Cine phase-contrast magnetic resonance imaging evaluation of cerebrospinal fluid flow dynamics in healthy pediatric subjects.

Objective: To evaluate cerebrospinal fluid dynamics, using cine phase-contrast magnetic resonance imaging (cine-PC MRI), in healthy pediatric subjects, determining the normal flow values in this population, as well as identifying differences related to age, sex, and body surface area. Materials and Methods: This was a descriptive cross-sectional study involving 32 healthy children and adolescents, in whom the flow of cerebrospinal fluid through the cerebral aqueduct was evaluated quantitatively with cine-PC MRI. We used specialized software to analyze the images obtained with cine-PC MRI, drawing a region of interest on the aqueduct. A flow-time curve was obtained, as were automated measurements of the various parameters. Results: The following normal (mean) values were obtained: net flow, 1.10 ± 0.99 mL/m; stroke volume, 12.2 ± 10.1 µL/cycle; mean velocity, 0.72 ± 1.00 cm/s; peak systolic velocity, 5.28 ± 2.30 cm/s; peak diastolic velocity, 4.51 ± 1.77 cm/s. These values were not affected by age or sex. In addition, body surface area was not found to correlate significantly with mean velocity or stroke volume. Conclusion: In children and adolescents, the basic cerebrospinal fluid flow parameters, as determined by cine-PC MRI, appear to be independent of age and sex.

Objetivo: Avaliar a dinâmica do fluxo do líquido cerebrospinal por cine-ressonância magnética com contraste de fase em crianças saudáveis, para determinar os valores normais de fluxo nesta população, bem como a diferença entre medições distintas de acordo com idade, sexo ou área da superfície corporal. Materiais e Métodos: Convocamos 32 crianças saudáveis em uma análise descritiva de prevalência para a avaliação quantitativa do fluxo do líquido cerebrospinal pelo aqueduto de Sylvius. A análise foi realizada por meio de software especializado, desenhando uma região de interesse no aqueduto. Uma curva de fluxo-tempo e medições automáticas de diversos parâmetros foram obtidas. Resultados: Os seguintes valores normais foram obtidos: fluxo efetivo de 1,10 ± 0,99 mL/m; volume médio de 12,2 ± 10,1 µL/ciclo; velocidade média de 0,72 ± 1,00 cm/s; pico de velocidade sistólica de 5,28 ± 2,30 cm/s; pico de velocidade diastólica de 4,51 ± 1,77 cm/s. Esses valores não foram afetados pela idade ou sexo das crianças. Além disso, não foi encontrada correlação significativa entre a área corporal, a velocidade média e o volume sistólico. Conclusão: De modo geral, os parâmetros básicos do fluxo do líquido cerebrospinal independem de idade e sexo em crianças.

Cine phase-contrast magnetic resonance imaging evaluation of cerebrospinal fluid flow dynamics in healthy pediatric subjects / Avaliação da dinâmica do fluxo do líquido cerebrospinal por cine-ressonância magnética com contraste de fase em crianças saudáveis

Abstract Objective: To evaluate cerebrospinal fluid dynamics, using cine phase-contrast magnetic resonance imaging (cine-PC MRI), in healthy pediatric subjects, determining the normal flow values in this population, as well as identifying differences related to age, sex, and body surface area. Materials and Methods: This was a descriptive cross-sectional study involving 32 healthy children and adolescents, in whom the flow of cerebrospinal fluid through the cerebral aqueduct was evaluated quantitatively with cine-PC MRI. We used specialized software to analyze the images obtained with cine-PC MRI, drawing a region of interest on the aqueduct. A flow-time curve was obtained, as were automated measurements of the various parameters. Results: The following normal (mean) values were obtained: net flow, 1.10 ± 0.99 mL/m; stroke volume, 12.2 ± 10.1 μL/cycle; mean velocity, 0.72 ± 1.00 cm/s; peak systolic velocity, 5.28 ± 2.30 cm/s; peak diastolic velocity, 4.51 ± 1.77 cm/s. These values were not affected by age or sex. In addition, body surface area was not found to correlate significantly with mean velocity or stroke volume. Conclusion: In children and adolescents, the basic cerebrospinal fluid flow parameters, as determined by cine-PC MRI, appear to be independent of age and sex.

Resumo Objetivo: Avaliar a dinâmica do fluxo do líquido cerebrospinal por cine-ressonância magnética com contraste de fase em crianças saudáveis, para determinar os valores normais de fluxo nesta população, bem como a diferença entre medições distintas de acordo com idade, sexo ou área da superfície corporal. Materiais e Métodos: Convocamos 32 crianças saudáveis em uma análise descritiva de prevalência para a avaliação quantitativa do fluxo do líquido cerebrospinal pelo aqueduto de Sylvius. A análise foi realizada por meio de software especializado, desenhando uma região de interesse no aqueduto. Uma curva de fluxo-tempo e medições automáticas de diversos parâmetros foram obtidas. Resultados: Os seguintes valores normais foram obtidos: fluxo efetivo de 1,10 ± 0,99 mL/m; volume médio de 12,2 ± 10,1 μL/ciclo; velocidade média de 0,72 ± 1,00 cm/s; pico de velocidade sistólica de 5,28 ± 2,30 cm/s; pico de velocidade diastólica de 4,51 ± 1,77 cm/s. Esses valores não foram afetados pela idade ou sexo das crianças. Além disso, não foi encontrada correlação significativa entre a área corporal, a velocidade média e o volume sistólico. Conclusão: De modo geral, os parâmetros básicos do fluxo do líquido cerebrospinal independem de idade e sexo em crianças.

