Other neurofibromatosis

This code represents a diagnosis of neurofibromatosis that does not fit the criteria for Neurofibromatosis Type 1 (NF1) or Type 2 (NF2). This includes conditions like schwannomatosis, which is characterized by multiple schwannomas without the typical NF1 or NF2 features, or other rare, atypical forms of neurofibromatosis.

Use this code when documentation specifies a diagnosis of schwannomatosis, segmental neurofibromatosis, or other forms of neurofibromatosis not classifiable as NF1 or NF2. Supporting documentation should clearly distinguish the condition from NF1 (e.g., absence of café-au-lait macules, Lisch nodules) and NF2 (e.g., absence of bilateral vestibular schwannomas).

• Ensure the provider's documentation explicitly rules out NF1 and NF2 before assigning this code.
• Do not use this code for unspecified neurofibromatosis; use Q85.00 for that scenario.
• Consider additional codes for specific manifestations or complications of the neurofibromatosis, such as tumors or neurological deficits.