Anencephaly and similar malformations (Q00)
The ICD-10 code section Q00 covers anencephaly and related neural tube defects, which are serious congenital malformations affecting the brain and spinal cord development.
This section includes specific codes like Q00.0 for anencephaly, a condition also known by terms such as congenital brain aplasia, acrania, and holoanencephaly, characterized by absence of major parts of the brain and skull. Code Q00.1 denotes craniorachischisis, where both the brain and spinal cord remain open due to failed closure of the neural tube. Meanwhile, Q00.2 identifies iniencephaly, a malformation involving severe neck and spinal defects often linked with abnormal cervical vertebrae positioning. These codes are crucial for accurately recording and reporting congenital nervous system anomalies. Knowing the synonyms helps medical coders correctly assign the ICD-10 code for conditions like acrania or craniorachischisis when documentation uses alternate terms, ensuring precise diagnosis coding and data capture.
Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
Congenital malformations of the nervous system (Q00-Q07)
Q00 Anencephaly and similar malformations
- Q00.0 Anencephaly
- Q00.1 Craniorachischisis
- Q00.2 Iniencephaly
Anencephaly and similar malformations (Q00)
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
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)
