2026 ICD-10-CM Diagnosis Code Q00.1Craniorachischisis

ICD-10-CM Codes›Q00-Q99›Q00-Q07›Q00

ICD-10-CM Q00.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q00.1 is a billable ICD-10-CM diagnosis code for craniorachischisis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Nervous system congenital anomalies.

Code Identity

ICD-10-CM Code
Q00.1
Billable Status
Yes — Valid for Submission
Code Describes
Craniorachischisis
Short Description
Craniorachischisis
Same as the full description in the CMS dataset.
Parent Code
Q00
Anencephaly and similar malformations

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ00-Q07Congenital malformations of the nervous system
CategoryQ00Anencephaly and similar malformations
This CodeQ00.1Craniorachischisis

Present on Admission (POA)Billing

Q00.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Craniorachischisis

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Craniorachischisis(totalis)

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL004
Nervous system congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

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.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q00.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
740.1 Craniorachischisis
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q00.1Overview

Is Q00.1 (Anencephaly and similar malformations) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report craniorachischisis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

Is Q00.1 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for craniorachischisis on inpatient claims.

What is the ICD-9 equivalent of Q00.1?

Under the General Equivalence Mappings, craniorachischisis converts to ICD-9-CM 740.1 (craniorachischisis). The mapping is a direct match.

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.