The correlation between intracranial pressure and venous sinus pressures changes after venous sinus stenting.

BACKGROUND: The pathophysiology of idiopathic intracranial hypertension (IIH) and other cerebral venous outflow disorders (CVD) has largely been unexplored. While a correlation between venous sinus pressure and opening pressure (OP) has been previously noted, there are limited data on this relationship in patients with prior venous sinus stenting (VSS). METHODS: A single center retrospective chart review was conducted on CVD patients who underwent diagnostic cerebral venography with manometry followed immediately by lateral decubitus lumbar puncture, from 2016 to 2024. RESULTS: 206 patients underwent 216 total procedures and were included. Among all patients, there was a moderate nearly one-to-one correlation between OP and torcular or superior sagittal sinus (SSS) pressures. Thirty-two patients underwent testing after having undergone VSS. Patients with previous VSS showed a significantly altered relationship between venous pressures and OP, whereby as venous sinus pressures increased, OP increased more gradually in previously stented patients compared with non-stented patients. For example, when OP was fixed at 21 mm Hg and body mass index at 30 kg/m2, predicted mean SSS pressure was 19.47 mm Hg in non-stented patients versus 16.91 mm Hg in stented patients. CONCLUSION: This study confirmed a strong relationship between OP and venous sinus pressure in CVD patients. However, patients with previous VSS demonstrated an altered relationship with higher CSF pressures relative to venous pressures compared with naïve patients. This finding may have important clinical implications in the management of IIH patients.

Cerebral venous pressures, sinus trans-stenosis gradients, and intracranial pressures are dramatically augmented by head position.

BACKGROUND: Cerebral venous pressures, sinus trans-stenosis gradients, and intracranial pressures are thought to be influenced by head position. OBJECTIVE: To investigate the intracranial manifestations of these changes in patients with cerebral venous outflow disorders (CVD). METHODS: A retrospective chart review was conducted on 22 consecutive adult patients who underwent diagnostic cerebral venography with rotational internal jugular vein (IJV) venography and superior sagittal sinus (SSS) pressure measurements in multiple head positions. Data on venous sinus pressures, IJV pressures, and lumbar puncture (LP) opening pressures (OP) were collected and analyzed. RESULTS: The study found that 21 (96%) patients experienced increases in SSS pressures with head rotation, with a mean increase of 25.4%. Intracranial trans-stenosis gradients showed significant variability with head position. Additionally, LP OP measurements increased by an average of 44.3% with head rotation. Dynamic IJV stenosis was observed in all patients during rotational testing. CONCLUSION: Head position significantly affects cerebral venous pressures, trans-stenosis gradients, and intracranial pressures in patients with CVD or intracranial hypertension. These findings highlight the need for dynamic venography in the diagnostic evaluation of these conditions to better understand their pathophysiology and improve treatment strategies.

Idiopathic intracranial hypertension pathogenesis: The jugular hypothesis.

In spite of expanding research, idiopathic intracranial hypertension (IIH) and its spectrum conditions remain challenging to treat. The failure to develop effective treatment strategies is largely due to poor agreement on a coherent disease pathogenesis model. Herein we provide a hypothesis of a unifying model centered around the internal jugular veins (IJV) to explain the development of IIH, which contends the following: (1) the IJV are prone to both physiological and pathological compression throughout their course, including compression near C1 and the styloid process, dynamic muscular/carotid compression from C3 to C6, and lymphatic compression; (2) severe dynamic IJV stenosis with developments of large cervical gradients is common in IIH-spectrum patients and significantly impacts intracranial venous and cerebrospinal fluid (CSF) pressures; (3) pre-existing IJV stenosis may be exacerbated by infectious/inflammatory etiologies that induce retromandibular cervical lymphatic hypertrophy; (4) extra-jugular venous collaterals dilate with chronic use but are insufficient resulting in impaired aggregate cerebral venous outflow; (5) poor IJV outflow initiates, or in conjunction with other factors, contributes to intracranial venous hypertension and congestion leading to higher CSF pressures and intracranial pressure (ICP); (6) glymphatic congestion occurs but is insufficient to compensate and this pathway becomes overwhelmed; and (7) elevated intracranial CSF pressures triggers extramural venous sinus stenosis in susceptible individuals that amplifies ICP elevation producing severe clinical manifestations. Future studies must focus on establishing norms for dynamic cerebral venous outflow and IJV physiology in the absence of disease so that we may better understand and define the diseased state.

Safety of intracranial venous stenting in patients with nickel allergy.

