ICD-10-CM Code Q00.0

Anencephaly

Version 2020 Billable Code POA Exempt

Valid for Submission

Q00.0 is a billable code used to specify a medical diagnosis of anencephaly. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code Q00.0 might also be used to specify conditions or terms like abnormal fetus, acardia, acephalobrachius, acephalocheiria, acephaly, acrania, etc The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

ICD-10:Q00.0
Short Description:Anencephaly
Long Description:Anencephaly

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code Q00.0:

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

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code Q00.0 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Abnormal fetus
  • Acardia
  • Acephalobrachius
  • Acephalocheiria
  • Acephaly
  • Acrania
  • Amyelencephalus
  • Anencephalus
  • Anencephaly without rachischisis
  • Congenital brain aplasia
  • Fetal anencephaly
  • Frog fetus
  • Hemianencephaly
  • Hemicephaly
  • Holoacardius acephalus
  • Holoanencephaly
  • Incomplete anencephaly
  • Parasitic twin of asymmetrical conjoined twins

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

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 CodePOA Reason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q00.0 to ICD-9

Code Classification

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

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients


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


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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.
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