All ICD10 Codes

Q89.81

Billable
ChapterQ00–Q99
Ranksubcategory
Official Description

Kabuki syndrome

Metadata & Insights
  • •Billable/specific code (valid for reimbursement).
  • •Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-Q99).

Instructional Notes

Includes
  • Kabuki syndrome, type 1, due to KMT2D mutation
  • Kabuki syndrome, type 2, due to KDM6A mutation
  • Niikawa-Kuroki syndrome
General instructions are available for the parent sections:
View CH17 NotesView Q89.8 Notes

Clinical Hierarchy

BillableHas subcodes
CH17Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)
Q80-Q89Other congenital malformations (Q80-Q89)
Q89Other congenital malformations, not elsewhere classified
Q89.8Other specified congenital malformations
Q89.81Kabuki syndromeBillable

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to Q89.81:

subcategoryQ89.8
Other specified congenital malformations
View context instructions
chapterCH17
Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)
View context instructions

Code History

  • FY 2025: This code replaced Q89.8 (Other specified congenital malformations)

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 564
Other Musculoskeletal System and Connective Tissue Diagnoses with MCC
DRG 565
Other Musculoskeletal System and Connective Tissue Diagnoses with CC
DRG 566
Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC

Cross Reference

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