Pseudotumor cerebri syndrome (PTCS): clinical analysis on a cohort of 37 pediatric subjects

OBJECTIVE: Pseudotumor Cerebri Syndrome (PTCS) is defined by the whole of signs and symptoms caused by elevated intracranial pressure of unclear etiology in the setting of normal brain parenchyma and cerebrospinal fluid. The diagnostic workup is important to differentiate this disorder from other conditions including brain tumors. The aim of this study is to emphasize the role of clinical signs as well as ophthalmologic evaluation for an accurate diagnosis, and to present a case-series of 37 children affected by this condition. PATIENTS AND METHODS: A retrospective study review has been performed using the medical records of the hospitalized children in the Policlinico-Vittorio Emanuele-G. Rodolico, Pediatric Units of Catania (Italy) from October 2005 to February 2020. The study was conducted ethically in accordance with World Medical Association Declaration of Helsinki and was approved by the Ethic Committee of the University of Catania, Italy (Catania 1 Clinical Registration n 138877/PO). We identified 37 children who were diagnosed with PTCS. In all the subjects, diagnosis Intracranial Idiopathic Hypertension has been verified in accordance to the Friedman 2013 criteria. Subjects with secondary intracranial hypertension have been excluded. RESULTS: A total of 37 subjects were included in this study, with a mean age, at the time of the admission, of 10 ± 3.5 years (range 4-18 years). Among these children, 56.7% were female (21 female/16 male) and almost all were overweight and/or obese (35/37, 94.5%). The most frequent symptoms were headache, vomiting and paralysis of the VI cranial nerve. In 16.2% of the cases, headache was not reported, 8.1% were asymptomatic and 5.4% had not manifested papilledema. CONCLUSIONS: In this study group, obesity and overweight were the main risk factors of intracranial Idiopathic hypertension, regardless of gender and age of the children. A different gender distribution was observed in the two groups of children based on age and pubertal development: Female gender was a risk factor for children over 10 years old and/or in pubertal stage, while the male gender was predominant in younger and/or prepubertal children. Our data suggest that, even if a minority, there is a number of children that remains under-diagnosed with a greater potential risk of developing long-term complications