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2007 ICD-9-CM Volume 1 Diagnosis Codes Home > Neoplasms 140-239 > Neoplasms Of Uncertain Behavior 235-238 > Neoplasm Of Uncertain Behavior Of Endocrine Glands And Nervous System 237.* >

2007 ICD-9-CM Diagnosis 237.71

Neurofibromatosis type 1 von recklinghausen's disease

 On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date.

The 2009 version of ICD-9-CM 237.71 can be accessed here.

  • 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 (GENES, NF1) gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).
  • except of bone
  • 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. -- 2003
  • 237.71 is a specific code that can be used to specify a diagnosis
  • 237.71 contains 13 index entries
  • View the ICD-9-CM Volume 1 237.* hierarchy

Index entries containing 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
  • multiple (M9540/1) 237.70
    • type 1 237.71
Neurofibromatosis (multiple) (M9540/1) 237.70
  • type 1 237.71
  • von Recklinghausen's 237.71
Neuroma (M9570/0) - see also Neoplasm, connective tissue, benignRecklinghausen'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