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PubMed This is a summary of 20 peer-reviewed journal articles Updated
Maternal-Fetal Medicine

Preparing for Birth, Prognosis, and Palliative Care

At a Glance

Alobar holoprosencephaly is a severe, life-limiting condition, with most babies passing away shortly after birth from respiratory challenges. Care focuses on perinatal palliative strategies to ensure the baby is kept comfortable, pain-free, and surrounded by loved ones.

Preparing for the birth of a child with alobar holoprosencephaly (HPE) is an act of profound love and courage. Between a mid-pregnancy diagnosis (often around the 20-week anatomy scan) and delivery, you will likely have frequent appointments with your Maternal-Fetal Medicine (MFM) specialist to monitor the baby’s growth and your health [1]. Because this is a life-limiting condition, the focus of care often shifts from “curing” to “comfort,” a practice known as perinatal palliative care [2][3]. This approach aims to maximize your baby’s quality of life and support your family’s emotional and spiritual needs during the limited time you may have together [4][5].

The Birth Experience and Early Mortality

Most babies with alobar HPE face significant challenges immediately after birth. The primary cause of early mortality is often respiratory failure [6]. This can happen because the brain’s respiratory center did not develop fully, or because facial differences—such as a proboscis or a single nostril (cebocephaly)—can physically block the airway [6][7].

Many families choose a comfort-first birth plan. This may include:

  • Prioritizing skin-to-skin contact and holding your baby immediately [3].
  • Compassionate Presentation: Your care team can gently wrap or position the baby so you can first focus on comforting features—like holding their hand or seeing their hair—before taking in the rest of your baby’s appearance [3].
  • Using non-invasive measures for comfort, such as positioning or suctioning, rather than intubation or mechanical ventilation [6][8].
  • Creating memories through photography, handprints, or religious rituals [3][9].

Challenges for Rare Survivors

While the prognosis for alobar HPE is typically fatal shortly after birth, a very small number of children may survive for weeks or, in extremely rare cases, months [6][10]. Interventions like feeding tubes or shunts are sometimes considered for these survivors, but it is important to know that these do not cure the underlying severe brain damage. They are used to manage symptoms [11]. These children face complex medical needs that require a dedicated multidisciplinary team [11].

Neurological Needs

  • Hydrocephalus: If fluid builds up in the brain, a ventriculoperitoneal (VP) shunt (a tube that drains fluid to the abdomen) may be considered to relieve pressure and pain [12]. However, these shunts can sometimes fail or become blocked in children with HPE [12][13].
  • Seizures: Most survivors will experience seizures, which are managed with anti-seizure medications to maintain comfort and stability [14].

Endocrine and Hypothalamic Issues

The hypothalamus (the brain’s control center for hormones and temperature) is often affected in HPE [15].

  • Diabetes Insipidus (DI): This is a condition where the body cannot balance fluids correctly, leading to excessive urination and dehydration [15][16]. It is managed with a medication called desmopressin (DDAVP) and careful monitoring of sodium levels [17][18].
  • Temperature Regulation: Children may struggle to maintain a normal body temperature, often running very high or very low temperatures without an infection being present [15].

Nutritional Support

Feeding is a major focus for survivors. Many babies have difficulty sucking and swallowing safely.

  • NG-tubes: A temporary tube through the nose to the stomach.
  • G-tubes: A more permanent tube placed through the abdomen for long-term nutrition [11].

Quality of Life as a Compass

In every discussion with your medical team, your family’s values and your child’s comfort should be the “compass” that guides the plan [19][20]. Whether your time together is measured in minutes, days, or months, the goal of care is to ensure that your child is known, loved, and kept free from pain [2][5].

If your family’s values align more with pursuing available life-prolonging interventions, your care team will continue to support you and will not abandon you [19]. Connecting with perinatal hospice programs and infant loss support networks can also provide critical emotional support and counseling during this devastating time [3]. Your palliative care team is there to help you navigate these choices, ensuring that your baby’s life—however short—is honored [3][9].

