Objective: Pediatric idiopathic intracranial hypertension (IIH) is a rare condition characterized by elevated intracranial pressure (ICP) without an identifiable cause. Although most patients respond to medical therapy, refractory cases might require surgical intervention, particularly if the patient experiences visual deterioration. Data on the outcomes of CSF shunting, optic nerve sheath fenestration (ONSF), and venous sinus stenting (VSS) for patients with pediatric IIH are inconsistent across studies.
Methods: A literature search was conducted from January 1990 until July 2025 across 3 databases. Eligible studies included patients younger than 18 years with primary, medically refractory IIH with visual impairment who underwent CSF shunt placement, ONSF, or VSS. Primary outcomes included improvement in headache, papilledema, visual acuity, and reoperation rates. Meta-analyses of the proportion of patients whose symptoms improved were performed with subgroup analyses by intervention type.
Results: The authors identified and included 54 studies with 150 patients (53% were female; in multipatient studies, the mean age range was 3.2-15 years, the mean BMI was 23.6-32 kg/m2, and the mean opening pressure was 270-550 mm H2O). Procedures comprised CSF shunting (n = 68), ONSF (n = 45), and VSS (n = 37). Across all studies, the headache rate improved by 80% (shunt 83%, ONSF 62%, VSS 84%), papilledema by 88% (shunt 90%, ONSF 88%, VSS 86%), and visual acuity by 67% (shunt 52%, ONSF 82%, VSS 70%). The overall reintervention rate was 22% (shunt 24%, ONSF 5%, VSS 30%). No primary outcome subgroup differences were statistically significant.
Conclusions: Symptom and ophthalmological improvement were similar across surgical modalities in pediatric patients with IIH with vision-threatening papilledema. ONSF offers high visual acuity preservation with low reintervention rates, making it well suited for urgent vision preservation. CSF shunt placement provides global intracranial pressure control that alleviates associated symptoms, but it is associated with frequent revisions. VSS is effective for patients with venous sinus stenosis and a pressure gradient and thus requires these important prerequisites before stent placement. Larger, pediatric-specific comparative studies are needed to optimize surgical decision-making.
Objective: The aim of this study was to characterize internal jugular vein (IJV) physiology across multiple head positions in patients undergoing evaluation for suspected cerebral venous outflow disorders (CVDs) and to expand prior work by presenting the largest dynamic cerebral venography and pressure manometry series to date.
Methods: A retrospective chart review was conducted on patients who underwent diagnostic cerebral venography with pressure manometry and rotational analysis between 2024 and 2025. Venography and manometry were performed in neutral, 90° rightward rotation, 90° leftward rotation, and chin flexion head positions for each IJV. The degree of stenosis was graded and the transstenotic pressure gradients were recorded.
Results: A total of 223 consecutive patients underwent diagnostic cerebral venography with pressure manometry during the study period. Ninety-four percent of patients developed at least moderate rotational stenosis with ipsilateral or contralateral head rotation and 84% developed severe or occlusive stenosis with head rotation in at least 1 IJV. Chin flexion resulted in severe or occlusive stenosis in 74% of the IJVs studied in this series. A rotational gradient of at least 6 mm Hg was found in 43.5% of patients, and 17.5% of patients had a gradient of at least 10 mm Hg. Contralateral head rotation resulted in stenosis predominately centered at C1, while ipsilateral head rotation resulted in stenosis predominately centered at C2-4.
Conclusions: Dynamic, position-dependent IJV stenosis is highly prevalent in patients evaluated for CVDs, often with large associated pressure gradients. These findings confirm and extend prior observations, highlight the consistent anatomical patterns of compression, and underscore the value of incorporating dynamic positional testing into routine diagnostic evaluation.
Objective: Growing evidence indicates that chronically raised intracranial pressure (ICP) might cause a spontaneous CSF leak. However, data on the prevalence of idiopathic intracranial hypertension (IIH) and associated clinical features of high ICP among patients presenting with spontaneous CSF leaks are limited.
