Magnetic Resonance Imaging of Dural Sinus Malformation in a Fetus: A Case Report.

BACKGROUND: Dural sinus malformation (DSM) is a rather rare congenital condition that can be encountered in the fetus and infants. The cause and etiology of DSM remain unclear. Obstetric ultrasound plays a key role in screening fetal brain malformations, and MRI is frequently used as a complementary method to confirm the diagnosis and provide more details. OBJECTIVE: Here, we present a fetus with DSM by multiple imaging methods to help better understand the imaging characteristics of this malformation. CASE PRESENTATION: A 22-year-old primipara was referred to our hospital at 25 weeks of gestation following the detection of a fetal intracranial mass without any symptoms. A prenatal ultrasound performed in our hospital at 25 + 2 gestational weeks showed a large anechoic mass with liquid dark space, while no blood flow was detected. After the initial evaluation, this primipara received a prenatal MRI in our hospital. This examination at 25 + 5 gestational weeks delineated a fan-shaped mass in the torcular herophili, which was iso-to hyperintense on T1WI and hypointense on T2WI. At the lower part of this lesion, a quasi-circular hyperintense on T1WI and a signal slightly hyperintense on T2WI could be seen. Meanwhile, the adjacent brain parenchyma was compressed by the mass. CONCLUSION: We reviewed the current literature to obtain a better understanding of the mechanisms, imaging characteristics, and survival status of DSM. Although the primipara of the present study regretfully opted for elective termination of pregnancy, the reevaluation of DSM survival deserves more attention because of the better survival data from recent studies.

Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review.

Dural sinus malformations (DSMs) are rare congenital vascular diseases characterized by a giant venous pouch with or without arteriovenous shunts. We present a neonatal case of DSM that was diagnosed prenatally and treated via endovascular intervention in the early postnatal period. The patient presented with a large DSM involving the torcular Herophilion prenatal magnetic resonance imaging (MRI). Enlargement of the head circumference and respiratory failure rapidly progressed after birth. On the 5th day after birth, the neonate underwent endovascular occlusion via the umbilical artery. The arteriovenous shunt was occluded, and the reflux from the enlarged venous pouch to the dural sinus was decreased. No additional procedure other than ventriculoperitoneal shunting was required. The neonate's development slowly caught up to normal parameters. Follow-up MRI demonstrated the successful development of the venous drainage system. DSMs are characterized by an abnormally dilated dural sinus, which can block the venous return and ultimately increase intracranial pressure and cerebral ischemia. Long-term follow-up indicates that an abnormally developed dural sinus can be reconstructed by appropriate and timely treatment.

Cerebral neurovascular embryology, anatomic variations, and congenital brain arteriovenous lesions.

Cerebral neurovascular development is a complex and coordinated process driven by the changing spatial and temporal metabolic demands of the developing brain. Familiarity with the process is helpful in understanding neurovascular anatomic variants and congenital arteriovenous shunting lesions encountered in endovascular neuroradiological practice. Herein, the processes of vasculogenesis and angiogenesis are reviewed, followed by examination of the morphogenesis of the cerebral arterial and venous systems. Common arterial anatomic variants are reviewed with an emphasis on their development. Finally, endothelial genetic mutations affecting angiogenesis are examined to consider their probable role in the development of three types of congenital brain arteriovenous fistulas: vein of Galen malformations, pial arteriovenous fistulas, and dural sinus malformations.

Associations among Audiometric, Doppler Hydroacoustic, and Subjective Outcomes of Venous Pulsatile Tinnitus.

OBJECTIVE: Venous pulsatile tinnitus (PT) has received increasing attention recently. As analyses of psychophysical and neuropsychological dimensions of venous PT are lacking, this study aimed to quantitatively and qualitatively investigate the correlation among audiometric, hydroacoustic, and subjective outcomes in patients with PT. METHODS: Fifty-five venous PT patients, with or without sigmoid sinus wall anomalies (SSWAs), were subdivided into SSWAs (n = 30) and non-SSWAs (n = 25) groups. Audiometric and hemodynamic evaluations were assessed. Questionnaires including the Tinnitus Handicap Inventory, Hospital Anxiety and Depression Scale (HADS), and Athens Insomnia Scale (AIS) were deployed to evaluate the psychological impacts of PT. RESULTS: Among 55 subjects, PT frequency-related pure-tone audiometry (PTA) was significantly different between ipsilesional non-PT frequency-related PTA (p < 0.01), ipsilateral jugular vein compression PTA (p < 0.01), and contralesional ear PTA (p < 0.01). In contrast with the pulsatility index and flow velocity, bilateral EOET and flow volume were significantly different (p < 0.01). Of the 3 questionnaire types, there was a strong correlation between HADS anxiety and AIS scores (r = 0.658, p < 0.01). The duration of PT was not correlated with subjective outcomes, and there was no statistical significance found among audiometric, hemodynamic, and subjective outcomes between SSWAs and non-SSWAs groups. CONCLUSIONS: (1) The duration of PT was irrelevant to the increase of PTA. (2) Venous PT is the perception of vascular flow sound, in which hydroacoustic characteristics can be highly independent. (3) Anxiety, depression, and sleep disorders commonly prevail among PT patients.

