Upright CT-based evaluation of the effects of posture on skull-base reconstruction after endoscopic endonasal surgery.

Cerebrospinal fluid (CSF) leakage is a common complication associated with endoscopic endonasal skull-base surgery (EESBS). Postoperative mobilization-associated postural changes are considered to cause CSF leakage. However, no study has demonstrated a robust relationship between postural changes and CSF leakage. We used upright computed tomography (CT) to clarify the effects of postural changes on the reconstructed skull base (RSB) after EESBS. Thirty patients who underwent EESBS at our institution were prospectively included, and their upright and supine CTs were compared to measure morphological changes in the RSB. Patient clinical data were also collected from medical charts and surgical videos, and their relationships with morphological changes were assessed. In upright CTs, the RSB shifted intracranially by 0.94 (0.0-2.9) mm on average. This shift was larger in cases with lesions extending to the sphenoid sinus, dural defects, intraoperative pulsation of the RSB, and large bone windows. The direction of the change was opposite to intuitive movement driven by gravity because of reduced intracranial pressure in the sitting position. Thus, these shifts can be directly associated with postoperative CSF leakage caused by reconstruction material displacement. Skull-base reconstruction and postoperative postural management accounting for these morphological changes may be necessary for preventing CSF leakage.

Vasa vasorum: The role in intracranial physiology and pathophysiology.

Background: Vasa vasorum (VVs) is a Latin word representing vessels of vessels. VVs are usually found on the adventitia of the parent vessel and infrequently reach the media and intima, depending on the size and type of the parent vessels and physiological and pathological conditions. The VVs include arteries, capillaries, veins, and lymphatic vessels, involving the oxygenation and nourishment of the vessel's wall to sustain its healthy state. Accumulated studies have revealed that VVs are involved in various intracranial lesions, including atherosclerotic diseases, aneurysms, and shunt diseases. The current review aims to review and integrate past and recent findings and knowledge on VVs and to facilitate our understanding of VVs and intracranial pathology involving VVs. Methods: A literature review was carried out with a focus on the role of VVs by searching the Pubmed database. Results: We identified 71 articles that discuss the role of VVs. We discussed the anatomical structure, physiological significance, and pathological significance of the VV. Conclusion: VV is not only involved in the nutrition and metabolism of the vascular wall but is also deeply involved in the pathogenesis of inflammation, ischemia, and thrombosis of the vascular wall. In addition, in the central nervous system, intracranial vascular wall nutrient particularities and VVs are closely related to the pathogenesis of cerebral aneurysms, subarachnoid hemorrhage, arteriovenous shunt disease, atherosclerotic lesions, and other conditions.

[Inferior Petrosal Sinus and Anterior Condylar Confluence and Surrounding Venous Anatomy].

Veins at the craniocervical junction are complex network structures. They empty into two main brain venous drainages, the internal jugular vein and internal vertebral venous plexus, and reroute venous blood according to postural changes. They are also involved in the etiology of dural arteriovenous shunts in this region. Hence, regional venous anatomy is crucial for interventional neuroradiologists to understand the pathophysiology and formulate therapeutic strategies. This article aims to provide a summary on venous anatomy, radiological findings, and related pathological conditions, especially for young and inexperienced interventional neuroradiologists.

Exploration of postural effects on the external jugular and diploic venous system using upright computed tomography scanning.

PURPOSE: Few studies have investigated the influence of posture on the external jugular and diploic venous systems in the head and cranial region. In this study, we aimed to investigate the effects of posture on these systems using upright computed tomography (CT) scanning. METHODS: This study retrospectively analysed an upright CT dataset from a previous prospective study. In each patient, the diameters of the vessels in three external jugular tributaries and four diploic veins were measured using CT digital subtraction venography in both supine and sitting positions. RESULTS: Amongst the 20 cases in the original dataset, we eventually investigated 19 cases due to motion artifacts in 1 case. Compared with the supine position, most of the external jugular tributaries collapsed, and the average size significantly decreased in the sitting position (decreased by 22-49% on average). In contrast, most of the diploic veins, except the occipital diploic veins, tended to increase or remain unchanged (increased by 12-101% on average) in size in the sitting position compared with the supine position. However, the changes in the veins associated with this positional shift were not uniform; in approximately 5-30% of the cases, depending on each vein, an opposite trend was observed. CONCLUSION: Compared to the supine position, the contribution of external jugular tributaries to head venous drainage decreased in the sitting position, whilst most diploic veins maintained their contribution. These results could enhance our understanding of the physiology and pathophysiology of the head region in upright and sitting positions.

