Q01.9

Encephalocele, unspecified

FY2027Chapter 17: Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders/Q00–Q07 Congenital malformations of the nervous system

BillableComplete code, valid to report

Q01.9 (also written Q019) is a billable ICD-10-CM diagnosis code for encephalocele, unspecified. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 5 codes in category Q01 (Encephalocele). The Alphabetic Index lists it under 3 entries. Its coding notes include 1 Excludes1 entry and 1 Excludes2 entry.

Guidelines tip

From the FY2027 Official Guidelines. ICD10Ref adds no rules of its own.

Chapter 17 codes can be used for life. Manifestations that are an inherent part of a specifically coded anomaly are not coded separately. A corrected malformation is reported with a personal history code.

Guidelines I.C.17Congenital malformations and genetic disordersRead the official text

Excludes1

Not coded here. The listed conditions generally should not be reported together.

Inherited from Q01 Encephalocele
  • Meckel-Gruber syndrome (Q61.9)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Also from chapter/block level (1 note)
Inherited from Chapter 17 Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders
  • inborn errors of metabolism (E70-E88)

Care settings

How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.

IRF 60% Rule

On the CMS list of diagnoses used to determine presumptive compliance with the IRF 60 percent rule.

Source: CMS IRF Presumptive Compliance-3, FY 2027 IRF PPS final rule files, effective for discharges on or after October 1, 2026

Hospital CC

Counts as a complication or comorbidity (CC) when reported as a secondary diagnosis on an inpatient claim. It does not count when the principal diagnosis is one of the 14 codes in its exclusion list. Some MS-DRGs also ignore a CC or MCC that defines the DRG.

Source: CMS MS-DRG Definitions Manual v44, effective October 1, 2026, Appendix C

Includes

Further defines or gives examples of the content of the category.

Inherited from Q01 Encephalocele
  • Arnold-Chiari syndrome, type III
  • encephalocystocele
  • encephalomyelocele
  • hydroencephalocele
  • hydromeningocele, cranial
  • meningocele, cerebral
  • meningoencephalocele

Notes

Also from chapter/block level (1 note)
Inherited from Chapter 17 Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders
  • Codes from this chapter are not for use on maternal records

Alphabetic Index terms

Terms the official Alphabetic Index lists for this code. 3 entries