DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

Q85 Phakomatoses, not elsewhere classified

  • Header · not billable

Q85 is not billable. The category heads 11 billable codes in FY2027, grouped under 4 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. Q85 Phakomatoses, not elsewhere classified
Billable codes
11
Subdivisions
4
CC / MCC codes
6 / 0
HCC V28 codes
11
New FY2027
0
Index entries
39

Codes in Q85

  1. Q85.0
    Neurofibromatosis (nonmalignant)
    Header
  2. Q85.00
    Neurofibromatosis, unspecified
    Billable23Exempt
  3. Q85.01
    Neurofibromatosis, type 1
    Billable23Exempt
    Inclusion terms 1

    Inclusion terms

    • Von Recklinghausen disease
  4. Q85.02
    Neurofibromatosis, type 2
    Billable23Exempt
    Inclusion terms 1

    Inclusion terms

    • Acoustic neurofibromatosis
  5. Q85.03
    Schwannomatosis
    Billable23Exempt
  6. Q85.09
    Other neurofibromatosis
    Billable23Exempt
  7. Q85.1
    Tuberous sclerosis
    BillableCC23Exempt
    Inclusion terms 2

    Inclusion terms

    • Bourneville's disease
    • Epiloia
  8. Q85.8
    Other phakomatoses, not elsewhere classified
    Header
    Excludes1 1

    Excludes1

    • Meckel-Gruber syndrome (Q61.9) Q61.9
  9. Q85.81
    PTEN hamartoma tumor syndrome
    BillableCC23Exempt
    Inclusion terms 2, Code also 1

    Inclusion terms

    • PHTS
    • PTEN related Cowden syndrome

    Code also

    • , if applicable, genetic susceptibility to malignant neoplasm (Z15.0-) Z15.0
  10. Q85.82
    Other Cowden syndrome
    BillableCC23Exempt
  11. Q85.83
    Von Hippel-Lindau syndrome
    BillableCC23Exempt
    Code also 1

    Code also

    • manifestations
  12. Q85.89
    Other phakomatoses, not elsewhere classified
    BillableCC23Exempt
    Inclusion terms 2

    Inclusion terms

    • Peutz-Jeghers syndrome
    • Sturge-Weber(-Dimitri) syndrome
  13. Q85.9
    Phakomatosis, unspecified
    BillableCC23Exempt
    Inclusion terms 1

    Inclusion terms

    • Hamartosis 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

At Q85

Excludes1

  • ataxia telangiectasia [Louis-Bar] (G11.3) G11.3
  • familial dysautonomia [Riley-Day] (G90.1) G90.1

Notes that also apply

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

Index entries

  • Neurofibromatosis (multiple) (nonmalignant) → Q85.00
  • Recklinghausen disease → Q85.01
  • Schwannomatosis → Q85.03
  • Bourneville's disease → Q85.1
  • Epiloia → Q85.1
  • Neurospongioblastosis diffusa → Q85.1
  • Pringle's disease (tuberous sclerosis) → Q85.1
  • Tuberous sclerosis (brain) → Q85.1
  • PHTS → Q85.81
  • Hippel's disease → Q85.83
  • Lindau (-von Hippel) disease → Q85.83
  • Von Hippel (-Lindau) disease or syndrome → Q85.83
  • Dimitri-Sturge-Weber disease → Q85.89
  • Kraft-Weber-Dimitri disease → Q85.89
  • Peutz-Jeghers disease or syndrome → Q85.89
  • Sturge (-Weber) (-Dimitri) (-Kalischer) disease or syndrome → Q85.89
  • Hamartoma, hamartoblastoma → Q85.9
  • Hamartosis → Q85.9
  • Phakomatosis → Q85.9
  • Neurofibromatosis (multiple) (nonmalignant)type 1 (von Recklinghausen) → Q85.01
  • Von Recklinghausendisease (neurofibromatosis) → Q85.01
  • Neurofibromatosis (multiple) (nonmalignant)acoustic → Q85.02
  • Neurofibromatosis (multiple) (nonmalignant)type 2 → Q85.02
  • Neurofibromatosis (multiple) (nonmalignant)specified NEC → Q85.09
  • Disease, diseasedBourneville (-Brissaud) (tuberous sclerosis) → Q85.1
  • Disease, diseasedPringle's (tuberous sclerosis) → Q85.1
  • Nevusmultiplex → Q85.1
  • PhakomatosisBourneville's → Q85.1
  • Sclerosis, sclerotictuberous (brain) → Q85.1
  • SyndromeBourneville (-Pringle) → Q85.1
  • SyndromePTEN (hamartoma) tumor → Q85.81
  • Syndromevon Hippel-Lindau → Q85.83
  • Angiomatosisencephalotrigeminal → Q85.89
  • Phakomatosisspecified NEC → Q85.89
  • SyndromePeutz-Jeghers → Q85.89
  • SyndromeSturge-Weber (-Dimitri) → Q85.89
  • Sclerosis, scleroticbrain (generalized) (lobular)tuberous → Q85.1
  • SyndromeCowdenPTEN related → Q85.81
  • SyndromeCowdenspecified NEC → Q85.82

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

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