Q89.81 ICD-10-CM Code: Kabuki syndrome
Q89.81 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC Buddy coding tools
HCC Buddy Code Card
Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.
FY 2026 Apr update / Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0) / Other congenital malformations (Q80-Q89)
Q89.81
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceKabuki syndrome
A rare genetic disorder present from birth characterized by distinctive facial features, developmental delays, and various organ abnormalities.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
N/A—
Not mapped
ACA/HHS
MappedHCC 97
Varies by metal level
ESRD/PACE
N/A—
Not mapped
RXHCC
N/A—
Not mapped
Code Book Path
Inclusion Terms
Official- Kabuki syndrome, type 1, due to KMT2D mutation
- Kabuki syndrome, type 2, due to KDM6A mutation
- Niikawa-Kuroki syndrome
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for Q89.81 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for Q89.81 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for Q89.81 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for Q89.81 in this effective period.
Use Additional
Official- code(s) to identify all associated manifestations
Code Also
OfficialICD-10-CM does not list Code Also instructions for Q89.81 in this effective period.
Buddy Documentation Tip
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is Q89.81 an HCC code?
No. Q89.81 is a billable ICD-10-CM code but does not map to any HCC category in V28, V24, ESRD, or RxHCC.
- Code
- Q89.81
- Description
- Kabuki syndrome
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF
- —
- Billable
- Yes
- Payment year
- 2026
Coder workflow notes
Get the V28 RAF + MEAT cheat sheet
One printable page: confirm a code's V28 HCC status, its RAF weight, and the MEAT your note needs to make it stick. Free, no card.
Free PDF. No card. Unsubscribe anytime.
This code does not map to an HCC category in any model (V28, V24, ESRD, RxHCC).
What This Code Means
Q89.81 is the ICD-10-CM diagnosis code for kabuki syndrome. A rare genetic disorder present from birth characterized by distinctive facial features, developmental delays, and various organ abnormalities. Q89.81 sits in the ICD-10-CM chapter for congenital malformations, deformations and chromosomal abnormalities (q00-qa0), within the section covering other congenital malformations (q80-q89).
Q89.81 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.
Kabuki syndrome is a specific recognizable pattern; do not use Q89.7 if Kabuki is confirmed.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for Q89.81 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Kabuki syndrome is a specific recognizable pattern; do not use Q89.7 if Kabuki is confirmed
- •Code associated complications such as cardiac defects or intellectual disability separately