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Objective:To explore the influencing factors of adult spontaneous meningoencephalocele, which occurs in the lateral recess of sphenoid sinus, in order to improve the level of clinical diagnosis and treatment. Methods:The clinical data of 27 adults with spontaneous meningoencephalocele in lateral recess of sphenoid sinus in Department of the Otorhinolaryngology, the First Affiliated Hospital of Zhengzhou University from January 2017 to December 2022 were retrospectively analyzed. Preoperative sinus CT and MRI were performed to confirm the diagnosis and location of meningoencephalocele. Results:â There were 0 cases of lateral recess of sphenoid sinus type â , 8 cases of lateral recess of sphenoid sinus type â ¡ and 19 cases of lateral recess of sphenoid sinus type â ¢. â¡Among the 27 adult patients with spontaneous meningoencephalocele, 9 were male and 18 were female, and the onset age was 19-72 years old, with an average age ofï¼50.7±12.4ï¼ years old. 18 cases were complicated with cerebrospinal fluid leakage, 11 cases with headache and dizziness, 3 cases with recurrent meningitisï¼complicated with cerebrospinal fluid leakageï¼, and 2 cases with epilepsy. â¢There were 20 patients with intracranial hypertension, 17 patients with body mass indexï¼BMIï¼ ≥25 kg/m², and 8 patients with empty sella. Conclusion:Type â ¢ of lateral recess of sphenoid sinus is the most common type in adult spontaneous meningoencephalocele, and intracranial hypertension and obesity are the influencing factors of this disease. Puncture, biopsy or operation should not be performed for patients suspected of spontaneous meningoencephalocele, and imaging examination should be performed to identify the source of the tumor.
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Hipertensión Intracraneal , Seno Esfenoidal , Adulto , Humanos , Masculino , Femenino , Persona de Mediana Edad , Adulto Joven , Anciano , Seno Esfenoidal/patología , Estudios Retrospectivos , Encefalocele/diagnóstico , Encefalocele/cirugía , Encefalocele/patología , Pérdida de Líquido Cefalorraquídeo , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/patología , Hipertensión Intracraneal/cirugíaRESUMEN
PURPOSE: This study aimed to investigate the efficacy of occipital emissary vein (OEV) detection in the diagnosis of idiopathic intracranial hypertension (IHH) in the pediatric age group, and to compare the prevalence and luminal diameter of OEV in patients with IHH and in healthy control subjects. METHODS: Conventional magnetic resonance imaging findings were assessed in the patients with IHH and in healthy control subjects who were under the age of 18, by two observers. The presence and luminal dimension of OEV and transverse sinus stenosis were also evaluated and compared between these two groups with magnetic resonance venography techniques. RESULTS: The rate of OEV existence was 7 times higher in the IIH group compared to the control group based on the second observer outcome (p = 0.010, OR = 7.0), with a very good interobserver agreement (Æ = 0.85). The dimension of OEV ranged between 0.6 and 2.5 mm. There was no correlation found between the opening pressure and the dimension of OEV (p = 0.834). CONCLUSION: In conclusion, OEV existence could be an additional radiological finding for diagnosing IHH among pediatric patients, alongside other conventional findings.
