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ICD-10-CM 2027 diagnosis code

Q89.81Kabuki syndrome

✓ Billable / specificPOA exemptCC — complication/comorbidity

Q89.81 is a valid, billable ICD-10-CM code for kabuki syndrome. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Code last changed in FY2026 (effective October 1, 2025). Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About Q89.81

Q89.81 is the ICD-10-CM diagnosis code for Kabuki syndrome. It belongs to category Q89 (other congenital malformations, not elsewhere classified), block Q80-Q89 (other congenital malformations) and chapter 17 (congenital malformations, deformations, chromosomal abnormalities, and genetic disorders). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within Q89.8 (other specified congenital malformations), Q89.81 is specifically for Kabuki syndrome. Related codes cover other specified congenital malformations (Q89.89).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 564 (Other Musculoskeletal System and Connective Tissue Diagnoses with MCC, relative weight 1.4908), DRG 565 (Other Musculoskeletal System and Connective Tissue Diagnoses with CC, relative weight 0.9783) and DRG 566 (Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC, relative weight 0.7378), in MDC 08 (Diseases and Disorders of the Musculoskeletal System and Connective Tissue), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It is exempt from present-on-admission (POA) reporting, so no POA indicator is required.

It was added in FY2026, effective October 1, 2025.

Coding notes

Applicable toConditions and synonyms classified to this code
  • Kabuki syndrome, type 1, due to KMT2D mutation
  • Kabuki syndrome, type 2, due to KDM6A mutation
  • Niikawa-Kuroki syndrome

Notes that apply from higher levels

Instructions written at a parent level also apply to Q89.81.

› From Q89.8 Other specified congenital malformations
Use additional code
  • code(s) to identify all associated manifestations

Broader instructions also apply from Chapter 17: Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders.

Alphabetic index entries

2 entries

Terms in the official ICD-10-CM index that lead to Q89.81.

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2026 (effective 10/1/2025): Added
  • No changes since FY2026.

Common questions about Q89.81

Is Q89.81 billable?

Yes. Q89.81 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can Q89.81 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict Q89.81 as a principal diagnosis.

Is Q89.81 a CC or MCC?

Q89.81 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.

What DRG does Q89.81 group to?

Q89.81 is used in the MS-DRG v44.0 logic for MS-DRG 564 (Other Musculoskeletal System and Connective Tissue Diagnoses with MCC), MS-DRG 565 (Other Musculoskeletal System and Connective Tissue Diagnoses with CC) and MS-DRG 566 (Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.