Q89.81BillableSave

Q89.81 - Kabuki syndrome

ICD-10-CM Chapter 17: Congenital malformations, deformations and chromosomal abnormalities · Q80-Q89 Other congenital malformations (Q80-Q89) · Subcategory of Q89.8 · FY2027

Applicable To

Inclusion terms listed under Q89.81 in the ICD-10-CM tabular list.

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

Includes, Excludes and Coding Notes

Use Additional CodeFrom Q89.8

Add a secondary code to identify the associated condition or cause when it is documented.

  • code(s) to identify all associated manifestations
Excludes 2 — Not Included HereFrom Chapter Q00-Q99

Not part of this code; report both codes when the patient has both conditions.

  • inborn errors of metabolism (E70-E88)
NotesFrom Chapter Q00-Q99

Instructional notes from the ICD-10-CM tabular list.

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

Documentation requirements, coding pitfalls and FAQs for conditions coded with Q89.81.