Common questions in this guide

What is a comfort-first birth plan for alobar holoprosencephaly?
A comfort-first birth plan prioritizes your baby's quality of life and your family's emotional needs. It often includes immediate skin-to-skin contact, gentle wrapping to focus on comforting features, and using non-invasive measures rather than mechanical ventilation to keep the baby comfortable.
How long can a baby survive with alobar holoprosencephaly?
Unfortunately, alobar holoprosencephaly is typically a fatal condition, and most babies pass away shortly after birth due to respiratory failure. In extremely rare cases, some children may survive for weeks or months, though they require complex medical support to manage symptoms.
What medical challenges do rare survivors of alobar HPE face?
Babies who survive past the first few days face significant challenges, including hydrocephalus, seizures, temperature regulation issues, and feeding difficulties. They require specialized interventions like feeding tubes, shunts, and anti-seizure medications to manage these symptoms.
How is diabetes insipidus managed in babies with alobar HPE?
Diabetes insipidus, caused by issues in the brain's hypothalamus, makes it hard for the baby's body to balance fluids. It is typically managed with careful monitoring of sodium levels and a medication called desmopressin to prevent severe dehydration.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Can we meet with the perinatal palliative care team to create a birth plan that focuses on our baby's comfort?
  2. 2.What specific signs of pain or respiratory distress should we look for, and how will they be managed?
  3. 3.If our baby survives the first few days, what are the options for feeding and seizure management that align with a comfort-first approach?
  4. 4.How will our child be monitored for diabetes insipidus, and what would the treatment involve?
  5. 5.If we choose to take our baby home, what equipment and nursing support will be available to us?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (20)
  1. 1

    The Role of Antenatal Ultrasound Scans in the Early Detection of Alobar Holoprosencephaly: A Case Report.

    Mohamed ME, Ahmed SR, Elsayed Ahmed EM, Ibrahim EH

    Cureus 2024; (16(10)):e70843 doi:10.7759/cureus.70843.

    PMID: 39493208
  2. 2

    Longitudinal Perinatal Palliative Care for Severe Fetal Neurologic Diagnoses.

    Humphrey LM, Schlegel AB

    Seminars in pediatric neurology 2022; (42()):100965 doi:10.1016/j.spen.2022.100965.

    PMID: 35868733
  3. 3

    Perinatal Palliative Care Birth Planning as Advance Care Planning.

    Cortezzo DE, Ellis K, Schlegel A

    Frontiers in pediatrics 2020; (8()):556 doi:10.3389/fped.2020.00556.

    PMID: 33014940
  4. 4

    Perinatal Palliative Care: ACOG COMMITTEE OPINION, Number 786.

    Obstetrics and gynecology 2019; (134(3)):e84-e89 doi:10.1097/AOG.0000000000003425.

    PMID: 31441826
  5. 5

    Recognising uncertainty: an integrated framework for palliative care in perinatal medicine.

    Wilkinson D, Bertaud S, Mancini A, et al.

    Archives of disease in childhood. Fetal and neonatal edition 2025; (110(3)):236-244 doi:10.1136/archdischild-2024-327662.

    PMID: 39567213
  6. 6

    Alobar holoprosencephaly with cebocephaly in a neonate: A rare case report from Northern Tanzania.

    Ariyo IJ, Mchaile DN, Magwizi M, et al.

    International journal of surgery case reports 2022; (93()):106960 doi:10.1016/j.ijscr.2022.106960.

    PMID: 35364389
  7. 7

    Alobar holoprosencephaly associated with a rare chromosomal abnormality: Case report and literature review.

    Ionescu CA, Calin D, Navolan D, et al.

    Medicine 2018; (97(29)):e11521 doi:10.1097/MD.0000000000011521.

    PMID: 30024536
  8. 8

    Alobar holoprosencephaly: Exploring mothers' perspectives on prenatal decision-making and prognostication.

    Elfarawi H, Tolusso L, McGowan ML, et al.

    Prenatal diagnosis 2022; (42(5)):617-627 doi:10.1002/pd.6130.

