NPI Atlas

ICD-10 Code for Arnold-Chiari Disease

The default ICD-10-CM 2027 code for arnold-chiari disease, obstruction or syndrome (type II) is Q07.00 – Arnold-Chiari syndrome without spina bifida or hydrocephalus. More specific codes depend on the details documented, as listed in the official index (5 codes, 5 billable).

Main code: Q07.00 – Arnold-Chiari syndrome without spina bifida or hydrocephalus
Billable: Yes

Index entries for Arnold-Chiari disease

with
- hydrocephalus – Q07.02
  - with spina bifida – Q07.03
- spina bifida – Q07.01
  - with hydrocephalus – Q07.03
type III – see Encephalocele
type IV – Q04.8

How to choose the right code

Start with the main term, then pick the indented subterm that matches the documentation (type, cause, site, laterality, episode of care). Always confirm the code in the tabular list: follow its Excludes1/Excludes2, “code first” and “use additional code” notes, and add a 7th character where required.

Source: CDC/NCHS ICD-10-CM FY2027 alphabetic index.