ICD 10 Coding Alert

Hint: Let disease type guide you to the right code.

Neurofibromatosis may pop up as a diagnosis if your surgeon performs reduction surgery to reduce the size of a neurofibroma. Look to the guidance that follows to select appropriate neurofibromatosis code by type.

Look at Definitive Diagnosis to Confirm Type 1

Neurofibromatosis type 1 is also called the von Recklinghausen's disease. This is one of the common types of neurofibromatosis that you will be reporting. Your clinician will clearly document the findings in the clinical record sheet and you will carefully read through to confirm that based on the clinical examination your clinician has made a diagnosis.

ICD-9: You report 237.71 (Neurofibromatosis type 1 von Recklinghausen's disease) when your clinician has been able to definitively document that the patient had von Recklinghausen's disease.

When ICD-10 is effective, you will report Q85.01 (Neurofibromatosis, type 1). The only difference in these codes is that the ICD-10 descriptor does not include the alternative name 'von Recklinghausen's disease.'

Hearing Loss Is Your Guide for Type 2

Neurofibromatosis type 2 is also called central neurofibromatosis. The main complaint you should specifically look for in the clinical records is the hearing loss. Typically, you will notice that your clinician was treating a younger patient for hearing loss.

ICD-9: You will report code 237.72 (Neurofibromatosis type 2 acoustic neurofibromatosis) for neurofibromatosis type 2.

ICD-10: The corresponding ICD-10 code will be Q85.02 (Neurofibromatosis, type 2). Once again, you will notice that the descriptor of the ICD-10 code does not include the term acoustic neurofibromatosis.

Note: Neurofibromatosis type 2 is characterized by bilateral acoustic neuromas. These are tumors of the vestibulocochlear nerve which is the eighth cranial nerve.

Learn How to Report the Other Variants

Schwannomatosis is a disorder characterized by multiple schwannomas. These are benign tumors involving the Schwann cells that produce the nerve sheath to provide insulation in the nerves. The schwannomas may be in the cranial, spinal, or peripheral nerves. You may also note that your surgeon documented segmental schwannomatosis. This term refers to schwannomas that are localized to a part of the body such as the leg or spine. Also, your clinician may notable record the absence of any hearing loss.

In this circumstance, you report ICD-9 code 237.73 (Schwannomatosis) and going further after ICD-10 implementation, you report code Q85.03 (Schwannomatosis).

You should also know the rare variants that your clinician may diagnose. These are not very common. These are variants like the type 3 and type 4 neurofibromatosis. Your clinician may document cutaneous neurofibromas in a patient who otherwise meets the requirement for a diagnosis of type 1 neurofibromatosis.

In this case, you will confirm that your clinician will document the diagnosis of neurofibromatosis type 4 and report ICD-9 code 237.79 (Other neurofibromatosis). Going further when ICD-10 is implemented, you will turn to code Q85.09 (Other neurofibromatosis).

When you aren't clear what the type of the neurofibromatosis was and your clinician hasn't clearly documented the same, you turn to code 237.70 (Neurofibromatosis unspecified). In future you can report ICD-10 code Q85.00 (Neurofibromatosis, unspecified).

"Some patients will only have a single sporadic neurofibroma, rather than one of the syndromes we have discussed so far. You will report this using the benign neoplasm of a nerve or connective tissue," says Gregory Przybylski, MD, director of neurosurgery, New Jersey Neuroscience Institute, JFK Medical Center, Edison.