DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

Q89 Other congenital malformations, not elsewhere classified

  • Header · not billable

Q89 is not billable. The category heads 10 billable codes in FY2027, grouped under 8 subdivisions.

Index → code

Where it sits

  1. Chapter 17 Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders (Q00-QA1)
  2. Q80-Q89 Other congenital malformations (Q80-Q89)
  3. Q89 Other congenital malformations, not elsewhere classified
Billable codes
10
Subdivisions
8
CC / MCC codes
6 / 1
HCC V28 codes
0
New FY2027
0
Index entries
182

Codes in Q89

  1. Q89.0
    Congenital absence and malformations of spleen
    Header
    Excludes1 1

    Excludes1

    • isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6) Q20.6
  2. Q89.01
    Asplenia (congenital)
    BillableCCExempt
  3. Q89.09
    Congenital malformations of spleen
    BillableCCExempt
    Inclusion terms 1

    Inclusion terms

    • Congenital splenomegaly
  4. Q89.1
    Congenital malformations of adrenal gland
    BillableExempt
    Excludes1 2

    Excludes1

    • adrenogenital disorders (E25.-) E25
    • congenital adrenal hyperplasia (E25.0) E25.0
  5. Q89.2
    Congenital malformations of other endocrine glands
    BillableExempt
    Inclusion terms 3, Excludes1 2

    Inclusion terms

    • Congenital malformation of parathyroid or thyroid gland
    • Persistent thyroglossal duct
    • Thyroglossal cyst

    Excludes1

    • congenital goiter (E03.0) E03.0
    • congenital hypothyroidism (E03.1) E03.1
  6. Q89.3
    Situs inversus
    BillableCCExempt
    Inclusion terms 6, Excludes1 1

    Inclusion terms

    • Dextrocardia with situs inversus
    • Mirror-image atrial arrangement with situs inversus
    • Situs inversus or transversus abdominalis
    • Situs inversus or transversus thoracis
    • Transposition of abdominal viscera
    • Transposition of thoracic viscera

    Excludes1

    • dextrocardia NOS (Q24.0) Q24.0
  7. Q89.4
    Conjoined twins
    BillableMCCExempt
    Inclusion terms 4

    Inclusion terms

    • Craniopagus
    • Dicephaly
    • Pygopagus
    • Thoracopagus
  8. Q89.7
    Multiple congenital malformations, not elsewhere classified
    BillableCCExempt
    Inclusion terms 2, Excludes1 1

    Inclusion terms

    • Multiple congenital anomalies NOS
    • Multiple congenital deformities NOS

    Excludes1

    • congenital malformation syndromes affecting multiple systems (Q87.-) Q87
  9. Q89.8
    Other specified congenital malformations
    Header
    Use additional code 1

    Use additional code

    • code(s) to identify all associated manifestations
  10. Q89.81
    Kabuki syndrome
    BillableCCExempt
    Inclusion terms 3

    Inclusion terms

    • Kabuki syndrome, type 1, due to KMT2D mutation
    • Kabuki syndrome, type 2, due to KDM6A mutation
    • Niikawa-Kuroki syndrome
  11. Q89.89
    Other specified congenital malformations
    BillableCCExempt
  12. Q89.9
    Congenital malformation, unspecified
    BillableExempt
    Inclusion terms 2

    Inclusion terms

    • Congenital anomaly NOS
    • Congenital deformity NOS

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

Notes that also apply

Chapter 17 instructions (Notes 1, Excludes2 1): printed on the chapter 17 page.

Index entries

  • Asplenia (congenital) → Q89.01
  • Ivemark's syndrome (asplenia with congenital heart disease) → Q89.01
  • Polysplenia syndrome → Q89.09
  • Retrosternal thyroid (congenital) → Q89.2
  • Thyrolingual duct, persistent → Q89.2
  • Kartagener's syndrome or triad (sinusitis, bronchiectasis, situs inversus) → Q89.3
  • Sinusitis-bronchiectasis-situs inversus (syndrome) (triad) → Q89.3
  • Situs inversus or transversus (abdominalis) (thoracis) → Q89.3
  • Conjoined twins → Q89.4
  • Craniopagus → Q89.4
  • Dicephalus, dicephaly → Q89.4
  • Epignathus → Q89.4
  • Ischiopagus → Q89.4
  • Janiceps → Q89.4
  • Pygopagus → Q89.4
  • Siamese twin → Q89.4
  • Sternopagus → Q89.4
  • Syncephalus → Q89.4
  • Thoracopagus → Q89.4
  • Xiphopagus → Q89.4
  • Monster, monstrosity (single) → Q89.7
  • Paracephalus → Q89.7
  • Teratism → Q89.7
  • Acardia, acardius → Q89.89
  • Acardiacus amorphus → Q89.89
  • Acephalobrachia monster → Q89.89
  • Acephalochirus monster → Q89.89
  • Acephalogaster → Q89.89
  • Acephalostomus monster → Q89.89
  • Acephalothorax → Q89.89
  • CHARGE association → Q89.89
  • Goldberg syndrome → Q89.89
  • Hyperekplexia → Q89.89
  • Hyperexplexia → Q89.89
  • Teratencephalus → Q89.89
  • Anomaly, anomalous (congenital) (unspecified type) → Q89.9
  • Defect, defective → Q89.9
  • Deformity → Q89.9
  • Embryopathia NOS → Q89.9
  • Absence (of) (organ or part) (complete or partial)spleen (congenital) → Q89.01

142 more, longer entries are not listed; the search box finds them by word.

NCHS Alphabetic Index. Index first, then the Tabular List.

Hierarchy