Correct understanding of the definition and management strategies for refractory hydrocephalus

Abstract Hydrocephalus refers to the abnormal accumulation of cerebrospinal fluid (CSF) in the central nervous system, typically resulting from an imbalance between CSF production and absorption. Traditional classifications of hydrocephalus do not incorporate management strategies (not classified ac...

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Main Authors: Zhixiong Lin, Hua Feng, Wangming Zhang, Gelei Xiao, Jingyu Chen, Zhiqiang Liu
Format: Article
Language:English
Published: BMC 2025-08-01
Series:Chinese Neurosurgical Journal
Subjects:
Online Access:https://doi.org/10.1186/s41016-025-00403-9
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author Zhixiong Lin
Hua Feng
Wangming Zhang
Gelei Xiao
Jingyu Chen
Zhiqiang Liu
author_facet Zhixiong Lin
Hua Feng
Wangming Zhang
Gelei Xiao
Jingyu Chen
Zhiqiang Liu
author_sort Zhixiong Lin
collection DOAJ
description Abstract Hydrocephalus refers to the abnormal accumulation of cerebrospinal fluid (CSF) in the central nervous system, typically resulting from an imbalance between CSF production and absorption. Traditional classifications of hydrocephalus do not incorporate management strategies (not classified according to the degree of difficulty of treatment). Clinically, hydrocephalus that is challenging to treat is often categorized as refractory hydrocephalus (RH). However, the absence of a unified definition of RH impedes the standardization of treatment approaches, raising clinical dilemmas. This article explores the definition, etiologies, classification, and management strategies for RH. Based on the literature and the Diagnosis-Related Group payment system principles, RH is clinically defined as progressive hydrocephalus meeting one or more of the following criteria: (1) the absence of significant clinical or radiological improvement within 60 days despite standard interventions, usually due to pathological factors, such as abnormal CSF characteristics, (2) inability to achieve curative surgical treatments attributable to complex anatomy such as abnormal dynamic changes or multiloculated compartments, and (3) failure to respond after two consecutive therapeutic procedures. RH consists of six distinct subtypes, with infectious hydrocephalus being the most common, followed by low-pressure hydrocephalus. Temporary management strategies for RH must be carefully tailored to patient-specific characteristics, considering the risk–benefit analysis of available measures. In cases of infectious RH, achieving CSF sterilization and evaluating the results are crucial. Curative surgery for infectious RH should be performed only after CSF has been completely sterilized to normal levels. In low-pressure RH, a critical focus is identifying and addressing the sites receiving CSF. 
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spelling doaj-art-34bc8194c86f43bf851cb113bbdcd4312025-08-20T03:07:24ZengBMCChinese Neurosurgical Journal2057-49672025-08-011111910.1186/s41016-025-00403-9Correct understanding of the definition and management strategies for refractory hydrocephalusZhixiong Lin0Hua Feng1Wangming Zhang2Gelei Xiao3Jingyu Chen4Zhiqiang Liu5Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, BeijingDepartment of Neurosurgery, Southwest Hospital, Third Military Medical UniversityThe National Key Clinical Specialty, Guangdong Provincial Key Laboratory on Brain Function Repair and Regeneration, Neurosurgery Center, The Engineering Technology Research Center of Education Ministry of China on Diagnosis and Treatment of Cerebrovascular Disease, Zhujiang Hospital, The Neurosurgery Institute of Guangdong Province, Southern Medical UniversityDepartment of Neurosurgery, Xiangya Hospital, Central South University, ChangshaDepartment of Neurosurgery, Southwest Hospital, Third Military Medical UniversityDepartment of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, BeijingAbstract Hydrocephalus refers to the abnormal accumulation of cerebrospinal fluid (CSF) in the central nervous system, typically resulting from an imbalance between CSF production and absorption. Traditional classifications of hydrocephalus do not incorporate management strategies (not classified according to the degree of difficulty of treatment). Clinically, hydrocephalus that is challenging to treat is often categorized as refractory hydrocephalus (RH). However, the absence of a unified definition of RH impedes the standardization of treatment approaches, raising clinical dilemmas. This article explores the definition, etiologies, classification, and management strategies for RH. Based on the literature and the Diagnosis-Related Group payment system principles, RH is clinically defined as progressive hydrocephalus meeting one or more of the following criteria: (1) the absence of significant clinical or radiological improvement within 60 days despite standard interventions, usually due to pathological factors, such as abnormal CSF characteristics, (2) inability to achieve curative surgical treatments attributable to complex anatomy such as abnormal dynamic changes or multiloculated compartments, and (3) failure to respond after two consecutive therapeutic procedures. RH consists of six distinct subtypes, with infectious hydrocephalus being the most common, followed by low-pressure hydrocephalus. Temporary management strategies for RH must be carefully tailored to patient-specific characteristics, considering the risk–benefit analysis of available measures. In cases of infectious RH, achieving CSF sterilization and evaluating the results are crucial. Curative surgery for infectious RH should be performed only after CSF has been completely sterilized to normal levels. In low-pressure RH, a critical focus is identifying and addressing the sites receiving CSF. https://doi.org/10.1186/s41016-025-00403-9HydrocephalusDiagnosisTreatmentRefractory
spellingShingle Zhixiong Lin
Hua Feng
Wangming Zhang
Gelei Xiao
Jingyu Chen
Zhiqiang Liu
Correct understanding of the definition and management strategies for refractory hydrocephalus
Chinese Neurosurgical Journal
Hydrocephalus
Diagnosis
Treatment
Refractory
title Correct understanding of the definition and management strategies for refractory hydrocephalus
title_full Correct understanding of the definition and management strategies for refractory hydrocephalus
title_fullStr Correct understanding of the definition and management strategies for refractory hydrocephalus
title_full_unstemmed Correct understanding of the definition and management strategies for refractory hydrocephalus
title_short Correct understanding of the definition and management strategies for refractory hydrocephalus
title_sort correct understanding of the definition and management strategies for refractory hydrocephalus
topic Hydrocephalus
Diagnosis
Treatment
Refractory
url https://doi.org/10.1186/s41016-025-00403-9
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