Inflammatory Markers in Cerebrospinal Fluid from Patients with Hydrocephalus: A Systematic Literature Review.

OBJECTIVE: The aim of this systematic review was to evaluate existing literature on inflammatory markers in CSF from patients with hydrocephalus and identify potential markers capable of promoting hydrocephalus development and progression. METHODS: Relevant studies published before December 3rd 2020 were identified from PubMed, Embase, and reference lists. Studies were screened for eligibility using the predefined inclusion and exclusion criteria. Data from eligible studies were extracted, and sources of bias were evaluated. We included articles written in English investigating inflammatory markers in CSF from patients with hydrocephalus and control subjects. The review was conducted according to the PRISMA guidelines by three independent reviewers. RESULTS: Twenty-two studies analyzed CSF from 311 patients with idiopathic normal pressure hydrocephalus (iNPH), 178 with posthemorrhagic hydrocephalus (PHH), 151 with other hydrocephalus diagnoses, and 394 control subjects. Fifty-eight inflammatory markers were investigated. The CSF of iNPH patients had increased CSF levels of IL-6, IL-1ß, and LRG compared with control subjects, whereas the CSF of PHH patients had increased levels of IL-6, IL-18, and VEGF. CSF from patients with "other hydrocephalus diagnoses" had elevated IFN-γ compared to control subjects, and VEGF was increased in congenital hydrocephalus, spina bifida, and hydrocephalus associated with tuberculous meningitis compared with controls. CONCLUSION: IL-6, IL-1ß, LRG, IL-18, VEGF, and IFN-γ are elevated in CSF from patients with hydrocephalus and may be involved in promotion of hydrocephalus development and progression. They may serve as novel disease biomarkers, and their signaling pathways may represent targets for pharmacological management of hydrocephalus.

Hidrocefalia canina ­ relato de caso / Canine hydrocephalus ­ case report / Hidrocefalia canina ­ relato de caso

Hidrocefalia é o acúmulo de líquido cefalorraquidiano de origens diversas. Tal acúmulo gera aumento da pressão intracraniana e pode causar compressão e danos a tecidos neurológicos. Estes danos são a causa dos sinais clínicos associados a esta afecção. O presente artigo relata um caso de hidrocefalia em uma fêmea canina da raça Shih Tzu de 45 dias de idade. O animal apresentava alterações comportamentais, vocalização excessiva, incoordenação motora, aumento de volume craniano, oligodpsia entre outros. A cadela foi diagnosticada com hidrocefalia por meio de exames de imagem. Foi instituída terapia medicamentosa com acetazolamida e furosemida para controle paliativo da afecção. Ocorreu melhora do quadro clínico após o início da terapêutica e o animal, apresentou sobrevida de aproximadamente um ano.(AU)

Hydrocephalus is the accumulation of cerebrospinal fluid in the brain coming from diverse origins. Such accumulation generates an increase in intracranial pressure and may cause compression and damage to neurological tissues. These damages are the cause of the onset of clinical signs associated with the condition. This study reports a case of hydrocephalus in a 45-day-old Shi Tzu female dog. The animal presented behavioral alterations, excessive vocalization, motor incoordination, cranial volume increase, polydipsia, among other clinical signals. Imaging tests were used for diagnosing it with hydrocephalus. Drug therapy with acetazolamide and furosemide was introduced for palliative control of the condition. The clinical scenario improved after the initiation of therapy and the animal presented a survival of approximately one year.(AU)

Hidrocefalia es el acúmulo de líquido cefalorraquídeo de diversos orígenes. Dicha acumulación genera un aumento en la presión intracraneal y puede causar compresión y daños a los tejidos neurológicos. Estos daños son la causa de los signos clínicos asociados a esta afección. El presente artículo informa sobre un caso de hidrocefalia en una hembra canina de la raza Shih Tzu de 45 días de edad. El animal presentaba alteraciones de comportamiento, vocalización excesiva, falta de coordinación motora, aumento del volumen craneal, oligodipsia entre otros. La perra fue diagnosticada con hidrocefalia por pruebas de imágenes. Se instituyó medicamentos con acetazolamida y furosemida para control paliativo de la enfermedad. El cuadro clínico mejoró después del inicio de la terapia y el animal presentó sobrevida de aproximadamente un año.(AU)

Diagnosis, Classification, and Management of Fourth Ventriculomegaly in Adults: Report of 9 Cases and Literature Review.

