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ICD Hub / Developmental anomalies (Q00–Q99) / LA00.2 - Acephaly
ICD-11

ICD-11 Codes
for LA00.2 - Acephaly
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Acephaly (ICD-11 Code LA00.2)

Acephaly is a severe congenital malformation characterized by the absence of the head. This condition arises from a failure of the neural tube to close properly during early embryonic development, specifically the failure of the neuropore to close. As a critical neural tube defect (NTD), acephaly is incompatible with life, and infants born with this condition do not survive. While the head is absent, infants may possess an intact spinal cord, cerebellum, and brainstem.

Alternative Names for Acephaly

This condition is also referred to by several other clinical terms, including Acephalia, Acephalism, and Congenital absence of head. These terms all describe the same severe developmental anomaly.

Factors Contributing to Acephaly

The primary cause of acephaly is the failure of the neural tube to close correctly during the initial weeks of pregnancy. Specifically, the neuropore fails to seal, leading to the absence of cranial structures. While the exact triggers are not always identified, factors associated with neural tube defects in general can increase the risk. These include genetic predispositions, nutritional deficiencies such as insufficient folic acid (vitamin B9) before and during early pregnancy, and certain maternal health conditions or exposures.

Identifying Acephaly

Diagnosis of acephaly is typically made during prenatal screening. Advanced imaging techniques such as ultrasound can reveal the absence of the head and skull structures. Blood tests, like the alpha-fetoprotein (AFP) test, may also indicate neural tube defects. In some instances, diagnosis might occur postnatally if prenatal screening was not performed or was inconclusive.

Managing Acephaly

Acephaly is a fatal condition with no available cure or treatment. Medical management focuses on supportive care for the mother and, if the infant survives birth, providing comfort and dignity. The prognosis for infants with acephaly is extremely poor, with survival limited to a very short period after birth.

Coding and Documentation for Acephaly

The official ICD-11 code for Acephaly is LA00.2. This code falls under the broader category of Anencephaly or similar anomalies (LA00). Accurate documentation is crucial for medical records and reporting, clearly identifying the condition as Acephaly (LA00.2) and noting its severe, fatal nature.

Frequently asked questions

What is the ICD-11 classification meaning of Acephaly (LA00.2)?
Acephaly (LA00.2) is classified in ICD-11 as a specific congenital malformation of the nervous system, categorized under "Anencephaly or similar anomalies" (LA00).
How is Acephaly (LA00.2) categorized within ICD-11, and what is its relationship to other similar conditions?
Acephaly (LA00.2) is a distinct entity within the ICD-11 category "Anencephaly or similar anomalies" (LA00), which is part of Chapter 20: Developmental anomalies. This category groups related malformations of the nervous system.
When would an unspecified category be used in relation to Acephaly (LA00.2) in ICD-11 coding?
In ICD-11, if a condition falls under the "Anencephaly or similar anomalies" group (LA00) but the specific type, such as Acephaly (LA00.2), cannot be determined from documentation, the unspecified category "Anencephaly or similar anomalies, unspecified" (LA00.Z) would be used.
How is Acephaly (LA00.2) used in ICD-11 documentation and coding?
Acephaly (LA00.2) is used as a specific code in ICD-11 documentation to identify and classify this particular congenital malformation of the nervous system, adhering to the structure and definitions provided by the classification.
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