ICD-11 code : LA00.0;
Preferred Label : Anencephaly;
ICD-11 definition : Anencephaly is a neural tube defect, characterized 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.;
ICD-11 synonym : anencephalic; congenital absence of brain; anencephalic monster; brain agenesis; brain absence; absent brain; anencephalus; brain aplasia;
ICD-11 inclusion : congenital absence of cerebrum; congenital hemicrania; hemicephalus; incomplete anencephaly; Hemianencephaly; anomaly of skull, with anencephaly; Holoanencephaly; bone anomaly of skull, with anencephaly; hemicephalia; Meroanencephaly; Hemicephaly; congenital skull deformity with anencephaly; absence of skull bone with anencephaly; skull agenesis with anencephaly;
ICD-11 "other" category code : LA00.0Y;
ICD-11 "unspecified" category code : LA00.0Z;
Origin ID : 1292761836;
UMLS CUI : C0002902;
Currated CISMeF NLP mapping
- ICD-10 Mapping
- See also inter- (CISMeF)
- Semantic type(s)
- Validated automatic mappings to NTBT
Anencephaly is a neural tube defect, characterized 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.
