Pediatric Pseudotumor Cerebri Research Articles

Systemic steroids in pediatric pseudotumor cerebri - A case report

Study design: Case report. Purpose: To study the efficacy of systemic steroids in pediatric pseudotumor cerebri. Introduction: Pseudotumor cerebri is a condition caused by elevated intracranial pressure presenting most commonly with headache. It is a diagnosis of exclusion. Methods: A 15-year-old girl presented in our outpatient department with a headache for 3 months, more in the posterior aspect, continuous type with severe intensity, non-radiating. On examination, the best corrected visual acuity in her right eye was 6/9, and her left eye was 6/6 parts. She had a normal anterior segment and normal Intraocular Pressure (IOP). Fundoscopy showed both optic discs edematous, pink in color with blurry disc margins, tortuous disc vessels, and edematous peripapillary area. The rest of the retina was normal. Lumbar puncture showed elevated opening CSF pressure and normal CSF composition. Hence she was diagnosed with IIH. She was started on oral prednisolone 1mg/kgbw once a day dosage, oral acetazolamide 250 mg once a day, topical nepafenac 0.1% one drop thrice a day and timolol 0.5% eye drops twice a day. Discussion: The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision. Steroids were commonly used for treating IIH in the past, but cause significant long-term side effects, such as weight gain, that are undesirable in IIH patients. Furthermore, withdrawal of steroids can cause rebound intracranial hypertension. Thus, steroids should not be used routinely for IIH treatment. Results: By the end of one month, the patient was relieved of symptoms, best corrected visual acuity was 6/6 in both eyes with normal IOP, normal optic disc in both eyes. Conclusion: Steroids are an effective way of managing idiopathic intracranial hypertension in pediatric patients.

Enhancing Diagnostic Accuracy Through Neuroimaging Revisions in Pediatric Pseudotumor Cerebri Syndrome: A Cross-Sectional Study

BackgroundThis cross-sectional study aimed to report all neuroimaging findings suggestive of raised intracranial pressure in children with pseudotumor cerebri syndrome (PTCS), before and after re-review by two neuroradiologists. MethodsWe included 48 children aged <18 years diagnosed with PTCS between 2016 and 2021. Clinical and radiological data were obtained from their medical files. Two neuroradiologists independently re-reviewed all neuroimages, and the average of their assessments was compared with the initial neuroimaging reports; an additional review was done to analyze inter- and intraclass correlation. ResultsThe initial neuroimaging reports showed under-reporting of findings, with only 26 of 48 (54.1%) patients identified with abnormal reports. After revision, the proportion of the reported findings increased to 44 of 48 (91.6%). Distention of the perioptic space was the most commonly reported finding after revision (36.5 of 48; 76%). Flattening of the posterior globe and empty sella were initially under-reported but improved after revision. Moreover, several findings suggestive of increased intracranial pressure not mandated by Friedman criteria were identified, such as narrowing of the Meckel cave, posterior displacement of the pituitary stalk, and narrowing of the cavernous sinus. Analysis of associations between neuroimaging findings and demographic and clinical characteristics yielded no statistically significant results. The inter- and intraclass correlation results demonstrated a significant agreement between raters and within each rater's assessment (P < 0.05). ConclusionsThis study highlights the impact of image revision in enhancing PTCS diagnosis. Intra- and interclass correlations underscore the reliability of the review process, emphasizing the importance of meticulous image analysis in clinical practice.

