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Q89.81 ICD-10-CM Code: Kabuki syndrome

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Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
3 inclusion terms · 1 use-additional code

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too; the Instructions section marks which is which.

Use additional codeReport with this code when documented
  • code(s) to identify all associated manifestations
IncludesWhat this code covers
  • Kabuki syndrome, type 1, due to KMT2D mutation
  • Kabuki syndrome, type 2, due to KDM6A mutation
  • Niikawa-Kuroki syndrome

CompareCheck ClaimView Related Codes

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v44, Appendix B.

  • MS-DRG 564 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH MCC (MDC 08)
  • MS-DRG 565 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH CC (MDC 08)
  • MS-DRG 566 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITHOUT CC/MCC (MDC 08)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q89.81 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on Q89.81 itself; “inherited from” names the category or block whose note applies here.

Inclusion Terms

Alternative terms the tabular list files under this code.

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

Use Additional Code

Supplementary codes the tabular list directs you to add.

  • Use additional code(s) to identify all associated manifestations

Source: inherited from Q89.8

Coder workflow for Q89.81

MedCoder structured workflow — derived from this code’s own official record

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
The conditions the Use Additional Code note names
Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

  • Use Additional Code — after identifying Q89.81(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q89.81 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

    See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: A condition the Use Additional Code note names is documented.

Coding question: Is a second code reported with Q89.81?

Path: Review the Use Additional Code note and the code it names.

Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Kabuki syndrome is a billable ICD-10-CM diagnosis code (Q89.81).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

  • CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder-derived relationships — computed from published CMS and AHRQ datasets

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 40 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 564 (MDC 08), DRG 565 (MDC 08), DRG 566 (MDC 08).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):MAL010 — Other specified and unspecified congenital anomalies (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.82 — Arterial tortuosity syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.86 — Kleefstra syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q87.A — Loeys-Dietz syndrome, Q89.89 — Other specified congenital malformations, Q99.2 — Fragile X chromosome, Q99.811 — Usher syndrome, type 1, Q99.812 — Usher syndrome, type 2, Q99.813 — Usher syndrome, type 3, Q99.818 — Other Usher syndrome, Q99.819 — Usher syndrome, unspecified, QA0.0101 — SCN2A-related neurodevelopmental disorder, QA0.0102 — CACNA1A-related neurodevelopmental disorder, QA0.0109 — Neurodevelopmental disorder related to pathogenic variant in other ion channel gene, +19 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified congenital anomalies).

Q87.43 — Marfan syndrome with skeletal manifestation, Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.82 — Arterial tortuosity syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.88 — CTNNB1 syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q89.01 — Asplenia (congenital), Q89.09 — Congenital malformations of spleen, Q89.1 — Congenital malformations of adrenal gland, Q89.2 — Congenital malformations of other endocrine glands, Q89.3 — Situs inversus, Q89.4 — Conjoined twins, Q89.7 — Multiple congenital malformations, not elsewhere classified, Q89.8 — Other specified congenital malformations, Q89.89 — Other specified congenital malformations, Q89.9 — Congenital malformation, unspecified, +59 more

Contextual Map

Every relationship of Q89.81 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Hierarchy

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 08 — Diseases and Disorders of the Musculoskeletal System and Connective Tissue[MDC crossing]: “Diseases and Disorders of the Musculoskeletal System and Connective Tissue — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 18,439 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Syndrome, Kabuki (type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Syndrome, Niikawa-Kuroki[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (12)

Change history

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Indexed Clinical Terms (2)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v44 Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder-derived relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder editorial explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 20, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "Q89.81 — Kabuki syndrome." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q89.81-kabuki-syndrome

Change history

  • FY2026 — October 1, 2025
    Added to the code set
    Kabuki syndrome
    FY2026 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q89.81 in its code family, with their registry titles.

View all codes in the Q89 family