Q89.81

Kabuki syndrome

Billable code

This code does not map to a risk-adjusting HCC in the loaded models.

Coding notes

Excludes 1 never coded together · Excludes 2 may co-exist

This code · Q89.81
Synonyms

Kabuki syndrome, type 1, due to KMT2D mutation Kabuki syndrome, type 2, due to KDM6A mutation Niikawa-Kuroki syndrome

Inherited from Q89.8Other specified congenital malformations
Use additional

code(s) to identify all associated manifestations

Inherited from chapter 17Congenital malformations, deformations and chromosomal abnormalities
Excludes 2

inborn errors of metabolism (E70-E88)

Note

Codes from this chapter are not for use on maternal records

Official Guidelines for Q89.81

FY 2026 ›
I.C.17Chapter-specific guidanceChapter 17: Congenital malformations, deformations, and chromosomal abnormalities (Q00-QA1)

Sections that reference Q89.81

Sections whose FY 2026 text names Q89.81, its Q89 category, or a range containing it — the chapter guidance above is the authoritative source.

Find Q89.81 in the alphabetic index

2 index paths lead to this code