ICD-11 classes
20 Developmental anomalies
Structural developmental anomalies primarily affecting one...
Structural developmental anomalies of the nervous system
LA00 — Anencephaly or similar anomalies
LA00.0 — Anencephaly
ICD-11 LA00.0 — Anencephaly
Anencephaly is a neural tube defect, characterised by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
The diagnosis includes nothing.
The diagnosis excludes nothing.
Diagnosis with code LA00.0 contains 3 clarifying diagnoses:
- LA00.00 — Craniorachischisis
- LA00.0Y — Other specified anencephaly
- LA00.0Z — Anencephaly, unspecified
The diagnosis is coded elsewhere:
- Anencephaly or similar anomalies #8672
- Anencephaly #8673