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Pediatric low-pressure hydrocephalus: a case series and re-analysis of a rare disease

Debajyoti Datta1,2, Albert Tu3,4

  • 1Division of Pediatric Neurosurgery, Children's Hospital of Eastern Ontario (CHEO), 401 Smyth Rd., Ottawa, Ontario, K1H 8L1, Canada.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|November 5, 2025
PubMed

Insights

Low or negative pressure hydrocephalus (LPH) is a rare pediatric condition. Management involves shunting into low-resistance settings or negative pressure cavities, with venous thrombosis possibly contributing to its development.

Area of Science:

  • Pediatric Neurosurgery
  • Neurology
  • Hydrocephalus Research

Background:

  • Low or negative pressure hydrocephalus (LPH) presents with hydrocephalus symptoms despite low or negative intracranial pressure (ICP).
  • This rare condition poses diagnostic and management challenges in pediatric patients.

Purpose of the Study:

  • To review and analyze the clinical characteristics, management strategies, and outcomes of pediatric patients diagnosed with LPH.
  • To investigate potential contributing factors, including venous thrombosis, in the pathogenesis of LPH.

Main Methods:

  • Retrospective study of pediatric patients (≤18 years) with LPH between November 2017 and December 2024.
  • Inclusion criteria: ventricular size changes, hydrocephalus symptoms, ICP ≤ 5 cmH2O, and clinical improvement after CSF drainage.
  • Data collected: demographics, etiology, clinical presentation, management, and outcomes.

Main Results:

  • Six pediatric patients with LPH were included in the study.
  • Management involved low-resistance ventriculoperitoneal shunts (n=3) or shunting into negative pressure cavities (n=3).
  • Two patients (1/3rd) had a history of jugular or sigmoid sinus thrombosis, suggesting a potential link.

Conclusions:

  • LPH in children is rare, challenging to manage, and may involve altered cerebral viscoelasticity or transmantle pressure gradients.
  • Effective management necessitates low-resistance valves or shunting into negative-pressure cavities.
  • Prior venous thrombosis is hypothesized as a potential contributing factor to LPH development.
Abstract

Keywords:
Cerebral venous sinus thrombosis+Cerebral viscoelasticityComplianceHydrocephalusLow pressureNegative pressureSubatmospheric drainageTransmantle pressure

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