Specific code 2014 ICD-9-CM Diagnosis Code 237.71
Neurofibromatosis, type 1 [von recklinghausen's disease]
  • Billable Code
  • Short description: Neurofibromatosis type I.
  • ICD-9-CM 237.71 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 237.71 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Neurofibromatosis, type 1
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.
  • Nf1. 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.
  • 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.
Convert to ICD-10-CM: 237.71 converts directly to:
  • 2014 ICD-10-CM Q85.01 Neurofibromatosis, type 1
ICD-9-CM Volume 2 Index entries containing back-references to 237.71:
  • Disease, diseased - see also Syndrome
    • Recklinghausen's (M9540/1) 237.71
      • bone (osteitis fibrosa cystica) 252.01
    • von Recklinghausen's (M9540/1) 237.71
      • bone (osteitis fibrosa cystica) 252.01
  • Elephant man syndrome 237.71
  • Elephantiasis (nonfilarial) 457.1
    • neuromatosa 237.71
  • Neurofibroma (M9540/0) - see also Neoplasm, connective tissue, benign
  • Neurofibromatosis (multiple) (M9540/1) 237.70
    • type 1 237.71
    • von Recklinghausen's 237.71
  • Neuroma (M9570/0) - see also Neoplasm, connective tissue, benign
  • Recklinghausen's disease (M9540/1) 237.71
    • bones (osteitis fibrosa cystica) 252.01
  • Syndrome - see also Disease
    • Elephant man 237.71
  • Tumor (M8000/1) - see also Neoplasm, by site, unspecified nature
    • von Recklinghausen's (M9540/1) 237.71
  • von Recklinghausen's
    • disease or syndrome (nerves) (skin) (M9540/1) 237.71
      • bones (osteitis fibrosa cystica) 252.01
    • tumor (M9540/1) 237.71
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 237.71 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.