Study on the correlation between hemodynamic status of the transverse sinus-sigmoid sinus and the clinical efficacy of sigmoid sinus wall reconstruction.

BACKGROUND AND PURPOSE: For patients with pulsatile tinnitus who have both transverse sinus stenosis and sigmoid sinus wall anomalies, sigmoid sinus wall reconstruction surgery is the first-choice treatment when the trans-stenotic pressure gradient less than 10 mmHg. However, not all patients are cured by surgery. We hypothesized the abnormal hemodynamics caused by transverse sinus stenosis is associated with the clinical efficacy of surgery. METHODS: Eight pulsatile tinnitus patients treated with surgery were retrospectively reviewed (4 rehabilitated, 4 nonrehabilitated). All patients had radiologically diagnosed transverse sinus stenosis and sigmoid sinus wall anomalies. A numerical simulation of the hemodynamics of the transverse sinus-sigmoid sinus was performed using computational fluid dynamics technology. Changes in the blood flow patterns before and after surgery were observed. The blood flow velocity at the stenosis, vorticity of blood flow in the sigmoid sinus and wall pressure distribution in the sigmoid sinus wall anomalies area were compared. RESULTS: The blood flow velocity in the stenosis (preoperative P = 0.04, postoperative P = 0.004) and vorticity in the sigmoid sinus (preoperative P = 0.02, postoperative P = 0.007) pre- and post-surgery were significantly higher in the non-rehabilitation group than in the rehabilitation group. No significant difference was found in the wall pressure distribution in the sigmoid sinus wall anomalies area (preoperative P = 0.12, postoperative P = 0.24). CONCLUSIONS: There is a clear correlation between the abnormal hemodynamic status caused by transverse sinus stenosis and the clinical efficacy of surgery. The blood flow velocity at the stenosis and vorticity of blood flow in the sigmoid sinus are factors influencing the clinical efficacy of surgery.

Fetal Cerebral Sinovenous Thrombosis and Dural Sinus Malformation.

BACKGROUND: Fetal cerebral sinovenous thrombosis (CSVT) and dural sinus malformation (DSM) are rare types of fetal cerebral venous pathology that are becoming increasingly recognized as fetal imaging advances. Fetal DSMs are a common source of fetal CSVT, although CSVT may occur without a DSM. The literature on these disorders is limited. METHODS: Cases of fetal CSVT and DSM were identified retrospectively through a query of the Indiana University Health fetal imaging archive from 2007 to 2021. RESULTS: Seven cases were identified, all of whom were alive at birth. A DSM was present in six. Treatments after birth included enoxaparin sodium (3), embolization (3), and shunt placements (1). Five cases had documented regression or complete resolution of the thrombus and/or malformation. One was lost to follow-up, one died from complications of hydrocephalus at nine months, one was receiving physical and occupational therapy at last follow-up at three months, one had concern for autism and mild gait abnormality at 21 months, two had concern for speech delay (18 months and 24 months), and one had normal development at most recent follow-up (four years). CONCLUSIONS: Positive short-term outcomes may occur for some cases of fetal CSVT and DSM. However, risk factors and best treatments are not clear, and long-term outcome data are limited. There is a need for further study.

Dural Venous Sinus Variations in Idiopathic Subarachnoid Hemorrhage: A New Indicator of the Venous Origin with Diagnostic Usefulness?