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Venas Cerebrales , Hipertensión Intracraneal , Seudotumor Cerebral , Senos Transversos , Humanos , Niño , Seudotumor Cerebral/patología , Imagen por Resonancia Magnética/métodos , Venas Cerebrales/patología , Cráneo , Hipertensión Intracraneal/patologíaRESUMEN
INTRODUCTION: Glioblastoma (GBM) is a tumor with rapid growth and a possible relationship to elevated intracranial pressure (ICP). High ICP may not always be associated with clinical signs. A non-invasive technique for assessment of ICP is measuring the optic nerve sheath diameter (ONSD). Identifying patients who need immediate intervention is of importance in neuro-oncological care. The goal of this study is to assess the available magnetic resonance imaging (MRI) of patients with GBM with respect to pre- and postoperative ONSD. METHODS AND MATERIALS: Retrospective data analysis was performed on all patients operated for GBM at a tertiary care center between 2010 and 2020. Two pre and one postoperative MRI had to be available. Clinical data and ONSD at multiple time points were analyzed and correlated, as well as preoperative volumetrics. RESULTS: Sixty-seven patients met the inclusion criteria. Clinical signs of elevated ICP were seen in 25.4% (n = 17), while significant perifocal edema was present in 67.2% (n = 45) of patients. Clinical signs of preoperatively elevated ICP were associated with significantly elevated ONSD at diagnosis (p < 0.001) as well as preoperative tumor volume (p < 0.001). Significant perifocal edema at the time of diagnosis was associated with elevated ONSD (p = 0.029) and higher tumor volume (p = 0.003). In patients with significant edema, ONSD increased significantly between preoperative MRIs (p = 0.003/005). In patients with clinical signs of raised ICP, ONSD also increased, whereas it was stable in asymptomatic patients (yes: 5.01+/-4.17 to 5.83+/-0.55 mm, p = 0.010, no: 5.17+/-0.46 mm to 5.38+/-0.41 mm, p = 0.81). A significant increase of ONSD from diagnosis to preoperative MRI and a significant decrease until 3 months postoperatively were observed (p < 0.001). CONCLUSIONS: ONSD might help identify high ICP in patients with GBM. In this first-of-its kind study, we observed a significant increase of ONSD preoperatively, likely associated with edema. Postoperatively, ONSD decreased significantly until 3 months after surgery and increased again at 12 months. Further prospective data collection is warranted.
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Glioblastoma , Glioma , Hipertensión Intracraneal , Humanos , Estudios Retrospectivos , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Presión Intracraneal/fisiología , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Glioma/patología , Glioblastoma/patología , Edema/patología , Ultrasonografía/métodosRESUMEN
OBJECTIVE: Optic nerve sheath(ONS) enhancement in idiopathic intracranial hypertension (IIH) patients has been reported in recent years. In this retrospective observation, we analyzed the clinical characteristics of IIH patients with enhancement of ONS. METHODS: Eighty-two patients with clinically diagnosed IIH from January 2017 to December 2019 were under observation. Then, based on the presence of contrast-enhancement (CE) in ONS on orbital magnetic resonance image (MRI), the IIH patients were divided into CE-ONS group and no-CE(NCE)-ONS group. Six months follow-up information was also included in the observation study. By comparing clinical data of the two groups of IIH patients, we tried to evaluate whether there is clinical heterogeneity in CE-ONS patients. RESULT: 12 patients were included in CE-ONS group, 10 females and 2 males. 70 patients were included in NCE-ONS group, 56 women and 14 men. We found that patients with CE-ONS had a longer course of disease (median disease duration before diagnosis, 5 months vs. 3months, P<0.01) and more likely had the sign of distension of the perioptic subarachnoid space (DPSS) (58.33 % vs. 24.29 %, P = 0.034). But no significant differences were found in demographic characteristics, clinical symptoms, degree of visual impairment, papilledema, opening pressure(OP) on lumbar puncture and clinical outcomes. CONCLUSION: As a rare sign on MRI, ONS enhancement can occur in patients with IIH. IIH patients with CE-ONS may have a longer course of disease and more prone to DPSS, but there is no significant difference in clinical manifestations, OP, and clinical outcomes compared with IIH patients without CE-ONS.
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Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Femenino , Humanos , Masculino , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/patología , Imagen por Resonancia Magnética , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Papiledema/diagnóstico por imagen , Seudotumor Cerebral/diagnóstico por imagen , Estudios RetrospectivosRESUMEN
BACKGROUND: Normal optic nerve diameter (OND) and optic nerve sheath diameter (ONSD) may be beneficial for describing optic nerve pathway abnormality reflecting increased intracranial pressure. Nonetheless, magnetic resonance imaging (MRI) measurement of the normal ONSD range and its associations with clinical factors and eyeball transverse diameter (ETD) are not well established in children. PURPOSE: To establish normal OND, ONSD, ETD, and OND/ONSD and ONSD/ETD measurements in children and their associations with age and sex. MATERIAL AND METHODS: We evaluated and analyzed 336 brain MRI studies of children aged 0.5 months to 18 years. We measured a total of 672 optic nerves. The OND and ONSD were measured 1â cm anterior to the optic foramina and 3â mm behind the optic disc on an axial T2 sequence. RESULTS: The mean OND 3â mm and 1â cm, ONSD 3â mm and 1â cm, and ETD were 0.23 ± 0.05â mm and 0.16 ± 0.04â mm, 0.53 ± 0.08â mm and 0.38 ± 0.06â mm, and 2.3 ± 0.13, respectively. Only ONSD 1â cm was independent of age (P = 0.247). ONSD 3â mm and ETD were significantly wider in boys compared to girls and significantly influenced by age (both P < 0.001). Age at scan and ETD were significantly correlated (P < 0.001). CONCLUSION: We established MRI-based OND, ONSD, ETD, and ONSD/ETD and OND/ONSD ratio normative values in children, which can be helpful in pediatric populations with disease.