Evaluación del cerebro fetal: nuevas herramientas / Fetal brain assessment: new tools

RESUMEN La evaluación del cerebro fetal es un punto imprescindible en el ultrasonido obstétrico, por la gran cantidad de malformaciones que pueden ser diagnosticadas. La guía de ISUOG nos brinda los cortes elementales para la sospecha de la patología cerebral; pero, podemos ampliar y mejorar nuestro ultrasonido con la visualización de estructuras fácilmente reproducibles, tales como el complejo anterior, cuerpo calloso, cisura de Silvio y el cuarto ventrículo. Presentamos algunas herramientas para complementar la evaluación del cerebro fetal.

ABSTRACT The evaluation of the fetal brain is an essential point in obstetric ultrasound due to the large number of malformations that can be diagnosed. The ISUOG guide provides us with the elementary sections for the suspicion of brain pathology; but we can extend and improve our ultrasound with the visualization of easily reproducible structures, such as the anterior complex, corpus callosum, Sylvian fissure and the fourth ventricle. We present some tools to complement the assessment of the fetal brain.

Two-Dimensional Ultrasound Evaluation of the Fetal Cerebral Aqueduct: Improving the Antenatal Diagnosis and Counseling of Aqueductal Stenosis.

OBJECTIVE: To describe a technique for the visualization and measurement of cerebral aqueduct diameter through a 2D sagittal median plane, and to report its aspect and measurement in fetuses with aqueductal stenosis (AS). METHODS: This was a cross-sectional study of 207 morphologically normal fetuses in low-risk pregnancies between 20 and 36 weeks of gestation. The cerebral aqueduct was visualized transvaginally in a midsagittal plane, and measurements of its greatest diameter (ampulla) were taken independently by an expert and a nonexpert sonographer. In addition, the aqueduct morphology from 7 fetuses with AS and complete follow-up were compared to the reference range. RESULTS: Aqueductal measurements were obtained in 206 of 207 normal fetuses. Aqueductal growth occurred linearly with gestational age. Our method demonstrated excellent interobserver reproducibility. Among the 7 fetuses with AS, the aqueductal lumen could not be identified in 6 and had a funneling aspect in 1. DISCUSSION: Our study demonstrated that it is possible to visualize and measure the cerebral aqueduct directly through a 2D ultrasound median plane. In fetuses with severe ventriculomegaly, the morphology and width of this structure could represent a relevant tool in improving AS diagnosis, differentiating it from other causes of significant ventricular dilation that carry a different outcome.

The value of early and comprehensive diagnoses in a human fetus with hydrocephalus and progressive obliteration of the aqueduct of Sylvius: Case Report.

BACKGROUND: Mutant rodent models have highlighted the importance of the ventricular ependymal cells and the subcommissural organ (a brain gland secreting glycoproteins into the cerebrospinal fluid) in the development of fetal onset hydrocephalus. Evidence indicates that communicating and non-communicating hydrocephalus can be two sequential phases of a single pathological phenomenon triggered by ependymal disruption and/or abnormal function of the subcommissural organ. We have hypothesized that a similar phenomenon may occur in human cases with fetal onset hydrocephalus. CASE PRESENTATION: We report here on a case of human fetal communicating hydrocephalus with no central nervous system abnormalities other than stenosis of the aqueduct of Sylvius (SA) that became non-communicating hydrocephalus during the first postnatal week due to obliteration of the cerebral aqueduct. The case was followed closely by a team of basic and clinic investigators allowing an early diagnosis and prediction of the evolving pathophysiology. This information prompted neurosurgeons to perform a third ventriculostomy at postnatal day 14. The fetus was monitored by ultrasound, computerized axial tomography and magnetic resonance imaging (MRI). After birth, the follow up was by MRI, electroencephalography and neurological and neurocognitive assessments. Cerebrospinal fluid (CSF) collected at surgery showed abnormalities in the subcommissural organ proteins and the membrane proteins L1-neural cell adhesion molecule and aquaporin-4. The neurological and neurocognitive assessments at 3 and 6 years of age showed neurological impairments (epilepsy and cognitive deficits). CONCLUSIONS: (1) In a hydrocephalic fetus, a stenosed SA can become obliterated at perinatal stages. (2) In the case reported, a close follow up of a communicating hydrocephalus detected in utero allowed a prompt postnatal surgery aiming to avoid as much brain damage as possible. (3) The clinical and pathological evolution of this patient supports the possibility that the progressive stenosis of the SA initiated during the embryonic period may have resulted from ependymal disruption of the cerebral aqueduct and dysfunction of the subcommissural organ. The analysis of subcommissural organ glycoproteins present in the CSF may be a valuable diagnostic tool for the pathogenesis of congenital hydrocephalus.