OBJECTIVE: An enlarged fourth ventricle, otherwise known as fourth ventriculomegaly (4th VM), has been reported previously in the pediatric population, yet literature on adults is scant. We report our experience with 4th VM in adults over an 11-year period and review the literature. MATERIALS AND METHODS: This was a retrospective chart review of adult patients with the diagnosis of 4th VM admitted to the intensive care unit in a tertiary care center. RESULTS: Nine patients were identified with 4th VM. Most presented with symptoms in the posterior fossa. Five cases were related to previous shunting and the underlying neurosurgical diseases, and average time interval to develop symptoms was 5.3 years. We divided our cases into primary, acquired, and degenerative based on the pathophysiology involved. Treatments included extended subzero cerebrospinal fluid diversion using a frontal external ventricular drain followed by low-pressure shunt revision, endoscopic third ventriculostomy, suboccipital decompression, and fourth ventricular catheter placement. Literature review identified additional published cases, and there were no reports of a formal classification scheme or treatment algorithm. CONCLUSIONS: This case series illustrates a narrow spectrum of etiologies associated with 4th VM in adults. We propose a simple classification scheme dividing 4th VM into 3 categories: primary, acquired, and degenerative. We recommend a stepwise treatment approach starting with extended subzero cerebrospinal fluid diversion followed by shunting for symptomatic primary and acquired 4th VM. Lower success rates and greater morbidity are associated with rescue procedures such as fourth ventricle drainage catheters, endoscopic third ventriculostomies, and skull base decompression.

Evaluation of hydrocephalus patients with 3D-SPACE technique using variant FA mode at 3T.

The major advantages of three-dimensional sampling perfection with application optimized contrasts using different flip-angle evolution (3D-SPACE) technique are its high resistance to artifacts that occurs as a result of radiofrequency or static field, the ability of providing images with sub-millimeter voxel size which allows obtaining reformatted images in any plane due to isotropic three-dimensional data with lower specific absorption rate values. That is crucial during examination of cerebrospinal-fluid containing complex structures, and the acquisition time, which is approximately 5 min for scanning of entire cranium. Recent data revealed that T2-weighted (T2W) 3D-SPACE with variant flip-angle mode (VFAM) imaging allows fast and accurate evaluation of the hydrocephalus patients during both pre- and post-operative period for monitoring the treatment. For a better assessment of these patients; radiologists and neurosurgeons should be aware of the details and implications regarding to the 3D-SPACE technique, and they should follow the updates in this field. There could be a misconception about the difference between T2W-VFAM and routine heavily T2W 3D-SPACE images. T2W 3D-SPACE with VFAM imaging is only a subtype of 3D-SPACE technique. In this review, we described the details of T2W 3D-SPACE with VFAM imaging and comprehensively reviewed its recent applications.

Hidrocefalia congênita bilateral total em felino: relato de caso / Total Congenital Bilateral Hydrocephalus in Feline: case report

O objetivo deste trabalho foi relatar um caso raro de hidrocefalia em um felino doméstico, da raça Persa, de 30 dias de idade. O animal foi atendido com histórico de impossibilidade de manter-se em estação, incoordenação motora, inabilidade para se alimentar sozinho e sustentar o peso da cabeça. No exame clínico, foi observada presença de fontanela aberta, aumento de calota craniana, ataxia, estrabismo unilateral e secreção ocular. A realização de ultrassonografia do crânio levou à confirmação do diagnóstico de hidrocefalia. O paciente permaneceu internado, sendo o protocolo de tratamento empregado constituído de corticosteroide, diurético e protetor gástrico em alta dose, além de alimentação por via oral e fluidoterapia. O animal veio a óbito após 24 horas, sendo encaminhado para realização de necropsia.(AU)

