Torcular dural sinus malformation

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.

Wasp controls oriented migration of endothelial cells to achieve functional vascular patterning

Endothelial cell migration and proliferation are essential for the establishment of a hierarchical organization of blood vessels and optimal distribution of blood. However, how these cellular processes are quantitatively coordinated to drive vascular network morphogenesis remains unknown. Here, using the zebrafish vasculature as a model system, we demonstrate that the balanced distribution of endothelial cells as well as the resulting regularity of vessel caliber, is a result of cell migration from veins towards arteries and cell proliferation in veins. We identify the Wiskott-Aldrich Syndrome protein (WASp) as an important molecular regulator of this process and show that loss of coordinated migration from veins to arteries upon wasb depletion results in aberrant vessel morphology and the formation of persistent arteriovenous shunts. We demonstrate that WASp achieves its function through the coordination of junctional actin assembly and PECAM1 recruitment and provide evidence that this is conserved in human. Overall, we demonstrate that functional vascular patterning in the zebrafish trunk is established through differential cell migration regulated by junctional actin, and that interruption of differential migration may represent a pathomechanism in vascular malformations.

TIMolol nasal spray as a treatment for epistaxis in hereditary hemorrhagic telangiectasia (HHT) – study protocol of the prospective, randomized, double-blind, controlled cross-over TIM-HHT trial

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an inherited orphan disease, in which the absence of capillary beds between arterioles and venules lead to arteriovenous shunts. Epistaxis is the core symptom. Several case reports have described the nonselective beta-adrenergic receptor antagonist timolol as a successful treatment method of nosebleeds due in HHT patients. OBJECTIVE: TIM-HHT is a single-site, prospective, randomized, placebo-controlled, double-blind, cross-over study to investigate whether the efficacy of standard laser treatment of epistaxis in HHT patients can be increased by the additional use of timolol nasal spray (1 mg/d). METHODS: Twenty patients will be randomly allocated to one of two treatment sequences. Primary outcome is the severity of epistaxis determined by the Epistaxis Severity Score (ESS). Secondary outcomes are subjective satisfaction, quality of life, as well as the hemoglobin, ferritin, and transferrin levels of the participating patients. Safety outcome is assessed by means of pulse, blood pressure, and adverse events. CONCLUSION: TIM-HHT will evaluate the efficacy and safety of timolol as an additional treatment of epistaxis in HHT patients in a three-month trial period. Benzalkonium chloride is used as a placebo, which has no documented positive effect on the nasal mucosa and hence on epistaxis in HHT patients (in contrast to saline). TRIAL REGISTRATION: German Clinical Trials Register (DRKS), DRKS00020994. Registered on 10 March 2020

Endovascular Arteriovenous Fistula Creation: A Review

Endovascular arteriovenous fistula (endoAVF) creation offers a minimally invasive method for the formation of arteriovenous shunts utilized for hemodialysis. Currently, there exist two similar yet unique devices: WavelinQ and Ellipsys. This review analyzes the anatomy, pre- and postoperative considerations, fistula creation methods, and outcomes associated with endoAVF. Currently, data are limited with regard to clinical efficacy of endoAVFs when compared to surgical AVFs. However, early data suggest endoAVFs are a useful technique in the creation of AVFs.

Abstract 1122‐000146: A Survey of Current Status and Roles of Interventional Neurologists in Japan