Pediatric Pseudotumor Cerebri Syndrome Secondary to Superior Sagittal Sinus Thrombosis Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection and Brief Literature Review

Pediatric Pseudotumor Cerebri Syndrome Secondary to Superior Sagittal Sinus Thrombosis Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection and Brief Literature Review

High CCL2 Levels Detected in CSF of Patients with Pediatric Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri (PTC) is a disorder characterized by increased intracranial pressure in the absence of a structural lesion or other identifiable cause. Cytokines, which are involved in the regulation of immune responses and inflammation, have been implicated in the pathogenesis of PTC. In a prospective, cross-sectional study at three centers in Israel, we analyzed cerebrospinal fluid (CSF) samples from 60 children aged 0.5-18 years, including 43 children with a definitive diagnosis of PTC and a control group of 17 children. Levels of IL-4, IL-10, IL-17, CCL2, CCL7, CCL8, CCL13, BDNF, and IFN-γ were measured using ELISA kits. Levels of CCL2 were significantly higher in the PTC group compared to the control group (p < 0.05), with no other significant differences in the measured cytokines between the two groups. The groups did not differ significantly in clinical presentation, imaging, treatment, or ophthalmic findings. Our findings provide preliminary evidence that CCL2 may be involved in the pathogenesis of PTC and may serve a potential target for therapy in PTC.

Clinical and prognostic importance of craniospinal elastance and pressure volume index in pediatric pseudotumor cerebri syndrome.

The aim of the study was to determine the clinical and prognostic significance of craniospinal elastance (Ecs), pressure volume index (PVI), and cerebrospinal fluid (CSF) total protein data in terms of resolution of papilledema and improvement of headache in children with pseudotumor cerebri syndrome (PTCS). This is a retrospective observational study of 33 children with definitive PTCS. The relationships between lumbar puncture (LP) measurements, especially Ecs and PVI, and papilledema and headache resolution time were investigated. In children with definite primary and secondary PTCS, higher opening pressure and decreased PVI were found to be associated with faster resolution of papilledema and faster improvement of headache (r = 0.904, P < 0.01; r = 0.894, P < 0.01 respectively). This effect was higher in children with secondary PTCS (P = 0.022). While papilledema resolution time and treatment time were statistically significantly higher in the secondary group (P = 0.035, P = 0.040), there was no significant difference between the two groups in terms of headache relief time (P = 0.051). Based on the primary and secondary groups, from the cut-off points determined, it was found that ≤ 41.60 ((AUC = 0.706, P = 0.0420), specificity 56%, sensitivity 86.7%) for opening pressure and ≤ 69.37 ((AUC = 0.702, P = 0.0448), specificity 48.2%, sensitivity 83.3%) for PVI were statistically significant. A very strong negative linear correlation was found between CSF total protein and lumbar puncture measurements and clinical outcomes (P < 0.001). Ecs and PVI may be markers that can provide potentially important data on pediatric PTCS prognosis. CSF total protein may also provide clinical benefit.

Increase in Pediatric Pseudotumor Cerebri Syndrome Emergency Department Visits, Inpatient Admissions, and Surgeries During the COVID-19 Pandemic.

Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It is unknown whether the increase in childhood obesity during the COVID-19 pandemic has affected the rates and characteristics of pediatric PTCS. We conducted a retrospective study of children evaluated for PTCS (inpatient or emergency department) at our children's hospital before (March 19, 2015 to March 19, 2020) and during (March 20, 2020 to February 20, 2021) the pandemic. We compared the monthly number of inpatient and emergency department encounters for pediatric PTCS before and during the pandemic. In addition, anthropometric and ophthalmologic characteristics of children evaluated for pediatric PTCS before and during the pandemic were compared. A total of 36 encounters in the 5 years before the pandemic and 26 encounters in the 11 months during the pandemic were identified. The median monthly number of encounters for pediatric PTCS was significantly higher during the pandemic compared with the 5 years before the pandemic (2 vs 0, P = 0.0021). Compared with prepandemic patients, children evaluated during the pandemic were older (median age 16 vs 14 years, P = 0.02), with higher rates of obesity (85% vs 66%, P = 0.05) and lower likelihood of reporting Caucasian race (4% vs 31%, P = 0.02). Pandemic patients had worse presenting visual acuity (median logMAR 0.14 vs 0.05, P = 0.05) and were more likely to have fulminant presentation (23% vs 6%, P = 0.04) and require surgical intervention (23% vs 6%, P = 0.04). At our children's hospital, the rate of inpatient admissions and emergency department visits for pediatric PTCS increased during the pandemic. The severity of disease and frequency of surgical treatment also increased. Racial and ethnic minorities seem to be disproportionately affected. These changes may be related to increasing rates of childhood obesity during the pandemic.