The objective of this work was to report a rare case of hydrocephalus in a domestic Persian feline, 30 days old. The animal was attended with a history of inability to keep in season, incoordination of the motor, inability to feed itself and support the weight of the head. In the clinical examination, the presence of an open fontanelle, an increase in the skull cap, ataxia, unilateral strabismus and ocular secretion were observed. The ultrasound examination of the skull led to confirmation of the diagnosis of hydrocephalus. The patient remained hospitalized, and the treatment protocol consisted of a corticosteroid, diuretic and gastric protector in high dose, besides oral feeding and fluid therapy. The animal died after 24 hours, being referred for necropsy.(AU)

[Strategy of the diagnosis and treatment for hydrocephalus associated with acoustic neuroma].

OBJECTIVE: To describe and analyze the strategy of the diagnosis and treatment for acoustic neuroma associated with hydrocephalus. METHODS: A retrospective review was performed in 29 patients with hydrocephalus associated with acoustic neuroma form Apr. 2004 to Apr. 2015. The patients' clinical information, the types of the hydrocephalus, the treatment and the prognosis of the hydrocephalus were recorded. RESULTS: There were 20 patients with obstructive hydrocephalus and 9 patients with communicating hydrocephalus preoperatively. Among the 29 cases, 3 patients had ventriculoperitoneal shunts, 5 patients had external ventricular drains, the remaining 21 patients had no further managements for hydrocephalus; after removing the acoustic neuroma, the hydrocephalus improved in 10 cases, the ventricle unchanged in 10 cases among the obstructive hydrocephalus group, the ventricle unchanged in all 9 cases among the communicating hydrocephalus group. Nineteen cases were diagnosed with communicating hydrocephalus and 10 cases with obstructive hydrocephalus postoperatively. CONCLUSIONS: Among the patients of acoustic neuroma associated with hydrocephalus, communicating hydrocephalus is more common than obstructive hydrocephalus. The optimal management of acoustic neuroma associated with hydrocephalus is complete removal of the tumor, with treatment only for patients with persistent hydrocephalus.

Imaging of Communicating Hydrocephalus.

Hydrocephalus basically means an increase in the volume of cerebrospinal fluid (CSF) and the ventricles. As simple as it may sound, the definition and classification of hydrocephalus have been a matter of debate over many decades. Many international neurosurgical and radiological workgroups have tried to develop a consensus and over the last 5-10 years have been able to put forth a more well-defined and standardized approach. Though, the debate and controversy surrounding this topic is expected to continue, we have tried to review the most recent and consensually accepted definition and classification. Although conventionally classified as either "obstructive and nonobstructive" or "communicating and noncommunicating," none of the classification schemes were without confusion and errors. A more precise nomenclature is to divide hydrocephalus as either "communicating": with or without obstruction of CSF absorption, or as "noncommunicating": with definite obstruction to CSF absorption. We discuss communicating hydrocephalus in the current article, and the subsequent article deals with noncommunicating hydrocephalus.

Noncommunicating Hydrocephalus.

Noncommunicating hydrocephalus is often referred to as obstructive hydrocephalus and is by definition an intraventricular obstruction of cerebrospinal fluid flow. Patient symptoms depend on the rapidity of onset. Acute obstructive hydrocephalus causes sudden rise in the intracranial pressure, which may lead to death, whereas in chronic hydrocephalus there may not be any symptoms. Computed tomography and magnetic resonance imaging play important roles in the diagnosis and management of hydrocephalus. Advances in magnetic resonance imaging such as the 3D sequences and phase-contrast imaging have revolutionized the preoperative and postoperative assessment of noncommunicating hydrocephalus. We would be discussing the various causes of noncommunicating hydrocephalus and their imaging.

Hydrocephalus in aqueductal stenosis--a retrospective outcome analysis and proposal of subtype classification.

