Overlap and Differences in Migraine and Idiopathic Intracranial Hypertension.

PURPOSE OF REVIEW: Migraine and idiopathic intracranial hypertension (IIH) are increasingly encountered but remain enigmatic. This review compares the similarities and differences of the diagnostic criteria, pathophysiology, and risk factors for chronic migraine and IIH. RECENT FINDINGS: While migraine and IIH are distinct diseases, both conditions are frequently found concurrently and may share a link. Increased intracranial pressure (ICP) in those with or without pre-existing migraine may present with migraine-like headaches and contribute to migraine chronification. Increased intracranial pressure may be a coincidental occurrence in patients with migraine and normalization of pressure does not always translate to headache improvement. Limited information is available regarding the standard of treatment for patients with chronic migraine and IIH without papilledema. There continues to be controversy over the normal range of cerebral spinal fluid (CSF) values. Recognizing the concurrence of both conditions advances our understanding of headache pathology and demonstrates a striking need for more research.

The role of Cushing's reflex and the vasopressin-mediated oligoanuric response to intracranial hypertension in patients with abdominal compartment syndrome.

BACKGROUND: We examined the link between increased intra-abdominal pressure, intracranial pressure, and vasopressin release as a potential mechanism. Intra-abdominal pressure, produced by abdominal-cavity insufflation with carbon dioxide (CO2) during laparoscopic abdominal procedures to facilitate visualization, is associated with various complications, including arterial hypertension and oliguria. METHODS: Mean arterial pressure, optic nerve sheath diameter, measured as a proxy for intracranial pressure, plasma vasopressin, serum and urine osmolarity, and urine output were measured 4 times during laparoscopic sleeve gastrectomy in 42 patients: before insufflation with CO2 (T0); after insufflation to 15 cm water (H2O) pressure, with 5 cm H2O positive end-expiratory pressure (T1); after positive end-expiratory pressure was raised to 10 cm H2O (T2); and after a return to the baseline state (T3). Mean values at T0 to T3 and the directional consistency of changes (increase/decrease/ unchanged) were compared among the 4 data-collection points. RESULTS: Statistically significant elevations (all P ≤ .001) were noted from T0 to T1 and from T0 to T2 in mean arterial pressure, optic nerve sheath diameter, and vasopressin, followed by decreases at T3. For optic nerve sheath diameter and vasopressin, the increases at T1 and T2 occurred in 98% and 100% of patients, ultimately exceeding normal levels in 88 and 97%, respectively. Conversely, urine output fell from T0 to T1 and T2 by 60.9 and 73.4%, decreasing in 88.1% of patients (all P < .001). Patients with class II obesity exhibited statistically greater increases in optic nerve sheath diameter and vasopressin, but statistically less impact on urine output, than patients with class III obesity. CONCLUSION: Increased mean arterial pressure, intracranial pressure, and vasopressin release appear to be intermediary steps between increased intra-abdominal pressure and oliguria. Further research is necessary to determine any causative links between these physiological changes.

Cerebrospinal fluid dynamics in idiopathic intracranial hypertension: a literature review and validation of contemporary findings.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a rare disease of unknown aetiology related possibly to disturbed cerebrospinal fluid (CSF) dynamics and characterised by elevated intracranial pressure (ICP) causing optic nerve atrophy if not timely treated. We studied CSF dynamics of the IIH patients based on the available literature and our well-defined cohort. METHOD: A literature review was performed from PubMed between 1980 and 2020 in compliance with the PRISMA guideline. Our study includes 59 patients with clinical, demographical, neuro-ophthalmological, radiological, outcome data, and lumbar CSF pressure measurements for suspicion of IIH; 39 patients had verified IIH while 20 patients did not according to Friedman's criteria, hence referred to as symptomatic controls. RESULTS: The literature review yielded 19 suitable studies; 452 IIH patients and 264 controls had undergone intraventricular or lumbar CSF pressure measurements. In our study, the mean CSF pressure, pulse amplitudes, power of respiratory waves (RESP), and the pressure constant (P0) were higher in IIH than symptomatic controls (p < 0.01). The mean CSF pressure was higher in IIH patients with psychiatric comorbidity than without (p < 0.05). In IIH patients without acetazolamide treatment, the RAP index and power of slow waves were also higher (p < 0.05). IIH patients with excess CSF around the optic nerves had lower relative pulse pressure coefficient (RPPC) and RESP than those without (p < 0.05). CONCLUSIONS: Our literature review revealed increased CSF pressure, resistance to CSF outflow and sagittal sinus pressure (SSP) as key findings in IIH. Our study confirmed significantly higher lumbar CSF pressure and increased CSF pressure waves and RAP index in IIH when excluding patients with acetazolamide treatment. In overall, the findings reflect decreased craniospinal compliance and potentially depleted cerebral autoregulation resulting from the increased CSF pressure in IIH. The increased slow waves in patients without acetazolamide may indicate issues in autoregulation, while increased P0 could reflect the increased SSP.