BACKGROUND: Nickel hypersensitivity is the most common metal related allergy. Nickel containing alloys are frequently used in endovascular devices. The use of intracranial stents in patients with nickel hypersensitivity appears to be safe, but these small series only evaluated arterial stent placement. This case series aimed to assess the safety of intracranial venous stent placement in patients with documented nickel allergy. METHODS: In this retrospective multicenter case series, patients with idiopathic intracranial hypertension and documented nickel allergy underwent treatment with a permanently implanted nickel containing stent in the dural venous sinuses. RESULTS: Nine patients with nickel allergy were included. All patients reported clinical improvement in their idiopathic intracranial hypertension symptoms. Of the five patients who had follow-up intracranial venous imaging, all stents remained patent. No patients experienced intraoperative, postoperative, or long term procedure related complications, with follow-up ranging from 1.8 weeks to 49.1 months. CONCLUSION: In this limited case series, the use of nickel containing stents in intracranial venous sinuses in patients with nickel allergy did not result in any allergic reaction or adverse outcome.

Dynamic internal jugular vein venography: a descriptive study in 89 patients with suspected cerebral venous outflow disorders.

BACKGROUND: Internal jugular vein (IJV) stenosis has recently been recognized as a plausible source of symptom etiology in patients with cerebral venous outflow disorders (CVD). Diagnosis and determining surgical candidacy remains difficult due to a poor understanding of IJV physiology and positional symptom exacerbation often reported by these patients. METHODS: A retrospective single-center chart review was conducted on adult patients who underwent diagnostic cerebral venography with rotational IJ venography from 2022 to 2024. Patients were divided into three groups for further analysis based on symptoms and diagnostic criteria: presumed jugular stenosis, near-healthy venous outflow, and idiopathic intracranial hypertension. RESULTS: Eighty-nine patients were included in the study. Most commonly, ipsilateral rotation resulted in ipsilateral IJV stenosis and gradient development at C4-6 and contralateral stenosis and gradient appearance in the contralateral IJV at C1, with stenosis and gradient development in bilateral IJVs at C1-3 bilaterally during chin flexion. In all patients, 93.3% developed at least moderate dynamic stenosis of at least one IJV, more than two-thirds (69.7%) developed either severe or occlusive stenosis during rightward and leftward rotation, and 81.8% developed severe or occlusive stenosis with head flexion. Dynamic gradients of at least 4 mmHg were seen in 68.5% of patients, with gradients of at least 8 mmHg in 31.5% and at least 10 mmHg in 12.4%. CONCLUSION: This study is the first to document dynamic changes in IJV caliber and gradients in different head positions, offering insights into the complex nature of venous outflow and its impact on CVD.

Redefining treatment expectations: exploring mid- and long-term outcomes of venous sinus stenting in idiopathic intracranial hypertension.

BACKGROUND: Venous sinus stenting (VSS) is recognized as a safe and effective intervention for medically-refractory idiopathic intracranial hypertension (IIH). However, its long-term efficacy remains uncertain. METHODS: This retrospective review analyzed a single-center database of adult patients with severe, medically-refractory IIH, who underwent VSS and had minimum 3-month follow-up (FU). Patients were divided into three groups based on post-stenting symptom trajectories: group 1 (sustained improvement without relapse), group 2 (temporary improvement with relapse), and group 3 (no improvement). RESULTS: Of 178 patients undergoing VSS, the majority were female (94%), with a median opening pressure (OP) of 31 cm H2O and trans-stenosis gradient of 14 mm Hg. Of these, 153 (86%) received transverse sinus (TS) stenting, and 19 (11%) underwent concurrent TS and superior sagittal sinus stenting. At a mean FU of 166 days, 53 patients (30%) showed long-term improvement without relapse (group 1). Symptomatic recurrence was noted in 101 patients (57%; group 2) within a mean FU of 390 days. Despite recurrent headache and tinnitus, the average OP reduction was 9.6 cm H2O on repeat lumbar puncture, with 75% showing papilledema improvement or resolution post-VSS. Only 17% required further surgical intervention. CONCLUSIONS: The most common clinical outcome post-VSS in IIH patients is initial symptomatic improvement followed by symptom recurrence in about 60% at a mean of 274 days, despite a consistent intracranial pressure reduction. These findings can guide physicians in setting realistic expectations with patients regarding VSS outcomes.

The Promise, Mystery, and Perils of Stenting for Symptomatic Internal Jugular Vein Stenosis: A Case Series.

