JEDI (jugular entrapment, dilated ventricles, intracranial hypertension) syndrome: a new clinical entity? A case report.

Patients with idiopathic intracranial hypertension are frequently obese women with normal/slit ventricles. Patients with high-pressure hydrocephalus, instead, present enlarged ventricles. We describe a 63-year-old woman with signs and symptoms of intracranial hypertension. Brain MRI revealed hydrocephalus. Venous Doppler ultrasound showed external compression of the omohyoid muscles on the internal jugular veins. During jugular vein decompression, intracranial pressure dropped from 18 to 6 mmHg. Patient is asymptomatic at 2-year follow-up, with decreased brain ventricles. These findings could represent a novel form of high-pressure hydrocephalus that can be successfully treated without a CSF shunt. We called this syndrome JEDI (jugular entrapment dilated ventricles intracranial hypertension).

Comparison Between Duplex Ultrasound and Multigate Quality Doppler Profile Software in the Assessment of Lower Limb Perforating Vein Direction.

OBJECTIVES: The aim was to assess more accurately the net flow of the lower limb perforating veins (PVs). MATERIAL AND METHODS: This was an observational prospective study. Two hundred and twenty one limbs with chronic venous disease (C1-6EpAs,pPr) of 193 patients underwent a duplex ultrasound (DUS). All identified PVs were scanned also by means of quality Doppler profile (QDP) multigate analysis in order to determine their net inward and outward flow direction. A comparison between the traditional pulsed wave Doppler analysis and QDP was performed to detect potential discrepancy between the traditional definition of PV incompetence and a net outward flow. RESULTS: The DUS investigation identified 774 PVs. Only 7.7% of the PVs showed an outward flow lasting more than 500 ms. Among the PVs showing a longer than 500 ms outward flow, QDP assessment revealed net outward flow in only 84% of the PVs along the thigh and in 28.6% along the lower leg. Among the PVs showing a shorter than 500 ms outward flow, QDP assessment reported a net outward flow in 2.4% of the PVs along the thigh and in 47.3% of those along the lower leg. The sensitivity of an outward flow lasting more than 500 ms in detecting an actual net outward flow was 13.9% (9-20.1%). The specificity of an outward flow lasting less than 500 ms in detecting a net inward flow was 96.4% (93.2-98.3%). CONCLUSIONS: A lack of overlap exists between the finding of a PV outward flow lasting more than 500 ms and the net outward flow of the same vessel. The traditional definition of PV incompetence is challenged by the reported data and further investigations are required to identify a gold standard assessment.

An ultrasound model to calculate the brain blood outflow through collateral vessels: a pilot study.

BACKGROUND: The quantification of the flow returning from the head through the cervical veins and the collaterals of the internal jugular vein (IJV), is becoming of prominent interest in clinical practice. We developed a novel model to calculate the cerebral venous return, normalized to the arterial inflow, in the different segments of the IJV. METHODS: We assessed, by established Echo Colour Doppler (ECD) methodology, the head inflow (HBinF) defined as the sum of common carotids and vertebral arteries, as well as the cerebral flow (CBF) defined as the sum of internal carotid and vertebral arteries. We also assessed the head outflow (HBoutF) defined as the sum of the measurements at the junction of the IJV and the vertebral veins. In addition, we also calculated the collateral flow index (CFI) by estimating the flow which re-enters directly into the superior vena cava as the amount of blood extrapolated by the difference between the HBinF and the HBoutF. We preliminarily tested the model by comparing ten healthy controls (HC) with ten patients affected by chronic cerebral spinal venous insufficiency (CCSVI), a condition characterized by some blockages in the IJV which are bypassed by collateral circulation. RESULTS: In HC the HBinF was 956+-105ml/min, whereas the HBoutF was > 90% of the HBinF, leading to a final CFI value of 1%. The last result shows that a very small amount of blood is drained by the collaterals. In upright we confirmed a reduction of the outflow through the IJV which increased CFI to 9%. When we applied the model to CCSVI, the HBinF was not significantly different from controls. In supine, the flow of CCSVI patients in the IJV junction was significantly lower (p < 0.001) while the correspondent CFI value significantly increased (61%, p < 0.0002). CONCLUSIONS: Our preliminary application of the novel model in the clinical setting suggests the pivotal role of the collateral network in draining the blood into the superior vena cava under CCSVI condition.

Comparison of signal to noise ratios from spatial and frequency domain formulations of nonprewhitening model observers in digital mammography.