Aneurysmal subarachnoid hemorrhage occurring during sleep: Clinical characteristics and risk factors.

BACKGROUND: Aneurysmal subarachnoid hemorrhage (aSAH) is known to be triggered by several specific human activities. Sleep, by contrast, has not been considered a triggering activity for aSAH, and clinical characteristics of patients who sustain aSAH during sleep have rarely been reported in the literature. METHODS: This is a retrospective analysis on the data acquired through a multicenter aSAH registry. Between January 2019 and December 2021, a total of 732 aSAH patients had been registered into our database. After excluding 109 patients whose activities at aSAH onset had been unidentifiable, the remaining 623 aSAH patients were dichotomized to 59 patients who sustained aSAH during sleep (Sleep group) and 564 patients who sustained aSAH during daytime activities (Awake group). Two-group comparison of demographic variables and multivariate logistic regression analysis were performed to clarify their clinical characteristics and identify potential risk factors. RESULTS: The Sleep group exhibited significantly higher frequencies of diabetes (15.5 % vs. 6.4 %, p = 0.01) and antiplatelet use (13.8 % vs. 4.6 %, p=0.004) than the Awake group. Furthermore, multivariate logistic regression analysis showed that diabetes (OR, 3.051; 95 % CI, 1.281-7.268; p = 0.012) and antiplatelet use (OR, 3.640; 95 % CI, 1.422-9.316; p = 0.007) were correlated with aSAH occurring during sleep. There were no significant inter-group differences in the patient outcomes evaluated at discharge. CONCLUSION: The current results indicate that risk factors may exist for aSAH occurring during sleep. Further investigations on how comorbidities such as diabetes, antiplatelet use and sleep apnea affect human hemodynamic and hemostatic parameters during sleep is warranted to better understand those relationships.

Detailed Anatomy of Bridging Veins Around the Foramen Magnum: a Multicenter Study Using Three-dimensional Angiography.

BACKGROUND AND PURPOSE: There has been limited literature regarding the bridging veins (BVs) of the medulla oblongata around the foramen magnum (FM). The present study aims to analyze the normal angioarchitecture of the BVs around the FM using slab MIP images of three-dimensional (3D) angiography. METHODS: We collected 3D angiography data of posterior fossa veins and analyzed the BVs around the FM using slab MIP images. We analyzed the course, outlet, and number of BVs around the FM. We also examined the detection rate and mean diameter of each BV. RESULTS: Of 57 patients, 55 patients (96%) had any BV. The median number of BVs was two (range: 0-5). The BVs originate from the perimedullary veins and run anterolaterally to join the anterior condylar vein (ACV), inferior petrosal sinus, sigmoid sinus, or jugular bulb, inferolaterally to join the suboccipital cavernous sinus (SCS), laterally or posterolaterally to join the marginal sinus (MS), and posteriorly to join the MS or occipital sinus. We classified BVs into five subtypes according to the draining location: ACV, jugular foramen (JF), MS, SCS, and cerebellomedullary cistern (CMC). ACV, JF, MS, SCS, and CMC BVs were detected in 11 (19%), 18 (32%), 32 (56%), 20 (35%), and 16 (28%) patients, respectively. The mean diameter of the BVs other than CMC was 0.6 mm, and that of CMC BV was 0.8 mm. CONCLUSION: Using venous data from 3D angiography, we detected FM BVs in most cases, and the BVs were connected in various directions.

Bridging veins at the craniocervical junction: from anatomy to clinical significance in dural arteriovenous shunts.