OBJECTIVE: Venous hypertension associated with a primitive basal vein of Rosenthal (BVR) has been noted as the most likely cause of idiopathic subarachnoid hemorrhage (iSAH). Other types of venous drainage variations have been scarcely studied but may further explain the cases not associated with a BVR anomaly. Our aim was to investigate if dural venous sinus (DVS) anomalies are related with iSAH. METHODS: A total of 76 patients diagnosed with iSAH were identified from a prospectively maintained database and their angiographic findings compared with 76 patients diagnosed with aneurysmal subarachnoid hemorrhage. RESULTS: On top of the BVR variations, our data showed a higher prevalence of transverse sinus hypoplasia (47.4% vs. 28.9%; P = 0.019), superior petrosal sinus hypoplasia (32.9% vs. 13.2%; P = 0.003), and clival plexus hyperplasia (65.8% vs. 43.4%; P = 0.005) in patients with iSAH. Analyzing by total number of angiograms, the iSAH group showed also a higher prevalence of inferior petrosal sinus hyperplasia (36.2% vs. 25%; P = 0.003). Of the patients with iSAH without a primitive BVR, 84% harbored ≥1 perimesencephalic DVS variation and the overall number of venous drainage variations was significantly higher in patients with iSAH. CONCLUSIONS: In addition to the well-documented BVR anomalies, there seems to be a significant relationship of other DVS variations in patients with iSAH. Transverse sinus hypoplasia, superior petrosal sinus hypoplasia, inferior petrosal sinus hyperplasia, and clival plexus hyperplasia were significantly more frequent in patients with iSAH. The presence of ≥3 of those variations would increase the suspicion of a nonaneurysmatic subarachnoid hemorrhage and could help avoid a second angiogram.

Successful endovascular therapy for an adolescent patient with cerebral venous sinus thrombosis: A case report.

RATIONALE: Cerebral venous sinus thrombosis associated with protein S deficiency is rare in adolescent patients and has high disability and fatality.Patient concerns: A 15-year-old male student presented in the hospital with sudden headache, nausea, and vomiting and was diagnosed with protein S deficiency by gene testing. DIAGNOSES: Cerebral venous sinus thrombosis due to protein S deficiency was diagnosed in this adolescent patient, who underwent successful endovascular therapy (EVT). INTERVENTIONS: The patient was treated with standard anti-coagulation therapy including low-molecular-weight heparin (90 IU/kg/Q12 h) and dehydrant (mannitol 125 mL Q8 h); however, the symptoms were not alleviated. Successful EVT was implemented. OUTCOMES: Both the superior sagittal sinus and bilateral transverse sinus were recanalized after thrombus clearance. The patient achieved a complete recovery without any other stroke recurrence during follow-up. LESSONS: EVT can be performed with favorable and effective clinical outcomes in adolescent cerebral venous sinus thrombosis patients with protein S deficiency. EVT associated with standard anti-coagulation therapy may improve the prognosis and reduce mortality among such patients.

Hydroacoustic analysis and extraluminal compression surgical insights of venous pulsatile tinnitus.

OBJECTIVE: This study aimed to quantitatively and qualitatively evaluate the hydroacoustic changes from "presence" to "disappearance" of pulsatile tinnitus (PT) with the extraluminal compression surgical technique. The recent issues of concern pertaining to the hydroacoustic characteristics of sigmoid sinus wall anomalies and distal transverse sinus stenosis (dTSS) were discussed. METHODS: This study was based on a retrospective case series. Seventy-seven patients with PT and transverse-sigmoid sinus enlargement with or without transverse-sigmoid sinus junction anomalies and transverse sinus stenosis (TSS) who had undergone extraluminal compression surgery under local anesthesia were included. Management of intractable intraoperative challenges and techniques for reversal extraluminal compression were introduced. Anatomical measurements, intraoperative color-coded Doppler ultrasonography, spectro-temporal analysis, and computational fluid dynamics were employed to analyze the hydroacoustic characteristics of PT. RESULTS: The efficacy of the extraluminal compression technique was evident with the significant reduction in peak turbulent kinetic energy, vorticity, and mean pressure gradient at the transverse-sigmoid junction, resulting in over 20% reduction in PT amplitude. dTSS is a common finding in patients with PT exhibiting transverse-sigmoid sinus enlargement. Patients with dTSS presented with significant differences in hemodynamic characteristics as compared to those without. Linear regression analysis showed that the flow disturbance (turbulent kinetic energy and vorticity) was closely associated with the degree of dTSS, whereas the flow amplitude was not related to the degree or location of TSS. Low-pulsatory vortex flow at the transverse-sigmoid junction was visualized during an intraoperative color-coded Doppler examination, and the displayed low-frequency PT sound corresponded to the patients' subjective perception of PT. CONCLUSION: (1) A reduction of over 20% of the flow-induced noise is the therapeutic goal of extraluminal compression technique. Since reductions in the magnitude of hemodynamic parameters, including turbulent kinetic energy, vorticity, and mean pressure gradient, render the flow-induced noise inaudible, besides sigmoid sinus wall anomalies, it is likely that PT develops from the aggregation of flow-based pathologies. (2) Although dTSS and diverticulum may greatly affect the hemodynamics at the transverse-sigmoid junction, in contrast to dehiscence, dTSS and diverticulum may not be the limiting factors for PT development.