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Hipertensión Intracraneal , Presión Intracraneal , Masculino , Femenino , Humanos , Niño , Presión Intracraneal/fisiología , Nervio Óptico/diagnóstico por imagen , Hipertensión Intracraneal/patología , Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética , UltrasonografíaRESUMEN
BACKGROUND: Caudal injections commonly used for neuraxial anaesthesia in children can displace cerebrospinal fluid cranially causing safety concerns in terms of raised intracranial pressure. Optic nerve sheath diameter (ONSD) is a noninvasive surrogate for the measurement of intracranial pressure. Regional cerebral oximetry (CrSO 2 ) can monitor brain oxygenation, which may decrease by a reduction in cerebral flow due to increased intracranial pressure. OBJECTIVES: Comparing how caudal injection volumes of 0.8 and 1.25âmlâkg -1 influence ONSD and CrSO 2 within the first 30âmin after injection. DESIGN: Prospective, randomised and parallel group trial. SETTING: Operating room. PATIENTS: Fifty-eight elective paediatric surgical patients between ages 1 and 7 years old, ASA class I or II, without previous intracranial or ocular pathology and surgery appropriate for single - shot caudal anaesthesia. INTERVENTION: Single-shot caudal anaesthesia with 0.8âmlâkg -1 (group L, n â=â29) and 1.25âmlâkg -1 (group H, n â=â29) of 2âmgâkg -1 bupivacaine solution. MAIN OUTCOME MEASURES: Optic nerve sheath diameter measured with ultrasonography and regional cerebral oximetry measured by near - infrared spectroscopy before (NIRS), immediately after, 10, 20 and 30âmin after the block. RESULTS: Mean ONSD values increased from a baseline of 4.4â±â0.2âmm to a maximum of 4.5â±â0.2âmm 20âmin after injection in group L and from a baseline of 4.5â±â0.3âmm to a maximum of 4.8â±â0.3âmm 10âmin after injection in group H. Eight of 29 patients in group H and none in group L had an ONSD increase by more than 10%. Both groups had a reduction of less than 2.5% in CrSO 2 . CONCLUSION: Caudal injection with 1.25âmlâkg -1 increased ONSD, an indirect measurement of ICP, more than 0.8âmlâkg -1 and neither volume caused a clinically important reduction in CrSO 2 . TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04491032.
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Circulación Cerebrovascular , Hipertensión Intracraneal , Humanos , Niño , Lactante , Preescolar , Estudios Prospectivos , Oximetría , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Ultrasonografía/métodos , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patologíaRESUMEN
BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the performance of magnetic resonance imaging (MRI) in measuring the optic nerve sheath diameter (ONSD) compared to the established method transorbital sonography (TOS) in patients with idiopathic intracranial hypertension (IIH). METHODS: Twenty-three patients with IIH were prospectively included applying IIH diagnostic criteria. All patients received a lumbar puncture with assessment of the cerebrospinal fluid (CSF) opening pressure to assure the IIH diagnosis. Measurement of ONSD was performed 3 mm posterior to inner sclera surface in B-TOS by an expert examiner, while three independent neuroradiologists took measurements in axial T-weighted MRI examinations. The sella turcica with the pituitary gland (and potential presence of an empty sella) and the trigeminal cavity were also assessed on sagittal and transversal T1-weighted MRI images by one independent neuroradiologist. RESULTS: The means of ONSD between ultrasound and MRI measurements were 6.3 mm (standard deviation [SD] = 0.6 mm) and 6.2 mm (SD = 0.8 mm). The interrater reliability between three neuroradiologists showed a high interclass correlation coefficient (ICC) (confidence interval: .573 < ICC < .8; p < .001). In patients with an empty sella, the ONSD evaluated by MRI was 6.6 mm, while measuring 6.1 mm in patients without empty sella. No correlation between CSF opening pressure and ONSD was found. CONCLUSIONS: MRI can reliably measure ONSD and yields similar results compared to TOS in patients with IIH. Moreover, patients with empty sella showed significantly larger ONSD than patients without empty sella.