PURPOSE: Bridging veins (BVs) serve as a route of pial venous reflux, and its anatomy is essential to understand the pathophysiology of dural arteriovenous shunts (dAVSs) around the craniocervical junction (CCJ) (from the jugular foramen level to the atlantal level). However, the anatomical variations of the BVs and their proximal connections remained poorly elucidated. This study aimed to radiologically investigate the anatomy of the bridging veins around CCJ and discuss the clinical significance of these BVs in the dAVS. METHODS: We investigated normal venous anatomy of the BVs from the jugular foramen level to the atlantal level using preoperative computed tomography digital subtraction venography in patients undergoing elective neurosurgery. BVs affected by the dAVSs in the same region were also evaluated. The three types of dAVS, craniocervical junction, anterior condylar, and proximal sigmoid sinus, were investigated. RESULTS: We identified six BV groups: superolateral, anterolateral, lateral, posterior, inferolateral, and inferoposterior. The superolateral and inferolateral groups, connected with the proximal sigmoid sinus and suboccipital cavernous sinus, respectively, were the largest groups. Each group has a specific downstream venous connection. The association with dVASs was observed only in the inferolateral group, which was typically the sole venous drainage in most dAVSs at the CCJ. CONCLUSION: We reported detailed anatomy of BVs from the jugular level to the atlantal level, which enhanced our understanding of the pathophysiology of dAVSs in the corresponding region.

A Case of Neurofibromatosis Type 1 Diagnosed after Idiopathic Rupture of Superficial Temporal Artery Pseudoaneurysm Requiring Endovascular Treatment.

Patients with neurofibromatosis type 1 not only have characteristic skin findings but are also known to have vascular disorders due to vascular vulnerability. A 44-year-old man with previously undiagnosed neurofibromatosis type 1 was brought to the emergency room due to a sudden subcutaneous hematoma with no history of trauma. Angiography revealed extravasation from the parietal branch of the right superficial temporal artery, which was embolized with n-butyl-2-cyanoacrylate. However, the next day, the patient exhibited an increased subcutaneous hematoma, and new extravascular leakage was detected at the frontal branch of the superficial temporal artery, which was also embolized with n-butyl-2-cyanoacrylate. The patient had physical findings characteristic of neurofibromatosis type 1, such as café-au-lait spots, and was subsequently diagnosed with neurofibromatosis type 1. No obvious neurofibroma or any other subcutaneous lesion associated with neurofibromatosis type 1 was identified in the affected area. Massive idiopathic arterial bleeding in the scalp, although infrequent, can be fatal. Neurofibromatosis type 1 should be considered when a subcutaneous scalp hematoma is observed without a history of trauma, even if the facial skin structure appears normal. Neurofibromatosis type 1 is also known to have multiple sources of hemorrhage. Thus, it is important to repeatedly evaluate vascular structures via cerebral angiography, contrast-enhanced computed tomography, and magnetic resonance imaging, if necessary.

"Missing-piece" sign with dural arteriovenous fistula at craniocervical junction: A case report.

OBJECTIVES: Spinal dural arteriovenous fistula (sDAVF) is a rare and often underdiagnosed spinal disease. Early diagnosis is required because the deficits are reversible and delays in treatment cause permanent morbidity. Although the abnormal vascular flow void is a critical radiographic feature of sDAVF, they are not always present. A characteristic enhancement pattern of sDAVF has been recently reported as the "missing-piece" sign which can lead to the early and correct diagnosis. METHODS: We presented imaging findings, treatment decisions, and the outcome of a rare case of sDAVF, in which the "missing-piece" sign appeared atypical. RESULTS: A 60-year-old woman developed numbness and weakness in her extremities. Spinal MRI revealed longitudinal hyperintensity in the T2-weighted image, extending from the thoracic level to medulla oblongata. At first, myelopathy with inflammation or tumor was suspected because of the lack of flow voids and vascular abnormalities in CT-angiography and MR-DSA. However, we administered intravenous methylprednisolone and her symptom got worse with the appearance of the "missing-piece" sign. Then, we successfully diagnosed sDAVF by angiography. The "missing-piece" sign was considered to derive from inconsistency of the intrinsic venous system of spinal cord, with the abrupt segments without enhancement. The same etiology was considered in our case. CONCLUSIONS: Detecting the "missing-piece" sign can lead to the correct diagnosis of sDAVF, even if the sign appeared atypical.

