Recent Advances and Current Controversies in Hydrocephalus

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: 25 February 2024 | Viewed by 2131

Special Issue Editors

Department of Neurosurgery and Neurotraumatology and Neurosurgery Research Unit (UNINN), Vall d’Hebron University Hospital, Universitat Autònoma de Barcelona, Passeig Vall d’Hebron 119–129, 08035 Barcelona, Spain
Interests: cerebrospinal fluid dynamics; normotensive hydrocephalus; traumatic brain injuries (TBI); pediatric neurosurgery; skull base surgery; surgery of the cranio-cervical junction
Special Issues, Collections and Topics in MDPI journals
Department of Neurosurgery and Neurotraumatology and Neurosurgery Research Unit (UNINN), Vall d’Hebron University Hospital, Universitat Autònoma de Barcelona, Passeig Vall d’Hebron 119–129, 08035 Barcelona, Spain
Interests: traumatic brain injury; neurocritical care; brain edema; brain energy metabolism; blood brain barrier; hydrocephalus; neurooncology; ischemic stroke; microdialysis; chiari malformations; cerebrospinal fluid dynamics; cerebral blood flow/metabolism; intracranial pressure
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

The past two decades have seen an unprecedented increase in our knowledge of the physiology and dynamics of human cerebrospinal fluid (CSF). New experimental and clinical investigations that can be summed up by the so-called Bulat–Klarica–Oreskovic hypothesis have recently cast doubt on the canonical understanding of CSF generation and reabsorption. Experimental investigations and human studies have shown that water enters every CNS compartment and that there is a constant water exchange between the CSF, interstitial fluid, and brain capillaries. The hypothesis of diffuse CSF generation and reabsorption has been supported by the finding of the significance of aquaporins, particularly AQP4 in astrocytes and endothelial cells. Another finding that supports these hypotheses is the description of the glymphatic system as a low-resistance network for the bulk flow of CSF running along the periarterial spaces and driven by a combination of arterial pulsatility, respiration, and CSF pressure gradients.

Variability in treatments is still the norm when treating hydrocephalus at any age. Shunting and endoscopic third ventriculostomy (ETV) are options that are applied despite a lack of consensus about indications or in the evaluation of outcomes. Because of the significant clinical and methodological heterogeneity, it is challenging for neurosurgeons to make decisions based on the best available evidence. The success–failure of ETV is still based in very crude estimators or suboptimal biomarkers such as the time to failure, in which ‘failure‘ is defined under the ambiguous term ‘need for surgical revision’.

Despite the popularity of ETV in the neurosurgical armamentarium, most patients with hydrocephalus are still managed with the implantation of a shunt in which the CSF’s flow is regulated by a differential-pressure valve. With today's shunt technology, neurosurgeons can choose from a wide range of hardware and customize it for each patient according to their age, height, weight, and clinical and neuroradiological parameters. This selection is especially important for patients with normal pressure hydrocephalus since it will help lower the number of non-responders and complications associated with shunts. In the pediatric population, the goal of selecting the most adequate shunt hardware is to reduce the number of shunt failures that infants and children will have until they become adults, an important factor in obtaining their best long-term outcomes and social integration.

In this Special Issue, we will present manuscripts that update contemporary concepts in the production, circulation, and reabsorption of the CSF. In order to individualize care and enhance surgical results, we will address contentious issues relating to screening procedures; diagnostic tests, including ICP monitoring; and the treatment of patients with hydrocephalus. We think that a more rational and individualized approach for choosing the best treatment, (neuroendoscopic or shunt implant) and the right choice of the best device—fixed or programmable valves with or without gravitational devices—can reduce variabilities in management, increase the likelihood of a successful outcome, and lessen long-term complications in patients with hydrocephalus. An individualized approach to treating hydrocephalus in children would aid in proper brain development and provide these kids the best chance of being able to adjust socially and professionally to a world that is becoming more and more demanding. If detected early and treated effectively, cognitive decline brought on by normal pressure hydrocephalus in the elderly, whose life expectancy is increasing, can be reversed. This will enhance the patient's and their family's quality of life.

Prof. Dr. Maria A. Poca
Prof. Dr. Juan Sahuquillo
Guest Editors

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Keywords

  • hydrocephalus
  • normal pressure hydrocephalus
  • adult chronic hydrocephalus
  • Hakim and Adams’ syndrome
  • CSF dynamic studies
  • intracranial pressure monitoring
  • shunt selection
  • congenital hydrocephalus
  • shunt overdrainage
  • shunt–patient mismatch
  • Hakim's Syndrome
  • ventriculo-peritoneal shunts
  • cerebrospinal fluid shunts

Published Papers (1 paper)

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Research

11 pages, 3368 KiB  
Article
Low- and Negative-Pressure Hydrocephalus: New Report of Six Cases and Literature Review
J. Clin. Med. 2023, 12(12), 4112; https://doi.org/10.3390/jcm12124112 - 18 Jun 2023
Cited by 1 | Viewed by 1745
Abstract
Low- or very-low-pressure hydrocephalus is a serious and rare phenomenon, which is becoming better known since it was first described in 1994 by Pang and Altschuler. Forced drainage at negative pressures can, in most cases, restore the ventricles to their original size, thus [...] Read more.
Low- or very-low-pressure hydrocephalus is a serious and rare phenomenon, which is becoming better known since it was first described in 1994 by Pang and Altschuler. Forced drainage at negative pressures can, in most cases, restore the ventricles to their original size, thus achieving neurological recovery. We present six new cases that suffered this syndrome from 2015 to 2020: two of them after medulloblastoma surgery; a third one as a consequence of a severe head trauma that required bifrontal craniectomy; another one after craniopharyngioma surgery; a fifth one with leptomeningeal glioneuronal tumor; and, finally, a patient with a shunt for normotensive hydrocephalus. Before the development of this condition, four of them had mid-low-pressure cerebrospinal fluid (CSF) shunts. Four patients required cerebrospinal fluid (CSF) drainage at negative pressures oscillating from zero to −15 mmHg by external ventricular drainage until ventricular size normalized, followed by the placement of a new definitive low-pressure shunt, one of them to the right atrium. The duration of drainage in negative pressures through external ventricular drainage (EVD) ranged from 10 to 40 days with concomitant intracranial pressure monitoring at the neurointensive care unit. Approximately 200 cases of this syndrome have been described in the literature. The causes are varied and superimposable to those of high-pressure hydrocephalus. Neurological impairment is due to ventricular size and not to pressure values. Subzero drainage is still the most commonly used method, but other treatments have been described, such as neck wrapping, ventriculostomy of the third ventricle, and lumbar blood patches when associated with lumbar puncture. Its pathophysiology is not clear, although it seems to involve changes in the permeability and viscoelasticity of the brain parenchyma together with an imbalance in CSF circulation in the craniospinal subarachnoid space. Full article
(This article belongs to the Special Issue Recent Advances and Current Controversies in Hydrocephalus)
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