Oncology & Hematology Coding Alert

Look for skin changes, hearing loss to ascertain the type of disease.

When reporting neurofibromatosis, you’ll need to confirm the type of neurofibromatosis your physician is treating, which can range from an isolated growing tumorous mass on a nerve to a collection of signs that make up a syndrome.

Definition: Neurofibromatosis is a genetic disorder that mainly affects how nerve cells form and grow. It can cause tumors to grow on nerves. There is no cure for neurofibromatosis, so clinicians plan treatment (such as surgery to remove the tumors, radiation or chemotherapy) to control symptoms.

"The ICD-9 Tabular section lists the diagnosis codes for the various neurofibromatosis conditions in the Neoplasms chapter in contrast to ICD-10 which includes the neurofibromatosis diagnoses in the Congenital Malformations, Deformations and Chromosomal Abnormalities chapter," says Marvel Hammer, RN, CPC, CCS-P, PCS, ACS-PM, CHCO, owner of MJH Consulting in Denver, Co.

Look at Definitive Skin Changes to Confirm Type 1

Neurofibromatosis type 1 (NF1 or von Recklinghausen’s disease) is one of the more common types of the disorder. This type causes skin changes and deformed bones, and usually begins at birth. Your provider should clearly document the findings in the patient record so a careful read-through will confirm that the clinical examination supports the diagnosis. See "Diagnosis of Neurofibromatosis 1" on page XX for more on the signs of type 1 neurofibromatosis.

ICD-9: Currently, you report 237.71 (Neurofibromatosis type 1 [von Recklinghausen’s disease]) when your clinician definitively documents that the patient has von Recklinghausen’s disease.

When ICD-10 goes into effect, you’ll report Q85.01 (Neurofibromatosis, type 1). "Even though ICD-10 does not include "von Recklinghausen’s disease" in the actual ICD-10 Q85.01 code descriptor it is listed underneath as an alternative term. Additionally, the ICD-10 Alphabetic Index has a listing under the term "Von Recklinghausen" and directs coders to the Q85.01 code for the "disease" subterm," says Hammer.

Hearing Loss Is Your Guide for Type 2

Neurofibromatosis type 2 is also called NF2 or central neurofibromatosis. This type of neurofibromatosis is characterized by bilateral acoustic neuromas, tumors of the vestibulocochlear nerve (8th Cranial nerve).

The main complaint you should specifically look for in the clinical records is hearing loss. Other symptoms can include tinnitus (ringing in the ears) or poor balance. Typically, you’ll notice that your clinician was treating a younger patient for hearing loss because signs of NF2 usually develop in the late teenage years or early 20s.

ICD-9: Under ICD-9, you report diagnosis 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 actual descriptor of the ICD-10 code does not include the additional name, however as with the NF1 ICD-10 code, the alternate term "acoustic neurofibromatosis" is listed below the NF2 Q85.02 code. "Additionally, the Alphabetic Index of ICD-10 also has a listing for the sub term ‘acoustic’ under the term neurofibromatosis. It directs coders to review the Q85.02 code," says Hammer.

Don’t Miss the Rarest Forms

Schwannomatosis is a disorder characterized by multiple schwannomas, and is one of the rarest types of neurofibromatosis. Schwannomas are benign tumors involving the cells that produce the nerve sheath insulating the nerves. The schwannomas may be found everywhere in the body, including cranial, spinal, or peripheral nerves. "The only exception is on the vestibular branch of the 8th Cranial nerve. The primary symptom is pain, which develops as the schwannoma tumors enlarge or compress nerves / adjacent tissue. Some patients also may have numbness, tingling or weakness in the fingers and toes," says Hammer.

On occasion, your clinician might document segmental schwannomatosis. This term refers to schwannomas that are localized to a part of the body such as an arm, leg or region of the spine. Also, your clinician might note the absence of any hearing loss.

When coding for schwannomatosis, report ICD-9 code 237.73 (Schwannomatosis). After ICD-10 implementation, you’ll report Q85.03 (Schwannomatosis).

Other possibilities: Some other variants of the disorder also exist that you should be aware of, even if you don’t code them very often. These might be known as type 3 or type 4 neurofibromatosis.

Example: Your clinician documents cutaneous neurofibromas in a patient who otherwise meets the requirement for a diagnosis of type 1 neurofibromatosis. Confirm that your clinician documents 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).

Backup plan: When you aren’t clear what type of neurofibromatosis the patient has and your clinician hasn’t clearly documented the same, turn to code 237.70 (Neurofibromatosis unspecified). Under ICD-10 you’ll report 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 with New Jersey Neuroscience Institute of JFK Medical Center in Edison.