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Hipertensión Intracraneal , Seudotumor Cerebral , Humanos , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/patología , Reproducibilidad de los Resultados , Nervio Óptico/diagnóstico por imagen , Ultrasonografía , Presión Intracraneal , Imagen por Resonancia Magnética , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/patologíaRESUMEN
BACKGROUND: Emergency departments (EDs) are typically the first medical contact for seizure patients, and early diagnosis and treatment is primarily the responsibility of emergency physicians. OBJECTIVES: Demonstrate the efficacy of bedside ocular ultrasonography for optic nerve sheath diameter (ONSD) measurement in differentiating provoked seizure from unprovoked seizure in the ED. DESIGN: Prospective observational study SETTINGS: Tertiary care hospital PATIENTS AND METHODS: Patients presenting to the ED with seizure were divided into two groups according to medical history, physical examination, laboratory results, cranial computed tomography findings and electroencephalography results. Patients with seizures that did not have a specific cause (unprovoked) were compared with patients who had seizures caused by underlying pathology (provoked). The measurement of the ONSD was taken at the bedside within 30 minutes of arrival. The study compared the ONSD values, age, sex, type of seizure, and Glasgow Coma Score between the two groups. MAIN OUTCOME MEASURE: Efficacy of ONSD to distinguish between provoked and unprovoked seizures. SAMPLE SIZE: 210 patients RESULTS: One hundred and fourteen (54.3%) patients were in the provoked seizure group and 96 (45.7%) were in the unprovoked seizure group. The ONSD measurements were significantly higher in the provoked seizure group compared with the unprovoked seizure group (median 6.1 mm vs. 5.2 mm, P<.001). The cut-off value of ONSD higher than 5.61 was significantly associated with the prediction of the provoked seizure (P<.001). The area under the curve value was 0.882 (95% CI: 0.830-0.922) with a sensitivity of 86.5 and specificity of 78.9%. CONCLUSIONS: Bedside ONSD measurement by means of ocular ultrasound is an effective method for differentiating provoked seizure from unprovoked seizure. LIMITATIONS: Statistical significance of age on ONSD and exclusion of pediatric patients. CONFLICT OF INTEREST: None.
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Hipertensión Intracraneal , Humanos , Niño , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Convulsiones/diagnóstico por imagen , Convulsiones/complicaciones , Servicio de Urgencia en Hospital , Ojo , UltrasonografíaRESUMEN
PURPOSE: The optic nerve (ON) is an extension of the central nervous system via the optic canal to the orbital cavity. It is accompanied by meninges whose arachnoid layer is in continuity with that of the chiasmatic cistern. This arachnoid layer is extended along the ON, delimiting a subarachnoid space (SAS) around the ON. Not all forms of chronic intracranial hypertension (ICH) present papilledema. The latter is sometimes asymmetric, unilateral, or absent. The radiological signs of optic nerve sheath (ONS) dilation, in magnetic resonance imaging, are inconsistent or difficult to interpret. The objective of this study was to analyze the anatomy, the constitution, and the variability of the SAS around the ON in its intraorbital segment to improve the understanding of the pathophysiologic mechanism of asymmetric or unilateral or absent papilledema in certain ICH. METHODS: The study was carried out on nine cadaveric specimens. In four embalmed specimens, macroscopic analysis of the SAS of the ONS were performed, with description about density of the arachnoid trabecular meshwork in three distinct areas (bulbar segment, mid-orbital segment and the precanal segment). In three other embalmed specimens, after staining of SAS by methylene blue (MB), we performed macroscopic analysis of MB progression in the SAS of the ONS. Then, in two non-embalmed specimens, light and electron microscopy (EM) analysis were also done. RESULTS: On the macroscopic level, after staining of SAS, we found in all cases that MB progressed on 16 mm average throughout the SAS of the ONS without reaching the papilla. In four embalmed specimens, in the SAS of the ONS, the density of the arachnoid trabecular meshwork showed inter-individual variability (100%) and intra-individual variability with bilateral variability (50%) and/or variability within the same ONS (88%). On the microscopic level, the arachnoid trabeculae of the ONS are composed of dense connective tissue. The EM perfectly depicted its composition which is mainly of collagen fibers of parallel orientation. CONCLUSION: The variability of the SAS around the ONS probably impacts the symmetrical or asymmetrical nature of papilledema in ICH.
