ABSTRACT
Because histopathological aspects of dural arteriovenous fistulas (DAVFs) are often lacking, there can be controversies regarding their angioarchitecture. Depending on various statements or DAVF types, the shunts can be situated directly on the sinuses, at the confluence of sinuses and cortical veins, in adjacent vascular structures surrounding the sinus, or, even, in the bone.1-5 Comprehensive knowledge of the intricate arteriovenous shunt anatomy is crucial for the secure and effective management of DAVFs.1 It has been confirmed that the dural arteries communicate with crack-like veins that connect ≥1 dural veins near the affected sinuses.3 In DAVFs, it is certain that ≥1 draining veins exist before draining into the venous sinus, in contrast to the commonly stated direct artery-to-sinus communication. Arteries branch and taper, and veins receive branches and thicken their lumens. We define fistulous points as the locations where the feeding arteries transition from thick to thin and the draining veins transition from thin to thick. We provide an image description based on superselective angiography.
Subject(s)
Central Nervous System Vascular Malformations , Cerebral Angiography , Humans , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Cerebral Veins/diagnostic imaging , Cranial Sinuses/diagnostic imaging , Cranial Sinuses/pathologyABSTRACT
OBJECTIVE: Intracranial dural arteriovenous fistulas (dAVFs) are rare vascular lesions that can be asymptomatic or can lead to devastating hemorrhage based on the dAVF's aggressiveness. Several approaches can be taken to treat dAVFs, such as endovascular embolization and surgical ligation. However, very few studies have evaluated the influence of surgery compared to endovascular approaches on patient outcomes. This study was performed to analyze the clinical characteristics and outcomes of patients who underwent treatment for intracranial dAVF in which either endovascular embolization or microsurgical ligation was used. METHODS: The Nationwide Readmissions Database was reviewed for all patients who underwent treatment for dAVFs (n = 18,152) between 2016 and 2019. Patients who received only surgical ligation or endovascular embolization (i.e., not both) were included. Variables regarding demographics, clinical outcomes, and healthcare utilization were queried. Primary outcome measures were nonroutine discharge, 1-year readmission, top quartile length of stay (LOS), and top quartile of inpatient all-payer cost. Propensity score matching was performed to evaluate the influence of either surgery or embolization on patient outcomes. Receiver operating characteristic (ROC) curves were created for each outcome measure. The area under the curve (AUC) of each ROC was used to estimate mixed-effects model performance. RESULTS: Following propensity score matching, 127 and 113 patients made up the surgical ligation and endovascular embolization cohort, respectively. There were no differences found in age (p = 0.16), sex (p = 0.57), or average Elixhauser Comorbidity Index (p = 0.32). Patients receiving surgical ligation had lower odds of readmission (OR 0.37, p = 0.028) and greater odds of nonroutine discharge (OR 2.21, p = 0.03) compared to patients who underwent endovascular embolization. The authors found no differences in the top quartile of LOS (p = 0.84), top quartile of cost (p = 0.38), or mortality (p > 0.99) between cohorts. ROC curves revealed that the mixed-effects models inclusive of approach outperformed models agnostic to approach with respect to nonroutine discharge (AUC with approach, 0.871; AUC without approach, 0.850; p = 0.018) and readmission (AUC with approach, 0.686; AUC without approach, 0.651; p = 0.019), but no differences were observed regarding top quartile of LOS (p = 0.17) and top quartile of cost (p = 0.40). CONCLUSIONS: Surgical approach may influence perioperative outcomes in patients treated for intracranial dAVF-most significantly discharge disposition and 1-year readmission. Future longitudinal prospective studies with more clinical detail will be required to fully capture the predictive utility of surgical approach in patients treated for intracranial dAVF, particularly for various dAVF subtypes.
