We recently encountered a patient with a rare congenital condition, Aicardi's syndrome, who required general anaesthesia for dental extractions and the filling of cavities. While descriptions of the condition are in the literature, we could not find specific details of anaesthetic techniques used in such patients or of any complications encountered or to anticipate. The aetiology of Aicardi's syndrome is unknown. It is characterized by infantile spasms, agenesis of the corpus callosum, lacunar chorioretinopathy and profound mental retardation . Over 100 cases have been reported and it occurs only in females; most die in the first few years. The condition is most probably an X-linked dominant one, which proves lethal in the hemizygous male. The differential diagnosis includes dysgenesis of the corpus callosum, although the chorioretinopathy is pathognomonic. Clinical diagnosis is usually confirmed with electroencephalography (EEG), followed by detailed magnetic resonance imaging (MRI) of the eyes. Vertebral abnormalities are also associated with the syndrome, and other skeletal anomalies have been described rarely . Cleft lip and palate occur in about 3% of affected patients  and palatal haemangioma has also been documented . The likelihood of airway difficulty exists, therefore, and neuraxial blockade might also be challenging. Prolongation of conduction times has been recorded in studies of visual-evoked potentials and auditory brainstem responses . An increased incidence of tumours is also believed to be a feature of the syndrome .
Our patient was a 23-yr-old, 40 kg, female with a mental handicap, scoliosis and a history of tonic-clonic epileptic seizures. Her condition was controlled with carbamazepine, lamotrigine, lansoprazole and clobazam. A diagnosis of Barrett's oesophagus was made at endoscopy 3 yr previously, at which time she had suffered episodes of haemoptysis. Before that, she had been anaesthetized in our hospital using inhalational anaesthesia with halothane on several occasions. In view of the development of oesophagitis, we thought it appropriate to use a rapid sequence induction. After placement of an intravenous cannula, we induced anaesthesia with sodium thiopental 250 mg and succinylcholine 70 mg, and intubated the patient's trachea without difficulty. Maintenance of anaesthesia was with sevoflurane in nitrous oxide and oxygen, and morphine 2 mg was given for analgesia. Routine monitoring of electrocardiography, blood pressure, oxygen saturation and capnography was instituted and no problems arose during a brief procedure. The patient was extubated uneventfully in the recovery room and after 4 h of observation in the day ward was allowed to return home with her family. They lived nearby and standard advice was given; no problems occurred.
We wanted to record, therefore, that the above approach allowed us to provide successful, safe and uncomplicated day case anaesthesia to a patient with this rare condition, and to inform colleagues about its potential perioperative implications.
Department of Anaesthesia; Beaumont Hospital; Dublin, Ireland
Department of Anaesthesia; University College Hospital; Galway, Ireland
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