PURPOSE: Image quality indices based upon model observers are promising alternatives to laborious human readings of contrast-detail images. This is especially appealing in digital mammography as limiting values for contrast thresholds determine, according to some international protocols, the acceptability of these systems in the radiological practice. The objective of the present study was to compare the signal to noise ratios (SNR) obtained with two nonprewhitening matched filter model observer approaches, one in the spatial domain and the other in the frequency domain, and with both of them worked out for disks as present in the CDMAM phantom. METHODS: The analysis was performed using images acquired with the Siemens Novation and Inspiration digital mammography systems. The spatial domain formulation uses a series of high dose CDMAM images as the signal and a routine exposure of two flood images to calculate the covariance matrix. The frequency domain approach uses the mathematical description of a disk and modulation transfer function (MTF) and noise power spectrum (NPS) calculated from images. RESULTS: For both systems most of the SNR values calculated in the frequency domain were in very good agreement with the SNR values calculated in the spatial domain. Both the formulations in the frequency domain and in the spatial domain show a linear relationship between SNR and the diameter of the CDMAM discs. CONCLUSIONS: The results suggest that both formulations of the model observer lead to very similar figures of merit. This is a step forward in the adoption of figures of merit based on NPS and MTF for the acceptance testing of mammography systems.

Altered velocity gradient in lower limb chronic venous disease.

BACKGROUND: In physiology, velocity of the deep venous compartment is higher than the one in the saphenous compartment which is higher than the one in the tributaries. Considering that velocity variation is associated with changes in the pressure gradient, aim of the present study is to assess changes in venous kinetics in case of superficial chronic venous disease, so to provide further clues in venous drainage direction determination. METHODS: Venous ultrasound scanning was performed on 40 lower limbs of 28 chronic venous disease patients (C2-6Ep,As,Pr). Velocities were measured in three different venous segments: great saphenous vein at 2 cm above the origin of the incompetent tributary (Group-A). great saphenous vein at 2 cm below the origin of tributary (Group-B). tributary at 2 cm from its origin from the great saphenous vein (Group-C.). RESULTS: Diastolic time average velocity was higher in group-C (-21.3 ± 8.5 cm/s) than in group-A (-15.7 ± 5.2 cm/s; p = .0001) and group-B (-11.1 ± 2.9 cm/s; p = .0001), thus indicating an inversion of the physiological velocity gradient in chronic venous disease patients. DISCUSSION: Chronic venous disease presents a subverted velocity gradient. These data introduce objective hemodynamics data, paving the way for further investigation in venous drainage direction determination.

Mechanical Function of Internal Jugular Vein Valve: Post-analysis of M-Mode Imaging under Cardiac Monitoring.

Because the internal jugular vein (IJV) valve is the only protective valve between the brain and heart, recent studies have focused on the dynamic behaviour of the valve and its importance in regulating the cerebral blood outflow pathway. However, the mechanism underlying valve opening and closure, as well as the normal opening time, has not been investigated before. The aim of the study described here was to investigate IJV physiology in healthy young adults by means of ultrasound imaging. Twenty-four normal young adults (16 male, 8 female, 21.79 ± 0.79 y of age) were enrolled in this study. Each participant underwent IJV B- and M-mode ultrasound scans of the neck veins in supine position. Data on IJV leaflet movement and IJV blood velocity were extracted from images with the associated electrocardiogram traces to analyze the opening and closure cycles of IJV leaflets. The normal opening time calculated in this study includes 70% of the dynamic valve cycle. The normal opening time of the IJV valve could be a new physiologic metric and serves as a premise for further studies in the field of cerebral venous return.

Lower limbs venous kinetics and consequent impact on drainage direction.

Background Literature concerning the lower limbs physiological venous haemodynamics is still lacking of reference velocity values and consequent impact on drainage direction. Aim of the present study is to assess the flow velocities in the different venous compartments, evaluating the possible Venturi effect role, thus finding clues for the identification of the physical model governing the flow direction. Methods Thirty-six lower limbs underwent a velocity and diameters echo-color-Doppler assessment in several anatomical point of analysis along both the deep and superficial venous systems. The investigation protocol included and compared two different manoeuvres to elicit the flow: manual calf compression/relaxation (CR) and active foot dorsiflexion (AFD). Both peak systolic (PSV) and time average velocities (TAV) were measured. Results The different venous segments demonstrated an overlap among the velocity values and the anatomical subdivision of the deep and superficial compartments. At the CR, TAV was 34 ± 12 cm/s in the deep venous system (N1), 15 ± 7 cm/s in the saphenous system (N2), 5 ± 2 cm/s in the saphenous tributaries (N3); PSV was 89 ± 35 cm/s in N1, 34 ± 16 cm/s in N2, 11 ± 4 cm/s in N3, p < 0.05. At the AFD, TAV was 33 ± 13 cm/s in N1, 15 ± 7 in N2, 9 ± 5 in N3; PSV was 83 ± 35 in N1, 32 ± 17 in N2, 15 ± 4 in N3, p < 0.05. A diameter decrease was reported from N1 to N3 ( p < 0.05). Conclusion This investigation provides evidences of the velocity decrease from the deepest to the most superficial compartments. These data introduce the Venturi effect as potential factor in the flow aspiration from the tributary to the deeper veins. The reported data represent a first step towards an objective evaluation of the physic laws governing the drainage. These values can constitute the basis for further investigations in pathological and post-procedural scenarios.

