2019 ICD-10-CM Diagnosis Code Q85.01

Neurofibromatosis, type 1

    2016 2017 2018 2019 Billable/Specific Code POA Exempt
  • Q85.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM Q85.01 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of Q85.01 - other international versions of ICD-10 Q85.01 may differ.
Applicable To
  • Von Recklinghausen disease
The following code(s) above Q85.01 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q85.01:
  • Q00-Q99
    2019 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    Note
    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    Congenital malformations, deformations and chromosomal abnormalities
  • Q85
    ICD-10-CM Diagnosis Code Q85

    Phakomatoses, not elsewhere classified

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)
    Phakomatoses, not elsewhere classified
Clinical Information
  • A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin.
  • An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic neural crest. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially optic nerve glioma and neurofibrosarcoma. Nf1 is caused by mutations which inactivate the nf1 gene (genes, neurofibromatosis 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (from Adams et al., Principles of Neurology, 6th ed, pp1014-18) there is overlap of clinical features with noonan syndrome in a syndrome called neurofibromatosis-noonan syndrome. Both the ptpn11 and nf1 gene products are involved in the signal transduction pathway of ras (ras proteins).
  • Neurofibromatosis is a genetic disorder of the nervous system. It mainly affects how nerve cells form and grow. It causes tumors to grow on nerves. You can get neurofibromatosis from your parents, or it can happen because of a mutation (change) in your genes. Once you have it, you can pass it along to your children. There are three types of neurofibromatosis:
    • type 1 (nf1) causes skin changes and deformed bones and usually starts at birth.
    • type 2 (nf2) causes hearing loss, ringing in the ears and poor balance. It often starts in the teen years.
    • schwannomatosis causes intense pain. It is the rarest type.
    there is no cure. Treatment is aimed at controlling symptoms. Depending on the type of disease and how bad it is, treatment may include surgery to remove tumors, radiation therapy and medicines.
  • Type 1 (peripheral) neurofibromatosis (von recklinghausen's disease), is the most common type of neurofibromatosis. It is characterized clinically by cutaneous and subcutaneous tumors with patches of hyperpigmentation. The hyperpigmented skin areas, are present from birth and found anywhere on the body surface. They can vary markedly in size and color. The dark brown areas are called cafe-au-lait spots. The multiple cutaneous and subcutaneous tumors are nerve sheath neoplasms, called neurofibromas. They can develop anywhere along the peripheral nerve fibers. Neurofibromas can become quite large, causing a major disfigurement, eroding bone, and compressing various peripheral nerve structures. Type 1 neurofibromatosis has dominant inheritance, with a gene locus on the proximal long arm of chromosome 17.
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q85.01 is considered exempt from POA reporting.
ICD-10-CM Q85.01 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 091 Other disorders of nervous system with mcc
  • 092 Other disorders of nervous system with cc
  • 093 Other disorders of nervous system without cc/mcc

Convert Q85.01 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change

Diagnosis Index entries containing back-references to Q85.01:

ICD-10-CM Codes Adjacent To Q85.01
Q84.2 Other congenital malformations of hair
Q84.3 Anonychia
Q84.4 Congenital leukonychia
Q84.5 Enlarged and hypertrophic nails
Q84.6 Other congenital malformations of nails
Q84.8 Other specified congenital malformations of integument
Q84.9 Congenital malformation of integument, unspecified
Q85 Phakomatoses, not elsewhere classified
Q85.0 Neurofibromatosis (nonmalignant)
Q85.00 Neurofibromatosis, unspecified
Q85.01 Neurofibromatosis, type 1
Q85.02 Neurofibromatosis, type 2
Q85.03 Schwannomatosis
Q85.09 Other neurofibromatosis
Q85.1 Tuberous sclerosis
Q85.8 Other phakomatoses, not elsewhere classified
Q85.9 Phakomatosis, unspecified
Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
Q86.0 Fetal alcohol syndrome (dysmorphic)
Q86.1 Fetal hydantoin syndrome
Q86.2 Dysmorphism due to warfarin

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.