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ICD Hub / Developmental anomalies (Q00–Q99) / LA00.0Y - Other specified anencephaly
ICD-11

ICD-11 Codes
for LA00.0Y - Other specified anencephaly
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Overview of Other Specified Anencephaly (ICD-11 LA00.0Y)

Other specified anencephaly, identified by the ICD-11 code LA00.0Y, represents a severe congenital malformation involving the incomplete development of the brain and skull. This condition is a type of neural tube defect, occurring when the neural tube fails to close properly during the early stages of fetal development. Infants with this diagnosis are typically born without major portions of their brain and skull. The classification 'other specified' indicates a particular variation or subtype of anencephaly that requires precise coding, distinguishing it from more general or unspecified forms. This condition is invariably life-limiting.

Causes and Risk Factors for Neural Tube Defects

The fundamental cause of anencephaly and its specified variations is a developmental anomaly in the formation and closure of the neural tube, which occurs within the first month of pregnancy. This tube is crucial for the development of the central nervous system, including the brain, skull, and spinal cord. Failure of the upper portion of the neural tube to close leads to the absence of key brain structures and the cranial vault. Several factors are known to increase the risk of neural tube defects:
  • Inadequate intake of folic acid (vitamin B9) before and during early pregnancy.
  • Maternal diabetes that is not adequately managed.
  • Exposure to certain medications during early pregnancy, such as some antiseizure drugs (e.g., phenytoin, carbamazepine, valproic acid) and opioids.
  • Maternal obesity.

Diagnosing Other Specified Anencephaly

The diagnosis of other specified anencephaly is typically established through prenatal screening and diagnostic procedures. Healthcare professionals utilise various methods to identify the condition during pregnancy:
  • Prenatal Ultrasound: This imaging technique allows visualisation of the fetal skull and brain, revealing the absence of major structures. Specific sonographic findings can suggest the diagnosis.
  • Maternal Serum Screening: Blood tests, including the quad marker screen, can detect elevated levels of alpha-fetoprotein (AFP), a marker often associated with neural tube defects.
  • Fetal Magnetic Resonance Imaging (MRI): For a more detailed assessment, MRI can provide enhanced images of the fetal brain and skull, confirming the extent of malformation.
  • Amniocentesis: Analysis of amniotic fluid may reveal elevated levels of AFP and acetylcholinesterase, further supporting the diagnosis.
Postnatal diagnosis can also occur via physical examination if prenatal testing is not performed.

Clinical Presentation of Anencephaly Variations

Infants diagnosed with other specified anencephaly present with severe physical characteristics due to the absence of critical brain and skull components. Key features include:
  • A significant portion of the skull and scalp is missing.
  • Brain tissue may be exposed and appear abnormal.
  • The head size is typically smaller than expected.
Newborns with this condition lack consciousness, vision, and hearing. While some may exhibit basic reflexes controlled by the brainstem, such as spontaneous breathing or heartbeat, they do not possess the higher brain functions necessary for awareness or sensory perception. The condition is invariably fatal, with most affected infants dying shortly after birth or experiencing intrauterine fetal demise. The ICD-11 code LA00.0Y, 'Other specified anencephaly,' is used to classify specific variations of anencephaly. Related terms and conditions that may fall under this classification include:
  • Anencephaly
  • Neural tube defect (NTD)
  • Congenital malformation of the central nervous system
  • Acalvaria: A condition characterised by the absence of the calvarial bones, dura mater, and associated muscles, while the skull base, facial bones, and brain remain intact. Acalvaria can be coded under LA00.0Y.
  • Meroanencephaly: A subtype involving partial absence of the brain and skull.
  • Holoanencephaly: A subtype involving complete absence of the brain.
  • Craniorachischisis: A severe form involving the absence of the brain, skull, and spine.

Coding and Documentation for Other Specified Anencephaly

Accurate clinical documentation is essential for correctly assigning the ICD-11 code LA00.0Y for 'Other specified anencephaly.' Healthcare providers should detail the specific findings that differentiate this condition from general anencephaly or other related neural tube defects. This precise coding ensures accurate medical records, facilitates appropriate clinical management, and supports billing processes. The ICD-11 code for other specified anencephaly is LA00.0Y, and it is used when the anencephaly presents with specific, documented characteristics that do not fit into broader or unspecified categories. This code is reportable and billable when associated with patient encounters requiring diagnosis.

Frequently asked questions

What is the ICD-11 classification for 'Other specified anencephaly'?
LA00.0Y is a specific category within the ICD-11 classification for Anencephaly or similar anomalies (LA00), used when the anencephaly is specified but does not fit into other defined subcategories.
What is the ICD-11 classification meaning of Anencephaly?
Anencephaly is classified under ICD-11 code LA00.0 and is characterized by the total or partial absence of the cranial vault and covering skin, with the brain being missing or reduced.
How does the ICD-11 code LA00.0Y, 'Other specified anencephaly', differ from 'Anencephaly, unspecified' (LA00.0Z)?
LA00.0Y is used when the anencephaly is specified but does not fit into more precise categories, while LA00.0Z is used when the anencephaly is not specified at all.
What is the purpose of the 'Y' suffix in ICD-11 codes like LA00.0Y?
The terminal letter 'Y' in ICD-11 codes indicates a residual category for 'other specified' conditions, used when a condition is specified but lacks a more precise code.
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