DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

Q87 Other specified congenital malformation syndromes affecting multiple systems

  • Header · not billable

Q87 is not billable. The category heads 21 billable codes in FY2027, grouped under 8 subdivisions.

FY2027 changes under Q87: 1 billable code added. All FY2027 changes.

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. Q87 Other specified congenital malformation syndromes affecting multiple systems
Billable codes
21
Subdivisions
8
CC / MCC codes
20 / 0
HCC V28 codes
0
New FY2027
1
Index entries
127

Codes in Q87

  1. Q87.0
    Congenital malformation syndromes predominantly affecting facial appearance
    BillableExempt
    Inclusion terms 9

    Inclusion terms

    • Acrocephalopolysyndactyly
    • Acrocephalosyndactyly [Apert]
    • Cryptophthalmos syndrome
    • Cyclopia
    • Goldenhar syndrome
    • Moebius syndrome
    • Oro-facial-digital syndrome
    • Robin syndrome
    • Whistling face
  2. Q87.1
    Congenital malformation syndromes predominantly associated with short stature
    Header
    Excludes1 2

    Excludes1

    • Ellis-van Creveld syndrome (Q77.6) Q77.6
    • Smith-Lemli-Opitz syndrome (E78.72) E78.72
  3. Q87.11
    Prader-Willi syndrome
    BillableCCExempt
  4. Q87.19
    Other congenital malformation syndromes predominantly associated with short stature
    BillableCCExempt
    Inclusion terms 8

    Inclusion terms

    • Aarskog syndrome
    • Cockayne syndrome
    • De Lange syndrome
    • Dubowitz syndrome
    • Noonan syndrome
    • Robinow-Silverman-Smith syndrome
    • Russell-Silver syndrome
    • Seckel syndrome
  5. Q87.2
    Congenital malformation syndromes predominantly involving limbs
    BillableCCExempt
    Inclusion terms 7

    Inclusion terms

    • Holt-Oram syndrome
    • Klippel-Trenaunay-Weber syndrome
    • Nail patella syndrome
    • Rubinstein-Taybi syndrome
    • Sirenomelia syndrome
    • Thrombocytopenia with absent radius [TAR] syndrome
    • VATER syndrome
  6. Q87.3
    Congenital malformation syndromes involving early overgrowth
    BillableCCExempt
    Inclusion terms 3

    Inclusion terms

    • Beckwith-Wiedemann syndrome
    • Sotos syndrome
    • Weaver syndrome
  7. Q87.4
    Marfan syndrome
    Header
  8. Q87.40
    Marfan syndrome, unspecified
    BillableCCExempt
  9. Q87.41
    Marfan syndrome with cardiovascular manifestations
    Header
  10. Q87.410
    Marfan syndrome with aortic dilation
    BillableCCExempt
  11. Q87.418
    Marfan syndrome with other cardiovascular manifestations
    BillableCCExempt
  12. Q87.42
    Marfan syndrome with ocular manifestations
    BillableCCExempt
  13. Q87.43
    Marfan syndrome with skeletal manifestation
    BillableCCExempt
  14. Q87.5
    Other congenital malformation syndromes with other skeletal changes
    BillableCCExempt
  15. Q87.8
    Other specified congenital malformation syndromes, not elsewhere classified
    Header
    Excludes1 1

    Excludes1

    • Zellweger syndrome (E71.510) E71.510
  16. Q87.81
    Alport syndrome
    BillableCCExempt
    Use additional code 1

    Use additional code

    • code to identify stage of chronic kidney disease (N18.1-N18.6) N18.1 N18.6
  17. Q87.82
    Arterial tortuosity syndrome
    BillableCCExempt
  18. Q87.83
    Bardet-Biedl syndrome
    BillableCCExempt
  19. Q87.84
    Laurence-Moon syndrome
    BillableCCExempt
  20. Q87.85
    MED13L syndrome
    BillableCCExempt
    Inclusion terms 2, Code also 5