Etiological Factors in Pediatric Pseudotumor Cerebri Cases

Objective: Pseudotumor cerebri (PTC) is the presence of the signs and symptoms of intracranial pressure increase in the absence of a tumoral, structural or vascular mass-occupying lesion. This study aimed to investigate the etiological factors involved in childhood PTC cases.Methods: Data of the patients presented to the Private Medical Park Samsun Hospital, pediatric neurology outpatient clinic with the preliminary diagnosis of PTC between June 2015 and December 2020 were retrospectively reviewed. Demographic data, presentation symptoms, neuro-ophthalmological examination findings, laboratory data, and radiological imaging (brain magnetic resonance imaging, magnetic resonance venography) findings. The female (i.e., Group 1) and male (i.e., Group 2) children were compared concerning the etiological factors.Results: The study cohort included 45 patients. There were 24 patients in Group 1 (i.e., female children) and 21 patients in Group 2 (i.e., male children). Among the 45 patients, 41 (91.1%) had an overt etiological factor. Mean age of the study patients were 9.06 year [2,5 months-18 years]. The most common symptom was headache (64.4%). Two groups were similar concerning mean patient age, pubertal stage, and rates of vitamin B12 deficiency, iron deficiency anemia, vitamin D deficiency, abnormal brain magnetic resonance imaging, and magnetic resonance venography findings. However, the obesity rate was significantly higher in Group 2 than Group 1 (32,7% vs. 12,5%, p&amp;lt;0.05). Conclusion: A thorough investigation concerning etiology is crucial in pediatric PTC cases regardless of patient gender. Timely investigations regarding etiology will lead to timely treatment of this clinical condition and prevent irreversible devastating complications such as permanent hearing loss.

Assessment of Pediatric Pseudotumor Cerebri Clinical Characteristics and Outcomes.

Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs in both children and adults, the pseudotumor cerebri literature is heavily dominated by adult studies. The aim of this study is to retrospectively describe the clinical presentation, imaging, treatment, and outcomes of a large pediatric pseudotumor cerebri population over a 23-year period. We also discuss secondary pseudotumor cerebri (44%) as well as the increasingly recognized patient subgroups without headache (13.3%) and without papilledema (7.3%). Female sex, obesity, and initial symptoms were consistent with the literature; however radiographic findings were surprisingly low in this cohort. Headache outcomes at 1 week, 1 month, and 3 months following initial lumbar puncture/treatment and visual function outcomes are reported.

Prevalence of Adrenal Insufficiency and Glucocorticoid Use in Pediatric Pseudotumor Cerebri Syndrome.

The pathophysiology underlying pseudotumor cerebri syndrome (PTCS) is complex and not well understood. There are clear differences between PTCS in adults and pediatrics. Few and isolated case reports have suggested that adrenal function may be involved, yet no large cohort study has examined this relationship. We conducted a retrospective single-center study of children who presented with a diagnosis of PTCS and had cortisol testing measured between January 2010 and September 2019. We included all subjects meeting the revised PTCS diagnostic criteria after the chart review. Based on morning, random or 1-μg cosyntropin stimulated cortisol levels, adrenal functioning was classified as: (1) insufficient (peak cortisol <16 μg/dL and AM cortisol <5 μg/dL), (2) at risk (peak cortisol 16-20 μg/dL, AM cortisol 5-13 μg/dL, or random <13 μg/dL), or (3) sufficient (peak cortisol >20 μg/dL and AM or random cortisol >13 μg/dL). A total of 398 individuals were reviewed, and 64 were included for analysis. Of these, 40.6% were men, of mixed race and ethnicity with a mean age of 10.5 (SD 4.7) years. Of these, 23% and 52% had insufficient or at-risk cortisol levels. The majority of those in the insufficient (70%) or at-risk (80%) groups were exposed to topical, nasal, or inhaled glucocorticoids but not systemic. Only 60% and 12% of those with PTCS with insufficient or at-risk cortisol testing, respectively, underwent definitive testing with a stimulation test. Glucocorticoid use and hypocortisolism are prevalent in PTCS and need consideration as a potential underlying cause. Most children had insufficient or at-risk cortisol levels, and many did not undergo further testing/workup. Children who present with PTCS, particularly young, males should be evaluated for adrenal insufficiency and its risk factors, including nonsystemic steroids. Prospective studies are necessary to further evaluate the effect of cortisol in relation to pediatric PTCS.