Prevalence of Incidentally Detected Signs of Intracranial Hypertension on Magnetic Resonance Imaging and Their Association With Papilledema.

Importance: Magnetic resonance imaging (MRI) signs of intracranial hypertension (IH) are traditionally associated with idiopathic intracranial hypertension (IIH), but these signs are also detected among individuals with primary headaches and among asymptomatic individuals without papilledema. Objective: To examine the prevalence of MRI signs of IH among consecutive outpatients undergoing brain MRI for any clinical indication and to explore their association with papilledema. Design, Setting, and Participants: This prospective cross-sectional study of outpatients undergoing brain MRI at 1 outpatient imaging facility was conducted between August 1, 2019, and March 31, 2020, with ocular fundus photographs taken concurrently. Radiographic images from consecutive adult patients who were undergoing brain MRI and able to participate in fundus photography were analyzed for MRI signs of IH. A univariate analysis using either Fisher exact tests or t tests was performed. Main Outcomes and Measures: Prevalence of MRI signs of IH and prevalence of papilledema detected on ocular fundus photographs. Radiographic signs of IH included empty sella, optic nerve head protrusion, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, enlarged Meckel caves, cephaloceles, cerebellar tonsillar descent, and bilateral transverse venous sinus stenosis. Results: A total of 388 patients were screened for eligibility; of those, 92 patients were excluded (58 declined participation, 16 were unable to consent, 14 were unable to complete fundus photography, and 4 completed MRI and fundus photography twice, so their second set of findings was removed). Among the 296 patients included in the study, the median age was 49.5 years (interquartile range, 37.8-62.0 years), and 188 patients (63.5%) were female. The most common indication for MRI was surveillance of a brain neoplasm (82 patients [27.7%]). Investigations of headaches (26 patients [8.8%]) and disorders of intracranial pressure (4 patients [1.4%]) were uncommon. At least 1 radiographic sign of IH was present in 145 patients (49.0%). Among 296 total study patients, 98 patients (33.1%) had empty sella, 47 patients (15.9%) had enlarged Meckel caves, 32 patients (10.8%) had increased perioptic cerebrospinal fluid, 23 patients (7.8%) had optic nerve tortuosity, 2 patients (0.7%) had scleral flattening, and 4 patients (1.4%) had cephaloceles. Bilateral transverse venous sinus stenosis was present in 6 of 198 patients (3.0%). Five patients (1.7%) had papilledema. Compared with patients without papilledema, those with papilledema had a significantly higher body mass index and history of IIH, in addition to an increased prevalence of empty sella, optic nerve tortuosity, and transverse venous sinus stenosis detected on MRI. The prevalence of papilledema increased from 2.8% among patients with at least 1 MRI sign of IH to 40.0% among patients with 4 or more MRI signs of IH. Conclusions and Relevance: Magnetic resonance imaging signs of IH were common among patients undergoing brain MRI in this study but rarely associated with papilledema. The management of patients with incidentally detected signs of IH likely does not require systematic lumbar puncture unless concerning symptoms or papilledema are present.

Ventriculoperitoneal Shunt for CNS Metastasis in Breast Cancer: Clinical Outcomes Based on Intrinsic Subtype.

