*3.4.1. Pregnancy management*

Anencephaly is the most common neural tube defect (NTD) [20]. The anencephalic fetus can be definitively identified by the 12th postmenstrual week by TVS; although in some cases, this diagnosis has been made as early as 9–10 postmenstrual weeks [21]. Early diagnosis can be made if the cranium is examined carefully at the time of nuchal translucency measurement [22].

Up to 75% of anencephalic infants are stillborn. Liveborn infants generally die within hours but occasionally survive for a few days or weeks. There are no neurosurgical management options. In most developed countries where abortion is legal, these pregnancies are interrupted earlier [23]. Because of their poor prognosis, anencephalic infants have been considered as potential organ donors for transplantation. The clinical cases reported that anencephalic infants are not good candidates for organ donation because they do not generally meet criteria for brain death until their clinical condition has declined to the point where the solid organs are damaged [24]. Polyhydramnios develops in up to 50% of the cases during the second and third trimester due to decreased fetal swallowing, but is not present during the first trimester [20].

Prevention is the most important aspect of management of anencephaly. Periconceptional folic acid supplementation is recommended for all women who are pregnant or who may become pregnant. Higher doses of folic acid supplements are usually recommended for women who are taking anticonvulsant drugs or who have had a previous pregnancy affected by a NTD.
