Short answer · Medically reviewed summary · Last updated: 2026-05-08
Anencephaly is classified in international medical billing systems under the ICD-10 code Q00.0 and the ICD-9 code 740.0. These codes are used by healthcare providers and insurance systems to identify this specific neural tube defect during prenatal screening and clinical documentation. What exactly is Anencephaly? Anencephaly is a severe, lethal congenital condition characterized by the absence of a major portion of the brain, skull, and scalp.
ICD10 code of Anencephaly and ICD9 code
ICD-10 and ICD-9 codes for Anencephaly, with classification details for clinicians, coders and patients.
Anencephaly is classified in international medical billing systems under the ICD-10 code Q00.0 and the ICD-9 code 740.0. These codes are used by healthcare providers and insurance systems to identify this specific neural tube defect during prenatal screening and clinical documentation.
What exactly is Anencephaly?
Anencephaly is a severe, lethal congenital condition characterized by the absence of a major portion of the brain, skull, and scalp. It occurs early in fetal development when the cephalic (head) end of the neural tube fails to close, usually between the 23rd and 26th day of gestation. Because the brain tissue remains exposed to amniotic fluid, it undergoes progressive degeneration throughout the pregnancy.
How is Anencephaly diagnosed?
Diagnosis is typically performed through routine prenatal ultrasound, often during the second-trimester anatomy scan. Other methods used to identify Anencephaly include:
- Maternal Serum Alpha-Fetoprotein (MSAFP) screening: High levels of this protein are often detected in the mother’s blood during the second trimester.
- Fetal MRI: Sometimes utilized to provide a more detailed assessment of the brain structure.
- Postnatal examination: A physical exam confirms the diagnosis immediately after birth if it was not identified prenatally.
What are the medical and emotional challenges?
For the 31 families in the DiseaseMaps.org community who have navigated a diagnosis of Anencephaly, the emotional burden is profound. Because Anencephaly is a terminal condition, the focus of clinical care shifts toward palliative measures, comfort, and support for the parents. While some infants may breathe briefly or show reflexive movements, vital brain functions cannot be sustained long-term, and the condition is currently considered non-correctable.
What is the genetic context of Anencephaly?
Anencephaly is a multifactorial condition, meaning it likely results from a combination of genetic predispositions and environmental factors. While most cases occur sporadically, parents who have had one affected pregnancy have an increased risk (approximately 2-5%) of recurrence in future pregnancies, often related to folate metabolism.
Next steps
- Consult with a genetic counselor to discuss recurrence risks and the role of folic acid supplementation.
- Connect with support organizations like the Anencephaly Support Foundation for specialized grief and bereavement resources.
- Engage with the 31 members on DiseaseMaps.org who have shared their personal experiences with Anencephaly.
- Discuss perinatal palliative care options with your obstetrician to create a birth plan focused on comfort.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Anencephaly
- Orphanet: Anencephaly (ORPHA:93240)
- OMIM (Online Mendelian Inheritance in Man): Anencephaly (#206500)
- CDC: Facts about Anencephaly
