2025 ICD-10-CM Diagnosis Code Q00.0

Anencephaly

ICD-10-CM Code:
Q00.0
ICD-10 Code for:
Anencephaly
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Q00.0 is a billable diagnosis code used to specify a medical diagnosis of anencephaly. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2024 through September 30, 2025. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    Q00-Q99
    • Congenital malformations of the nervous system
      Q00-Q07
      • Anencephaly and similar malformations
        Q00

Approximate Synonyms

The following list of clinical terms are approximate synonyms, alternative descriptions, or common phrases that might be used by patients, healthcare providers, or medical coders to describe the same condition. These synonyms and related diagnosis terms are often used when searching for an ICD-10 code, especially when the exact medical terminology is unclear. Whether you're looking for lay terms, similar diagnosis names, or common language alternatives, this list can help guide you to the correct ICD-10 classification.

  • Acardia
  • Acephalobrachius
  • Acephalocheiria
  • Acephaly
  • Acrania
  • Amyelencephalus
  • Anencephalus
  • Anencephaly without rachischisis
  • Congenital absence of left hand
  • Congenital absence of right hand
  • Congenital brain aplasia
  • Hemianencephaly
  • Hemicephaly
  • Holoacardius acephalus
  • Holoanencephaly
  • Incomplete anencephaly

Clinical Classification

Clinical Classifications group individual ICD-10-CM diagnosis codes into broader, clinically meaningful categories. These categories help simplify complex data by organizing related conditions under common clinical themes.

They are especially useful for data analysis, reporting, and clinical decision-making. Even when diagnosis codes differ, similar conditions can be grouped together based on their clinical relevance. Each category is assigned a unique CCSR code that represents a specific clinical concept, often tied to a body system or medical specialty.

Nervous system congenital anomalies

CCSR Code: MAL004

Inpatient Default: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.

Outpatient Default: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Clinical Information

  • Anencephaly

    a malformation of the nervous system caused by failure of the anterior neuropore to close. infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. the skull is only partially formed but the eyes are usually normal. this condition may be associated with folate deficiency. affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (from menkes, textbook of child neurology, 5th ed, p247)

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Acephaly
  • Acrania
  • Amyelencephaly
  • Hemianencephaly
  • Hemicephaly

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Present on Admission (POA)

Q00.0 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA Indicator: Y

Reason: Diagnosis was present at time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: N

Reason: Diagnosis was not present at time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: U

Reason: Documentation insufficient to determine if the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: W

Reason: Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: 1

Reason: Unreported/Not used - Exempt from POA reporting.

CMS Pays CC/MCC DRG? NO

Convert Q00.0 to ICD-9-CM

Below are the ICD-9 codes that most closely match this ICD-10 code, based on the General Equivalence Mappings (GEMs). This ICD-10 to ICD-9 crosswalk tool is helpful for coders who need to reference legacy diagnosis codes for audits, historical claims, or approximate code comparisons.

Anencephalus

ICD-9-CM: 740.0

This is a direct match with no additional mapping qualifiers. The absence of a flag generally means the mapping is considered exact or precise. In other words, the ICD-10 code maps cleanly to the ICD-9 code without qualification, approximation, or needing multiple codes.

Patient Education


Neural Tube Defects

Neural tube defects are birth defects of the brain, spine, or spinal cord. They happen in the first month of pregnancy, often before a woman even knows that she is pregnant. The two most common neural tube defects are spina bifida and anencephaly. In spina bifida, the fetal spinal column doesn't close completely. There is usually nerve damage that causes at least some paralysis of the legs. In anencephaly, most of the brain and skull do not develop. Babies with anencephaly are usually either stillborn or die shortly after birth. Another type of defect, Chiari malformation, causes the brain tissue to extend into the spinal canal.

The exact causes of neural tube defects aren't known. You're at greater risk of having an infant with a neural tube defect if you:

  • Have obesity
  • Have poorly controlled diabetes
  • Take certain antiseizure medicines

Getting enough folic acid, a type of B vitamin, before and during pregnancy prevents most neural tube defects.

Neural tube defects are usually diagnosed before the infant is born, through lab or imaging tests. There is no cure for neural tube defects. The nerve damage and loss of function that are present at birth are usually permanent. However, a variety of treatments can sometimes prevent further damage and help with complications.

NIH: National Institute of Child Health and Human Development


[Learn More in MedlinePlus]

Anencephaly

Anencephaly is a condition that prevents the normal development of the brain and the bones of the skull. This condition results when a structure called the neural tube fails to close during the first few weeks of embryonic development. The neural tube is a layer of cells that ultimately develops into the brain and spinal cord. Because anencephaly is caused by abnormalities of the neural tube, it is classified as a neural tube defect.

Because the neural tube fails to close properly, the developing brain and spinal cord are exposed to the amniotic fluid that surrounds the fetus in the womb. This exposure causes the nervous system tissue to break down (degenerate). As a result, people with anencephaly are missing large parts of the brain called the cerebrum and cerebellum. These brain regions are necessary for thinking, hearing, vision, emotion, and coordinating movement. The bones of the skull are also missing or incompletely formed.

Because these nervous system abnormalities are so severe, almost all babies with anencephaly die before birth or within a few hours or days after birth.


[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.