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ICD Hub / Developmental anomalies (Q00–Q99) / LA00 - Anencephaly or similar anomalies
ICD-11

ICD-11 Codes
for LA00 - Anencephaly or similar anomalies
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Alternative Names and Classifications for LA00

This condition, identified by the ICD-11 code `LA00`, is known by several terms. It is officially classified as "Anencephaly or similar anomalies." Common clinical synonyms include anencephaly, congenital absence of the brain, and absence of the brain and skull. It is also recognized as a severe type of neural tube defect.

Understanding Anencephaly or Similar Anomalies (ICD-11 Code LA00)

The ICD-11 code `LA00` represents a severe structural developmental anomaly of the nervous system. This condition arises from the failure of the neural tube to close properly during early fetal development. Infants born with this anomaly lack the formation of neural structures above the brainstem and cerebellum, including the cerebrum and cranial vault. While the skull is only partially formed, the eyes are typically normal.

Factors Contributing to Anencephaly or Similar Anomalies

The primary cause of anencephaly or similar anomalies (ICD-11 code `LA00`) is the incomplete closure of the neural tube in the developing fetus. While the exact triggers are often not identified, a combination of genetic predispositions and environmental factors is suspected. Key risk factors include insufficient intake of folic acid by the mother before and during early pregnancy, exposure to certain medications during pregnancy, and specific maternal health conditions.

Diagnosing Anencephaly or Similar Anomalies

Diagnosis of anencephaly or similar anomalies (ICD-11 code `LA00`) can often be made during routine prenatal care.
  • Prenatal Ultrasound: This imaging technique can typically detect the absence of the skull and major parts of the brain as early as the first trimester.
  • Maternal Serum Screening: Blood tests performed on the mother can indicate an elevated risk for neural tube defects.
  • Postnatal Examination: Following birth, the diagnosis is confirmed through a physical examination of the infant.

Accurate Coding for Anencephaly or Similar Anomalies

For accurate medical billing and record-keeping, precise ICD-11 coding is essential. The primary ICD-11 code for this condition is `LA00` (Anencephaly or similar anomalies). Specific sub-classifications are available for more detailed reporting:
  • `LA00.0` Anencephaly
  • `LA00.1` Iniencephaly
  • `LA00.2` Acephaly
  • `LA00.3` Amyelencephaly
  • `LA00.Y` Other specified anencephaly or similar anomalies
  • `LA00.Z` Anencephaly or similar anomalies, unspecified
Healthcare providers should use the most specific code applicable to the patient's condition. This ICD-11 code for anencephaly ensures proper documentation for clinical and statistical purposes.

Managing Anencephaly or Similar Anomalies

Anencephaly or similar anomalies (ICD-11 code `LA00`) is a condition incompatible with sustained life. Management strategies focus on palliative care and comprehensive support for the affected family. Decisions regarding pregnancy management, including continuation or palliative birth, are sensitive and require careful consideration. Postnatal care is typically focused on comfort and support.

Frequently asked questions

What is classified under ICD-11 code LA00?
ICD-11 code LA00 classifies anencephaly or similar anomalies, which are malformations of the nervous system caused by the failure of neuropore closure. This category encompasses conditions where neural structures above the brainstem are absent or underdeveloped, often with partial or complete absence of the cranial vault.
How are specific types of anencephaly or similar anomalies coded within ICD-11 LA00?
ICD-11 provides specific codes for subtypes of anencephaly or similar anomalies, including LA00.0 for Anencephaly, LA00.1 for Iniencephaly, LA00.2 for Acephaly, and LA00.3 for Amyelencephaly.
When should unspecified or other specified codes be used for anencephaly or similar anomalies in ICD-11?
For cases where the specific type of anencephaly or similar anomaly is not documented, LA00.Z (Anencephaly or similar anomalies, unspecified) should be used. If the anomaly is specified but does not fit into a more specific category, LA00.Y (Other specified anencephaly or similar anomalies) is applicable.
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