Anencephaly

Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development.^[1] It is a cephalic disorder that results from a neural tube defect that occurs when the rostral (head) end of the neural tube fails to close, usually between the 23rd and 26th day following conception.^[2] Strictly speaking, the Greek term translates as "without a brain" (or totally lacking the inside part of the head), but it is accepted that children born with this disorder usually only lack a telencephalon,^[3] the largest part of the brain consisting mainly of the cerebral hemispheres, including the neocortex, which is responsible for cognition. The remaining structure is usually covered only by a thin layer of membrane—skin, bone, meninges, etc., are all lacking.^[4] With very few exceptions,^[5] infants with this disorder do not survive longer than a few hours or days after birth.

Anencephaly is a severe neural tube defect typically considered incompatible with prolonged postnatal survival, and as such, surgical intervention is not commonly indicated. However, a unique case report published in documented the first known surgical procedure performed on an infant diagnosed with anencephaly who survived beyond the neonatal period. The case, managed by Menekse and colleagues, describes a multidisciplinary approach and a neurosurgical intervention performed to improve the patient's quality of life. This report represents a rare and unprecedented instance in the clinical management of anencephaly and has contributed to the evolving ethical and medical discussions surrounding the condition.^[6]