BACKGROUND AND OBJECTIVES: Cerebral venous outflow disorders (CVDs) secondary to internal jugular vein (IJV) stenosis are becoming an increasingly recognized cause of significant cognitive and functional impairment in patients. There are little published data on IJV stenting for this condition. This study aims to report on procedural success. METHODS: A single-center retrospective analysis was performed on patients with CVD that underwent IJV stenting procedures. RESULTS: From 2019 to 2023, 29 patients with CVD underwent a total of 33 IJV stenting procedures. Most patients (20; 69%) had an underlying connective tissue disorder diagnosis. The mean age of the included patients was 36.3 years (SD 12.4), 24 were female (82.8%), and all were Caucasian except for 2 patients (27; 93.0%). Twenty-eight procedures (85%) involved isolated IJV stenting under conscious sedation, whereas 5 procedures (15%) involved IJV stenting and concomitant transverse sinus stenting under general anesthesia. Thirteen (39%) patients underwent IJV stenting after open IJV decompression and styloidectomy. Three patients had stents placed for stenosis below the C1 tubercle, one of which was for carotid compression. Periprocedural complications occurred in 11 (33%), including intracardiac stent migration in 1 patient, temporary shoulder pain/weakness in 5 (15%), and persistent and severe shoulder pain/weakness in 2 patients (6%). Approximately 75% of patients demonstrated improvement after stenting although only 12 patients (36%) had durable improvement over a mean follow-up of 4.5 months (range 6 weeks-3.5 years). CONCLUSION: Our experience, along with early published studies, suggests that there is significant promise to IJV revascularization techniques in these patients; however, stenting carries a high complication rate, and symptom recurrence is common. Most neurointerventionalists should not be performing IJV stenting unless they have experience with these patients and understand technical nuances (stent sizing, anatomy, patient selection), which can maximize benefit and minimize risk.

Intensive care unit admission is not necessary after venous sinus stenting.

BACKGROUND: Venous sinus stenting (VSS) has emerged as a safe and effective treatment option for idiopathic intracranial hypertension. Many physicians routinely admit patients to the intensive care unit (ICU) for close monitoring, but little data exists on whether this is necessary. METHODS: Electronic medical records of consecutive patients who underwent VSS by the senior author from 2016 to 2022 at a single center were reviewed. RESULTS: 214 patients were included. The mean (SD) age was 35.5 (11.6) and 196 (91.6%) patients were female. A total of 166 (77.6%) patients underwent transverse sinus stenting alone; 9 (4.2%) underwent superior sagittal sinus (SSS) stenting alone, 37 (17.3) concomitant transverse and SSS stenting, and 2 (0.9%) underwent stenting at alternate sites. All patients were planned admission to the regular ward (27.6%) or day hospital (72.4%). Twenty (9.3%) patients were discharged to home the same day as the procedure and 182 (85%) patients were discharged the following day. Major periprocedural complications were identified in 2 (0.93%) patients and minor complications were identified in 16 (7.4%). Only one patient with a subdural hematoma identified in the post-anesthesia care unit (PACU) had care escalated to the ICU. No severe complications were identified after the PACU stay. During the next 48 hours after discharge, 4 (1.9%) patients returned to any emergency room to be evaluated without requiring readmission. CONCLUSION: Routine ICU admission following uncomplicated VSS is unnecessary. Overnight admission to a low-acuity ward, or even same-day discharge in select patients, appears to be a safe and cost-effective strategy.

Characterizing a new clinical phenotype: the co-existence of cerebral venous outflow and connective tissue disorders.

Background: There is increasing recognition of connective tissue disorders and their influence on disease in the general population. A conserved clinical phenotype involving connective tissue disorders and idiopathic intracranial hypertension (IIH) and associated cerebral venous outflow disorders (CVD) has not been previously described. Methods: A single-institution retrospective review of a prospectively maintained database of patients with connective tissue disorders and CVD was performed. Results: A total of 86 patients were identified. The majority of these patients carried a diagnosis of Ehlers-Danlos syndrome (55%) and most were non-obese (mean body mass index 29.7 kg/m2), Caucasian (90%) females (87%). Most prevalent presenting symptoms included pressure headache (98%), dizziness (90%), tinnitus (92%), and cognitive dysfunction (69%). Aside from CVD and IIH, the most common associated conditions were postural orthostatic tachycardia syndrome (POTS; 55.8%), cerebrospinal fluid (CSF) leaks (51.2%), dysautonomia (45.3%), cranio-cervical instability (37.2%), mast cell activation syndrome (25.6%), and tethered cord syndrome (23.3%). Allergies to medications (87.2%) and surgical tape (19.8%) were also frequent. Despite significantly lower opening pressures on lumbar puncture, headache severity and quality of life scores were reported with the same severity of classic IIH patients, suggesting an underlying hypersensitivity to intracranial pressures and cerebral venous congestion. Conclusion: There is a rare but conserved clinical phenotype that has not been described previously that presents with severe IIH symptoms in predominantly young, non-obese Caucasian women with a high associated incidence of dysautonomia, POTS, craniocervical instability, and CSF leaks, among others.