UNLABELLED: Treatment of aqueductal stenosis (AQS) has undergone several paradigm shifts during the past decades. Currently, endoscopic ventriculostomy (ETV) is recommended as treatment of choice. Several authors have addressed the issue of variable ETV success rates depending on age and pathogenetic factors. However, success rates have usually been defined as "ETV non-failure." The aim of the study was a retrospective analysis of radiological and neurological treatment response after ETV or VP-shunting (VPS) in age-dependent subtypes of AQS. PATIENTS AND METHODS: Eighty patients (median age 12.0 years, range 0-79 years) have been treated for MRI-proven aqueductal stenosis. Neurological treatment success was defined by neurological improvement and, in childhood, head circumference. Radiological response was measured as Evan's index in follow-up MRI. Initial signs and symptoms, type of surgery, and complications were analyzed. RESULTS: Four types of AQS have been defined with distinct age ranges and symptomatology: congenital type I (n = 24), chronic progressive (tectal tumor-like) type II (n = 23), acute type III (n = 10), and adult chronic (normal-pressure hydrocephalus-like) type IV (n = 23). Retrospective analysis of neurological and radiological outcome suggested that congenital type I (<1 years of age) may be more successfully treated with VPS than with ETV (81 vs. 50 %). Treatment of chronic juvenile type II (age 2-15) by ETV 19 % compared to 57 % after VP-shunt, but similar neurological improvement (>80 %). There has been no influence of persistent ventriculomegaly in type II after ETV in contrast to VPS therapy for neurological outcome. Adult acute type III (age > 15 years) responded excellent to ETV. Chronic type IV (iNPH-like) patients (age > 21) responded neurologically in 70 % after ETV and VPS, but radiological response was low (5 %). CONCLUSION: AQS can be divided into four distinct age groups and types in regards of clinical course and symptomatology. Depending on the AQS type, ETV cannot be unequivocally recommended. Congenital type I AQS may have a better neurological outcome with VP-shunt whereas acute type III offers excellent ETV results. Chronic progressive type II still requires prospective investigation of long-term ETV outcome, especially when ventriculomegaly persists. Late chronic type IV seems to result in similar outcome after VP-shunt and ETV.

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences.

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms.

The lack of relationship between intracranial pressure and cerebral ventricle indices based on brain computed tomography in patients undergoing ventriculoperitoneal shunt.

BACKGROUND: In this study we investigated whether cerebral ventricle indices based on brain computed tomography (CT) scans are reliable for predicting intracranial pressure (ICP) in hydrocephalic patients. METHODS: Electronic medical records of 221 patients undergoing ventriculoperitoneal shunt due to hydrocephalus were retrospectively reviewed. Cerebral ventricle indices including Evans' index, third ventricle index, cella media index, and ventricular score were calculated from transverse diameters measured at various levels on preoperative brain CT scans. ICP was considered as CSF opening pressure. Patients were categorized into three groups: communicating hydrocephalus, non-communicating hydrocephalus, and normal pressure hydrocephalus (NPH). The non-communicating hydrocephalus group was further divided according to the obstruction site; aqueduct, fourth ventricle outlet, third ventricle, and the foramen of Monro. The primary endpoint was the extent of the correlation between cerebral ventricle indices and ICP in each hydrocephalus group. RESULTS: No cerebral ventricle index correlated with ICP in patients with communicating hydrocephalus (n = 113) and NPH (n = 62). In the non-communicating hydrocephalus group (n = 46), only the third ventricle index revealed moderate negative correlation with ICP (r = -0.395, p < 0.01). In subgroup analyses, the third ventricle index showed a strong negative relationship with ICP only in patients with the third ventricle obstruction (r = -0.779, p < 0.05). CONCLUSIONS: In this study we showed that although an inverse correlation existed between ICP and the third ventricle index only in patients with non-communicating hydrocephalus due to obstruction of the third ventricle, cerebral ventricle indices based on brain CT scan were non-reliable predictors of ICP in hydrocephalic patients.

Intracranial pressure for the characterization of different types of hydrocephalus: A Permutation Entropy study.

