Anencephaly is a condition in which a baby’s skull and most of the brain do not form. It cannot be treated or cured, so babies born with it live a very short time, usually minutes to hours and only occasionally days.
How Long Babies With Anencephaly Live
Among babies born alive, half had died within about 55 minutes.1PubMed. The natural history of anencephaly The range was wide, though, from 10 minutes to 8 days.2PubMed. The natural history of anencephaly No doctor can say exactly how long one baby will live, only the likely range.
Some babies do live a day or more. In a larger group of liveborn babies, a little over 4 in 10 lived longer than 24 hours.3PubMed. Survival in infants with anencephaly Boys were more likely to pass the first day than girls, about 51% versus 34%.4PubMed. Survival in infants with anencephaly Of the babies who lived past a day, only about 5% were still alive on the seventh day.5PubMed. Survival in infants with anencephaly There are rare exceptions. One baby girl with anencephaly lived to 28 months without breathing machines or other life support, the longest such case recorded.6PubMed Central. Prolonged unassisted survival in an infant with anencephaly. Doctors cannot predict cases like this.
The reason is the brainstem, the part of the brain that runs breathing and heartbeat. It may partly work, so a baby can breathe for a while. Without the rest of the brain and skull, though, these functions usually fail soon, and breathing pauses or stops, often within the first hours. Life support cannot fix the missing brain, so it does not change the outcome.
A baby who cries, breathes steadily, or looks pink at birth can still die within hours. Those signs come from the brainstem and do not show that the rest of the body can keep going. A good first hour does not predict a longer life.
During those hours, the baby may breathe, move, and respond to touch. Parents can hold the baby, take photos, and have family present. Breathing may become irregular near the end. The care team focuses on warmth and comfort throughout.
Many parents ask whether the baby will be in pain. Babies with anencephaly are generally understood to have little or no awareness, because the parts of the brain that produce conscious experience are largely missing. Experts are cautious, though, and care teams still treat the baby as though discomfort is possible. Reflex actions such as grimacing, crying out, stretching, or startling can look like distress. They usually come from the brainstem and do not necessarily mean the baby is suffering. You can ask the team to tell you what they are seeing as it happens, so you are not left guessing about each movement or sound.
Many Babies Die Before or During Birth
In one group of pregnancies continued to term, about 23% of babies died in the womb before labour began.7PubMed. The natural history of anencephaly Another 35% died during labour.8PubMed. The natural history of anencephaly In that group, fewer than half the babies were born alive.
Labour is risky for these babies because the exposed head is unprotected during birth, and the stress of contractions can be too much for the limited brainstem. Doctors usually do not use a C-section to try to prevent this, since it would not change survival.
A birth plan can cover both a live birth and a stillbirth. Parents can still hold, name, and photograph a stillborn baby, and many hospitals arrange keepsakes such as footprints. Whether to have monitoring during labour is a real choice, since intervention will not save the baby.
It also helps to ask the team in advance what they will do if no heartbeat is found at admission or during labour, and who will be with you. If the baby is born alive, staff can step back so the family has time alone, while a nurse stays within reach in case you want help or reassurance.
Caring for a Baby in the Hospital or at Home
Comfort care means keeping the baby warm, fed, and free of pain rather than trying to prolong life. Hospitals generally regard warmth, nutrition, and fluids as the standard care for babies with anencephaly.9Cambridge Quarterly of Healthcare Ethics. The Baby K Case: A Search for the Elusive Standard of Medical Care In a well-known U.S. case, however, a court ruled that a hospital must provide emergency breathing support when the mother asked for it.10Cambridge Quarterly of Healthcare Ethics. The Baby K Case: A Search for the Elusive Standard of Medical Care
Some parents ask for breathing support at birth. This is usually a trial rather than a promise, and it can be stopped if the baby is clearly not benefiting. Decide with the team before birth which interventions you want, which you decline, and when they should stop. Writing these wishes down, and sharing them with every shift, helps avoid confusion in the delivery room. Parents interested in donation, such as tissue or research, can ask the hospital what is allowed.