Pediatric Pseudotumor Cerebri: Epidemiological Features.

Objective: The purpose was to study the epidemiological and clinical features of Pediatric Pseudotumor Cerebri; we especially evaluated age, sex, sexual maturity, weight, symptoms, duration of the episode, visual acuity, correlation with systemic diseases or drugs. Methods: We investigated a population of 40 children (80 eyes), aged between 2 and 18 years, that referred to our Centre (Città della Salute e della Scienza di Torino - presidio Ospedale Regina Margherita) from 2000 to 2018. All the patients were monitored with visual field exam, fundus exam and Frisen classification, pupillary reflexes, best corrected visual acuity, orthoptic exam. Then, the demographic characteristics, BMI (body max index) and weight changes, the correlation with systemic diseases or drugs, the Relative Afferent Pupillary Defect (RAPD), the VI cranial nerve deficit, the time required for the diagnosis, the modality of onset, the correlation between diagnosis delay and best corrected visual acuity, the resolution time and the probability of recurrence were studied. Results: We observed 2 age peaks in our population, one consisting of adolescents (aged between 11 and 14 years) and one consisting of younger children aged between 4 and 7 years, with a statistically significant correlation between age and BMI. The deficit of the VI cranial nerve and the diplopia were resolved within the first month, despite the longer time of resolution of the papilledema. Headache was the main onset symptom, but after reclassifying onset symptoms per weight classes, in overweight children, we found an increased variability of symptoms (dizziness, postural instability, photophobia, nausea, vomit, strabism and diplopia, visual fogging, drowsiness, irritability). Moreover, we found only one totally asymptomatic case, during a routine visit, in a 6 years old overweight child with Down syndrome. Conclusion: The Pediatric Pseudotumor Cerebri is, even nowadays, a little known pathology. To provide a correct diagnostic assessment, it will be imperative to recollect the epidemiological features of this disease.

Update in Pediatric Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences between prepubertal and postpubertal children, further elucidating the complex pathophysiology. Management requires brain imaging, lumbar puncture, and frequent eye exams with medical and rarely surgical treatment. Visual outcomes in children are favorable if caught early and management can be prolonged over years. Pediatric PTCS is different from adult PTCS in many ways, and this review will focus on the most updated definitions of the disease, theories of pathophysiology, management, and treatment in the pediatric population.

Magnetic Resonance Imaging Findings in Pediatric Pseudotumor Cerebri Syndrome

BackgroundRevised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children. MethodsAs part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis. ResultsThe presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%). ConclusionsIn children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.

Demographics, clinical features, and response to conventional treatments in pediatric Pseudotumor Cerebri syndrome: a single-center experience.

