ICD-11 Codes
for LA00.Z - Anencephaly or similar anomalies, unspecified

Teodor Jurukovski January 1, 1970

Fact checked by: Teodor Jurukovski

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Understanding Anencephaly or Similar Anomalies, Unspecified (LA00.Z)

The ICD-11 code LA00.Z designates 'Anencephaly or similar anomalies, unspecified'. This is a severe congenital anomaly, classified as a neural tube defect, characterised by the absence or significant underdevelopment of parts of the brain and skull. It arises from the failure of the neural tube to close properly during the early stages of embryonic development, typically within the first month of pregnancy. This condition affects the central nervous system and is invariably fatal, with affected infants usually surviving only a few hours or days after birth, or not surviving gestation at all.

Factors Contributing to Neural Tube Defects like Anencephaly

The primary cause of anencephaly and similar anomalies is a developmental issue where the neural tube, which forms the central nervous system, fails to close completely. This failure typically occurs very early in pregnancy, often before a woman may even know she is pregnant. While the exact trigger is often unknown, several risk factors are associated with an increased likelihood of these conditions. These include insufficient intake of folic acid (vitamin B9) before and during pregnancy, pre-existing maternal diabetes that is not well-managed, and exposure to certain medications, such as some antiseizure drugs (e.g., phenytoin, carbamazepine, valproic acid) or opioid use during early pregnancy. Although not typically inherited, a history of a previous neural tube defect in a prior pregnancy can increase the risk for subsequent pregnancies.

How Anencephaly or Similar Anomalies are Identified

Diagnosis of anencephaly or similar anomalies can often be made during pregnancy through routine prenatal screening. Elevated levels of alpha-fetoprotein (AFP) detected in the mother's blood or amniotic fluid can be an early indicator. Diagnostic imaging, such as ultrasound or fetal magnetic resonance imaging (MRI), can visualise the absence of skull and brain structures. In cases where specific details are not documented, the unspecified code LA00.Z may be used. Postnatal diagnosis is typically confirmed via physical examination of the newborn.

Outlook and Supportive Care for Anencephaly

There is currently no cure or specific medical treatment for anencephaly or similar anomalies. The condition is universally fatal. Management focuses on supportive care for the mother during pregnancy and after delivery, and providing comfort and dignity for the infant. The prognosis is extremely poor, with most affected pregnancies ending in miscarriage or stillbirth, and infants born alive typically surviving only for a very short period. Affected newborns do not experience pain.

Alternative Terminology and Related Conditions

When documenting or searching for information related to ICD-11 code LA00.Z, various terms may be encountered. These include Anencephaly, absence of brain, absence of skull, and general terms for neural tube defects or cephalic disorders. The "unspecified" designation indicates that the specific type or extent of the anomaly is not detailed in the diagnostic record. Related conditions under the LA00 block include Iniencephaly (LA00.1), Acephaly (LA00.2), and Amyelencephaly (LA00.3).

ICD-11 Coding for LA00.Z: Anencephaly or Similar Anomalies, Unspecified

The ICD-11 code LA00.Z, 'Anencephaly or similar anomalies, unspecified,' is used for medical record documentation and billing when a diagnosis of anencephaly or a related anomaly is made, but specific details regarding the type or extent are not provided or are unknown. This code ensures that the condition is recorded for statistical purposes and health system reporting. It is crucial for accurate health data collection and can be relevant for billing and administrative processes within healthcare settings.