Q89.81: Kabuki syndrome

New code · FY2026

Billable: use this code on claims.

• New code, effective October 1, 2025 (FY2026).

Code breakdown

Coding notes

Inclusion terms

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

Use additional code (from Q89.8)

• code(s) to identify all associated manifestations

Medicare coverage

Q89.81 is listed as a covered diagnosis in 4 Medicare billing and coding articles:

• A56726 Billing and Coding: Ophthalmology: Posterior Segment Imaging (Extended Ophthalmoscopy and Fundus Photography)
• A56767 Billing and Coding: Heavy Metal Testing
• A56781 Billing and Coding: Transthoracic Echocardiography (TTE)
• A57071 Billing and Coding: Ophthalmology: Posterior Segment Imaging (Extended Ophthalmoscopy and Fundus Photography)

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All FY2026 ICD-10-CM changes

ICD-10-CM codes and descriptions are from the CMS and NCHS code files. Coverage is from the CMS Medicare Coverage Database billing and coding articles.