Q89.81 Kabuki syndrome

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis. Exempt from present-on-admission (POA) reporting.

Applicable to

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

Applies from Q89.8 Other specified congenital malformations

Use additional code

• code(s) to identify all associated manifestations

MS-DRG index

Defines the logic of DRG(s) 564-566 in MDC 08.

Diagnosis Index entries for Q89.81

• Syndrome › Kabuki (type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)
• Syndrome › Niikawa-Kuroki

Nearby codes

• 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
• Q89.81 Kabuki syndrome (this code)
• Q89.89 Other specified congenital malformations
• Q89.9 Congenital malformation, unspecified