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ICD-10-CM 2027

ICD-10 code for Craniorachischisis

Q00.1

Craniorachischisis

From the official ICD-10-CM alphabetic index entry “Craniorachischisis (totalis)”. Page updated September 29, 2026.

About coding Craniorachischisis

The ICD-10-CM code for Craniorachischisis is Q00.1 (Craniorachischisis).

Within Q00 (anencephaly and similar malformations), Q00.1 is specifically for Craniorachischisis. Related codes cover anencephaly (Q00.0) and Iniencephaly (Q00.2).

As a secondary diagnosis it is an MCC (major complication or comorbidity), which can move an inpatient stay into the highest-paying "with MCC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 091 (Other Disorders of Nervous System with MCC, relative weight 1.7046), DRG 092 (Other Disorders of Nervous System with CC, relative weight 1.0239), DRG 093 (Other Disorders of Nervous System without CC/MCC, relative weight 0.7783), DRG 791 (Prematurity with Major Problems, relative weight 4.1075) and DRG 793 (Full Term Neonate with Major Problems, relative weight 4.2195), in MDC 01 (Diseases and Disorders of the Nervous System) and MDC 15 (Newborns and Other Neonates with Conditions Originating in the Perinatal Period), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

Common questions about Craniorachischisis ICD-10 codes

What is the ICD-10 code for Craniorachischisis?

Q00.1 — Craniorachischisis.

Is Q00.1 billable?

Yes. Q00.1 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can Q00.1 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict Q00.1 as a principal diagnosis.

Is Q00.1 a CC or MCC?

Q00.1 is an MCC (major complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.