Hidrocefalia ligada al cromosoma X o síndrome de Bickers-Adams: presentación de un caso / X-linked hydrocephalus or Bickers-Adams syndrome: a case presentation

Introducción: la hidrocefalia es una enfermedad con transmisión recesiva ligada al cromosoma X, y una tasa de recurrencia del 50 por ciento en los fetos masculinos. Es la forma más común genética de hidrocefalia congénita, con una incidencia de 1 / 30, 000 nacimientos de varones. Se caracteriza por la dilatación de los ventrículos laterales y el retraso mental, suelen ser graves. Caso clínico: recién nacido que al momento de su nacimiento se observa con macrocefalia evidente con antecedente familiar de un primo con el mismo problema, con igual cuadro clínico y la misma neuroimagen por lo que se diagnosticó una hidrocefalia ligada al cromosoma X. Conclusiones: el caso que se presentó por sus características clínicas y el estudio de scanner cerebral se pudo llegar a un diagnóstico de hidrocefalia ligada al cromosoma X o llamado síndrome de Bickers-Adams.

Introduction: hydrocephalus (HL) is a recessive transmission disease linked to X- chromosome. It presents a 50 % recurrence rate in male fetuses. It is the commonest form of congenital hydrocephalus, with an incidence of 1/30 000 male births. It is characterized by dilation of lateral ventricles and mental retardation, usually severe. Clinical case: a newborn at birth is observed with evident macrocephaly with a family history of a cousin with the same problem. X-linked hydrocephalus (HLX) was diagnosed. Conclusions: a case of X- linked hydrocephalus or Bickers-Adams syndrome was presented, diagnosed by their clinical characteristics and brain scanner study.

Transtorno de aprendizagem e alteração cognitiva secundária a hidrocefalia por estenose do aqueduto de Sylvius: relato de caso / Learning disorder and cognitive impairment secondary to hydrocephalus due to stenosis of aqueduct of Sylvius: case report

JUSTIFICATIVA E OBJETIVOS: A estenose do aqueduto de Sylvius é responsável por um terço dos casos congênitos de hidrocefalia.A hipertensão intracraniana com consequente dilatação ventricular pode comprometer o desenvolvimento neuropsicomotor. Em crianças em idade escolar com alterações cognitivas ou comportamentais de início recente, sem causa aparente, este é um diagnóstico a ser pensado. Exames de imagem são fundamentais para a confirmação diagnóstica. Com tratamento adequado é esperada evolução favorável. O objetivo deste estudo foi relatar um caso de hidrocefalia por estenose do aqueduto de Sylvius como causa de transtorno de aprendizagem e alteração cognitiva e discutir seus aspectos clínicos, evolutivos e terapêuticos, ressaltando a importância da avaliação criteriosa de alterações neuropsiquiátricas em crianças em idade escolar. RELATO DO CASO: Paciente do sexo masculino, 10 anos, apresentando quadro progressivo de baixo rendimento escolar, déficit de concentração e compreensão, apatia e isolamento social há cinco meses. Após o diagnóstico de hidrocefalia por estenose do aqueduto de Sylvius foi submetido à cirurgia para derivação liquórica ventrículo peritoneal, evoluindo com melhora progressiva. CONCLUSÃO: Os efeitos da hidrocefalia em crianças podem comprometer o desenvolvimento cerebral, portanto, o diagnóstico precoce, a fim de instituir a terapêutica adequada, são essências para a reabilitação neuropsicomotora nesses pacientes.

BACKGROUND AND OBJECTIVES: Sylvius aqueduct stenosis accounts for one third of congenial hydrocephalus cases. Intracranial hypertension with subsequent ventricular dilatation may impair neuro-psychomotor development. In school-aged children with cognitive and/or behavioral alterations of recent onset, without apparent cause, this is a diagnosis to be considered. Imaging tests are of crucial importance for diagnostic confirmation. Favorable outcomes are expected with an effective treatment. The aim of this study was to report a case of hydrocephalus due to Sylvius aqueduct stenosis as a cause of learning disorders and cognitive impairment and to discuss its clinical, evolutionary and therapeutic aspects, emphasizing the importance of a careful assessment of neuropsychiatric alterations in school-aged children. CASE REPORT: A 10-year-old male patient that presented progressive history of poor academic performance, deficits of concentration and comprehension, apathy and social isolation for five months. The diagnosis was hydrocephalus due to Sylvius aqueduct stenosis; the patient underwent ventriculoperitoneal shunting surgery and there was significant improvement. CONCLUSION: The effects of hydrocephalus in children can impair brain development; therefore, early diagnosis to institute appropriate therapy is essential for neuro-psychomotor rehabilitationin these patients.