Cerebral Inflow and Outflow Discrepancies in Severe Sudden Sensorineural Hearing Loss.

The aim of this study is to evaluate whether cerebral inflow and outflow abnormalities, assessed by the means of a validated ultrasound model, could be associated with Sudden Sensorineural Hearing Loss (SSNHL). According to Clark, a total of 42 patients affected by severe SSNHL and 19 healthy volunteers matched by gender without any history of sudden hearing impairment have been included in this study. Patients and controls underwent EchocolorDoppler assessment of brain hemodynamics. All subjects affected by SSNHL were also assessed with Auditory Brainstem Responses (ABR) and Magnetic Resonance Imaging (MRI) in order to exclude retrocochlear pathology. The head inflow through the common carotid artery was practically equivalent between groups, but at the level of the carotid bifurcation, the external carotid artery showed a highly significant flow rate in SSNHL 5.4±2 vs 3.9±1.1 ml/s in controls (p=0.01). The brain inflow was similar between patients and controls, but interestingly the flow rate of the vertebral artery was significantly reduced in SSNHL 1.6±0.8 vs 2.8±0.9 ml/s (p=0.01). The brain outflow was found significantly restricted at the level of the jugular outlet 6.6±6 vs 9.9±6 ml/s (p=0.002); consequently, the collateral flow index was significantly increased in SSNHL (p=0.001). The present study shows a discrepant distribution of the brain inflow which seems to penalize the posterior segments of the Willis polygon in patients affected by severe SSNHL. In addition, our study confirms the presence of chronic cerebrospinal venous insufficiency in SSNHL with significant activation of venous collateral circulation.

Ultrasound Monitoring of Jugular Venous Pulse during Space Missions: Proof of Concept.

The jugular venous pulse (JVP) is one of the main parameters of cardiac function and is used by cardiologists in diagnosing heart failure. Its waveform comprises three positive waves (a, c and v) and two negative waves (x and y). Recently, it was found that JVP can be extrapolated from an ultrasound (US) video recording of the internal jugular vein (IJV), suggesting its application in space missions, on which US scanners are already widely used. To date, the feasibility of assessing JVP in microgravity (microG) has not been investigated. To verify the feasibility of JVP assessment in microG, we tested a protocol of self-performed B-mode ultrasound on the International Space Station (ISS). The protocol consisted of a video recording of IJV synchronized with electrocardiogram that produces a cross-sectional area time trace (JVP trace) (in cm2). The scans were acquired in six experimental sessions; two pre-flight (BDC1 and -2), two in space (ISS1 and -2) and two post-flight (Houston PF1, Cologne PF2). We measured the mean and standard deviation of the JVP waves and the phase relationship between such waves and P and T waves on the electrocardiogram. We verified that such parameters had the same accuracy on Earth as they did under microG, and we compared their values. The sensitivity, specificity and accuracy of JVP trace in microgravity are higher than those on Earth. The sequence of (a, c, and v) ascents and (x and y) descents along the cardiac cycle in microG is the same as that on Earth. The cause-and-effect relationship between the P and T waves on the electrocardiogram and a and v waves, respectively, of JVP is also confirmed in microG. Our experiment indicated the feasibility of deriving a JVP trace from a B-mode US examination self-performed by an astronaut in microG.

High Resolution M-mode Evaluation of Jugular Vein Valves in Patients with Neurological and Neurosensory Disorders.