BACKGROUND: Leptomeningeal metastasis (LM) is associated with a grave prognosis in breast cancer (BC) and can be controlled with a ventriculoperitoneal shunt (VPS). Information regarding LM and VPS based on intrinsic subtype is limited; thus, we investigated the clinical outcomes of BC treated with VPS. PATIENTS AND METHODS: The present retrospective study comprised 70 patients diagnosed with LM who received a VPS. The patients were divided into 4 groups based on BC subtype: hormone receptor (HR)+/human epidermal growth factor receptor 2 (HER2)-, HR+/HER2+, HR-/HER2+, and triple negative BC (TNBC). RESULTS: The most common indications for VPS were uncontrolled intracranial pressure (57.1%) and uncontrolled headache (55.7%), which improved in 54 (77.1%) of 70 patients after VPS. The median overall survival (OS) after brain or LM and overall survival after VPS were 7.6 and 2.3 months, respectively. Anti-HER2 treatment was a significant prognostic factor for better OS after brain or LM based on multivariate analysis (hazard ratio, 0.15; 95% confidence interval, 0.04-0.57; P = .005), whereas TNBC was correlated with shorter OS after central nervous system metastasis (hazard ratio, 2.82; 95% confidence interval, 1.46-5.48; P = .002). CONCLUSIONS: There were significant differences in clinical outcome based on the intrinsic subtype of patients with BC with LM who received a VPS. Anti-HER2 treatment in patients with HER2+ BC was associated with better survival in patients with metastatic BC with VPS insertion compared with those without. Survival of metastatic BC with VPS remained poor, especially in the TNBC subgroup.

Robot-Assisted Stereotactic Biopsies in 377 Consecutive Adult Patients with Supratentorial Diffuse Gliomas: Diagnostic Yield, Safety, and Postoperative Outcomes.

BACKGROUND: Multiple biopsy samples are warranted for the histomolecular diagnosis of diffuse gliomas in the current molecular era, which possibly increases morbidity. OBJECTIVE: We assessed diagnostic yield, safety, and risk factors of postoperative morbidity after robot-assisted serial stereotactic biopsy sampling along 1 biopsy trajectory for diffuse gliomas. METHODS: Observational retrospective analysis of consecutive magnetic resonance imaging-based robot-assisted stereotactic biopsies performed at a single institution to assess the diagnosis of nonresectable newly diagnosed supratentorial diffuse gliomas in adults (2006-2016). RESULTS: In 377 patients, 4.2 ± 1.9 biopsy samples were obtained at 2.6 ± 1.2 biopsy sites. The histopathologic diagnosis was obtained in 98.7% of cases. Preoperative neurologic deficit (P = 0.030), biopsy site hemorrhage ≥20 mm (P = 0.004), and increased mass effect on postoperative imaging (P = 0.014) were predictors of a new postoperative neurologic deficit (7.7%). Postoperative neurologic deficit (P < 0.001) and increased mass effect on postoperative imaging (P = 0.014) were predictors of a Karnofsky Performance Status decrease ≥20 points postoperatively (4.0%). Increased intracranial pressure preoperatively (P = 0.048) and volume of the contrast-enhanced area ≥13 cm3 (P = 0.048) were predictors of an increased mass effect on postoperative imaging (4.4%). Preoperative Karnofsky Performance Status <70 (P = 0.045) and increased mass effect on postoperative imaging (P < 0.001) were predictors of mortality 1 month postoperatively (2.9%). Preoperative neurologic deficit (P = 0.005), preoperative Karnofsky Performance Status <70 (P < 0.001), subventricular zone contact (P = 0.004), contrast enhancement (P = 0.018), and steroid use (P = 0.003), were predictors of the inability to discharge to home postoperatively (37.0%). CONCLUSIONS: Robot-assisted stereotactic biopsy sampling results in high diagnostic accuracy with low complication rates. Multiple biopsy sites and samples do not increase postoperative complications.

Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension.

BACKGROUND: The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. OBJECTIVE: To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. METHODS: Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. RESULTS: Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). CONCLUSION: Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby "treating" the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

Isolated intracranial hypertension associated with COVID-19.

