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Assessment of Pediatric Pseudotumor Cerebri Clinical Characteristics and Outcomes
Erin Yamamoto1, Darren Farber2, David Rothner3
1443553Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
Insights
This study details pediatric pseudotumor cerebri (idiopathic intracranial hypertension) cases over 23 years, noting common symptoms and outcomes. It highlights less common presentations, including those without headache or papilledema.
Area of Science:
- Neurology
- Pediatrics
- Ophthalmology
Background:
- Pseudotumor cerebri, or idiopathic intracranial hypertension (IIH), is a rare condition with unknown causes.
- Existing literature on IIH predominantly features adult studies, leaving a gap in pediatric understanding.
- This study focuses on a large cohort of pediatric patients to better understand IIH in this demographic.
Purpose of the Study:
- To retrospectively analyze the clinical presentation, diagnostic imaging, treatment strategies, and patient outcomes in a substantial pediatric pseudotumor cerebri population.
- To investigate the occurrence and characteristics of secondary IIH and atypical presentations in children, such as those lacking headache or papilledema.
- To provide a comprehensive overview of pediatric IIH over a 23-year period.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with pseudotumor cerebri over a 23-year period.
- Analysis of clinical data including symptoms, physical examination findings (papilledema), neuroimaging results, and treatment interventions.
- Evaluation of patient outcomes, focusing on headache resolution and visual function at multiple time points post-treatment.
Main Results:
- The study identified a significant proportion of secondary pseudotumor cerebri cases (44%) and recognized subgroups presenting without typical symptoms like headache (13.3%) or papilledema (7.3%).
- Demographic factors such as female sex and obesity aligned with existing literature.
- Radiographic findings were notably less prevalent in this pediatric cohort compared to adult studies.
Conclusions:
- This research provides valuable insights into the clinical spectrum and outcomes of pediatric pseudotumor cerebri, expanding upon the limited pediatric literature.
- The findings underscore the importance of considering IIH even in the absence of classic symptoms like headache or papilledema in children.
- Further research is warranted to elucidate the pathophysiology and optimize management strategies for pediatric idiopathic intracranial hypertension.
Abstract:
Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs in both children and adults, the pseudotumor cerebri literature is heavily dominated by adult studies. The aim of this study is to retrospectively describe the clinical presentation, imaging, treatment, and outcomes of a large pediatric pseudotumor cerebri population over a 23-year period. We also discuss secondary pseudotumor cerebri (44%) as well as the increasingly recognized patient subgroups without headache (13.3%) and without papilledema (7.3%). Female sex, obesity, and initial symptoms were consistent with the literature; however radiographic findings were surprisingly low in this cohort. Headache outcomes at 1 week, 1 month, and 3 months following initial lumbar puncture/treatment and visual function outcomes are reported.
