Q89.81 — Kabuki syndrome
Is Q89.81 billable?
Yes — Q89.81 is billable for FY2027. Q89.81 is a valid, billable ICD-10-CM code at the highest level of specificity in its branch. Q89.81 may be submitted for encounters from October 1, 2026 through September 30, 2027.
Q89.81 at a glance
| Code | Q89.81 |
|---|---|
| Description | Kabuki syndrome |
| Billable | Yes |
| Code set | ICD-10-CM FY2027 |
| Valid for encounters | October 1, 2026 – September 30, 2027 |
Which MS-DRGs does Q89.81 group to?
Q89.81 sits in MDC 08 and helps define the logic of 3 MS-DRGs (version 44, FY2027). Which one a stay actually groups to also depends on procedures and secondary diagnoses.
- 564Other Musculoskeletal System and Connective Tissue Diagnoses with MCCmedical
- 565Other Musculoskeletal System and Connective Tissue Diagnoses with CCmedical
- 566Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCCmedical
As a secondary diagnosis, Q89.81 is a complication or comorbidity (CC), which can move a stay into a higher-paying DRG.
How is Q89.81 listed in the Alphabetic Index?
These are the routes through the Alphabetic Index that lead to Q89.81. They show the wording a clinician may have documented, which often differs from the Tabular description.
- Syndrome › Kabuki
- Syndrome › Niikawa-Kuroki
Inclusion terms
Alternative wording in documentation that is classified to Q89.81.
- Kabuki syndrome, type 1, due to KMT2D mutation
- Kabuki syndrome, type 2, due to KDM6A mutation
- Niikawa-Kuroki syndrome
Use additional code — inherited from Q89.8
Report an additional code alongside Q89.81 to fully describe the condition. Q89.81 is sequenced first.
- code(s) to identify all associated manifestations
Excludes2 — not included here — inherited from Chapter 17
The conditions below are not part of Q89.81, but a patient may have both at the same time. When documentation supports it, Q89.81 and the excluded code may both be reported.
- inborn errors of metabolism (E70-E88)
How long has Q89.81 existed?
Q89.81 first appears in FY2026.
Derived from the official order files for FY2016–FY2026. FY2017 and FY2020 publish no order file, so those years are not covered.