Pseudotumor cerebri in childhood: a new vision to clinical spectrum and predictors for visual outcome

Objective: To evaluate the clinical spectrum and visual outcome in children with pseudotumor cerebri (PTC) through a new vision using a scoring model based-on magnetic resonance imaging (MRI) findings with respect to cerebrospinal fluid (CSF) pressure. Methods: A total of 47 children with PTC, had been treated with departmental pharmacotherapy and surgical modalities included in the cohort: group I:elevated CSF pressure (>250-280 mm-H2O) and group II:normal CSF pressure. A scoring model was designed based on the four MRI parameters: (P1)decreased pituitary gland size, (P2)posterior globe flattening, (P3)horizontal tortuosity of the optic nerve, (P4)transverse sinus stenosis. The scoring model applied for the prediction of the early visual outcome and the long-term outcome. Results: Female to male ratio was 1.23:1. The mean age at the time of diagnosis was 10.3±3.9 (1-16) years. Group I had 35 patients and CSF pressure (mean 464±177 mm-H2O, range:260-930). Group II involved 12 (25.5%) patients with following MRG parameters: P1 in 5, P2 in 7, P3 in 7, P4 in 4 patients. Optic nerve surgery was performed in 5 (10.6%) patients in the early period due to resistance to medical therapy or progress very fast and 21 (44.7%) patients had visual field defects in the long-term outcome in all group. No statistically significant correlation was found between the total score of MRI findings, CSF pressure values, and visual outcome. Conclusion: The diagnostic challenges of PTC in childhood are still problematic without CSF analysis. MRI findings provide a significant impact on the clinical diagnosis of PTC in children.
Keywords: Pseudotumor cerebri