Pseudotumor Cerebri Syndrome in Children
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Published on: June 12, 2017
Rebecca Barmherzig1,2, Christina L Szperka3,4
1Division of Neurology, Women's College Hospital Centre for Headache, University of Toronto, Toronto, Canada.
Insights
Pseudotumor cerebri syndrome (PTCS) in children presents uniquely, often with subtle signs. Early identification and management are crucial due to risks of vision loss and reduced quality of life.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Neurology
Background:
- Pseudotumor cerebri syndrome (PTCS) affects children and adults, but risk factors and presentation differ.
- Unique considerations for PTCS in pediatric populations require specific attention.
Purpose of the Study:
- To review the epidemiology, demographics, and clinical presentation of PTCS in children.
- To discuss revised diagnostic criteria, evaluation, management, and prognosis for pediatric PTCS.
- To highlight unique aspects of PTCS in the pediatric population.
Main Methods:
- This is a review article.
- It synthesizes current knowledge on pediatric PTCS.
- No specific experimental methods were employed.
Main Results:
- Clinical presentation of PTCS in children can be subtle and challenging to diagnose.
- Papilledema diagnosis and upper limits for opening pressure (25 cm H2O, or 28 cm H2O in sedated/obese children) require specific pediatric considerations.
- Morbidity includes visual loss, pain, and reduced quality of life, underscoring the need for prompt management.
Conclusions:
- Accurate identification, evaluation, and management of pediatric PTCS are urgent due to potential morbidity.
- Evidence-based recommendations for PTCS management in children are lacking due to the absence of randomized controlled trials.
- Further research is needed to establish consolidated management approaches for PTCS in children.
Purpose Of Review:
Pseudotumor cerebri syndrome (PTCS) may affect both children and adults; however, the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS.
Recent Findings:
Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cut-off of 25 cm H20 (or 28 cm H2O in a sedated or obese child). Morbidity related to visual loss, pain and reduced quality of life lends urgency towards accurately identifying, evaluating and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.
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