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Hipertensión Intracraneal , Papiledema , Electrones , Humanos , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Microscopía Electrónica , Nervio Óptico/anatomía & histología , Nervio Óptico/diagnóstico por imagen , Papiledema/patología , Espacio Subaracnoideo/anatomía & histología , Espacio Subaracnoideo/diagnóstico por imagenRESUMEN
OBJECTIVES: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure. We investigated the optic nerve, Meckel's cavity, internal carotid artery (ICA) and pituitary findings of IIH by Cranial Magnetic Resonance Imaging (MRI). METHODS: Cranial MRI images of 35 adult patients with IIH and 35 adult subjects with normal cranial MRI results (control) were evaluated. Optic nerve diameter (OND), optic nerve sheat diameter (ONSD), OND/ONSD ratio, ON tortuosity, ON protrusion, posterior scleral flattening, ICA transverse diameter, CSF distance in Meckel's cavity and ICA transverse diameter/CSF distance in Meckel's cavity ratio, and pituitary gland measurements (height and transverse dimension; and Optic chiasm- pituitary gland distance) were measured. RESULTS: OND and ONSD of the IIH group were significantly higher than those of the control groups at anterior and posterior measurements (p < 0.05). OND/ONSD ratio of the IIH group was lower at anterior measurement; and higher at the posterior measurement than the control group (p < 0.05). Right ICA transverse diameter and bilateral CSF distance in Meckel's cavity of the IIH group were higher than those of the control. Optic chiasm- pituitary gland distance of the IIH group was significantly higher than that of the control group (p < 0.05). CONCLUSION: OND/ONSD ratio is different in anterior and posterior measurements. So we recommend measuring OND and ONSD separately in IIH patients to use in the clinical practice. Similarly, optic chiasm-pituitary gland distance is also another point to note for IIH patients on MRI. Our paper adds new approach to IIH in terms of OND/ONSD ratio.
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Hipertensión Intracraneal , Seudotumor Cerebral , Adulto , Humanos , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/patología , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/patología , Nervio Óptico/diagnóstico por imagen , Cráneo , Imagen por Resonancia Magnética/métodosRESUMEN
INTRODUCTION: Central nervous system xanthogranulomas are uncommon clinical entities, and symptomatic bilateral choroid plexus xanthogranulomas are rare. CASE PRESENTATION: We present the case of a 15-year-old male patient with bilateral choroid plexus xanthogranulomas with symptoms of increased intracranial pressure. Gross total resection of the tumor in the left lateral ventricle was performed. The patient improved, and asymptomatic right-sided tumor was monitored at follow-up. CONCLUSION: The main treatment objective in these tumors is gross total resection. Asymptomatic tumors can be followed without intervention. However, surgery should be performed for symptomatic tumors that cause hydrocephalus or symptoms of increased intracranial pressure.
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Neoplasias del Plexo Coroideo , Hidrocefalia , Hipertensión Intracraneal , Xantomatosis , Adolescente , Niño , Plexo Coroideo/diagnóstico por imagen , Plexo Coroideo/patología , Plexo Coroideo/cirugía , Neoplasias del Plexo Coroideo/diagnóstico por imagen , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/cirugía , Granuloma/diagnóstico por imagen , Granuloma/patología , Granuloma/cirugía , Humanos , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/etiología , Hidrocefalia/cirugía , Hipertensión Intracraneal/patología , Ventrículos Laterales/patología , Masculino , Xantomatosis/complicaciones , Xantomatosis/diagnóstico por imagen , Xantomatosis/cirugíaRESUMEN
ABSTRACT: This study aimed to determine pain characteristics in patients with persistent headache after COVID-19 and to investigate the role of increased intracranial pressure (ICP) in the pathogenesis of this headache. This is a case-control study comparing the parameters and measurements indicating increased ICP based on magnetic resonance imaging between COVID-19-diagnosed patients with persistent headache and a control group. Optic nerve sheath diameter (ONSD) and eyeball transverse diameter (ETD) were performed on the left eye of each participant. Seventeen of the patients (53.12%) met the diagnostic criteria for new daily persistent headache. Seven patients (21.87%) had migraine, and eight (25%) had tension headache characteristics. No significant difference was observed between the patient and control groups in terms of the ONSD and ETD values. It is possible that the etiopathogenesis is multifactorial. We consider that future studies that will evaluate ICP measurements in large patient groups can present a different perspective for this subject.
