(polygyry, microgyry). Absence of the cerebral cortex and other malformations of the brain also occur in conditions other than Aicardi syndrome. On eye examination, the retinal lacunae appear as white spots in the fundus, where the retina is missing. Sometimes there are slits in the eye (coloboma), retinal detachment and abnormally small or differently sized eyes. When X-raying the skeleton, it is sometimes possible to see that there are vertebral changes and extra ribs or that ribs are missing. Treatment Treatment of Aicardi syndrome primarily involves management of seizures and early/continuing intervention programs for developmental delays.Additional comorbidities and complications sometimes seen with Aicardi syndrome include porencephalic cysts and hydrocephalus, and gastro-intestinal problems. Treatment for porencephalic cysts and/or hydrocephalus is often via a shunt or endoscopic fenestration of the cysts, though some require no treatment. Placement of a feeding tube, fundoplication, and surgeries to correct hernias or other gastrointestinal structural problems are sometimes used to treat gastro-intestinal issues. Girls with Aicardi syndrome come into contact with many different specialists in healthcare early on. It is therefore important that efforts are coordinated. The drug treatment given for infantile spasms and other types of epilepsy is also given to girls with Aicardi syndrome. Epilepsy is often difficult to treat. If medication does not help, after an examination at a regional hospital, a decision can be made as to whether another treatment may be appropriate. Since the cause of the epileptic seizures is found in many different places in the brain, however, epilepsy surgery is rarely an option in Aicardi syndrome. Treatment with a ketogenic diet may be considered. It involves a carefully calculated diet that is rich in fat, contains a minimum of carbohydrates and provides the daily need for protein. The excess of fat forms starvation bodies (ketones) which can be used instead of glucose as a fuel source for the metabolism in the brain. For some children, this leads to fewer seizures. Treatment is started at regional hospitals by special teams with doctors, nurses and dieticians. Another treatment option when the drug treatment of the epilepsy does not help is a so-called vagus nerve stimulator (VNS). The vagus nerve is one of twelve nerves that originate directly from the brain (cranial nerves). A small battery-powered box (generator) is operated under the skin under the left collarbone and a thin wire (electrode) from the generator is operated around the left vagus nerve.