    Inclusion terms

    • Asadollahi-Rauch syndrome
    • Mediator complex subunit 13L syndrome

    Code also

    • , if applicable, any associated manifestations such as:
    • autism spectrum disorder (F84.0-) F84.0
    • congenital malformations of cardiac septa (Q21.-) Q21
    • epilepsy and recurrent seizures (G40.-) G40
    • intellectual disability (F70-F79) F70 F79
  21. Q87.86
    Kleefstra syndrome
    BillableCCExempt
  22. Q87.87
    Hao-Fountain Syndrome
    BillableCCExempt
    Inclusion terms 1, Use additional code 6

    Inclusion terms

    • HAFOUS

    Use additional code

    • code, if applicable, for associated conditions such as:
    • autism spectrum disorder (F84.0) F84.0
    • developmental speech disorder (F80.-) F80
    • epilepsy, by specific type (G40.-) G40
    • intellectual disabilities (F70-F79) F70 F79
    • pervasive developmental disorders (F84.-) F84
  23. Q87.88
    CTNNB1 syndrome
    BillableCCExempt
    Use additional code 7

    Use additional code

    • code, if applicable, for associated conditions such as:
    • cerebral palsy (G80.-) G80
    • congenital heart malformations (Q20.0-Q24.9) Q20.0 Q24.9
    • developmental disorder of speech and language (F80.-) F80
    • exudative retinopathy (H35.02-) H35.02
    • intellectual disability (F70-F79) F70 F79
    • microcephaly (Q02) Q02
  24. Q87.89
    Other specified congenital malformation syndromes, not elsewhere classified
    BillableCCExempt
  25. Q87.A
    Loeys-Dietz syndrome New FY2027
    BillableCCExempt
    Excludes1 4

    Excludes1

    • arterial tortuosity syndrome (Q87.82) Q87.82
    • Ehlers-Danlos syndrome (Q79.6) Q79.6
    • Marfan syndrome (Q87.40-Q87.43) Q87.40 Q87.43
    • other systemic involvement of connective tissue (M35) M35

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

At Q87

Use additional code

  • code(s) to identify all associated manifestations

Notes that also apply

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

Index entries

  • Acrocephalopolysyndactyly → Q87.0
  • Acrocephalosyndactyly → Q87.0
  • Aglossia-adactylia syndrome → Q87.0
  • Apert's syndrome → Q87.0
  • Carpenter's syndrome → Q87.0
  • Cyclopia, cyclops → Q87.0
  • Cyclopism → Q87.0
  • Dyscranio-pygo-phalangy → Q87.0
  • Fraser's syndrome → Q87.0
  • Freeman Sheldon syndrome → Q87.0
  • Goldenhar (-Gorlin) syndrome → Q87.0
  • Gorlin-Chaudry-Moss syndrome → Q87.0
  • Hallerman-Streiff syndrome → Q87.0
  • Hanhart's syndrome → Q87.0
  • Marchesani (-Weill) syndrome → Q87.0
  • Meyer-Schwickerath and Weyers syndrome → Q87.0
  • Mohr's syndrome (Types I and II) → Q87.0
  • Papillon-Léage and Psaume syndrome → Q87.0
  • Pierre Robin deformity or syndrome → Q87.0
  • Robin (-Pierre) syndrome → Q87.0
  • Ullrich-Feichtiger syndrome → Q87.0
  • Whistling face → Q87.0
  • Prader-Willi syndrome → Q87.11
  • Aarskog's syndrome → Q87.19
  • Amsterdam dwarfism → Q87.19
  • Bonnevie-Ullrich syndrome → Q87.19
  • Bruck-de Lange disease → Q87.19
  • Cockayne's syndrome → Q87.19
  • Cornelia de Lange syndrome → Q87.19
  • De Lange's syndrome → Q87.19
  • Dubowitz' syndrome → Q87.19
  • Noonan's syndrome → Q87.19
  • Prader-Willi-like syndrome → Q87.19
  • Robinow-Silverman-Smith syndrome → Q87.19
  • Russell-Silver syndrome → Q87.19
  • Seckel's syndrome → Q87.19
  • Silver's syndrome → Q87.19
  • Sjögren-Larsson syndrome → Q87.19
  • Turner-like syndrome → Q87.19
  • Ullrich (-Bonnevie)(-Turner) syndrome → Q87.19

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

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

Hierarchy