Discover free eBooks, guides and med spa templates on our new resources page

Book a demo
☰
Why us
ICD Hub / Developmental anomalies (Q00–Q99) / LA00.0Z - Anencephaly, unspecified
ICD-11

ICD-11 Codes
for LA00.0Z - Anencephaly, unspecified
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
Use Code

Understanding Anencephaly, Unspecified

Anencephaly, unspecified, identified by the ICD-11 code LA00.0Z, is a severe congenital anomaly affecting the central nervous system. It is characterised by the total or partial absence of the cranial vault and the scalp, with the brain being either missing or reduced to a small mass. This condition arises from a failure of the neural tube to close properly during early fetal development, specifically around 28-29 days post-fertilisation, due to a neurulation defect in the rostral part of the neural plate. It represents a significant developmental defect where the brain and cranial vault are grossly malformed.

Factors Contributing to Anencephaly, Unspecified

The aetiology of anencephaly, unspecified (LA00.0Z) is considered multifactorial, resulting from complex gene-environment interactions. While most cases are sporadic, familial instances with a potential autosomal recessive inheritance pattern have been documented. Identified risk factors include deficiencies in folic acid and zinc, as well as maternal obesity. The primary underlying issue is the failure of cranial neurulation.

Clinical Presentation of Anencephaly, Unspecified

Infants diagnosed with anencephaly, unspecified (LA00.0Z) are typically stillborn. In rare instances, some may survive for a few hours after birth. Autopsy findings frequently reveal the absence of adrenal glands. The condition is visibly characterised by the absence of bony covering over the back of the head, missing bones around the front and sides of the skull, and often a folding of the head. The brain tissue present is usually significantly reduced or malformed.

Diagnostic Approaches for Anencephaly, Unspecified

The diagnosis of anencephaly, unspecified (LA00.0Z) is often made during prenatal screening through ultrasound imaging, which can detect the absence of the cranial vault and significant brain malformations. At birth, the condition is immediately apparent due to the severe physical characteristics. While specific diagnostic tests are not typically required for confirmation due to the obvious presentation, prenatal imaging is crucial for early identification.

Coding and Documentation for LA00.0Z

The ICD-11 code for Anencephaly, unspecified is LA00.0Z. This code is used for accurate medical record documentation and billing purposes when a patient is diagnosed with this specific condition. It falls under the broader category of Anencephaly or similar anomalies (LA00). Proper coding ensures clear communication among healthcare providers and facilitates statistical tracking of this rare birth defect.

Alternative Names for Anencephaly, Unspecified

Anencephaly, unspecified (LA00.0Z) is also known by several other clinical terms and synonyms. These include brain absence, brain agenesis, brain aplasia, absent brain, congenital absence of brain, anencephalus, and anencephalic monster. Less common terms like congenital absence of cerebrum and congenital hemicrania may also be encountered. The term exencephaly is also related.

Frequently asked questions

What is the ICD-11 classification meaning of LA00.0Z Anencephaly, unspecified?
LA00.0Z is used when the documentation does not provide sufficient detail to select a more specific category for anencephaly.
Under what circumstances is the 'unspecified' category for anencephaly used in ICD-11 coding?
The 'unspecified' category for anencephaly is used when the available documentation lacks the necessary information to assign a more specific code within the anencephaly block.
How is the ICD-11 code LA00.0Z applied in clinical documentation?
LA00.0Z is assigned when the diagnosis of anencephaly is recorded without further specification that would allow for a more detailed ICD-11 code.
How does LA00.0Z fit within the ICD-11 structure for anencephaly?
LA00.0Z is a specific code for 'Anencephaly, unspecified' within the broader ICD-11 block 'Anencephaly or similar anomalies' (LA00).
What distinguishes LA00.0Z from specified codes for anencephaly in ICD-11?
LA00.0Z is used when the specific type or details of the anencephaly are not documented, unlike specified codes which would reflect such details.
×