Perfusion gradients promote delayed perihaematomal oedema in intracerebral haemorrhage.

Perihaematomal oedema is a potential therapeutic target to improve outcome of patients with intracerebral haemorrhage, but its pathophysiology remains poorly elucidated. We investigated the longitudinal changes of cerebral perfusion and their influence on perihaematomal oedema development in 150 patients with intracerebral haemorrhage who underwent computed tomography perfusion within 6 h from onset, at 24 h and at 7 days. Perfusion parameters were measured in haemorrhagic core, perihaematomal rim, surrounding normal appearing and contralateral brain tissue. Computed tomography perfusion parameters gradually improved from the core to the periphery in each time interval with an early increase at 24 h followed by a delayed decline at 7 days compared with admission values (P < 0.001). Multivariable linear regression analysis showed that haematoma volume and cerebral blood flow gradient between normal appearing and perihaematomal rim were independently associated with absolute perihaematomal oedema volume in the different time points (within 6 h, B = 0.128, P = 0.032; at 24 h, B = 0.133, P = 0.016; at 7 days, B = 0.218, P < 0.001). In a secondary analysis with relative perihaematomal oedema as the outcome of interest, cerebral blood flow gradient between normal appearing and perihaematomal rim was an independent predictor of perihaematomal oedema only at 7 days (B = 0.239, P = 0.002). Our findings raise the intriguing hypothesis that perfusion gradients promote perihaematomal oedema development in the subacute phase after intracerebral haemorrhage.

Role of endovascular treatment for ruptured aneurysms involving the anterior spinal artery at the craniocervical junction.

Ruptured aneurysms at the craniocervical junction (CCJ) involving the anterior spinal artery (ASA) are rare and consist of heterogenous lesions with variable clinical entities. However, the standard therapeutic strategy for the lesions has not been well-established. Moreover, despite advances in modern neurointervention, reports describing endovascular treatment for this specific lesion have been few. Here, we report three cases of ruptured aneurysms on the pial tributary of the ASA at the CCJ, which were subsequently treated by transarterial glue injection or coil embolization. Endovascular treatment can be a therapeutic option, particularly for these ruptured aneurysms. Either transarterial glue injection or coil embolization can be effective depending on the type of etiology and the surrounding vasculature anatomy.

A case of refractory chronic subdural hematoma and internal carotid artery stenosis sequentially treated with surgical drainage, middle meningeal artery embolization, and carotid artery stenting.

Background: Both chronic subdural hematoma (CSDH) and ischemic cerebrovascular disease are commonplace in the clinical context, and their combination is sometimes experienced. We describe a unique and challenging case in which both therapeutic interventions were indispensable and performed in a sequential manner. This report aims to discuss the management of hemorrhagic and ischemic conditions where CSDH and carotid artery stenosis coexist. Case Description: An 83-year-old male presented with the left cerebral infarction due to the left internal carotid artery (ICA) stenosis. The coexisting left CSDH was surgically drained first. Then, the left middle meningeal artery (MMA) was endovascularly embolized to prevent hematoma recurrence under antiplatelet therapy, before the left carotid artery stenting (CAS) was successfully conducted. The subdural hematoma gradually grew but remained asymptomatic. However, he later presented with another stroke due to the progressive right ICA stenosis that had been conservatively treated initially. Emergency right CAS was required eventually. Conclusion: Under the circumstances where CSDH is present but antiplatelet therapy is inevitable, MMA embolization could be a reasonable treatment option to avoid additional surgical procedures. Furthermore, early intervention should be considered even for asymptomatic carotid stenosis in terms of shortening the administration period of antiplatelet agents.

Meningiomas Involving the Hypoglossal Canal: A Case Report and Literature Review.