Transmastoid Hydroxyapatite Resurfacing for Sigmoid Sinus Wall Anomalies Causing Pulsatile Tinnitus.

OBJECTIVE: Comprising 4% of tinnitus, pulsatile tinnitus (PT) can be particularly difficult for affected patients as well as surgeons looking to address their symptoms. Often the cause is not identified but can be secondary to turbulent flow in or near the sigmoid sinus, particularly if there is an identifiable sigmoid sinus dehiscence (SSDe) and/or diverticulum (SSDi). These sigmoid sinus wall anomalies (SSWA) may be treated with transmastoid sigmoid sinus resurfacing; however, this intervention remains relatively novel and its technique, materials used, resolution success, and complications need to be continuously reviewed. METHODS: A retrospective case series of patients with PT due to SSWA at a tertiary referral center was reviewed. A total of 6 patients (7 ears) treated by transmastoid resurfacing using hydroxyapatite (HA) were retrospectively assessed. Pre-operative demographics and symptoms, pre- and post-operative hearing results, and post-operative outcomes were reviewed. RESULTS: All patients were female with an average BMI of 32.9 (±5.4) and a mean age of 45.5 years (±15.3). Mean follow-up was 648 days. Objective tinnitus was noted in all ears with SSDi (100%); however, no objective tinnitus was noted with purely SSDe. In 100% of ears, PT was diminished with ipsilateral jugular compression and was amplified with contralateral head turn. Pre-operative symptoms of PT resolved in all patients, but delayed recurrence (>1 year) occurred in 1 ear (14%). No patient had pre- or post-operative hearing loss. No major complications were encountered. CONCLUSIONS: Transmastoid resurfacing for SSWA with HA bone cement is a safe, reliable intervention in properly identified PT patients.

Two cases of persistent falcine and occipital sinuses.

BACKGROUND: The coexistence of falcine and occipital sinuses is rare and its natural course has not been reported. CASE REPORTS: Two patients with persistent falcine and occipital sinuses are described. Both patients had straight sinuses. In one, both the transverse and sigmoid sinuses were hypoplastic and the patient had an acquired Chiari I malformation. The other patient had no other venous anomalies and had a normal posterior cranial fossa. CONCLUSION: The coexistence of falcine and occipital sinuses can lead to an acquired Chiari I malformation. These cases suggest the importance of checking other venous and brain anomalies in this situation.

Association of pulse synchronous tinnitus and sigmoid sinus wall abnormalities in patients with idiopathic intracranial hypertension.

PURPOSE: Pulse synchronous tinnitus (PT) is common in patients with idiopathic intracranial hypertension (IIH) and in those with sigmoid sinus wall abnormalities (SSWAs). Although patients with SSWAs and IIH share many clinical features, the incidence of SSWAs in patients with IIH and its relationship to PT in this cohort is less well established. The purpose of this study is to assess the incidence of SSWAs in patients with IIH and PT, and to determine if there is an association between SSWAs and PT in this population. MATERIALS AND METHODS: Prospective computed tomography (CT) study of adults with IIH. Subjective PT was correlated with presence or absence of SSWAs on CT. RESULTS: 22 subjects were enrolled and 14 subsequently underwent CT. The incidence of SSWAs was significantly higher in subjects with PT than without (70% vs. 0%, p = 0.02). Mean age, BMI and opening pressures did not differ between those with and without SSWAs or PT. CONCLUSIONS: There is a high incidence of SSWAs in subjects with IIH and PT. These findings support an association between SSWAs and PT, and implicate SSWAs as a possible cause of, or contributing factor to, PT in patients with IIH. Patients with IIH and PT that does not resolve with reducing intracranial pressure should undergo diagnostic CT and consider treatment of a SSWA if present.

