Q00.0

Billable
ChapterQ00–Q99
Ranksubcategory
Official Description

Anencephaly

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Billable/specific code (valid for reimbursement).
  • •Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-Q99).

Instructional Notes

Includes
  • Acephaly
  • Acrania
  • Amyelencephaly
  • Hemianencephaly
  • Hemicephaly
General instructions are available for the parent sections:
View CH17 Notes

Clinical Hierarchy

CH17Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)
Q00-Q07Congenital malformations of the nervous system (Q00-Q07)
Q00Anencephaly and similar malformations
Q00.0Anencephaly

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to Q00.0:

chapterCH17
Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to Q00.0. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

22 raw paths
Absence (of) (organ or part) (complete or partial)2 unique routes
brain→Q00.0
skull bone (congenital)
with
anencephaly→Q00.0
Acephalia, acephalism, acephalus, acephaly1 unique routes
Direct index entry→Q00.0
Acrania1 unique routes
Direct index entry→Q00.0
Agenesis2 unique routes
brain→Q00.0
skull (bone)
with
anencephaly→Q00.0
Amyelencephalus, amyelencephaly1 unique routes
Direct index entry→Q00.0
Anencephalus, anencephaly1 unique routes
Direct index entry→Q00.0
Anomaly, anomalous (congenital) (unspecified type)5 unique routes
bone
skull
with
anencephaly→Q00.0
hemianencephaly→Q00.0
hemicephaly→Q00.0
hemicrania→Q00.0
skull
with
anencephaly→Q00.0
Aplasia1 unique routes
brain→Q00.0
Deformity1 unique routes
skull (acquired)
congenital
with
anencephaly→Q00.0
Distortion (s) (congenital)1 unique routes
skull bone (s) NEC
with
anencephalus→Q00.0
Hemianencephaly1 unique routes
Direct index entry→Q00.0
Hemicephalus, hemicephaly1 unique routes
Direct index entry→Q00.0
Hemicrania1 unique routes
congenital malformation→Q00.0
Hypoplasia, hypoplastic1 unique routes
skull (bone)
with
anencephaly→Q00.0
Imperfect1 unique routes
closure (congenital)
skull
with
anencephaly→Q00.0
Monster, monstrosity (single)1 unique routes
acephalic→Q00.0

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 091
Other Disorders of Nervous System with MCC
DRG 092
Other Disorders of Nervous System with CC
DRG 093
Other Disorders of Nervous System without CC/MCC
DRG 791
Prematurity with Major Problems
DRG 793
Full Term Neonate with Major Problems

Cross Reference

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