1. Q85 Phakomatoses, not elsewhere classified


    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)
    1. Q85.0 Neurofibromatosis (nonmalignant)

      1. Q85.00 Neurofibromatosis, unspecified

      2. Q85.01 Neurofibromatosis, type 1

        Inclusion term(s):

        • Von Recklinghausen disease
      3. Q85.02 Neurofibromatosis, type 2

        Inclusion term(s):

        • Acoustic neurofibromatosis
      4. Q85.03 Schwannomatosis

      5. Q85.09 Other neurofibromatosis

    2. Q85.1 Tuberous sclerosis

      Inclusion term(s):

      • Bourneville's disease
      • Epiloia
    3. Q85.8 Other phakomatoses, not elsewhere classified

      1. Q85.81 PTEN hamartoma tumor syndrome

        Inclusion term(s):

        • PHTS
        • PTEN related Cowden syndrome
      2. Q85.82 Other Cowden syndrome

      3. Q85.83 Von Hippel-Lindau syndrome

      4. Q85.89 Other phakomatoses, not elsewhere classified

        Inclusion term(s):

        • Peutz-Jeghers syndrome
        • Sturge-Weber(-Dimitri) syndrome
    4. Q85.9 Phakomatosis, unspecified

      Inclusion term(s):

      • Hamartosis NOS
Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.