Q89.81 — Kabuki syndrome
Q89.81 is a billable, specific ICD-10-CM code for kabuki syndrome. It is valid for submission on a claim.
Inclusion terms
Alternative wordings a physician may use in the chart for this same code.
- Kabuki syndrome, type 1, due to KMT2D mutation
- Kabuki syndrome, type 2, due to KDM6A mutation
- Niikawa-Kuroki syndrome
Risk adjustment
Q89.81 does not map to an HCC and does not risk-adjust under CMS-HCC V28. That is normal — most ICD-10 codes do not. It still needs to be coded correctly; it just will not move a RAF score.
Medicare coverage
Q89.81 is named in 5 Medicare coverage policies — 5 listing it as supporting medical necessity. Which of them applies to you depends on your Medicare contractor, and coverage genuinely differs by state.
- A56726Billing and Coding: Ophthalmology: Posterior Segment Imaging (Extended Ophthalmoscopy and Fundus Photography)
- A56767Billing and Coding: Heavy Metal Testing
- A56781Billing and Coding: Transthoracic Echocardiography (TTE)
- A56781Billing and Coding: Transthoracic Echocardiography (TTE)
How Q89.81 is indexed
Coders do not find codes by browsing the tabular list — they look them up in the alphabetic index, under the word the physician wrote. These are the index entries that lead here, so you can see which chart wordings map to Q89.81.
- Syndrome›Kabuki(type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)
- Syndrome›Niikawa-Kuroki
Related codes at this level
Codes that share Q89.8. If Q89.81 is not quite right, the correct code is usually one of these.
Questions about Q89.81
- Is Q89.81 a billable ICD-10-CM code?
- Yes. Q89.81 is a billable, specific ICD-10-CM code and is valid for submission on a claim.
- Where does Q89.81 sit in the tabular list?
- Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0), in the block Other congenital malformations (Q80-Q89).