    PMID: 35253927
  9. 9

    Compassion and Community in Perinatal Palliative Care: Understanding the Necessity of the Patient Perspective Through Narrative Illustration.

    Kuchemba-Hunter J

    Journal of palliative care 2019; (34(3)):160-163 doi:10.1177/0825859719827020.

    PMID: 30776963
  10. 10

    Alobar Holoprosencephaly Associated with Meningomyelocoele and Omphalocoele: An Unusual Coexistence.

    Waghmare TP, Sathe PA, Goel NA, Kandalkar BM

    Journal of clinical and diagnostic research : JCDR 2016; (10(11)):ED23-ED24 doi:10.7860/JCDR/2016/22453.8932.

    PMID: 28050387
  11. 11

    Prenatal diagnosis of holoprosencephaly.

    Kousa YA, du Plessis AJ, Vezina G

    American journal of medical genetics. Part C, Seminars in medical genetics 2018; (178(2)):206-213 doi:10.1002/ajmg.c.31618.

    PMID: 29770996
  12. 12

    Surgical Nuances in Ultrasound-Guided Percutaneous Distal Catheter Placement in Pediatric Ventriculoatrial Shunts.

    Reynoso LG, Rodríguez Lezama A, Hernández Martínez CA, et al.

    Cureus 2025; (17(5)):e84345 doi:10.7759/cureus.84345.

    PMID: 40535372
  13. 13

    Congenital complete arhinia with alobar holoprosencephaly.

    Boakye-Yiadom AP, Nguah SB, Mahama H, Plange-Rhule G

    Ghana medical journal 2022; (56(3)):231-235 doi:10.4314/gmj.v56i3.14.

    PMID: 37449001
  14. 14

    Holoprosencephaly: Review of Embryology, Clinical Phenotypes, Etiology and Management.

    Malta M, AlMutiri R, Martin CS, Srour M

    Children (Basel, Switzerland) 2023; (10(4)) doi:10.3390/children10040647.

    PMID: 37189898
  15. 15

    [Alobar holoprosencephaly associated with diabetes insipidus and hypothyroidism in a 10-month old infant].

    Seck N, Basse I, Keita Y, et al.

    The Pan African medical journal 2017; (28()):193 doi:10.11604/pamj.2017.28.193.11288.

    PMID: 29599891
  16. 16

    Management of Arginine Vasopressin Deficiency (Central Diabetes Insipidus) in Neonates and Infants.

    Pearlstein H, Dayno A, Zook J, et al.

    Hormone research in paediatrics 2025; 1-11 doi:10.1159/000547155.

    PMID: 40602377
  17. 17

    Sublingual Administration of Desmopressin Oral Disintegrating Tablet in a Neonate With Central Diabetes Insipidus.

    Watanabe D, Yagasaki H, Tsukahara M, et al.

    Cureus 2025; (17(7)):e87902 doi:10.7759/cureus.87902.

    PMID: 40809652
  18. 18

    Use of Chlorothiazide in the Management of Central Diabetes Insipidus in Early Infancy.

    Raisingani M, Palliyil Gopi R, Shah B

    Case reports in pediatrics 2017; (2017()):2407028 doi:10.1155/2017/2407028.

    PMID: 28553553
  19. 19

    Role of palliative care in fetal neurological consultations: Guiding through uncertainty and hope.

    Cortezzo DE, Vawter-Lee M, Shoaib A, Venkatesan C

    Frontiers in pediatrics 2023; (11()):1205543 doi:10.3389/fped.2023.1205543.

    PMID: 37334218
  20. 20

    The Path Is Made by Walking-Mapping the Healthcare Pathways of Parents Continuing Pregnancy after a Severe Life-Limiting Fetal Diagnosis: A Qualitative Interview Study.

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    PMID: 36291491

This page provides educational information about alobar holoprosencephaly prognosis and palliative care. Always consult your maternal-fetal medicine specialist or perinatal palliative care team to create a birth plan tailored to your baby's specific needs.

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