BACKGROUND: Headache is a frequent complaint in COVID-19 patients. However, no detailed information on headache characteristics is provided in these reports. Our objective is to describe the characteristics of headache and the cerebrospinal fluid (CSF) profile in COVID-19 patients, highlighting the cases of isolated intracranial hypertension. METHODS: In this cross-sectional study, we selected COVID-19 patients who underwent lumbar puncture due to neurological complaints from April to May 2020. We reviewed clinical, imaging, and laboratory data of patients with refractory headache in the absence of other encephalitic or meningitic features. CSF opening pressures higher than 250 mmH2O were considered elevated, and from 200 to 250 mmH2O equivocal. RESULTS: Fifty-six COVID-19 patients underwent lumbar puncture for different neurological conditions. A new, persistent headache that prompted a CSF analysis was diagnosed in 13 (23.2%). The pain was throbbing, holocranial or bilateral in the majority of patients. All patients had normal CSF analysis and RT-qPCR for SARS-CoV-2 was negative in all samples. Opening pressure >200 mmH2O was present in 11 patients and, in six of these, > 250 mmH2O. 6/13 patients had complete improvement of the pain, five had partial improvement, and two were left with a daily persistent headache. CONCLUSIONS: In a significant proportion of COVID-19 patients, headache was associated to intracranial hypertension in the absence of meningitic or encephalitic features. Coagulopathy associated with COVID-19 could be an explanation, but further studies including post-mortem analysis of areas of production and CSF absorption (choroid plexuses and arachnoid granulations) are necessary to clarify this issue.

The incidence of obesity, venous sinus stenosis and cerebral hyperaemia in children referred for MRI to rule out idiopathic intracranial hypertension at a tertiary referral hospital: a 10 year review.

BACKGROUND: Children referred to a tertiary hospital for the indication, "rule out idiopathic intracranial hypertension (IIH)" may have an increased risk of raised venous sinus pressure. An increase in sinus pressure could be due to obesity, venous outflow stenosis or cerebral hyperemia. The purpose of this paper is to define the incidence of each of these variables in these children. METHODS: Following a data base review, 42 children between the ages of 3 and 15 years were found to have been referred over a 10 year period. The body mass index was assessed. The cross sectional areas and circumferences of the venous sinuses were measured at 4 levels to calculate the hydraulic and effective diameters. The arterial inflow, sagittal and straight sinus outflows were measured. Automatic cerebral volumetry allowed the brain volume and cerebral blood flow (CBF) to be calculated. The optic nerve sheath diameter was used as a surrogate marker of raised intracranial pressure (ICP). The sagittal sinus percentage venous return was used as a surrogate marker of elevated venous pressure. Age and sex matched control groups were used for comparison. RESULTS: Compared to controls, the obesity rates were not significantly different in this cohort. Compared to controls, those at risk for IIH had a 17% reduction in transverse sinus and 14% reduction in sigmoid sinus effective cross sectional area (p = 0.005 and 0.0009). Compared to controls, the patients at risk for IIH had an arterial inflow increased by 34% (p < 0.0001) with a 9% larger brain volume (p = 0.02) giving an increase in CBF of 22% (p = 0.005). The sagittal and straight sinus venous return were reduced by 11% and 4% respectively (p < 0.0001 and 0.0009) suggesting raised venous sinus pressure. Forty five percent of the patients were classified as hyperemic and these had optic nerve sheath diameters 17% larger than controls (p < 0.0002) suggesting raised ICP. CONCLUSION: In children with the chronic headache/ IIH spectrum, the highest associations were with cerebral hyperemia and mild venous sinus stenosis. Obesity was not significantly different in this cohort. There is evidence to suggest hyperemia increases the venous sinus pressure and ICP.

The incidence of significant venous sinus stenosis and cerebral hyperemia in childhood hydrocephalus: prognostic value with regards to differentiating active from compensated disease.