Hydrocephalus is a condition characterized by altered cerebrospinal fluid (CSF) dynamics and chronic rises in intracranial pressure (ICP). However, the reason why hydrocephalic physiologies fail to inhibit dangerously high ICP levels is not known. Infusion studies are used to raise ICP and evaluate CSF circulation disorders. In this pilot study, ICP signals recorded during infusion tests from 33 patients with normal pressure hydrocephalus and 36 patients having developed a secondary form of normal pressure hydrocephalus were characterized using Permutation Entropy (PE), a symbolic non-linear method to quantify complexity. Each ICP signal was divided into four epochs--baseline (before infusion begins), infusion, plateau, and recovery (after infusion has stopped)--and the mean PE was calculated for each epoch. Statistically significant differences were found between PE for most epochs (p<;0.00833, Bonferroni-corrected Wilcoxon tests), with a significant decrease in the plateau phase. However, differences between PE for normal pressure and secondary hydrocephalus were not significant. Results suggest that the increase in ICP during infusion studies is associated with a significant decrease in PE. PE analysis of ICP signals could be useful for increasing our understanding of CSF dynamics in normal pressure hydrocephalus.

Infantile hydrocephalus: a review of epidemiology, classification and causes.

Hydrocephalus is a common but complex condition caused by physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Though hydrocephalus was recently estimated to affect 1.1 in 1000 infants, there have been few systematic assessments of the causes of hydrocephalus in this age group, which makes it a challenging condition to approach as a scientist or as a clinician. Here, we review contemporary literature on the epidemiology, classification and pathogenesis of infantile hydrocephalus. We describe the major environmental and genetic causes of hydrocephalus, with the goal of providing a framework to assess infants with hydrocephalus and guide future research.

Ventricular and lumbar cerebrospinal fluid concentrations of Alzheimer's disease biomarkers in patients with normal pressure hydrocephalus and posttraumatic hydrocephalus.

BACKGROUND: Little information is available on the rostro-caudal concentration gradient of Alzheimer's disease (AD) biomarkers. OBJECTIVE: We studied the concentrations of amyloid-ß (Aß) peptides 1-42 and 1-40 as well as the Tau and pTau proteins in simultaneously collected ventricular and lumbar cerebrospinal fluid (CSF) samples. METHODS: The samples were simultaneously collected from the ventricle and the lumbar spinal canal in two groups of patients: 10 subjects being treated for normal pressure hydrocephalus (NPH) by the placement of a ventriculo-peritoneal shunt and 5 patients treated simultaneously with an external ventricular drain and a lumbar CSF drain due to posttraumatic hydrocephalus (PTH). RESULTS: The ventricular-lumbar (V/L) concentration ratio for Aß1-40 was 0.81 in NPH patients and 0.71 in PTH patients. The V/L-ratio for Aß1-42 was 0.84 in NPH, reflecting significantly higher concentrations in lumbar CSF than in ventricular CSF, and 1.02 in PTH patients. The V/L-ratios for Tau and pTau differed significantly depending on the diagnostic group: the median V/L-ratio for Tau was 6.83 in NPH patients but only 0.97 in PTH patients. The median V/L-ratio for pTau was 2.36 in NPH patients and 0.91 in PTH patients. CONCLUSIONS: We conclude that the rostro-caudal concentration gradient for brain-derived proteins (Tau and pTau in this study) depends on the diagnosis and clinical status of the patient, which were largely neglected in the previously postulated models.

Transsylvian-transinsular approach for the removal of basal ganglia hemorrhage under a Modified Intracerebral Hemorrhage score.

