Application of endoscopic third ventriculostomy for treating hydrocephalus-correlated Chiari type I malformation in a single Chinese neurosurgery centre

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Abstract

The correlation between hydrocephalus and Chiari type I malformation (CIM) has been debated since Chiari’s first descriptions of CIM but some studies have shown that CIM and hydrocephalus (HCP) could cause symptoms/disease of each other or vice versa. Recent research has found that treatment focused on hydrocephalus with ventricle enlargement also provides alleviation of CIM and even of syringomyelia. However, the lack of consensus among previous studies left unanswered the question of how endoscopic third ventriculostomy (ETV) addresses CIM and why it fails. Ten symptomatic hydrocephalic patients associated with CIM underwent ETV from October 2002 to May 2012. The clinical features and neuroimaging of all patients were reviewed. Statistical analysis was applied to evaluate the changes in the tonsillar ectopia and the ventricle dilation after operation. The mean follow-up period of this series was 92 months (range 24–163 months). Eight patients (80%) remained shunt free or experienced symptom relief following ETV. The remaining two patients were identified as failures due to the deterioration of symptoms or subsequent hindbrain decompression. Endoscopic third ventriculostomy provides an effective treatment for hydrocephalus associated with CIM, which can relieve HCP and improve the symptoms of CIM in most patients. The clinical outcomes are related to the major cause of the tonsillar herniation.

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Most cited references 22

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Radiological assessment of hydrocephalus: new theories and implications for therapy.

Dan Greitz (2004)

It is almost a century since Dandy made the first experimental studies on hydrocephalus, but its underlying mechanism has been unknown up to now. The conventional view is that cerebrospinal fluid (CSF) malabsorption due to hindrance of the CSF circulation causes either obstructive or communicating hydrocephalus. Analyses of the intracranial hydrodynamics related to the pulse pressure show that this is an over-simplification. The new hydrodynamic concept presented here divides hydrocephalus into two main groups, acute hydrocephalus and chronic hydrocephalus. It is still accepted that acute hydrocephalus is caused by an intraventricular CSF obstruction, in accordance with the conventional view. Chronic hydrocephalus consists of two subtypes, communicating hydrocephalus and chronic obstructive hydrocephalus. The associated malabsorption of CSF is not involved as a causative factor in chronic hydrocephalus. Instead, it is suggested that increased pulse pressure in the brain capillaries maintains the ventricular enlargement in chronic hydrocephalus. Chronic hydrocephalus is due to decreased intracranial compliance, causing restricted arterial pulsations and increased capillary pulsations. The terms "restricted arterial pulsation hydrocephalus" or "increased capillary pulsation hydrocephalus" can be used to stress the hydrodynamic origin of both types of chronic hydrocephalus. The new hydrodynamic theories explain why third ventriculostomy may cure patients with communicating hydrocephalus, a treatment incompatible with the conventional view.

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Chiari I malformations: clinical and radiologic reappraisal.

Eric A. Elster, Hong Chen (1992)

Clinical findings and magnetic resonance (MR) images in 68 patients with Chiari I malformations were retrospectively analyzed to identify those radiologic features that correlated best with clinical symptoms. A statistically significant (P = .03) female predominance of the malformation was observed, with a female: male ratio of approximately 3:2. Associated skeletal anomalies were seen in 24% of patients. Syringomyelia was detected in 40% of patients, most commonly between the C-4 and C-6 levels. Of the 25 patients who presented with spinal symptoms, 23 (92%) proved to have a syrinx at MR imaging. When the syrinx extended into the medulla (n = 3), however, brain stem symptoms predominated. Patients with objective brain stem or cerebellar signs had the largest mean tonsillar herniations. Patients with tonsillar herniations greater than 12 mm were invariably symptomatic, but approximately 30% of patients with tonsils herniating 5-10 mm below the foramen magnum were asymptomatic at MR imaging. "Incidental" Chiari I malformations are thus much more common than previously recognized, and careful clinical assessment remains the cornerstone for proper diagnosis and management.

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The natural history of the Chiari Type I anomaly.

Antonio Massa, Benedetta Pettorini, Luca Massimi … (2008)

Since the advent of MR imaging, an increasing number of asymptomatic or oligosymptomatic patients have been diagnosed with Chiari malformation Type I (CM-I). The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease. The authors report on their experience in a series of 22 patients with CM-I who were evaluated at the authors' institution, and for whom a conservative approach to treatment was adopted. The patients ranged in age from 1 to 16 years (mean 6.3 years) at diagnosis. Neuroradiological and complete clinical examinations were performed in all patients 6 months after the first observation and every year thereafter. The follow-up period ranged from 3 to 19 years (mean 5.9 years). Chiari malformation Type I was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up. The authors' data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations.

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Author and article information

Contributors

Yiping Wu: jesse522@163.com

Chuzhong Li: lichuzhong@163.com

Xuyi Zong: zongxuyi@163.com

Xinsheng Wang: xshwang@126.com

Songbai Gui: guisongbai@hotmail.com

Caiping Gu: gcp5256@163.com

Yazhuo Zhang: zyz2004520@yeah.net

Journal

Journal ID (nlm-ta): Neurosurg Rev

Journal ID (iso-abbrev): Neurosurg Rev

Title: Neurosurgical Review

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Print): 0344-5607

ISSN (Electronic): 1437-2320

Publication date (Electronic): 22 March 2017

Publication date PMC-release: 22 March 2017

Publication date (Print): 2018

Volume: 41

Issue: 1

Pages: 249-254

Affiliations

[1 ]ISNI 0000 0004 0369 153X, GRID grid.24696.3f, Beijing Neurosurgical Institute, , Capital Medical University, ; Beijing, China

[2 ]ISNI 0000 0004 0369 153X, GRID grid.24696.3f, Department of Neurosurgery, , Beijing Tiantan Hospital, Capital Medical University, ; Beijing, China

[3 ]ISNI 0000 0004 0369 153X, GRID grid.24696.3f, Beijing Institute for Brain Disorders Brain Tumor Center|, ; Beijing, China

[4 ]ISNI 0000 0004 0642 1244, GRID grid.411617.4, China National Clinical Research Center for Neurological Diseases, ; Beijing, China

[5 ]ISNI 0000 0000 9255 8984, GRID grid.89957.3a, Department of Neurosurgery, , Affiliated Wuxi People’s Hospital of Nanjing Medical University, ; Wuxi, China

Article

Publisher ID: 844

DOI: 10.1007/s10143-017-0844-x

PMC ID: 5748424

PubMed ID: 28326451

SO-VID: 565da016-a61e-4d8c-b7b9-a210f220c885

License:

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

History

Date received : 13 December 2016

Date revision received : 1 March 2017

Date accepted : 9 March 2017

Funding

Funded by: Research Special Fund For Public Welfare Industry of Health

Award ID: 201402008

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ScienceOpen disciplines: Surgery

Keywords: endoscopic third ventriculostomy,hydrocephalus,chiari type i malformation,tonsillar herniation

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ScienceOpen disciplines: Surgery

Keywords: endoscopic third ventriculostomy, hydrocephalus, chiari type i malformation, tonsillar herniation

Application of endoscopic third ventriculostomy for treating hydrocephalus-correlated Chiari type I malformation in a single Chinese neurosurgery centre

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Chinese Journal of General Practice

Most cited references 22

Radiological assessment of hydrocephalus: new theories and implications for therapy.

Chiari I malformations: clinical and radiologic reappraisal.

The natural history of the Chiari Type I anomaly.

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