Q89.81
Billable codeKabuki syndrome
The ICD-10 code for kabuki syndrome is Q89.81.
Clinical notes
Also known as
Alternate wording, synonyms, or specific conditions that fall under this code, listed to help confirm you've picked the right one.
- Kabuki syndrome, type 1, due to KMT2D mutation
- Kabuki syndrome, type 2, due to KDM6A mutation
- Niikawa-Kuroki syndrome
Documentation support
General coding-documentation guidance — not a substitute for payer-specific requirements or professional coding judgment.
- •As a general rule, code to the highest level of specificity supported by the documentation in the medical record.
Related codes in this category
- Q89.89Other specified congenital malformations
- Q89.81Kabuki syndrome
Associated MS-DRGs
Q89.81 can serve as the principal diagnosis for these Medicare Severity Diagnosis-Related Groups.
Informational only — actual DRG assignment also depends on procedures, complications/comorbidities (CC/MCC), discharge status, and payer-specific rules not reflected here.