BACKGROUND: Spontaneous intracerebral hemorrhages account for 20% of all strokes. The Modified Intracerebral Hemorrhage (MICH) score provides a simple, reliable system for decision making regarding surgical treatment. The transsylvian-transinsular approach had previously been neglected because of the dependence on great surgical experience. We believe this approach not only compares favorably with the minimally invasive surgery concept but also preserves most of the cerebral functional cortex with a maximum hematoma evacuation rate. METHODS: From May 2007 to September 2008, a single surgeon treated 32 patients with basal ganglia hemorrhage using the transsylvian-transinsular approach. Of these, 20 had MICH scores of 2 to 3; 5 had MICH scores of 4; and 7 had MICH scores of 5. After 24 postoperative hours, we evaluated the hematoma evacuation rate by a computed tomography scan. The functional recovery was evaluated by the Barthel Index at 1, 3, and 6 months postoperatively. RESULTS: All data were analyzed according to MICH score. The hematoma evacuation rates were in the following order: MICH scores 2 to 3 (97%) > MICH score 4 (92%) > MICH score 5 (90%). Surgery-related mortality was MICH2, 3 (0%) < MICH4 (20%) < MICH5 (43%). The Barthel Index of the MICH2, 3 patients (n = 18) improved from 16.9 at 1 postoperative month to 41.94 at 6 postoperative months. CONCLUSIONS: The transsylvian-transinsular approach for the removal of an ICH was not difficult, and it was found to be a safe method for treating a spontaneous basal ganglion ICH. In addition, this approach conformed with the spirit of minimally invasive surgery.

Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus.

This study analyzed 156 cases of fetal hydrocephalus treated at Osaka National Hospital from 1992 to 2011 to review current methods for diagnosing and treating fetal hydrocephalus, and for estimating its clinical outcome. This was a retrospective study of a single institute (Osaka National Hospital). Of 156 cases in total, 37% were diagnosed as isolated ventriculomegaly, 50% as another type of malformation (36 cases of myelomeningocele, six of holoprosencephaly, three of Dandy-Walker syndrome, one case of Joubert syndrome, 12 of arachnoid cyst, nine of encephalocele, three of atresia of Monro and eight of corpus callosum agenesis, and 13% as secondary hydrocephalus. Diagnoses were made between 13 and 40 weeks of gestation (average 27 weeks). Diagnosis was made before 21 weeks of gestation in 24% of cases, from the first day of 22 weeks to the sixth day of 27 weeks in 27%, and after the first day of 28 weeks in 49%. With the exclusion of 17 aborted cases and 40 cases in which the patients were too young to evaluate or lost during follow-up, the final outcome was analyzed for 90 cases. Of these, 17% of the patients died, 21% showed severe retardation, 13% moderate retardation, 26% mild retardation, and 23% showed a good outcome. The long-term outcome was mostly influenced by the basic disease and accompanying anomaly. The time of diagnosis showed no correlation with outcome. Hydrocephalus associated with arachnoid cyst, atresia of Monro, and corpus callosum agenesis, and hydrocephalus due to fetal intracranial hemorrhage, resulted in good outcomes. By contrast, holoprosencephaly, hydrocephalus associated with encephalocele, syndromic hydrocephalus, and hydrocephalus due to fetal virus infection led to poor outcomes. For accurate diagnosis and proper counseling, established protocols are important for the diagnosis and treatment of fetal hydrocephalus, including not only fetal sonography, fetal magnetic resonance imaging, and TORCH (toxoplasma, rubella, cytomegalovirus, herpes simplex) screening test, but also chromosomal and gene testing.

[New treatment strategies for intraventricular hemorrhage]. / Neue Therapiestrategien bei zerebraler intraventrikulärer Blutung.

The presence of additional intraventricular hemorrhage (IVH) in patients with intracerebral hemorrhage (ICH) is associated with a much higher mortality and worse functional outcome. Although evidence-based specific treatment options for this entity are still lacking, knowledge about the pathophysiology of IVH has grown in recent decades, leading to the development of promising treatment strategies. Intraventricular fibrinolysis (IVF) accelerates IVH resolution and removal from the ventricular system. The additional usage of lumbar drains probably reduces the incidence of permanent posthemorrhagic hydrocephalus. The influence of these treatment modalities on functional outcome is currently being investigated in ongoing studies. The present article gives an overview of pathophysiological and clinical aspects of IVH, emphasizing novel treatment options.