We report a rare case of hypoglossal canal meningioma in a 65-year-old woman who presented with dysphagia. Neurological examination revealed left hypoglossal nerve palsy. Head computed tomography and magnetic resonance imaging revealed a lesion around the left hypoglossal canal. She underwent a total resection with a midline suboccipital transcondylar approach. There were no postoperative complications, and the hypoglossal nerve palsy improved. There was no recurrence nine months after the surgery. Choosing a surgical approach that considers the site of origin and extent of tumor extension is important.

Extracranial prevertebral venous network of the craniocervical junction: CT-digital subtraction venography analysis.

PURPOSE: Although the craniocervical junction has a complex anatomical structure associated with clinical diseases, its ventral venous network has not been well studied. This study aimed to clarify the extracranial ventral venous structure at the craniocervical junction. METHODS: Head computed tomography digital subtraction venography (CT-DSV) images of 273 patients (age 6 months to 93 years) taken at our department were retrospectively analyzed. We analyzed the frequency and anatomical features of the venous channels, as well as their upstream and downstream connections with the surrounding channels at the ventral craniocervical junction, from the level of the hypoglossal canal to the second cervical vertebra. RESULTS: In 54% of the cases, the vein descended from the anterior condylar confluence, running medially along the basioccipital and fusing with its counterpart in the midline at the level of the atlanto-occipital membrane. Furthermore, 24% of this vein was connected caudally to the anterior external vertebral venous plexus. We also identified venous channels, either as a sole vein or venous plexus, on the tip of the odontoid process (10%), which has not been well described previously. The vein around the odontoid process was connected to several veins, including the aforementioned vein anterior to the condyle and the anterior internal vertebral venous plexus. CONCLUSIONS: CT-DSV analysis revealed a detailed venous architecture ventral to the craniocervical junction. Venous structures identified in this study may be involved in diseases in this area.

Imaging of the venous plexus of Rektorzik using CT-digital subtraction venography: a retrospective study.

PURPOSE: The venous plexus of Rektorzik (VPR), first described by Rektorzik in 1858, is a venous plexus around the internal carotid artery in the carotid canal. However, the VPR has never been investigated using the recently developed imaging modalities. In this study, we analyzed the VPR using computed tomography-digital subtraction venography (CT-DSV). METHODS: This study included 253 patients who had undergone head CT-DSV. The presence or absence of the right and left VPRs and their connecting veins were visually examined by two researchers. RESULTS: The VPR was observed in 60 patients (24%), 39 of which showed VPR only on the right side, 10 only on the left side, and 11 on both sides. VPR was significantly more common on the right side (p = 0.0002) and was observed more frequently around the horizontal segment of the internal carotid artery than around the vertical segment. The most common veins identified as distal and proximal VPR connections were the cavernous sinus (63/71, 89%) and the anterior condylar confluence (27/71, 38%), respectively. The mean age was significantly lower in patients with the VPR than in those without (53 vs. 57 years, p = 0.02). CONCLUSION: The VPR was significantly more frequent on the right side and in younger patients but was not a radiographically constant structure. In most cases, the VPR connected the cavernous sinus and anterior condylar confluence. Preoperative evaluation of VPR may lead to refined surgical procedures.

Application of Deep Learning Techniques for Automated Diagnosis of Non-Syndromic Craniosynostosis Using Skull.

ABSTRACT: Non-syndromic craniosynostosis (NSCS) is a disease, in which a single cranial bone suture is prematurely fused. The early intervention of the disease is associated with a favorable outcome at a later age, so appropriate screening of NSCS is essential for its clinical management. The present study aims to develop a classification and detection system of NSCS using skull X-ray images and a convolutional neural network (CNN) deep learning framework. A total of 56 NSCS cases (scaphocephaly [ n = 17], trigonocephaly [n = 28], anterior plagiocephaly [n = 8], and posterior plagiocephaly [n = 3]) and 25 healthy control infants were included in the study. All the cases underwent skull X-rays and computed tomography scan for diagnosis in our institution. The lateral views obtained from the patients were retrospectively examined using a CNN framework. Our CNN model classified the 4 NSCS types and control with high accuracy (100%). All the cases were correctly classified. The proposed CNN model may offer a safe and high-sensitivity screening of NSCS and facilitate early diagnosis of the disease and better neurocognitive outcome for patients.

