Clinical features and prognosis of children diagnosed with pseudotumor cerebri syndrome

Objective: Pseudotumor cerebri syndrome (PTC), also known as idiopathic intracranial hypertension (IIH), is a clinical condition characterized by increased intracranial pressure without any apparent cause such as a mass lesion, hydrocephalus, ventriculomegaly, or infection, despite normal cerebrospinal fluid (CSF) composition. While there is a substantial amount of research on PTC in adults, studies on children are limited. This study aims to identify the clinical features and factors affecting the prognosis of children diagnosed with pseudotumor cerebri syndrome, and to determine the necessary measures to prevent the most severe complication, permanent vision loss, in high-risk patients, thereby contributing to the literature. Materials and Methods: The study received ethical approval from the Non-Interventional Clinical Research Ethics Committee of Çukurova University Faculty of Medicine with decision number 47 dated 09.07.2021. The study included 53 patients under the age of 18 who were diagnosed with PTC according to the revised diagnostic criteria by Friedman et al. in 2013, and who presented to the Pediatric Neurology Department of Çukurova University Faculty of Medicine over the past 5 years. Discussion/Conclusion: The average age of our patients at presentation was 11 years, with 28.3% being prepubertal and 71.7% being pubertal. In our study, the male/female ratio was almost equal in the prepubertal age group, whereas 76.3% of the pubertal age group were girls. A total of 18 (34%) patients were overweight or obese, with 3 (20%) in the prepubertal period and 15 (39.5%) in the pubertal period being overweight or obese. The most common symptoms in our study were headache, blurred vision, nausea, vomiting, and diplopia. There was no statistically significant difference in headache status between patients with normal or elevated opening CSF pressure (p=0.609). CSF pressure may not always correlate with the presence and severity of headache. Therefore, eye findings should be carefully monitored along with headache during the diagnosis and treatment process. In our study, thrombosis was detected in MR venography in 4 (7.6%) patients. Genetic tests performed in all four patients with thrombosis revealed pathologies in genes predisposing to thrombophilia. Iron deficiency was observed in 60.6% (n=20) of female patients, compared to 26.7% (n=4) in male patients, and this difference was statistically significant (p=0.029). Among those who fully recovered, patients receiving dual medical therapy were more common than those receiving multiple medical therapies, and this was found to be statistically significant (p=0.009). Headache and permanent vision loss are the two main complications affecting quality of life in PTC. In our study, none of the 53 patients developed the complication of permanent vision loss. Keywords: Pseudotumor cerebri syndrome, headache, papilledema, cerebrospinal fluid opening pressure