Introduction : In Japan, there are more board‐certified neurosurgeons than board‐certified neurologists, and a significant part of stroke practice is provided by neurosurgeons. In neuroendovascular therapy practice, the trend of neurosurgeons to be in majority is more pronounced. The most of neuroendovascular therapy specialists (n = 1,586) certified by the Japanese Society for Neuroendovascular Therapy (JSNET) consists of neurosurgeons, and the proportion of neurologists/internists is only 8% (n = 128) as of April 2021. The aim of this study is to investigate the current status and roles of interventional neurologists, the minority providing neuroendovascular therapy, in Japanese clinical setting. Methods : Between 16th and 28th February 2021, the Japan Society of Vascular and Interventional Neurology (JSVIN) conducted a survey for society‐member neurologists and internists using questionnaires on Google Forms. The questionnaires consisted of 11 items regarding years after graduation, facility location, department, work style (full‐time or concurrent interventionalist), diseases for which respondents were engaged, disease/procedure preferences which respondents would treat by themselves, respondents’ roles in their own neuroendovascular therapy team, and others. Results : Replies were obtained from 112 (67.1%) out of all JSVIN‐member neurologists and internists (n = 167). The respondents included 71 JSNET‐certified specialists and they consisted of 56.3% of all the JSNET‐certified neurologists/internists. The departments to where the respondents belonged were Neurology in 66%, Cerebrovascular medicine in 25%, Neuroendovascular therapy in 4%, and Neurosurgery in 3%. The median years after graduation was 15 years (interquartile range, 10 – 21 years) and the proportion of respondents who have graduated 10 years or less was 30%. Respondents’ facilities were distributed around 27 prefectures of all 47 Japanese prefectures and a significant proportion of those was located in urban area; 20% in Osaka and 13% in Tokyo. The number of full‐time interventionalist was only 1. Sixty‐three (56%) were concurrently engaged in general neurology practice, and 33 (30%) in stroke neurology practice. The proportions of diseases for which the respondents were engaged were ischemic cerebrovascular diseases (acute large vessel occlusion strokes, carotid stenoses, and others) in 100%, hemorrhagic cerebrovascular diseases (cerebral aneurysms, arteriovenous shunts, and others) in 44%, and other diseases (tumors, spinal vascular disorders, and others) in 28%. The proportions of disease/procedure preferences which respondents would treat by themselves were acute stroke thrombectomy in 90%, carotid/intracranial stenoses in 87%, ruptured cerebral aneurysms in 38%, unruptured cerebral aneurysms in 31%, cerebral arteriovenous shunts in 33%, brain tumors in 29%, spinal vascular disorders in 13%, and pediatric diseases in 3%. Respondents’ roles in their own neuroendovascular therapy team were diagnostic performances based on neurologist’s skills in 89%, comorbidity assessment and management based on internist’s skills in 88%, precise neurological evaluation in 77%, neurosonological evaluation in 75%, and establishments of in‐hospital workflow/multi‐disciplinary collaboration in 71%. Conclusions : Most of interventional neurologists in Japan were engaged in neuroendovascular therapy mainly for ischemic cerebrovascular diseases in parallel with general neurology and/or stroke neurology practices. Interventional neurologists’ skill set developed in neurology/internist trainings and practices might contribute to the quality improvement of neuroendovascular therapy in Japan.

Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease

Hereditary hemorrhagic telangiectasia (HHT; Rendu-Osler-Weber syndrome) affects the capillary and larger vessels, leading to arteriovenous shunts. Epistaxis is the main symptom impairing quality of life. The aim of the Osler Calendar is to offer information about the extent of the systemic disease and the current state of treatment. A care plan with information on the rare disease and self-treatment of epistaxis was created. Organ examinations and ongoing treatments were recorded. A questionnaire documents the treatment success, including patient satisfaction, frequency of hemorrhage and hemoglobin levels. The patients using the Osler Calendar for at least one year (n = 54) were surveyed. Eighty-five percent of patients (n = 46) used the calendar to gain information about HHT. Seventy-two percent (n = 39) used the Osler Calendar for instructions on the self-treatment of nosebleeds. The calendar increased patients’ understanding for the need for organ screenings from 48% (n = 26) to 81% (n = 44). Seventy-nine percent (n = 43) of patients confirmed that the Osler Calendar documented their therapeutic process either well or very well. Fifty-two percent (n = 28) saw an improvement in the therapeutic process due to the documentation. The Osler Calendar records the individual intensity of the disease and facilitates the communication between attending physicians. It is a tool for specialists to review treatment strategies. Furthermore, the calendar enhances patients’ comprehension of their condition.

Incidence and clinical characteristics of spinal arteriovenous shunts: hospital-based surveillance in Okayama, Japan

