Q89.81

Kabuki syndrome

Billable / specificPOA exempt

Q89.81 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.

Inclusion terms

Applicable to

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

Includes (from Q89)

  • Note: Codes from this chapter are not for use on maternal records

Excludes2

Not included here — may be reported together when both conditions exist.

  • inborn errors of metabolism (E70-E88) (from Q89)

Use additional code

Add a code to identify the following, if applicable.

  • code(s) to identify all associated manifestations (from Q89.8)

Also known as

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

Parent code

  • Q89.8Other specified congenital malformationsHeader

Related codes

  • Q89.89Other specified congenital malformationsBillable

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