Patterns of audiometric threshold shifts from pulsatile tinnitus due to sigmoid sinus wall anomalies.

PURPOSE: To determine the severity and nature of audiometric threshold shifts for patients with pulsatile tinnitus (PT) due to sigmoid sinus wall anomalies (SSWA). MATERIALS AND METHODS: 38 patients with SSWAs and available pre-operative audiograms were examined. Low- and high-frequency pure tone averages (LF-PTA, HF-PTA) were calculated. Audiometric data were compared between affected and unaffected ears, with the interaural difference (affected-unaffected PTA) representing the change in hearing due to PT. Additionally, post-operative change was examined in 14 patients with available data. RESULTS: The average pre-operative air conduction (AC) LF-PTA was 17.04 dB on the affected side and 11.38 dB on the unaffected side (p < 0.001). The mean AC HF-PTA was significantly higher on the affected side as well (16.45 dB vs. 14.08 dB, p = 0.008). All shifts were sensorineural, with no significant air-bone gaps, and most subjects still had low-frequency thresholds in the normal range. Though the post-op change was not significant due to attrition, 5/14 patients (35.7%) had complete resolution of their pre-op interaural difference. A similar number developed a HF-PTA post-op threshold elevation in the surgical ear. CONCLUSIONS: PT due to SSWAs causes a mean 6 dB low-frequency bone-conduction threshold elevation, and smaller high-frequency threshold shifts, due to masking. Patients with larger threshold shifts should have other potential causes of hearing loss explored. LEVEL OF EVIDENCE: Level IV.

Evolution of Developmental Venous Anomalies in the Setting of a Torcular Dural Arteriovenous Fistula and Cerebrofacial Venous Metameric Syndrome.

BACKGROUND: We describe evolution of a developmental venous anomaly (DVA) over time in a patient with a complex intracranial vascular malformation. CASE DESCRIPTION: A 26-year-old male patient initially presented with a scalp vascular malformation and was later diagnosed to have a torcular dural arteriovenous fistula resembling a dural sinus malformation. The dural fistula increased in size over 4 years. The dural fistula also was associated with multiple complex developmental venous anomalies draining the bilateral cerebral hemispheres and cerebellum. The DVA was only faintly demonstrated on the baseline magnetic resonance imaging but appeared to increase in size and extent over time as the dural arteriovenous fistula developed more aggressive angioarchitecture features. In addition to the evolution manifestation of the DVAs, the patient developed multiple de novo cavernous malformations in the venous radicles of the DVA. Increased venous hypertension in the superficial venous system from the dural fistula likely resulted in growth of the DVAs, as they served as the primary means of venous drainage for the bilateral cerebral hemispheres. The patient also had reopening of the persistent falcine sinus, which was not present at baseline. CONCLUSIONS: This would be the first reported case of growth or evolution of a DVA in association with a dural arteriovenous fistula in an adult patient and highlights the dynamic nature of both the medullary venous and dural venous sinuses of the cerebral venous system, even into adulthood.

Malformación congénita de seno dural con resolución espontánea / Congenital malformation of dural sinus that resolved spontaneously

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Different normal anatomical variations of the transverse dural sinus in magnetic resonance venography (MRV): do age and sex matter?

The anatomical variations of the intracranial venous dural sinuses must be put in consideration in diagnosing magnetic resonance venography (MRV) to avoid the diagnostic pitfalls resulting from over-diagnosis of cerebral venous dural sinus occlusion or thrombosis. The available data regarding the age and sex difference of the magnetic resonance venography (MRV) anatomical variations is still limited. A retrospective study is done for 500 patients ranging from 20 to 70 years. Only 363 patients (142 males and 221 females) were included in our final analysis: all have normal MRI brain & posterior fossa. Magnetic resonance venography (MRV) is done to detect the presence or absence of the transverse venous dural sinuses and to detect any age-or sex-related differences. Also 64 dry Egyptian skulls (41 males and 23 females) were employed to detect symmetry of transverse sulcus and to determine age and sex difference. Hypoplastic left transverse sinus was by far the commonest asymmetrical transverse sinus variants representing 22.0% of total: it was noted in 38 male and 38 female. Even if the asymmetrical transverse sinus is more common in females, there is no significant difference between both genders. In the dry skull, symmetrical transverse sulcus was observed in 67.2% of total, while asymmetrical transverse sulcus was recorded in 32.8% of total, which were more observed in female skull 17.2% of total with no significant difference

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