Orbitotemporal neurofibromatosis: classification and treatment.

PURPOSE: To review the clinical findings in orbitotemporal neurofibromatosis and discuss treatment options. Clinical features, histopathologic characteristics, and treatment options are reviewed.

METHODS: A Medline literature search from 1966 to 2004 was performed, using the key words: orbitotemporal neurofibromatosis, orbitopalpebral neurofibromatosis, orbitofacial neurofibromatosis, cranio-orbital neurofibromatosis, and cranio-orbital-temporal neurofibromatosis, and the pertinent literature was reviewed. Additionally, our experience with two patients is reported. The surgical procedures are discussed.

CONCLUSION: The management of orbitotemporal neurofibromatosis is challenging. The planned surgical approach and extent of resection depend on the severity of the orbital soft tissue and bony involvement and on the visual potential. Ultimately, orbital exenteration may be needed for rehabilitation and cosmesis.