Molecular investigation of brain tumors progressing during pregnancy or postpartum period: the association between tumor type, their receptors, and the timing of presentation.

OBJECTIVE: Brain tumors often become clinically evident during pregnancy; however, the mechanism has not been well elucidated. Purpose of this study is to investigate the influence of molecular genetic factors on the progression of brain tumors during pregnancy or the postpartum period. METHODS: Twelve cases of brain tumors that presented during pregnancy or postpartum period were included: five gliomas, three meningiomas, two vestibular schwannomas, and two chordomas. Tumor samples were investigated by metaphase comparative genomic hybridization and immunohistochemistry, for chromosomal copy number aberration (CNA) and receptor expression of sex hormones and growth factors. RESULTS: The results were correlated with the timing of tumor presentation in relation to the stage of pregnancy. EGFR, VEGFR-1/2, AR, and c-Myc were expressed in gliomas, PgR, ER, HER-2, VEGFR-1, EGF and VEGFR2 in meningiomas, VEGFR-1 in vestibular schwannomas, and EGFR, VEGFR-1/2, and c-Myc in chordomas. The CNAs of the tumors varied. Four of the five gliomas presented in the 2nd trimester, all three meningiomas in the 3rd trimester or postpartum period, and both of the two schwannomas in the late 2nd trimester. Expression of VEGFR-1/2 and EGFR was observed regardless of the timing of tumor presentation, whereas female hormone receptors and HER-2 were exclusively found in meningiomas. Interestingly, one anaplastic astrocytoma (IDH mut, non-codeleted) that progressed from precedent grade 2 tumor harbored amplification of the MYC locus. CONCLUSION: Progression of brain tumors during pregnancy is associated with various growth factors as well as sex hormones. The timing of presentation is likely dependent on molecular receptors specific to each tumor type.

Mechanical Thrombectomy in Distal Residual Occlusions of the Middle Cerebral Artery after Large Vessel Recanalization in Acute Stroke: 2b or not 2b? A Pragmatic Approach in Real-Life Scenarios.

BACKGROUND /OBJECTIVE: Recent studies have suggested that a recanalization grade of modified Thrombolysis in Cerebral Infarction (mTICI) score ≥2c is strongly related with good clinical outcome rather than the current therapeutic angiography target ≥2b. To achieve better recanalization, additional further maneuvers on distal residual vessel occlusion (RVO) may be required. The aim of this study was to evaluate the safety and efficacy of rescue treatment in RVOs after recanalization of large vessel occlusions in the anterior circulation. METHODS: A single-center retrospective review of a prospectively maintained stroke databank was performed. Patients presenting with RVOs after mechanical thrombectomy on the M1/internal carotid artery terminus were included and further divided into treated and untreated groups: the former underwent additional maneuvers on RVOs, whereas the latter did not. Baseline and posttreatment clinical, radiologic, and angiographic data were compared between the 2 groups. End points included good functional outcome (modified Rankin Scale [mRS] score ≤2) rates of hemorrhagic transformations, neurologic deterioration and mortality. RESULTS: RVOs were observed in 183/488 patients (37.5%). 74/183 (40.4%) underwent rescue treatment, showing a better outcome in terms of median 24 hours National Institutes of Health Stroke Scale score (13 vs. 18; P < 0001), 24 hours Alberta Stroke Programme Early CT Score (6 vs. 5; P < 0.001) and 3 months mRS score 0-2 (47.3% vs. 33.1%; P = 0.06). Recanalization of the superior (frontal) branch of the middle cerebral artery was particularly critical in terms of outcome. Hemorrhagic transformation was higher in the untreated group (53.6% vs. 66.6%; P = 0.1) as well as symptomatic intracranial hemorrhage (13.1% vs. 29.4%; P = 0.01). Neurologic deterioration occurred more often among untreated patients (16.2% vs. 25.7%; P = 0.1). Three complications (1.3%) occurred during rescue treatment. CONCLUSIONS: When feasible, improving mTICI score 2a-2b recanalization to mTICI 2c/3 is safe and associated with a better clinical outcome, particularly for residual occlusions involving the superior branch of bifurcation.