BACKGROUND: Symptomatic or active hydrocephalus in children is linked to an elevation in intracranial pressure (ICP), which is likely to be multifactorial in origin. The CSF outflow resistance, venous sinus resistance and total cerebral blood flow are likely factors in the ICP elevation. The purpose of this paper is to define the incidence, site and significance of venous sinus stenosis and/or cerebral hyperemia in a cohort of children diagnosed with hydrocephalus at a tertiary referral hospital. METHODS: The imaging database was reviewed over a 10 year period and the index MRI of all children between the ages of 4 months and 15 years, who were diagnosed with treatment naive hydrocephalus of any type (excluding secondary to tumor) and had magnetic resonance venography (MRV) and flow quantification were selected. Patients were compared with children undergoing an MRI with MRV and flow quantification who were subsequently shown to have no abnormality. The cross-sectional area and circumference of the sinuses were measured at 4 levels. The hydraulic and effective diameters were calculated. An area stenosis of 65% or greater was deemed significant. A total cerebral blood flow greater than two standard deviations above the mean for controls was taken to be abnormal. RESULTS: There were a total of 55 children with hydrocephalus compared to 118 age matched control MRV's and 35 control flow quantification studies. A high grade stenosis occurred in 56% of patients but in none of the controls (p < 0.0001). The commonest site of narrowing was in the distal sigmoid sinus. Cerebral hyperemia occurred in 13% of patients but did not occur in the controls. CONCLUSIONS: The elevation in ICP in symptomatic hydrocephalus is multifactorial. Both high grade venous stenosis and cerebral hyperemia are common in childhood hydrocephalus. High grade stenosis was noted to be a risk factor for conservative management failure. Hyperemia was a good prognostic indicator.

Fulminant Idiopathic Intracranial Hypertension.

PURPOSE OF REVIEW: The goal of this review is to describe the presenting features of fulminant idiopathic intracranial hypertension (IIH) and outline the multimodal approach to its treatment. RECENT FINDINGS: Venous sinus stenting may be an appropriate alternative to optic nerve sheath fenestration or cerebrospinal fluid shunting in select patients with fulminant IIH. Prompt surgical intervention maximizes the chance of visual recovery in patients with fulminant IIH. "Fulminant IIH" is defined as intracranial hypertension with no secondary cause, severe vision loss within 4 weeks of symptom onset, and progressive vision loss over days. Rapid recognition of the fulminant phenotype of IIH by emergency department physicians, neurologists, and ophthalmologists is critical. Without appropriate triage and rapid medical and surgical intervention, patients with fulminant IIH are at high risk for profound, permanent vision loss. Prompt surgical intervention with optic nerve sheath fenestration, cerebrospinal fluid shunting, or venous sinus stenting minimizes the chance of poor visual outcome. If a delay is anticipated, serial lumbar punctures or temporary cerebrospinal fluid drainage and medical therapy may forestall irreversible vision loss.

Impact of Obesity and Obstructive Sleep Apnea in Lateral Skull Base Cerebrospinal Fluid Leak Repair.

OBJECTIVE: To investigate the prevalence and impact of obstructive sleep apnea (OSA) and obesity in lateral skull base cerebrospinal fluid leak repair (LSBR) of various etiologies. METHODS: Retrospective case review at a tertiary skull base center was conducted of consecutive adults undergoing LSBR via transmastoid, middle cranial fossa, or combined approach between 2013-2018. The following data were collected: demographics, comorbidities, radiology and intraoperative findings, and surgical outcomes including complications and need for revision surgery or shunt placement. Patients with incomplete data or leaks following skull base surgery, trauma or chronic ear disease were excluded. RESULTS: Ninety-four patients (67.4% female, mean age 53.5 ± 12.9 years) underwent repair for spontaneous (sCSFL, 44%) and other etiology (nsCSFL) leaks. nsCSFL served as a comparison group consisting of leaks status-post lateral skull base surgery, temporal bone fractures, and chronic ear disease. Class III obesity (P = .02), OSA (P = .03), and imaging findings of empty sella (OR = 3.32, P = .02), and skull base thinning including contralateral tegmen thinning (31%, OR = 4.3, P = .02), arachnoid granulations (26%, OR = 4.35, P = .02), and superior canal dehiscence (15.8%, OR = 8.57, P = .04) were more common in sCSFL. Four patients (4.2%) required surgical revision for recurrence, and another four (4.2%) resolved with shunting. Evidence of elevated intracranial hypertension was present in nine patients with sCSF leaks and was predictive of need for revision or shunt procedures (P < .01). CONCLUSION: Obesity, OSA, and imaging consistent with elevated intracranial pressures were more common among patients with sCSFL. Elevated intracranial pressure predicted outcomes following multilayer repair of spontaneous CSF leaks LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2234-2240, 2020.

Intracranial Electroencephalography in Pediatric Severe Traumatic Brain Injury.