OBJECTIVE There have been no accurate surveillance data regarding the incidence rate of spinal arteriovenous shunts (SAVSs). Here, the authors investigate the epidemiology and clinical characteristics of SAVSs. METHODS The authors conducted multicenter hospital-based surveillance as an inventory survey at 8 core hospitals in Okayama Prefecture between April 1, 2009, and March 31, 2019. Consecutive patients who lived in Okayama and were diagnosed with SAVSs on angiographic studies were enrolled. The clinical characteristics and the incidence rates of each form of SAVS and the differences between SAVSs at different spinal levels were analyzed. RESULTS The authors identified a total of 45 patients with SAVSs, including 2 cases of spinal arteriovenous malformation, 5 cases of perimedullary arteriovenous fistula (AVF), 31 cases of spinal dural AVF (SDAVF), and 7 cases of spinal epidural AVF (SEAVF). The crude incidence rate was 0.234 per 100,000 person-years for all SAVSs including those at the craniocervical junction (CCJ) level. The incidence rate of SDAVF and SEAVF combined increased with advancing age in men only. In a comparative analysis between upper and lower spinal SDAVF/SEAVF, hemorrhage occurred in 7/14 cases (50%) at the CCJ/cervical level and in 0/24 cases (0%) at the thoracolumbar level (p = 0.0003). Venous congestion appeared in 1/14 cases (7%) at the CCJ/cervical level and in 23/24 cases (96%) at the thoracolumbar level (p < 0.0001). CONCLUSIONS The authors reported detailed incidence rates of SAVSs in Japan. There were some differences in clinical characteristics of SAVSs in the upper spinal levels and those in the lower spinal levels.

Umbilical vessel catheter retro-exchange technique (U-RET) for repeat use of the umbilical artery for neonatal vascular intervention: Technical note

A high flow arteriovenous shunts in newborns may require urgent endovascular treatment right immediately after delivery if high output cardiac failure is resistant to medical treatment. The umbilical approach is often the first choice of the access route for endovascular treatment in the newborn. It is, however, not infrequent that the patient has an extensive lesion, which necessitates a second session of treatment because of the limitation of the usable amount of the contrast material in one session. In such a case, re-puncturing the femoral artery is difficult and carries the risk of leg ischemia. On the other hand, leaving the umbilical sheath for the second procedure carries risks of infection, thrombosis, and vessel injury. Herein we introduce our umbilical vessel catheter (UVC) retro-exchange technique (U-RET) in which we replace the umbilical sheath to a 3.5Fr UVC at the end of the first endovascular procedure to preserve the umbilical artery access and prepare for the repeated use. We believe that this method minimizes the risks of infection and vessel injury.

Traumatic cervical vertebral artery aneurysm associated with suicidal stabs

Background: Cervical vertebral artery (VA) aneurysm occasionally develops in association with penetrating injury. However, its treatment strategy is not yet determined. Case Description: A 50-year-old woman with bipolar disorder attempted suicide by stabbing herself in the lateral neck. At presentation, focal neurological deficits were not observed. Spinal computed tomography (CT) showed unclear delineation of the VA in the right C4/5 intervertebral foramen. CT performed 7 days later identified an aneurysm of the right VA at C4/5, with abnormal arteriovenous shunts between the aneurysm and paravertebral venous plexus. The patient underwent coil embolization of the VA segment involving the aneurysm on the same day that was complicated by cerebellar ataxia due to procedure-associated infarction. Conclusion: Traumatic VA aneurysms associated with penetrating injuries should be carefully managed with a detailed presurgical evaluation of the relevant cranial and spinal structures.

Natural History and Clinical Outcomes of Paravertebral Arteriovenous Shunts

Background and Purpose: Paravertebral arteriovenous shunts (PVAVSs) are rare. Whether the intradural venous system is involved in drainage may lead to differences in clinical characteristics through specific pathophysiological mechanisms. This study aims to comprehensively evaluate the natural history and clinical outcomes of PVAVSs with or without intradural drainage. Methods: Sixty-four consecutive patients with PVAVSs from 2 institutes were retrospectively reviewed. Lesions were classified as type A (n=28) if the intradural veins were involved in drainage; otherwise, they were classified as type B (n=36). The clinical course from initial presentation to the last follow-up was analyzed. Results: The patients with type A shunts were older at presentation (52.5 versus 35.5 years, P <0.0001) and more likely to have lower spinal segments affected than patients with type B PVAVSs (67.8% versus 13.9%, P =0.00006). After presentation, the deterioration rates related to gait and sphincter dysfunction were significantly higher in patients with type A than type B shunts (gait dysfunction: 71.8%/y versus 17.0%/y, P =0.0006; sphincter dysfunction: 63.7%/y versus 11.3%/y, P =0.0002). According to the angiogram at the end of the latest treatment, 79% of type A and 75% of type B PVAVSs were completely obliterated. If the lesions were partially obliterated, a significantly higher clinical deterioration rate was observed in patients with type A shunts than those with type B shunts (69.9%/y versus 3.2%/y, P =0.0253). Conclusions: Type A PVAVSs feature rapid progressive neurological deficits; therefore, early clinical intervention is necessary. For complex lesions that cannot be completely obliterated, surgical disconnection of all refluxed radicular veins is suggested.