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  6. 2026 ICD-10-CM Code Q89.81

2026 ICD-10-CM Diagnosis Code Q89.81

Kabuki syndrome

ICD-10-CM Code:
Q89.81
ICD-10 Code for:
Kabuki syndrome
Is Billable?
Yes - Valid for Submission
Code Navigator:

Q89.81 is a billable diagnosis code used to specify a medical diagnosis of kabuki syndrome. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2025 through September 30, 2026. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    Q00-Q99
    • Other congenital malformations
      Q80-Q89
      • Other congenital malformations, not elsewhere classified
        Q89

Approximate Synonyms

The following list of clinical terms are approximate synonyms, alternative descriptions, or common phrases that might be used by patients, healthcare providers, or medical coders to describe the same condition. These synonyms and related diagnosis terms are often used when searching for an ICD-10 code, especially when the exact medical terminology is unclear. Whether you're looking for lay terms, similar diagnosis names, or common language alternatives, this list can help guide you to the correct ICD-10 classification.

  • Kabuki make-up syndrome

Clinical Information

  • 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.

New 2026 ICD-10-CM Code

Q89.81 is new to ICD-10-CM code set for the FY 2026, effective October 1, 2025. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2025. This is a new and revised code for the FY 2026 (October 1, 2025 - September 30, 2026).

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Kabuki syndrome, type 1, due to KMT2D mutation
  • Kabuki syndrome, type 2, due to KDM6A mutation
  • Niikawa-Kuroki syndrome

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Index of External Cause of Injuries

References found for this diagnosis 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

Present on Admission (POA)

Q89.81 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA Indicator: Y

Reason: Diagnosis was present at time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: N

Reason: Diagnosis was not present at time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: U

Reason: Documentation insufficient to determine if the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: W

Reason: Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: 1

Reason: Unreported/Not used - Exempt from POA reporting.

CMS Pays CC/MCC DRG? NO

Replacement Code

Q8981 replaces the following previously assigned ICD-10-CM code(s):

  • Q89.8 - Other specified congenital malformations

Code History

  • FY 2026 - Code Added, effective from 10/1/2025 through 9/30/2026