OBJECTIVES: Electroencephalography is used in neurocritical care for detection of seizures and assessment of cortical function. Due to limited resolution from scalp electroencephalography, important abnormalities may not be readily detectable. We aimed to identify whether intracranial electroencephalography allows for improved methods of monitoring cortical function in children with severe traumatic brain injury. DESIGN: This is a retrospective cohort study from a prospectively collected clinical database. We investigated the occurrence rate of epileptiform abnormalities detected on intracranial electroencephalography when compared with scalp electroencephalography. We also investigated the strength of association of quantitative electroencephalographic parameters and cerebral perfusion pressure between both intracranial and scalp electroencephalography. SETTING: This is a single-institution study performed in the Phoenix Children's Hospital PICU. PATIENTS: Eleven children with severe traumatic brain injury requiring invasive neuromonitoring underwent implantation of a six-contact intracranial electrode as well as continuous surface electroencephalography. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Visual detection of epileptiform abnormalities was performed by pediatric epileptologists. Association of intracranial and scalp electroencephalography total power, alpha percentage, and alpha-delta power ratio to cerebral perfusion pressure was performed using univariate dynamic structural equations modeling. Demographic data were assessed by retrospective analysis. Intracranial and scalp electroencephalography was performed in 11 children. Three of 11 children had observed epileptiform abnormalities on intracranial electroencephalography. Two patients had epileptiform abnormalities identified exclusively on intracranial electroencephalography, and one patient had seizures initiating on intracranial electroencephalography before arising on scalp electroencephalography. Identification of epileptiform abnormalities was associated with subsequent identification of stroke or malignant cerebral edema. We observed statistically significant positive associations between intracranial alpha-delta power ratio to cerebral perfusion pressure in nine of 11 patients with increased strength of association on intracranial compared with scalp recordings. CONCLUSIONS: These findings suggest that intracranial electroencephalography may be useful for detection of secondary insult development in children with traumatic brain injury.

Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction.

PURPOSE: Delayed intracranial hypertension (DIH) occurs most frequently in children with syndromic or multi-suture synostosis after surgical correction. The rarity of DIH in children with isolated non-syndromic sagittal synostosis (ISS) warrants follow-up evaluation by large craniofacial centers until skeletal maturity. This study reports the incidence of DIH in children following open repair for ISS by our center's craniofacial team. METHODS: A single-center retrospective study of patients who underwent open calvarial vault remodeling (CVR) for ISS at our institution between November 2000 and November 2012 was performed. Syndromic and multi-suture synostosis patients were excluded. Demographic and follow-up data were extracted from the medical record for analysis until July 2017. RESULTS: One hundred five patients with ISS were identified who had undergone CVR in the aforementioned timeframe. Average age at initial surgery was 11.7 ± 15.32 months. Mean follow-up in our craniofacial clinic was 4.94 ± 3.53 years, with 69 patients (65.7%) having follow-up in craniofacial clinic ≥ 3 years and 74 (70.5%) having follow-up ≥ 3 years in any clinic at our institution. Four patients (3.8%) had intracranial pressure (ICP) monitors placed for symptoms concerning for DIH, one of which (0.95%) had confirmed DIH and underwent a second surgical procedure at 7.4 years of age. The patient presented late initially, having his first operation at 1.56 years of age. CONCLUSION: One patient out of 105 (0.95%) developed DIH, confirmed by ICP monitoring, and required reoperation. The occurrence of DIH, albeit rare, remains an important topic to include in parental discussions and mandates long-term follow-up in this population.

Events associated with the occurrence of intracranial hypertension in pediatric patients with severe cranioencephalic trauma and monitoring of intracranial pressure / Eventos associados à ocorrência de hipertensão intracraniana em pacientes pediátricos com traumatismo cranioencefálico grave e monitoração da pressão intracraniana