BACKGROUND: High prevalence of valve absence was found in the internal jugular vein (IJV) of healthy volunteers by means of M-mode high-resolution Echo Colour Doppler (ECD). However, the prevalence of valve in neurovascular disorders linked to Chronic Cerebrospinal Venous Insufficiency (CCSVI) is still unknown. METHODS: A cohort of 83 Healthy Controls (HC), 71 Multiple Sclerosis (MS), 99 Inner Ear Disorders (IED) underwent ECD investigation of the IJV valve, including M-mode evaluation and related hemodynamics. The primary outcome measure was characterization of valve presence, morphology and motility, whereas the secondary outcome was the rate of flow alteration. RESULTS: Bilateral valve presence was found in 38% of HC, 58% of MS and 25% of IED, whereas, bilateral valve absence was recorded in 16% of HC, 10% of MS and 31% of IED (p<0.003). Bicuspid morphology was more prevalent in HC 56%, while monocusp was more prevalent in patients: 75% MS and 57% IED (p<0.0001). The main finding was the presence of mobile valve leaflets in 98% of HC, contrarily fixed valve leaflets were recorded in 82% of MS and in 41% of IED, p< 0.0001. Finally, by stratifying the entire cohort according to the presence of mobile and not mobile valve leaflets, normal monodirectional and phasic flow were commonly found in the mobile leaflets subgroup, p<0.0001. CONCLUSION: In patients with miscellaneous neurological disorders, a significant higher rate of defective valves was found with respect to HC. The latter condition is strongly associated to brain outflow abnormalities described in CCSVI condition.

Fixing the jugular flow reduces ventricle volume and improves brain perfusion.

OBJECTIVE: Increased ventricle volume and brain hypoperfusion are linked to neurodegeneration. We hypothesized that in patients with restricted jugular flow, surgical restoration may reduce brain ventricle volume, because it should improve the pressure gradient, hence promoting cerebrospinal fluid reabsorption into the venous system. METHODS: The effects of restoring the jugular flow were assessed by means of a validated echocardiography with color Doppler (ECD) protocol of flow quantification, magnetic resonance venography, and single-photon emission computed tomography combined with computed tomography (SPECT-CT). The main outcome measurement was the cerebral ventricle volume blindly assessed at SPECT-CT. Secondary outcomes were brain perfusion in the whole brain and in another 12 cerebral regions. The mean follow-up of the SPECT-CT and ECD parameters was 30 days. Patency rate was subsequently monitored by means of the same ECD protocol every 3 months. RESULTS: Among 56 patients (28 male and 28 female; mean age, 44 ± 10 years) with ECD screening positive for chronic cerebrospinal venous insufficiency due to nonmobile jugular leaflets, 15 patients were excluded from the initial cohort because they did not meet the inclusion and exclusion criteria. Of the remaining 41 patients, 27 patients (14 male, 13 female; mean age, 48 ± 7 years) underwent endophlebectomy and autologous vein patch angioplasty. Omohyoid muscle section was performed when appropriate. The control group comprised 14 patients matched by age and gender (8 male, 6 female; mean age, 44 ± 11 years) who were not treated. Comorbidity was multiple sclerosis without significant differences in relapsing remitting (RR) and secondary progressive (SP) clinical course among groups. In the control group, neither ECD nor SPECT-CT showed any significant changes at follow-up. On the contrary, in the group operated on, the collateral flow index went from 70% to 30% (P < .0003) thanks to improved flow through the internal jugular vein. Correspondingly, ventricle volume dramatically decreased in the treated group (from 34 ± 14 cm(3) to 31 ± 13 cm(3); P < .01). The effect was much more evident in the RR subgroup (P = .009), whereas in the SP subgroup, it was not significant. Perfusion was found to be improved in the surgical group with respect to controls, particularly in the occipital and parietal regions of the RR subgroup (P < .0001 and P = .017, respectively), but not in the SP subgroup. The probability of reducing ventricle size is increased by 13-fold (P < .03) when restoration of the jugular flow achieves a postoperative collateral flow index ≤20%. Finally, the 18-month patency rate was 74%. CONCLUSIONS: Fixing the flow in the jugulars in patients with chronic cerebrospinal venous insufficiency might significantly reduce brain ventricle volume and improve cerebral perfusion. These changes are more evident in patients in the earlier stages of neurodegenerative disease.

Clinical Applicability of Assessment of Jugular Flow over the Individual Cardiac Cycle Compared with Current Ultrasound Methodology.