The goal of this study was to better understand pediatric Pseudotumor Cerebri syndrome, and its relationship to age, obesity, and other medical conditions; and to evaluate response to conventional treatments. A retrospective chart review was performed on consecutive patients who were diagnosed with PTCS between January 1, 2007, and July 31, 2014. A total of 78 patients were included in this study: 54 female (69.3%) and 24 male (30.7%). Variables including age, sex, body mass index, concomitant medical conditions, secondary causes, associated symptoms, physical exam findings, imaging results, recurrence of symptoms, and treatment modalities were analyzed. Patients were grouped into "pre-kindergarten," "elementary," and "adolescent" based on their age; and weight categories of underweight, normal weight, overweight, moderately, and severely obese. Mean age of symptom onset was 11.92 ± 4.09years. Elementary and adolescent age patients were more likely to be overweight, moderately obese, and severely obese, while this finding was not found for patients in pre-kindergarten group. Headache (83.3%) and visual disturbances (48.7%) were the most common presenting complaints. Asthma (16.6%) was the most common associated concomitant medical condition. Medical management resulted in resolution in 84% of population, 15% required surgical interventions, and the recurrence rate was found to be 20.5%. There was a statistically significant trend in success with medical management in younger patients (p= 0.04), while medically refractory PTCS was seen in adolescent females. Recurrence of PTCS had a linear trend with increased occurrence in adolescent age group with higher BMI. Asthma was observed to be frequently associated with PTCS in our cohort. Obesity is strongly associated with PTCS, not only in the adolescent group but also in the younger elementary age group. Treatment remains similar to management in the adults with a good response (84%) to medical management and a low relapse rate.

Clinical and Prognostic Significance of Cerebrospinal Fluid Opening and Closing Pressures in Pediatric Pseudotumor Cerebri Syndrome

BackgroundThe purpose of this study was to determine the prognostic utility of closing pressure and volume of cerebrospinal fluid removed with respect to papilledema resolution and headache improvement in pediatric pseudotumor cerebri syndrome. MethodsThis is a retrospective observational study of 93 children with definite pseudotumor cerebri syndrome. The primary outcome measure was time to resolution of papilledema, and the secondary outcome measure was time to resolution of headache. ResultsThere were no significant differences in gender, age, or body mass index z score observed between subjects with (N = 35) and without (N = 58) documented closing pressure. The median time to resolution of papilledema was not statistically different between children above or equal to and those below the median closing pressure (170 mm of cerebrospinal fluid, n = 31, P = 0.391) or the volume of median cerebrospinal fluid removed (16 mL, n = 19, P = 0.155). There was no statistically significant difference detected in days of headache between the children with opening pressure above and equal to the median (400 mm of cerebrospinal fluid) and the children with opening pressure below the median (n = 44, P = 0.634). ConclusionsNo significant association between closing pressure, amount of cerebrospinal fluid removed, and time to resolution of papilledema due to pseudotumor cerebri syndrome was detected. The diagnostic and therapeutic purposes of either measuring the closing pressure or maximizing the volume of cerebrospinal fluid removed were not evident in these analyses.

Pediatric Pseudotumor Cerebri Syndrome: Diagnosis, Classification, and Underlying Pathophysiology

Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered.

Pediatric pseudotumor cerebri.

Recent findings in the literature regarding the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri (PTC) are described. In the past, children with PTC have been treated like adult cases; however, the literature highlights important differences between children and adults with PTC. Revised diagnostic criteria define an opening cerebrospinal fluid pressure greater than 28 cm water as elevated in the pediatric population. Obesity is an important risk factor for primary and secondary PTC in post-pubertal children. Magnetic resonance imaging shows findings suggestive of elevated intracranial pressure in children with PTC, similar to those of adults with PTC. Diamox and weight loss are effective treatments for PTC patients with mild visual field loss. Severe papilledema, decreased vision, and optical coherence tomography measures at presentation identify patients at increased risk for subsequent visual loss. Findings summarized in the recent literature may change practice patterns in the diagnosis and treatment of pediatric PTC. Although there are many similarities between the adult and pediatric populations, the differences should be considered when managing children with PTC.

Octreotide: A Therapeutic Option for Pediatric Pseudotumor Cerebri Syndrome?