Going home is also possible. Parents who took their babies home worried most about transport, feeding, changing the head dressing, managing pain and seizures, uncertainty, and a peaceful death.11PubMed. Providing Palliative Care to Neonates With Anencephaly in the Home Setting Asking a palliative or hospice team about each of these before discharge helps.
Before the birth, you can ask the care team:
- Who will be in the room, and whether a palliative team is involved
- How the baby’s head will be covered and dressed
- Whether feeding by mouth or tube is offered
- What medicine is available for pain or seizures
- Whether the family can take the baby home
The brainstem controls sucking and swallowing, so some babies feed by mouth and others cannot. The team can show you how to offer small amounts by dropper, syringe, or bottle. Feeding is mostly about comfort and closeness, not long-term nutrition, so a baby who takes very little is not being failed. Mothers who want to express milk can ask for help, as some find this meaningful whatever the baby takes.
What Continuing the Pregnancy Means for the Mother
Polyhydramnios, too much amniotic fluid, is common in these pregnancies. It has appeared in about 15% to 27% of cases in most groups studied,12PubMed Central. Anencephaly: Do the Pregnancy and Maternal Characteristics Impact the Pregnancy Outcome? though one group in Iran reported it in 90%.13PubMed. Anencephaly and pregnancy outcome in Iran It can raise the risk of early delivery and breathing trouble for the mother.14PubMed Central. Anencephaly: Do the Pregnancy and Maternal Characteristics Impact the Pregnancy Outcome? In a few women, severe fluid caused breathlessness and was drained with a needle more than once.15PubMed Central. What’s Happening When the Pregnancies Are Not Terminated in Case of Anencephalic Fetuses?
A baby without the usual brain structures may swallow less amniotic fluid than normal, which lets fluid build up. This often appears later in pregnancy, so a normal check early on does not mean it will not develop. A large amount of fluid stretches the womb, which is why it can bring on early contractions and pressure on the lungs.
Tell your team if your belly is growing faster than expected, if you are short of breath or cannot lie flat, or if you notice tightness or early contractions. Doctors track fluid with ultrasound at checkups.
Draining fluid with a needle through the belly, guided by ultrasound, is called amnioreduction. It is done only when the fluid is causing real symptoms. It can bring quick relief from breathlessness, but fluid may return, so it sometimes needs repeating, as in the cases reported. It carries small risks, such as triggering labour, so it is a decision to weigh with your obstetrician.
Vaginal birth is the recommended way to deliver, because a C-section adds risk to the mother without helping the baby.16Indonesian Journal of Obstetrics and Gynecology. Anencephaly with Prior Caesarean Section: A Management Dilemma for Lethal Fetal Condition to Perform Labor Induction If induced labour stalls, a C-section may be needed for the mother’s safety.17Jurnal Biomedika dan Kesehatan. Dilemma of Labor Management in Lethal Anencephalic Fetus Shoulders getting stuck and heavy bleeding after birth occurred in a few cases and were managed without surgery.18PubMed Central. What’s Happening When the Pregnancies Are Not Terminated in Case of Anencephalic Fetuses? The delivery plan should center on the mother’s health and wishes, including emotional ones.19Jurnal Biomedika dan Kesehatan. Dilemma of Labor Management in Lethal Anencephalic Fetus
Some parents continue to term, while others choose earlier induction. Timing can be discussed with your obstetrician. A prior C-section makes induction more complicated but is still often possible.
How Certain the Diagnosis Is
Anencephaly can sometimes be seen as early as 8 weeks but can be missed in the first trimester.20lumen.luc.edu. Anencephaly Second-trimester ultrasound diagnosis is essentially always accurate.21lumen.luc.edu. Anencephaly A mid-pregnancy diagnosis is therefore very unlikely to be a mistake, though a second scan is reasonable.
A normal early scan does not rule out anencephaly. If an early scan is unclear, the usual step is a repeat scan a week or two later, not waiting for the routine mid-pregnancy scan.
