2026 ICD-10-CM Diagnosis Code Q89.81Kabuki syndrome

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q89

ICD-10-CM Q89.81
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q89.81 is a billable ICD-10-CM diagnosis code for kabuki syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 564 through 566. The code is exempt from POA reporting. Coders also document this condition as kabuki make-up syndrome.

Code Identity

ICD-10-CM Code
Q89.81
Billable Status
Yes — Valid for Submission
Code Describes
Kabuki syndrome
Short Description
Kabuki syndrome
Same as the full description in the CMS dataset.
Parent Code
Q89.8
Other specified congenital malformations

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ89Other congenital malformations, not elsewhere classified
This CodeQ89.81Kabuki syndrome

Present on Admission (POA)Billing

Q89.81 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Kabuki make-up syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Syndrome
      • Kabuki (type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)
    • Syndrome
      • Niikawa-Kuroki

Clinical InformationClinical

  • Stickler Syndrome

    a rare autosomal dominant syndrome caused by mutations in the col11a1, col11a2, and col2a1 genes which affect the production of type ii and xi collagen. it is characterized by a range of signs and symptoms including cleft palate, large tongue, small lower jaw, hearing loss, myopia, glaucoma, retinal detachment, skeletal, and joint abnormalities.

  • Stickler Syndrome Type 1|STL1

    stickler syndrome inherited in an autosomal dominant pattern, caused by mutation(s) in the col2a1 gene, encoding collagen alpha-1(ii) chain.

  • Stickler Syndrome Type 2|Stickler Syndrome Type II

    a rare autosomal dominant syndrome caused by mutations in the col11a1 gene. it is characterized by an abnormal ocular vitreous architecture (beaded vitreous phenotype). other signs and symptoms include retinal detachment, joint hypermobility, hearing loss, and midline clefting.

Code History & ChangesHistory

New Code Q89.81 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.

Replacement Q89.81 replaces the following previously assigned code(s):

  • Q89.8 - Other specified congenital malformations
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About Q89.81Overview

Is Q89.81 (Other specified congenital malformations) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report kabuki syndrome on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q89.81 group to?

When kabuki syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 565, 566, with relative weights from 0.7493 to 1.5436 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q89.81 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for kabuki syndrome on inpatient claims.