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COVID-19/complicaciones , Cefalea/etiología , Hipertensión Intracraneal/patología , Hipertensión Intracraneal/virología , Presión Intracraneal , Adulto , Estudios de Casos y Controles , Ojo/patología , Femenino , Humanos , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/fisiopatología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Nervio Óptico/patología , SARS-CoV-2 , Adulto JovenRESUMEN
This article introduces the basic concepts of intracranial physiology and pressure dynamics. It also includes discussion of signs and symptoms and examination and radiographic findings of patients with acute cerebral herniation as a result of increased as well as decreased intracranial pressure. Current best practices regarding medical and surgical treatments and approaches to management of intracranial hypertension as well as future directions are reviewed. Lastly, there is discussion of some of the implications of critical medical illness (sepsis, liver failure, and renal failure) and treatments thereof on causation or worsening of cerebral edema, intracranial hypertension, and cerebral herniation.
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Encefalocele , Hipertensión Intracraneal , Encefalocele/diagnóstico , Encefalocele/etiología , Encefalocele/patología , Humanos , Hipertensión Intracraneal/complicaciones , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/patologíaRESUMEN
Although the central canal is an integral component of the cerebral ventricular system, central canal dilation has not been examined adequately during the progression of subarachnoid hemorrhage-related hydrocephalus (SAH-H). Central canal dilation-associated ependymal cell desquamation or subependymal membrane rupture has been rarely reported. Herein, we try to describe possible mechanisms of central canal dilation "Hydromyelia," developing after SAH. A total of 25 New Zealand hybrid female rabbits were recruited. Five served as controls, and five received sham operations. In the remaining animals (n = 15), 0.5 mL/kg of autologous blood was injected into the cisterna magna twice on 0 and 2nd days. Five of these animals died within a few days. A total of 10 survivor animals decapitated 3 weeks later, and the brains and cervical spinal cords were histologically examined. Central canal volumes, ependymal cell numbers on the canal surfaces, and the Evans' indices of the ventricles were compared. On histological examination, central canal occlusion with desquamated ependymal cells and basement membrane rupture were evident. The mean Evans' index of the brain ventricles was 0.31, the mean central canal volume was 1.054 mm3, and the normal ependymal cell density was 4.210/mm2 in control animals; the respective values were 0.34, 1.287 mm3, and 3.602/mm2 for sham-operated animals, and 0.41, 1.776 mm3, and 2.923/mm2 in the study group. The differences were statistically significant (p < 0.05). Hydromyelia, an ignored complication of SAH-H, features ependymal cell desquamation, subependymal basement membrane destruction, blood cell accumulation on the subependymal cell basement membrane, and increased CSF pressure. Hydromyelia may be a significant complication following SAH.
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Encéfalo/patología , Modelos Animales de Enfermedad , Hidrocefalia/patología , Médula Espinal/patología , Hemorragia Subaracnoidea/patología , Animales , Ventrículos Cerebrales/patología , Cisterna Magna/patología , Femenino , Hidrocefalia/etiología , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Conejos , Hemorragia Subaracnoidea/complicacionesAsunto(s)
Lesiones Traumáticas del Encéfalo/complicaciones , Costo de Enfermedad , Diagnóstico por Imagen/métodos , Imagenología Tridimensional/métodos , Hipertensión Intracraneal/complicaciones , Hipertensión Intracraneal/diagnóstico por imagen , Lesiones Traumáticas del Encéfalo/patología , Estudios de Cohortes , Humanos , Hipertensión Intracraneal/patología , Reino UnidoRESUMEN
BACKGROUND: ERF-related craniosynostosis are a rare, complex, premature trisutural fusion associated with a broad spectrum of clinical features and heterogeneous aetiology. Here we describe two cases with the same pathogenic variant and a detailed description of their clinical course. CASE PRESENTATION: Two subjects; a boy with a BLSS requiring repeated skull expansions and his mother who had been operated once for sagittal synostosis. Both developed intracranial hypertension at some point during the course, which was for both verified by formal invasive intracranial pressure monitoring. Exome sequencing revealed a pathogenic truncating frame shift variant in the ERF gene. CONCLUSIONS: Here we describe a boy and his mother with different craniosynostosis patterns, but both with verified intracranial hypertension and heterozygosity for a truncating variant of ERF c.1201_1202delAA (p.Lys401Glufs*10). Our work provides supplementary evidence in support of previous phenotypic descriptions of ERF-related craniosynostosis, particularly late presentation, an evolving synostotic pattern and variable expressivity even among affected family members.
