Q00.2Iniencephaly

Q00.2 is the ICD-10-CM diagnosis code for Iniencephaly. It belongs to category Q00 (anencephaly and similar malformations), block Q00-Q07 (congenital malformations of the nervous system) and chapter 17 (congenital malformations, deformations, chromosomal abnormalities, and genetic disorders). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

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

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.

It is exempt from present-on-admission (POA) reporting, so no POA indicator is required.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 740.2 (Iniencephaly).