Pseudotumor cerebri syndrome (PTCS) is a condition of unclear etiology, characterized by increased intracranial pressure (ICP) with normal cerebrospinal fluid contents and no associated anomalies evident in the brain parenchyma. It may be associated with concomitant risk factors, including obesity, endocrine disorders, chronic medical illness, and medications. Clinical signs and symptoms of PTCS in children are heterogeneous, but they usually include headache, papilledema at the funduscopic examination, and visual disturbances (e.g., diplopia and/or vision loss). Irreversible blindness, due to persistently raised ICP, has been also reported in up to 10% of cases. Treatment goal of PTCS is the relief of symptoms and the preservation of visual function, by reducing ICP. Diuretics (e.g., acetazolamide, furosemide) could be regarded as the first-line treatments in PTCS; however, up to 30% of patients do not have a clinical response, making important the identification of alternative (conservative) treatment's choices to avoid the PTCS-related complications (e.g., permanent visual loss) and the necessity of invasive surgical (cerebrospinal fluid diverting) procedures. In this context, octreotide (i.e., a synthetic somatostatin analog) has been previously proposed in some studies as a potential (etiologically targeted) treatment able to revert to PTCS-related manifestations. We hereby discuss the possible role of octreotide in PTCS and hypothesize the possible mechanism of action of this drug in reducing ICP.

Pediatric Pseudotumor Cerebri Syndrome: Recent Insights and Future Directions

Pediatric Pseudotumor Cerebri Syndrome: Recent Insights and Future Directions

Clinical Assessment and Differential Diagnosis of Pediatric Pseudotumor Cerebri Syndrome: Pearls and Pitfalls

Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and normal cytologic and chemical analyses of the cerebrospinal fluid. Pathophysiology of this syndrome remains unclear, while its incidence may be potentially rising in children. The presenting signs and symptoms of PTCS are heterogeneous to usually include headache, papilledema, and visual disturbances (i.e., vision loss or double vision). The clinical features of PTCS in children have changed over the years, making the diagnosis challenging in some cases. Young children may present with atypical symptoms, such as those mimicking a posterior fossa tumor. There are reports of PTCS in children in the absence of headache. Papilledema, considered the acute hallmark of PTCS at onset, can also be absent. It is thus important for pediatricians to recognize atypical presentations especially as previous studies have suggested children presenting with headache in the absence of papilledema or those with papilledema in the absence of any other clinical signs may have increased risk of developing visual constriction deficits and permanent loss of vision, respectively. Moreover, many conditions in the pediatric age group may mimic PTCS, such as migraine and tension headaches. Cerebral venous sinus thrombosis and some neoplastic processes such as gliomatosis cerebri and leptomeningeal spread of hematologic malignancies are known to augment intracranial pressure and have to be carefully excluded. To avoid misdiagnosis and potential long-term sequel, a thorough clinical assessment is crucial to promptly and correctly recognize PTCS.

Brain Imaging in Pediatric Pseudotumor Cerebri Syndrome

Pseudotumor cerebri syndrome (PTCS) is a clinical entity of unclear etiology characterized by raised intracranial pressure but normal cerebrospinal fluid contents and no associated anomalies evident in the brain parenchyma. Idiopathic intracranial hypertension, which is the primary (idiopathic) form of PTCS, generally affects adult obese females of childbearing age. Pediatric PTCS has been reported in association with various potential comorbidities, including chronic medical illness, infections, endocrine disorders, and ingestion and/or withdrawal of some drugs. Clinical signs and symptoms of PTCS in children are heterogeneous, but they classically include headache, papilledema, and visual disturbances (e.g., diplopia and/or vision loss). An important criterion for diagnosing PTCS involves imaging. The role of computed tomography is useful to rule out some secondary causes of elevated increased intracranial pressure (e.g., hydrocephalus, hemorrhage, mass effect); magnetic resonance imaging and magnetic resonance venography are helpful in many diagnostic challenges and in the detection of subtle intracranial anomalies (e.g., isodense tumors, meningeal infiltrations, pathologies of posterior fossa, sinus venous thrombosis). In addition, magnetic resonance imaging is able to identify some secondary signs supportive of a diagnosis of PTCS, such as flattened posterior globe/sclera, empty sella, partially empty sella/decreased pituitary height, patulous optic nerve sheath, intraocular protrusion of optic nerve head, and enhanced and increased tortuosity of the optic nerve, though these are not specific. We briefly discuss the application of imaging in the assessment of pediatric PTCS patients.