Normal pressure hydrocephalus in pediatrics: a comprehensive review and practical management algorithm

Abstract

Background
Normal pressure hydrocephalus (NPH) is a well-recognized entity in adults but remains poorly characterized in the pediatric population, with fewer than 150 cases described to date. Early diagnosis is crucial, as untreated cases may result in irreversible neurodevelopmental decline. Despite its rarity, timely intervention can significantly improve neurological outcomes in affected children.


Methods
A narrative review of the literature was performed using the PubMed database (1987–2024). Clinical studies, systematic reviews, and case reports with at least three patients and outcome data were included. Priority was given to articles reporting both diagnostic approaches and therapeutic outcomes.


Results
Pediatric NPH most commonly presents with cognitive impairment and motor disturbances, with urinary incontinence being less common. Besides clinical suspicion, diagnosis hinges on neuroimaging, CSF flow studies, normal CSF opening pressure, with the tap test serving as a key predictive tool. Treatment is surgical and programmable shunts represent current standard of care, in selected cases, endoscopic third ventriculostomy (ETV) has shown favourable outcomes, while endoscopic coagulation of the choroid plexus (ECCP) has been proposed in high-risk cases. We integrate this evidence to propose a structured diagnostic and management algorithm. Nevertheless, significant knowledge gaps persist, especially regarding validated biomarkers and standardised diagnostic criteria for children.


Conclusion
Pediatric NPH is a distinct, underdiagnosed condition. This review highlights the imperative for age-specific criteria and presents a practical algorithm to guide clinical decision-making. Future multicentre studies validating this framework and focusing on surgical outcomes are essential to optimize long-term neurodevelopmental prospects.