Q89.81 ICD-10-CM Code: Kabuki syndrome
A clear reference for the official code description, hierarchy, and related coding information from the FY 2027 U.S. release.
Code definition
Official description
Q89.81Kabuki syndrome
ICD-10-CM diagnosis code · United States clinical modification
Classification
Where Q89.81 sits
- 1
Chapter 17
Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders (Q00-QA1)
- 2
Section
Other congenital malformations (Q80-Q89)
- 3
Category Q89
Other congenital malformations, not elsewhere classified
- 4
Subcategory Q89.8
Other specified congenital malformations
- 5
Code Q89.81
Kabuki syndrome
Documentation details
Notes for this code
Inclusion terms
- Kabuki syndrome, type 1, due to KMT2D mutation
- Kabuki syndrome, type 2, due to KDM6A mutation
- Niikawa-Kuroki syndrome
Code first
No “code first” instruction is listed for Q89.81.
Use additional code
No “use additional code” instruction is listed for Q89.81.
Excludes1
Conditions that should not be reported together with this code.
No Excludes1 note is listed for Q89.81.
Excludes2
Conditions not included here that may be reported separately when present.
No Excludes2 note is listed for Q89.81.
Transparency
Source and release
This reference is generated from Vero’s ICD-10-CM description, hierarchy, and code-note dataset. The visible content and structured data share that source.
Review the CDC/NCHS ICD-10-CM sourceCode-specific guidance
Frequently asked questions
What does ICD-10-CM code Q89.81 mean?
Q89.81 is the FY 2027 U.S. ICD-10-CM code for “Kabuki syndrome.”
Is Q89.81 a billable or specific ICD-10-CM code?
Yes. In the FY 2027 reference data used on this page, Q89.81 has no child codes and is presented as a billable/specific code. Always confirm the active code set and payer requirements before use.
Where is Q89.81 located in ICD-10-CM?
Q89.81 is listed in Chapter 17, Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders (Q00-QA1), within Other congenital malformations (Q80-Q89) (Q80–Q89), under category Q89, Other congenital malformations, not elsewhere classified and subcategory Q89.8, Other specified congenital malformations.
What coding notes are listed for Q89.81?
Its listed inclusion terms are Kabuki syndrome, type 1, due to KMT2D mutation; Kabuki syndrome, type 2, due to KDM6A mutation; Niikawa-Kuroki syndrome. No code-first instruction is listed. No Excludes1 note is listed. No Excludes2 note is listed.
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