ABSTRACT Objective: To determine the events associated with the occurrence of intracranial hypertension (ICH) in pediatric patients with severe cranioencephalic trauma. Methods: This was a prospective cohort study of patients 18 years old and younger with cranioencephalic trauma, scores below nine on the Glasgow Coma Scale, and intracranial pressure monitoring. They were admitted between September, 2005 and March, 2014 into a Pediatric Intensive Care Unit. ICH was defined as an episode of intracranial pressure above 20 mmHg for more than five minutes that needed treatment. Results: A total of 198 children and adolescents were included in the study, of which 70.2% were males and there was a median age of nine years old. ICH occurred in 135 (68.2%) patients and maximum intracranial pressure was 36.3 mmHg, with a median of 34 mmHg. A total of 133 (97.8%) patients with ICH received sedation and analgesia for treatment of the condition, 108 (79.4%) received neuromuscular blockers, 7 (5.2%) had cerebrospinal fluid drainage, 105 (77.2%) received mannitol, 96 (70.6%) received hyperventilation, 64 (47.1%) received 3% saline solution, 20 (14.7%) received barbiturates, and 43 (31.9%) underwent a decompressive craniectomy. The events associated with the occurrence of ICH were tomographic findings at the time of admission of diffuse or hemispheric swelling (edema plus engorgement). The odds ratio for ICH in patients with Marshall III (diffuse swelling) tomography was 14 (95%CI 2.8-113; p<0.003), and for those with Marshall IV (hemispherical swelling) was 24.9 (95%CI 2.4-676, p<0.018). Mortality was 22.2%. Conclusions: Pediatric patients with severe cranioencephalic trauma and tomographic alterations of Marshall III and IV presented a high chance of developing ICH.

RESUMO Objetivo: Determinar eventos associados à ocorrência de hipertensão intracraniana (HIC) em pacientes pediátricos com traumatismo cranioencefálico grave. Métodos: Trata-se de coorte prospectiva de pacientes de até 18 anos, com traumatismo cranioencefálico, pontuação abaixo de nove na Escala de Coma de Glasgow e monitoração da pressão intracraniana, admitidos entre setembro de 2005 e março de 2014 em unidade de terapia intensiva pediátrica. A HIC foi definida como episódio de pressão intracraniana acima de 20 mmHg por mais de cinco minutos e com necessidade de tratamento. Resultados: Incluídas 198 crianças e adolescentes, 70,2% masculinos, mediana de idade de nove anos. A HIC ocorreu em 135 (68,2%) pacientes; valor máximo de pressão intracraniana de 36,3; mediana 34 mmHg. Receberam sedação e analgesia para tratamento da HIC 133 (97,8%) pacientes, 108 (79,4%) receberam bloqueadores neuromusculares, 7 (5,2%) drenagem de líquor, 105 (77,2%) manitol, 96 (70,6%) hiperventilação, 64 (47,1%) solução salina a 3%, 20 (14,7%) barbitúricos e 43 (31,9%) foram submetidos à craniectomia descompressiva. Os eventos associados à ocorrência de HIC foram os achados tomográficos à admissão de swelling (edema mais ingurgitamento) difuso ou hemisférico. A razão de chance para que pacientes com classificação tomográfica Marshall III (swelling difuso) apresentassem HIC foi 14 (IC95% 2,8-113; p<0,003) e para aqueles com Marshall IV (hemisférico) foi 24,9 (IC95% 2,4-676; p<0,018). A mortalidade foi de 22,2%. Conclusões: Pacientes pediátricos com traumatismo cranioencefálico grave e alterações tomográficas tipo Marshall III e IV apresentaram grande chance de desenvolver HIC.

Surgical intervention in pediatric intracranial hypertension: incidence, risk factors, and visual outcomes.

PURPOSE: To evaluate the incidence of surgical intervention in pediatric intracranial hypertension (IH), evaluate the visual outcomes of surgically managed patients, and identify potential predictors for surgical intervention. METHODS: The medical records of patients with primary and secondary IH at Nationwide Children's Hospital from 2010 to 2017 were reviewed retrospectively. Presenting characteristics of medically and surgically managed patients were compared, and the clinical courses of surgically managed patients were reviewed. RESULTS: A total of 129 medically and 14 surgically managed patients were included. The surgical incidence was 9.8%. Final visual acuity in 27 of 28 surgically managed eyes was 20/25 or better. In combined primary and secondary IH patients, elevations in body mass index (BMI; OR = 1.06; 95% CI, 1.01-1.11; P = 0.022) and lumbar puncture opening pressures ≥52 cm H2O (OR = 6.17; 95% CI, 1.93-19.67; P = 0.002) were significantly associated with the likelihood of surgical intervention when assessed by univariate logistic regression; grade of papilledema >2 was of marginal significance. After controlling for BMI, a lumbar puncture opening pressure of ≥52 cm H2O was more likely to result in surgery (adjusted OR = 4.69; 95% CI = 1.39-15.98; P = 0.013). CONCLUSIONS: Most pediatric IH can be treated medically. Patients with lumbar puncture opening pressures ≥52 cm H2O at the time of diagnosis are at a higher risk of surgical intervention and should be monitored closely. Elevations in presenting BMI and grade of papilledema may also increase the odds of surgery.