Methods: The authors performed a retrospective review of patients presenting with spontaneous skull base CSF leaks from 2013 to 2020 at a quaternary care center. Demographic, clinical, and imaging characteristics between groups with versus without IIH were compared using the t-test, chi-square test, or Fisher's exact test. Generalized estimating equations estimated visual acuity differences. The authors performed a sensitivity analysis to evaluate the sensitivity, specificity, and predictive value of one or more radiological findings suggestive of high ICP for the diagnosis of IIH among patients with CSF leaks.
Results: Among 55 patients with CSF leaks with complete data, 34 (62%) met criteria for the diagnosis of definite IIH, with 23 having a known diagnosis of IIH prior to CSF leak diagnosis and an additional 11 meeting criteria after surgical CSF leak repair. Compared with patients without confirmed IIH, these patients were more likely to be female (97% vs 76%, p = 0.03), were younger (mean age 44.4 ± 10.4 vs 56.7 ± 10.7 years, p < 0.0001), and had a higher maximum recorded BMI (mean 40.5 ± 8.9 vs 35.47 ± 5.9, p = 0.03). The presence of 1 or more radiological findings suggestive of high ICP (including empty sella, vertical optic nerve tortuosity, posterior globe flattening, venous sinus stenosis, and low-lying cerebellar tonsils) had a specificity of 0.62 and a positive predictive value (PPV) of 0.75 for diagnostic confirmation of IIH. Specificity and PPV increased to 0.91 and 0.88, respectively, with the presence of 2 or more radiological signs. Both specificity and PPV equaled 1 when 3 or more radiological signs suggestive of high ICP were present.
Conclusions: Nearly two-thirds of patients with CSF leaks in this study met criteria for definite IIH, although strict reliance on IIH diagnostic criteria in the setting of CSF leaks might underestimate the true prevalence. In the setting of a CSF leak, the presence of even 1 radiological sign of high ICP has a high PPV for IIH diagnosis. In this study, PPV increased to 100% when 3 radiological signs of high ICP were present.
Objective: The aim of this study was to evaluate the impact of conscious deep breathing on intracranial venous sinus pressures (VSPs) in awake patients undergoing cerebral venography and venous manometry for suspected cerebral venous congestion (CVC).
Methods: Authors of this retrospective study conducted a chart review of adult patients who, between 2023 and 2025, had undergone cerebral venography and manometry for high clinical suspicion of CVC and had been instructed to engage in deep breathing after baseline VSP measurement at the torcular Herophili. Both the inspiratory and expiratory phases were standardized to 5 seconds in duration to achieve a rate of 6 breaths/minute. After 5 cycles, torcular pressure measurements were repeated. VSP changes before and after deep breathing were assessed for a fast or slow return to baseline. Statistically significant differences between pressure measurements before and after 5 deep breathing cycles as well as differences in blood pressure measurements before and after diagnostic cerebral venography and manometry were assessed using the paired Wilcoxon signed-rank test.
Results: All 28 patients included in the study exhibited a reduction in torcular VSP after 5 deep breathing cycles, with a median pressure change of -2.5 (IQR -2.0, -4.0) mm Hg. The median torcular pressure decreased from 8.5 (IQR 5.75, 12) to 5 (2, 9) mm Hg, a change that was statistically significant (p = 3.5 × 10-6). The majority of patients (92.86%) returned to baseline pressures within 5 seconds of deep breathing cessation, whereas 7.14% showed a prolonged return to baseline (>15 seconds), resolving within 2 minutes.
Conclusions: As insights into the mechanisms and effects behind CVC grow, noninvasive therapies that improve venous outflow, such as deep breathing, may be impactful to patients, potentially delaying or even obviating the need for surgical treatments. Where surgery is needed, deep breathing may allow for tighter VSP control. Understanding VSPs may thus improve the medical and surgical management of patients with venopathic intracranial hypertension. More research is needed to understand these phenomena.
Objective: The role of extracranial venous compression of the internal jugular vein (IJV) in patients who have symptoms consistent with intracranial hypertension is a recently described phenomenon. If medical treatment fails to control symptoms, several surgical techniques have been described to address the IJV compression that typically occurs, as the IJV passes anterior to the C1 transverse process. Techniques include styloidectomy, IJV fasciotomy, IJV repositioning, C1 transverse process resection, and IJV stenting, in isolation or in various combinations. The aim of this study was to evaluate outcomes (symptom severity, quality of life [QOL], and radiological change in IJV stenosis) in patients who underwent a single institution's standard surgical protocol of styloidectomy, IJV fasciotomy, and CI transverse process resection.
