Neurosurgical Presentations of Neurofibromatosis Type 1: Our Experience in a Regional Neurosurgical Centre, Sokoto, Nigeria

Abstract

Objective: The objective of this paper was to highlight various neurosurgical presentations of patients with neurofibromatosis type 1 seen in our center. Design: This is retrospective analysis of all cases of neurofibromatosis type 1 seen in our neurosurgical center over five- year period. Data was extracted from case files retrieved from medical records department of the hospital. Biodemographic characteristics, clinical presentations, neuroimaging findings and treatment offered were noted. Setting: A single center report conducted at department of neurosurgery, regional neurosurgical center, Usmanu Danfodiyo university teaching Hospital Sokoto. Participants: All patients presenting to neurosurgery department with a diagnosis of neurofibromatosis type 1 over five-year period. Interventions: Among the patients seen, three had surgical intervention for disfiguring plexiform neurofibroma. While the remaining two patients refused any form of surgery. Cosmesis was main study outcome which was satisfactory to both the surgeons and the patients. Results: We found a total of five cases managed over the study period. Three males and two females, the youngest was 2 weeks old while the oldest was a 22-year-old man. Three patients had major cranial lesions while two presented with spinal/paraspinal lesions. Also, only three patients met NIH criteria for the diagnosis of NF-1, two presented with only plexiform neurofibromatosis. Two patients declined any form of surgical intervention. Cosmesis was the only reason for presenting to the hospital. Postoperative outcome was good for the three patients that accepted surgery. Conclusion: Neurofibromatosis type 1 occurs at a rate of one case per year in our setting. Various cranial and spinal/paraspinal manifestations were observed.