Dural sinus collapsibility, idiopathic intracranial hypertension, and the pathogenesis of chronic migraine.

Available evidences suggest that a number of known assumption on idiopathic intracranial hypertension (IIH) with or without papilledema might be discussed. These include (1) the primary pathogenetic role of an excessive dural sinus collapsibility in IIH, allowing a new relatively stable intracranial fluids pressure balance at higher values; (2) the non-mandatory role of papilledema for a definite diagnosis; (3) the possibly much higher prevalence of IIH without papilledema than currently considered; (4) the crucial role of the cerebral compliance exhaustion that precede the raise in intracranial pressure and that may already be pathologic in cases showing a moderately elevated opening pressure; (5) the role as "intracranial pressure sensor" played by the trigeminovascular innervation of dural sinuses and cortical bridge veins, which could represent a major source of CGRP and may explain the high comorbidity and the emerging causative link between IIHWOP and chronic migraine (CM). Accordingly, the control of intracranial pressure is to be considered a promising new therapeutic target in CM.

Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study.

BACKGROUND: Certain headache characteristics and associated symptoms are commonly attributed to increased intracranial pressure, but they have not been systematically studied among children in the context of revised diagnostic criteria for pseudotumor cerebri syndrome (PTCS). METHODS: We performed a retrospective cohort study of patients treated for suspected or confirmed PTCS. Charts were reviewed for PTCS and headache diagnostic criteria and associated characteristics. Chi-squared or Fisher's exact tests were used to compare the frequency of headache characteristics between groups. RESULTS: One hundred and twenty-seven individuals were identified: 61 had definite PTCS, 10 had probable PTCS, 31 had elevated opening pressure (OP) without papilledema, and 25 had normal OP without papilledema. Eleven children had no headache (6 with definite PTCS, 5 with probable PTCS). Headache pattern was episodic in 49% (95% CI: 34-64%) of those with definite PTCS, 18% (95% CI 6-37%) of those with elevated OP without papilledema, and 16% (5-36%) of those with normal OP without papilledema. Headache location was more likely to involve the head along with neck or shoulders in those with definite PTCS compared with elevated OP without papilledema (OR = 7.2, 95% CI: 1.9-27.6) and normal OP (OR = 4.5, 95% CI: 1.3-15.6) groups. DISCUSSION: While missing data and small cohort size are limitations, this study suggests that headache in PTCS is more likely to involve the head along with neck/shoulders, and that headache in PTCS may be episodic or constant. Headache is occasionally absent in PTCS.

Clinical outcome and prognostic analysis of meningeal carcinomatosis treated by intrathecal chemotherapy.

OBJECTIVE: To evaluate the clinical efficacy of meningeal carcinomatosis (MC) treated by intrathecal chemotherapy (IC) and analyzes the prognostic factors. METHODS: The clinical and follow-up data of 33 MC patients was collected and retrospectively analyzed. Potential prognostic factors such as age, gender, primary cancer, transfer time, presence of brain metastasis, intracranial pressure, protein, glucose, vascular endothelial growth factor (VEGF), eastern cooperative oncology group (ECOG) performance status (PS), systemic treatment and IC were discussed. RESULTS: The most common clinical manifestation of MC was high intracranial pressure (30 cases). The negative rate of cerebrospinal fluid cytology was 50% after MC patients treated by IC. The remission rate of IC and no-IC group was 71.4% and 15.7%, respectively, of which the stability rate was 92.8% and 57.8% with significant difference. The median survival time was 5.200 months (95% CI 0.000-11.491) and the survival rate of 3, 6, 12 months were respectively 71.6%, 49.2%, 30.7%. CONCLUSION: IC can relieve the symptoms of MC and extend life expectancy. Early treatment can improve prognosis. Presence of brain metastasis, ECOG PS and IC were correlated with prognosis of patients, whereas IC is an independent prognostic factor.