Methods: Adult patients who had symptoms consistent with intracranial hypertension and radiological evidence of IJV compression were evaluated from January 2023 to January 2025 in a multidisciplinary team meeting. Those patients who remained significantly impaired despite medical therapy and who elected to undergo surgery were included. Demographic data, symptom severity and QOL data, and radiological data were collected preoperatively and postoperatively (3-6 months). Descriptive and comparative statistical analyses were performed.
Results: Twenty-seven patients (22 female, median age 40 years) were included in this analysis. Improvements were observed in all 3 domains during the postoperative follow-up period. The mean IJV stenosis grade improved by 1.89 points (p < 0.0001). All symptom severity scores, except for dizziness, showed a statistically significant improvement, with headache demonstrating the most improvement (Δ = 1.85, p = 0.0002). A statistically significant improvement (Δ = 1.13, p = 0.006) was seen in overall QOL, as well as 10 other measures markers.
Conclusions: This case series demonstrated that combined styloidectomy, IJV fasciotomy, and C1 transverse process resection yielded meaningful improvements in symptom burden, QOL, and venous outflow on imaging in a carefully selected cohort of patients who were refractory to medical therapy.
Objective: Accurate risk stratification in idiopathic intracranial hypertension (IIH) remains challenging, and surgical selection criteria lack standardization. The aim of this study was to evaluate a multidisciplinary diagnostic algorithm combining optic nerve sheath diameter (ONSD) measurement on ultrasonography and 48-hour invasive intracranial pressure (ICP) monitoring to classify patients with IIH by risk of vision loss and to guide individualized management.
Methods: The prospective study consecutively enrolled patients with suspicion of IIH treated with medical therapy, but with minimal response, from January 2021 to December 2024 at a single center. Exclusion criteria included secondary causes of intracranial hypertension or significant transverse sinus stenosis. The diagnostic protocol consisted of baseline measurement of ONSD; 48-hour cisternal pressure monitoring via external lumbar drainage, including a 24-hour CSF subtraction phase; and subsequent repeat of ONSD measurement. Patients were stratified into three groups (no IIH, IIH CSF subtraction nonresponders, and IIH CSF subtraction responders) based on the opening pressure, change in mean cisternal pressure (Δp), and ONSD change. Borderline cases were included in the pressure waveform analysis using mean absolute error and Dynamic Time Warping relative to a normative curve. Area under the curve (AUC) analysis was performed to determine optimal cutoff values for ONSD and Δp.
Results: Thirty-one patients (24 female, mean age 38.7 years, mean BMI 30.2) met inclusion criteria. ONSD and Δp were significantly associated with IIH diagnosis (p = 0.004 and p = 0.031, respectively). AUC analysis identified optimal cutoffs of 5.6 mm for ONSD (AUC 0.98, 92.5% sensitivity, 100% specificity) and 3.0 cm H2O for Δp (AUC 0.93, 81.7% sensitivity, 100% specificity). At the 3-month follow-up, IIH CSF subtraction responders (with shunting) had normalization of ONSD and visual field improvement in most cases, while the IIH CSF subtraction nonresponders remained stable on medical therapy.
Conclusions: The integration of ultrasonography-determined ONSD with invasive cisternal pressure monitoring and CSF subtraction provided an objective and reproducible approach for diagnosing IIH and stratifying patients by risk of vision loss. The proposed ONSD and Δp thresholds could support standardized surgical decision-making and reduce variability in IIH management. Multicenter validation and long-term follow-up are warranted.
Objective: Cerebral venous sinus stenting (VSS) is an established treatment for transverse sinus (TS) stenosis associated with idiopathic intracranial hypertension (IIH). The authors' early practice favored shorter stents that only spanned the focal narrowing to reduce the metal burden. However, stent-adjacent stenosis (SAS) emerged as a significant failure mode prompting the use of longer constructs. In this study, the authors sought to compare the incidence of SAS, clinical outcomes, and angiographic findings between shorter and full-length stent constructs.