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Craneosinostosis/genética , Predisposición Genética a la Enfermedad , Hipertensión Intracraneal/genética , Proteínas Represoras/genética , Adulto , Craneosinostosis/complicaciones , Craneosinostosis/patología , Craneosinostosis/cirugía , Femenino , Heterocigoto , Humanos , Lactante , Hipertensión Intracraneal/complicaciones , Hipertensión Intracraneal/patología , Hipertensión Intracraneal/cirugía , Masculino , Madres , Cráneo/patología , Cráneo/cirugíaRESUMEN
BACKGROUND: Senior-Løken syndrome (SLS) is a rare autosomal recessive disease characterised by nephronophthisis and retinal degeneration, and belongs to a group of genetically heterogeneous disorders known as the ciliopathies. MATERIALS AND METHODS: Case report of a patient with genetically proven SLS presenting with headaches and swollen optic nerve heads, review of medical notes and ophthalmic imaging, with retinal photography, fundus autofluorescence, and OCT retinal nerve fibre layer analysis. RESULTS: We present findings in a 15 year old girl with Senior-Løken syndrome associated with compound heterozygous mutations in the SDCCAG8 gene, who initially presented with a retinal dystrophy, and subsequent renal failure requiring renal transplantation and immunosuppression. Four and a half years later, she presented with headaches, reduced vision and clinical findings of papilloedema. Cerebrospinal fluid analysis revealed a high opening pressure of 37cmH20 and neuroimaging was otherwise unremarkable. Treatment with a reduced dose of oral acetazolamide resulted in symptomatic relief of headaches, and resolution of optic nerve swelling. CONCLUSION: The association of intracranial hypertension in a ciliopathy is a rare occurrence. The aetiology of intracranial hypertension in this case is likely multi-factorial, due to renal transplantation, post-renal transplant medications and/ or weight gain. With evidence of cilia involvement in the central nervous system, ciliary dysfunction may contribute to intracranial hypertension, and should be considered in these patients presenting with headaches. Diagnosis may be difficult with advanced retinal degeneration and baseline retinal nerve fibre layer thinning. Treatment requires careful monitoring of renal function.
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Ciliopatías/patología , Hipertensión Intracraneal/patología , Enfermedades Renales Quísticas/patología , Amaurosis Congénita de Leber/patología , Atrofias Ópticas Hereditarias/patología , Adolescente , Ciliopatías/complicaciones , Femenino , Humanos , Hipertensión Intracraneal/complicaciones , Enfermedades Renales Quísticas/complicaciones , Amaurosis Congénita de Leber/complicaciones , Atrofias Ópticas Hereditarias/complicaciones , PronósticoRESUMEN
Transorbital sonography provides reliable information about the estimation of intra-cranial pressure by measuring the optic nerve sheath diameter (ONSD), whereas the optic nerve (ON) diameter (OND) may reveal ON atrophy in patients with multiple sclerosis. Here, an AUTomatic Optic Nerve MeAsurement (AUTONoMA) system for OND and ONSD assessment in ultrasound B-mode images based on deformable models is presented. The automated measurements were compared with manual ones obtained by two operators, with no significant differences. AUTONoMA correctly segmented the ON and its sheath in 71 out of 75 images. The mean error compared with the expert operator was 0.06 ± 0.52 mm and 0.06 ± 0.35 mm for the ONSD and OND, respectively. The agreement between operators and AUTONoMA was good and a positive correlation was found between the readers and the algorithm with errors comparable with the inter-operator variability. The AUTONoMA system may allow for standardization of OND and ONSD measurements, reducing manual evaluation variability.
