated ethers. However, the weight of experience thus far indicates that individuals with AS tolerate anesthetic agents well. Convalescence from surgical procedures also appears to occur relatively normally. For example, rather significant surgical interventions of scoliosis repair with rod replacement and bone grafting is not uncommon to be well tolerated in those with AS.
There have been recent reports of bradycardia in individuals with AS and the presumption has been that these rhythm problems were due to increased activity of the vagus nerve [1-3]. These reports are somewhat difficult to interpret because of the complexities associated with hospitalization such as multiple medication use and the variables of the surgical procedure. At this point, it seems unclear if individuals with AS have an increased risk for cardiac rhythm disturbances. Anesthesiologist should certainly be aware of these case reports however and of the possibility that agents that increase vagal tone may not be well tolerated in individuals with AS.
A final note should be made about seizures since precautions are always advised whenever someone with a seizure disorder undergoes general anesthesia or undergoes any type of operative procedure. At this point, it appears that the usual precautions that would be given for anyone with a seizure disorder would as well apply to someone with AS. The surgical procedure or the anesthesia event itself does not necessarily leads to an exacerbation of seizures and AS. This is not to say that a surgical procedure will not be complicated by the onset of seizures since that is always a possibility and it is generally known that significant health events predispose to exacerbation of seizures whether one has AS or any other type of seizure condition.
1. Bujok G and Knapik P. Angelman syndrome as a rare anaesthetic problem. Paediatr Anaesth, 2004. 14(3): p. 281-3.
2. Gardner JC, Turner CS and Ririe DG. Vagal hypertonia and anesthesia in Angelman syndrome. Paediatr Anaesth, 2008. 18(4): p. 348-9.
3. Vanagt WY, Pulles-Heintzberger CF, Vernooy K, et al. Asystole during outbursts of laughing in a child with Angelman syndrome. Pediatr Cardiol, 2005. 26(6): p. 866-8.