Methods: The authors conducted a retrospective analysis of patients with IIH who underwent TS stenting at Tufts Medical Center in the period from 2013 to 2023. Patients with medically refractory IIH symptoms and a significant cerebral venous pressure gradient were included. Symptom presentation, neuroimaging, venous manometry, preoperative and follow-up angiography, and clinical examination findings were recorded and analyzed across two categories of stent length: shorter (40 or 60 mm) and full length (80 mm). SAS was defined as de novo narrowing of the TS contiguous with the stent edge.
Results: Endovascular treatment was successful in all 60 patients included in the study (48 females, mean patient age of 39 years). Three patients were treated with 40-mm stents, 13 with 60-mm stents, and 44 with full-length 80-mm stents. SAS was documented in 8 patients (13%) and was significantly more frequent (p < 0.01) among those with shorter stents (6/16), with 3 patients requiring reintervention using a second overlapping stent. Among the 44 patients with full-length constructs, 2 demonstrated SAS at the residual unstented TS lateral to the torcular Herophili. After a mean follow-up of 40 months, 29 patients (48%) reported persistent symptoms, although there was no significant difference in this rate between the stent groups. Full-length constructs demonstrated a more favorable postoperative reduction in TS pressure gradients (p < 0.01) and lower gradients on follow-up (p < 0.05).
Conclusions: SAS is a clinically relevant complication following VSS in patients with IIH and may contribute to persistent symptoms and the need for reintervention. Full-length stent constructs extending from the sigmoid sinus to the torcula significantly reduce the incidence of SAS and are associated with better hemodynamic outcomes following VSS.
Objective: Venous sinus stenting (VSS) has emerged as a promising and less invasive treatment alternative to CSF diversion for patients with idiopathic intracranial hypertension (IIH). Fulminant IIH (fIIH) is a rare manifestation of IIH, affecting 2%-3% of IIH patients and characterized by rapid, progressive, and severe visual loss. Outcomes following different treatment modalities for fIIH remain unclear.
Methods: A dual-center retrospective cohort study was done comparing patients who underwent VSS or ventriculoperitoneal shunting (VPS) for fIIH over a 5-year time frame, defined as rapid visual loss secondary to IIH refractory to medical management. Patient demographic characteristics, baseline symptom characteristics focusing on headache and visual decline, operative parameters, postoperative complications, and outcomes were collected. The decision to proceed with VSS or VPS was based on surgeon discretion and institutional preference. Data were analyzed using the bivariate t-test or chi-square test as appropriate, and multivariate logistic regression was used to identify significant predictors.
Results: A total of 39 patients who underwent treatment for fIIH were identified, of whom 46% (n = 18) underwent VPS and 54% (n = 21) underwent VSS. The VSS cohort included more females (100% vs 76%, p = 0.03), otherwise no differences were seen in age, BMI, rate of preoperatively diagnosed headache disorder or psychiatric disorder, acetazolamide usage, preoperative papilledema grade, preoperative average visual acuity, or preoperative opening pressure. On univariate regression, VSS was associated with improved headache reduction (58% vs 25%, p < 0.001) as well as decreased headache recurrence (27% vs 65%, p = 0.03). Both cohorts showed a high rate of improvement in papilledema postoperatively (93.3% for VSS vs 92.8% for VPS, p > 0.99), and there was no significant difference in the rates of postoperative visual acuity improvement (83% for VSS vs 50% for VPS, p = 0.17). VPS patients were more likely to have reductions in acetazolamide usage postoperatively (85% vs 22%, p = 0.001). No postoperative hemorrhages, access site complications, or stent thromboses were observed. On multivariate regression, no significant predictors of improvement in headache severity, papilledema, or visual acuity were observed.
Conclusions: Among patients presenting with fIIH, VSS is comparable to VPS with respect to visual outcomes defined as improvement in papilledema. Larger randomized studies are required to determine differences in outcomes between VSS and VPS in this subpopulation of IIH patients.