For confirmation, ask for a scan with a fetal medicine specialist. The missing top of the skull is clearly visible by mid-pregnancy.
When the diagnosis is first given, the sonographer may be quiet or call in a doctor, which is normal when something unexpected is seen. You may ask for time, for your partner or a support person to be there, and for the findings to be explained in plain words. A written summary helps, since much of what is said at this moment is hard to take in.
Why It Happens and Lowering the Risk Next Time
Doctors do not know all the causes, but genes and other factors combined likely play a role.22Centers for Disease Control and Prevention. Anencephaly Low folate in early pregnancy, poorly controlled diabetes, and overheating from fever or hot tubs raise the risk.23Centers for Disease Control and Prevention. Anencephaly In rare families, a single inherited gene change has caused it.24PubMed. A homozygous mutation in TRIM36 causes autosomal recessive anencephaly in an Indian family Many cases have no known cause, so most parents did nothing wrong.
Nothing a parent did in an ordinary day, such as exercise, stress, work, or a missed vitamin after the pregnancy was under way, is a known cause. The neural tube closes so early that searching for a reason usually has no answer.
Anencephaly arises when the neural tube, the early structure that becomes the brain and spinal cord, fails to close. This happens around four weeks after conception, often before a woman knows she is pregnant, which is why folic acid must be in place beforehand.
The evidence from whole populations is strong. After Canada added folic acid to flour, anencephaly fell by about 38%.25PubMed. Reduction in neural-tube defects after folic acid fortification in Canada Worldwide, required folic acid fortification is linked to about 44% fewer of these defects.26PubMed Central. The effectiveness of mandatory folic acid fortification compared with pre-fortification periods on reducing neural tube defects (NTDs): a systematic review and meta-analysis Taking folic acid before conceiving and early in pregnancy is the main step. After a pregnancy affected by anencephaly, doctors usually recommend a much higher dose, often 4 mg daily. That is why a prescription is generally needed. It is usually taken from before conception until early in pregnancy, as your doctor advises. Even a high dose lowers the risk but does not remove it, so a future scan is still part of care.
If you are planning another pregnancy:
- See a doctor before trying to conceive again.
- Start high-dose folic acid at least a month before conception.
- Get diabetes well controlled beforehand.
- Treat fevers promptly and avoid hot tubs and saunas early on.
- Consider genetic counselling to learn your family’s own risk.
After one affected pregnancy, the chance of another is higher than for the general population, but still low in absolute terms. Most later pregnancies are unaffected, and detailed ultrasound can check early on.
Coping With the Diagnosis and Loss
About 3 in 4 parents in one group reported intense grief.27PubMed. The impact of anencephaly on parents: A mixed-methods study Parents who felt they had control over their care tended to grieve less intensely.28PubMed. The impact of anencephaly on parents: A mixed-methods study Parents who continued the pregnancy described deep sadness alongside meaningful time as parents and personal growth.29PubMed. Anencephaly; the maternal experience of continuing with the pregnancy
Grief after this diagnosis often starts at the scan, not at the birth, because parents are mourning the future they expected while still pregnant. Some people feel numb, angry, guilty, or relieved at times, and none of these reactions means the person is coping badly. Others may ask how the pregnancy is going, which can be painful, so some parents prepare a short answer in advance.
Perinatal hospice programs support families through pregnancy and after. Bereavement support groups connect parents with others who have been through it. Partners may grieve differently and need their own support. Memory-making, like photos and handprints, is valued by many parents.
Children usually cope better with short, honest explanations, such as that the baby’s brain did not grow properly and the baby will die soon. You can decide whether children meet the baby, and a nurse or child-life specialist can prepare them for what they will see. Relatives may say unhelpful things, so it can help to ask one person to share news and set expectations for everyone else.
Physical recovery continues after birth, including milk coming in, and the team can explain how to handle that before you leave. Grief can return around due dates and anniversaries, and seeking counselling later is common and not a sign of failing.