Asunto(s)
Procesamiento de Imagen Asistido por Computador/métodos , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Ultrasonografía/métodos , Algoritmos , Humanos , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/patología , Presión Intracraneal , Esclerosis Múltiple/diagnóstico por imagen , Atrofia Óptica/diagnóstico por imagen , Reproducibilidad de los ResultadosRESUMEN
Objective: To investigate the frequency of neuroimaging findings of intracranial hypertension in patients with idiopathic intracranial hypertension (IIH) and cerebral venous thrombosis (CVT). We also aimed to compare these findings in two patient groups and investigate the association of these findings with some clinical parameters.Methods: Patients with IIH and CVT admitted to the neurology, neuro-ophthalmology outpatient clinics, and emergency services between 2017 and 2019 were enrolled in this study. The presence of eight previously defined neuroimaging signs of intracranial hypertension were evaluated on MRIs. The values of total MRI scores were formed to use in the analyses investigating the association between neuroimaging findings and clinical parameters.Results: Twenty-three patients with proven IIH and seven patients with CVT with signs of intracranial hypertension were included in this study. Body mass index (BMI) values were higher in the IIH group than in the CVT group (31.0/25.9; p = 0.022). There were no differences in terms of gender distribution, age, ortotal MRI scores between the patient groups. The most common neuroimaging parameters found in the IIH group were the following: increased peri-optic cerebrospinal fluid (CSF) (n = 20) and optic nerve tortuosity (n = 12). There were no significant correlations between values of total MRI scores, lumbar puncture opening pressure, and BMI.Conclusion: Neuroimaging findings are useful tools with high diagnostic sensitivity in IIH. The most sensitive finding was increased peri-optic CSF. The utility of these neuroimaging correlates and their potential significance as solely diagnostic markers in IIH should be investigated in future large-scale studies.
Asunto(s)
Hipertensión Intracraneal/diagnóstico por imagen , Trombosis Intracraneal/diagnóstico por imagen , Trombosis de la Vena/diagnóstico por imagen , Adolescente , Adulto , Anciano , Femenino , Humanos , Hipertensión Intracraneal/complicaciones , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/patología , Trombosis Intracraneal/complicaciones , Trombosis Intracraneal/diagnóstico , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neuroimagen/estadística & datos numéricos , Nervio Óptico/patología , Trombosis de la Vena/complicaciones , Trombosis de la Vena/diagnóstico , Adulto JovenRESUMEN
OBJECTIVES: The objectives of this study were to test: (1) the repeatability of ultrasonographic examination of the optic nerve sheath diameter (ONSD) in the cat; (2) the association between the ONSD and age, sex and body weight in healthy cats; and (3) the difference in the ONSD between healthy cats and those suffering from presumed intracranial hypertension (ICH). METHODS: This study had a prospective, blinded, observational cross-sectional study design. Two groups of animals were considered: healthy cats (group A) and cats with a diagnosis of presumed ICH (group B). The ONSD was evaluated, measured and compared between the two groups via an ultrasonographic transpalpebral approach. Repeatability of the procedure was evaluated through the intraclass correlation coefficient (ICC). Data were statistically compared using the Student's t-test and linear regression analysis. RESULTS: A strong inter- and intraobserver ICC indicating good repeatability was observed. The interobserver ICC was 0.965 (P <0.05) for the right eye and 0.956 (P <0.05) for the left eye. The intraobserver ICC was 0.988 (P <0.05) and 0.984 (P <0.05) for the right and left eyes, respectively. In healthy cats the mean ± SD ONSD was 1.23 ± 0.11 mm (range 1-1.47 mm) and 1.23 ± 0.10 (range 1-1.4 mm) for right and left eyes, respectively. The ONSD was not related to sex or weight; a weak relationship was observed with age. In group B, the mean ONSD was 1.68 ± 0.13 mm (range 1.5-1.9 mm) and 1.61 ± 0.15 mm (range 1.4-1.9 mm) for the right and left eyes, respectively. In group B, the ONSD was statistically significantly larger than in group A, the healthy cats (P <0.001). CONCLUSIONS AND RELEVANCE: The transpalpebral ultrasonographic technique is a non-invasive, feasible and reproducible method to measure ONSD both in healthy cats and in cats suffering from suspected ICH.