There is growing interest in measuring cerebral venous outflow with ultrasound (US). However, results obtained with the current US Doppler methodology, which uses just a single value of cross-sectional area (CSA) of the vessel, are highly variable and inconclusive. The product of CSA and time-averaged velocity in the case of pulsatile vessels may be a possible source of error, particularly for a pulsatile vein like the internal jugular vein (IJV), where the cardiac pump transmits a sequence of well-established waves along the conduit. We herein propose a novel technique for US IJV flow assessment that accurately accounts for IJV CSA variations during the cardiac cycle. Five subjects were investigated with a high-resolution real-time B-mode video, synchronized with an electrocardiography trace. In this approach, CSA variations representing the pulsatility of the IJV are overlapped with the velocity curve obtained by the usual spectral Doppler trace. The overlap is then phased point by point using the electrocardiography pacemaker. This allows us to experimentally measure the velocity variation in relation to the change in CSA precisely, ultimately enabling calculation of IJV flow. (i) The sequence of CSA variation with respect to the electrocardiography waves corresponds exactly to the jugular venous pulse as measured in physiology. (ii) The methodology permits us to phase the velocity and CSA, which is ultimately what is currently lacking to precisely calculate the flow in the IJV with US. (iii) The time-averaged flow, calculated with the described technique, is very close to that calculated assuming a constant IJV CSA, whereas the time-dependent flow shows differs as much as 40%. (iv) Finally, we tested the accuracy of the technique with a methodology that may allow for universal assessment of the accuracy of each personal US-based evaluation of flow rate.

The Oscillating Component of the Internal Jugular Vein Flow: The Overlooked Element of Cerebral Circulation.

The jugular venous pulse (JVP) provides valuable information about cardiac haemodynamics and filling pressures and is an indirect estimate of the central venous pressure (CVP). Recently it has been proven that JVP can be obtained by measuring the cross-sectional area (CSA) of the IJV on each sonogram of an ultrasound B-mode sonogram sequence. It has also been proven that during its pulsation the IJV is distended and hence that the pressure gradient drives the IJV haemodynamics. If this is true, then it will imply the following: (i) the blood velocity in the IJV is a periodic function of the time with period equal to the cardiac period and (ii) the instantaneous blood velocity is given by a time function that can be derived from a flow-dynamics theory that uses the instantaneous pressure gradient as a parameter. The aim of the present study is to confirm the hypothesis that JVP regulates the IJV blood flow and that pressure waves are transmitted from the heart toward the brain through the IJV wall.

An ultrasonographic technique to assess the jugular venous pulse: a proof of concept.

The purpose of the work described here was to investigate the feasibility of assessing the jugular venous pulse (JVP) using ultrasound (US) equipment. Three young healthy subjects underwent a B-mode US scan of the internal jugular vein (IJV) to acquire a sonogram sequence in the transverse plane. On each acquired sonogram, the IJV contour was manually traced, and both the cross-sectional area (CSA) and the perimeter were measured. The CSA data set represents the US jugular diagram (USJD). The arterial distension waveform of the subjects was compared with its USJD. The correlation between the CSA and the perimeter was assessed during the cardiac cycle to verify IJV distension. For each subject, a short sonogram sequence of a few seconds was recorded, and the USJD obtained exhibited periodic behavior. Furthermore, for all subjects, the CSA was found to be correlated with the perimeter (Pearson coefficient, R > 0.9), indicating that the IJV in supine position is distended. We compared 390 manually traced contours of the IJV cross-sectional area with corresponding values semi-automatically calculated by an algorithm developed in-house. For all subjects, the sensitivity, specificity and accuracy were around 95%, 85% and 90% respectively. We found that a diagram reflecting the JVP can be obtained by analyzing a B-mode sonogram sequence of the IJV; such a diagram can result in a new methodology to assess the IJV functionality.

Human internal jugular valve M-mode ultrasound characterization.

In humans the mechanism governing the internal jugular vein (IJV) valve opening and closure is still unclear. M-mode is used in echo-cardiology for the heart valves assessment. Sometimes it was performed also in deep peripheral veins and in vena cava assessment, but never in the IJV valve. Aim of the present study is to investigate the IJV valves physiology in healthy volunteers, by means of both B and M-mode ultrasound. Eighty-three (83) healthy volunteers (35 Male, 48 Female, 25.7±6.7 y.o.), for a total of 166 IJVs, were enrolled. The entire cohort underwent IJVs high-resolution B and M-mode evaluation, in standardized postural and respiratory conditions. Presence, motility, and number of cusps, as well as their opening and closure mechanism have been assessed. Bilateral valve absence occurred in 13/83 (16%), whereas at least a one side absence was recorded in 38/83 (46% of the cohort) (p<0.0356). Valve leaflets were always mobile and respectively bi-cusps in 34%, or mono-cusp in 27%. The latter was significantly more frequent on the left side (35%) than on the right side (19%) (p<0.0013). In supine, M-mode valve opening was synchronous with the cardiac cycle. To the contrary, in an upright position, the valve remained always open and saddled to the wall, independently from the cardiac cycle. In healthy subjects, the IJV valve leaflets are always mobile, but the significant rate of mono and bilateral absence could suggest a progressive phylogenetic importance loss of such apparatus. M-mode ultrasound enhances the characterization of IJV valve, for this reason it should be taken into consideration to routinely add it to the cerebral venous return investigation.