Intradural spinal cord arteriovenous shunts in a personal series of 210 patients: novel classification with emphasis on anatomical disposition and angioarchitectonic distribution, related to spinal cord histogenetic units.

OBJECTIVE: Few classifications of intradural spinal arteriovenous shunts (ID-SAVSs) have considered their anatomical localization in relation to their phenotype and angioarchitectonics. The authors propose another vision of ID-SAVSs allowing a reappraised classification based on analysis of the anatomical disposition, angioarchitecture, and histogenetic location of these vascular malformations. METHODS: The radiological and clinical records of 210 patients with ID-SAVSs were retrospectively reviewed, considering their localization, vascular architectonics, and correlation with the 5 histogenetic units of the spinal cord. Among these, 183 files with complete data allowed precise analysis of the ID-SAVSs. RESULTS: Among these 183 files (162 and 21 cases with single and multiple lesions, respectively), different entities were identified: 13 pial macro arteriovenous fistulas (MAVFs), 92 pial micro arteriovenous fistulas (mAVFs), 33 superficial pial niduses, and 69 intramedullary niduses. Thirteen sulcal shunts (either fistulas or niduses) were considered subtypes of pial lesions. Among the 21 multiple cases, 11 were monomyelomeric while 10 were multimyelomeric. Pial lesions, either fistulas or niduses, were dominantly vascularized by pial arteries (anterior or posterior depending on the localization of the shunt) and occasionally (except for MAVFs) by transmedullary arteries. Pial niduses occasionally extended into the funiculus by recruiting intrinsic veins or by extension of the nidus itself inside the white matter. Intramedullary niduses were always vascularized by both centrifugal and centripetal feeders, respectively, from sulcal arteries (SAs) and pial arteries. Sulcal lesions are pial lesions located within the ventral median sulcus and vascularized by SAs and veins. Single or multiple ID-SAVSs can be part of various syndromes such as hereditary hemorrhagic telangiectasia, Parkes-Weber, RASA1, CLOVES, and spinal arteriovenous metameric syndromes. Histogenetic analyses revealed a specific distribution of each ID-SAVS in the 5 histogenetic units of the spinal cord: intramedullary niduses were found almost equally from cervical to thoracic units, while MAVFs and mAVFs were mostly found from thoracic to postcrural ones. Pial niduses showed intermediate features between intramedullary and fistulous lesions and were mostly distributed from brachial to crural segments. CONCLUSIONS: Precise analysis of the anatomical disposition of ID-SAVSs in relation to functional histogenetic units allows a better understanding of these lesions and improved therapeutic management.

De novo arteriovenous shunts after endovascular cure of cerebrospinal macro arteriovenous fistulas. A role for the vasa vasorum?

BACKGROUND AND PURPOSE: Macro-arteriovenous fistulas (MAVFs) are arteriovenous shunts draining into a giant venous ectasia. They can be treated by surgery or embolisation. Angiographic controls are usually performed rapidly after treatment in order to prove the cure of the lesion but no long term angiographies are generally scheduled. We wanted to control the stabilities of such lesions at follow-up. METHOD: Clinical history and imaging of ninety-five patients with high flow shunts draining into venous ectasias (MAVFs, Vein of Galen malformations and dilatations) were reviewed. RESULTS: De novo arteriovenous shunts related to angiogenesis involving vasa vasorum developed in three patients with MAVFs at various intervals. Genetic underlying conditions as HHT or RASA 1 mutations were suspected in each patient. CONCLUSIONS: Neo-angiogenesis can occur after cure of MAVFs. Long term imaging follow-ups should be considered as the natural history of such recurrent shunts is currently unknown.