Neurofibromatosis and its Management in Nigeria

Abstract

Neurofibromatosis (NF) is a relatively common disease with multisystemic manifestation. It is incurable and may be associated with learning difficulties and tumors such as optic nerve gliomas, brain tumors, and malignant peripheral nerve sheath tumors. This review seeks to increase awareness among physicians, surgeons, and ophthalmologists of the prevalence and manifestations of NF in the Nigerian environment, consequently, improving recognition, facilitating early diagnosis, follow-up, and treatment of the disabling complications of this disorder. The databases of “MedLine/PubMed,” “African-Journals-On-Line (AJOL),” “Google scholar,” and “UpToDate” were searched using the keywords Von Recklinghausen disease, familial NF, schwannomatosis, vestibular schwannoma, acoustic neuroma, meningioma, classical NF, central NF, neurofibroma, and other synonyms for NF were mapped to the key phrases “Nigeria,” “neurological,” and “complications.” Relevant MeSH terms were used alone and in combination, adding Boolean operators and wildcards to broaden, restrict, and modify the search, as required. This scoping review describes the historical origins, classification, diagnostic criteria, and clinical presentation of the three major types of NF, with a focus on neurologic complications. Thereafter, it reviews the literature on NF in Nigeria (Nigerians), discussing the implications for ophthalmologists and suggesting guidelines for multidisciplinary team management and follow-up in light of current advances in NF research. Neurologic complications of NF include epilepsy, headaches, learning difficulties, cranial and peripheral neuropathies, and intracranial tumors. Screening, early recognition, long-term monitoring, and rehabilitation by a multidisciplinary team, is required to enhance the quality of life of patients with NF. There are currently very few published studies describing the neurologic complications of NF among Nigerians. However, Nigerian physicians, surgeons, and ophthalmologists should be aware that patients with NF may develop these neurologic complications and should seek to identify them early, in the management and follow-up of patients with NF.