Subject(s)
Central Nervous System Vascular Malformations , Embolization, Therapeutic , Humans , Prospective Studies , Propensity Score , Retrospective Studies , Central Nervous System Vascular Malformations/surgery , Central Nervous System Vascular Malformations/pathology , Embolization, Therapeutic/methodsABSTRACT
OBJECTIVE: The mechanism behind SDAVF is still unclear. We discovered that the vessel wall of the SDAVF-DV occasionally showed enhancement in MRI, and this study assessed the relationship between the enhancement of the draining vein's wall and its histology. METHODS: For histopathologic analysis, 16 draining vein samples from 16 patients with SDAVF were included, 3 normal arteries and 3 normal veins were chosen as comparison. We assessed the imaging and microscopic characteristics of the draining veins in SDAVF patients. The former included the presence of significant enhancement of the wall of the draining vein in MRI, and the latter included the adherence, aggregation, infiltration of pro-inflammatory factors and inflammatory cells. Immuno-histochemical staining was performed using IL-1ß, IL-8, TGF-ß as well as MPO and MMP-9, and positive results were counted. Multiple logistic regression analysis was used to determine whether the infiltration of inflammatory cells was connected to vessel wall enhancement in the SDAVF-DV. RESULTS: Infiltration of inflammatory cells was significantly higher in SDAVF-DV compared to normal vessels, 7 out of 16 patients significantly had enhancement of the vessel wall of SDAVF-DV, and logistic regression analysis showed that samples with more infiltration of inflammatory cells were more likely to show enhancement of the SDAVF-DV walls. CONCLUSION: There was considerable inflammatory cells infiltration in SDAVF-DV, and this may explain why their vessel wall had such a significant enhancement in MRI.
Subject(s)
Central Nervous System Vascular Malformations , Magnetic Resonance Imaging , Humans , Male , Female , Middle Aged , Aged , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Magnetic Resonance Imaging/methods , Adult , Veins/pathology , Veins/diagnostic imaging , Spinal Cord/diagnostic imaging , Spinal Cord/pathology , Spinal Cord/blood supplyABSTRACT
Cavernous-type malformations are venous lesions that occur in multiple locations throughout the body, and when present in the CNS, they have canonically been referred to as cavernomas, cavernous angiomas, and cerebral cavernous malformations. Herein all these lesions are referred to as "cavernous venous malformations" (CavVMs), which is congruent with the current International Society for the Study of Vascular Anomalies classification system. Even though histologically similar, depending on their location relative to the dura mater, these malformations can have different features. In Part 1 of this review, the authors discuss and review pertinent clinical knowledge with regard to CavVMs as influenced by anatomical location, starting with the dural and extradural malformations. They particularly emphasize dural CavVMs (including those in the cavernous sinus), orbital CavVMs, and spinal CavVMs. The genetic and histopathological features of CavVMs in these locations are reviewed, and commonalities in their presumed mechanisms of pathogenesis support the authors' conceptualization of a spectrum of a single disease entity. Illustrative cases for each subtype are presented, and the pathophysiological and genetic features linking dural and extradural to intradural CavVMs are examined. A new classification is proposed to segregate CavVMs based on the location from which they arise, which guides their natural history and treatment.
Subject(s)
Central Nervous System Vascular Malformations , Hemangioma, Cavernous, Central Nervous System , Hemangioma, Cavernous , Humans , Central Nervous System/pathology , Hemangioma, Cavernous, Central Nervous System/diagnostic imaging , Hemangioma, Cavernous, Central Nervous System/genetics , Hemangioma, Cavernous, Central Nervous System/surgery , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/surgery , Central Nervous System Vascular Malformations/pathology , Veins/pathologyABSTRACT
BACKGROUND AND OBJECTIVES: Arteriovenous fistulas involving the deep venous system have often been treated with microsurgery or transarterial embolization. Increasing familiarity with transvenous navigation and improved endovascular access systems may facilitate transvenous embolization (TVE) for these rare and challenging lesions. METHODS: We performed a retrospective study of neurointerventional databases of 6 high-volume centers. We identified all cases of arteriovenous fistulas with deep transvenous embolizations for arteriovenous fistula. Details regarding demographics, fistula characteristics, treatment considerations, clinical outcomes, and fistula occlusion were obtained and analyzed. The meta-analysis used the same inclusion criteria. RESULTS: Seventeen cases of TVE were identified. The most common reasons for TVE included prior treatment failure with microsurgery (n = 2) or transarterial embolization (n = 3) or inaccessible arterial pedicles (n = 4). For patients with full clinical outcome data (n = 14), 2 patients had worsened modified Rankin Scale, 8 patients had no change, and 4 were improved at a median clinical follow-up of 3.5 months. Angiographic obliteration was achieved in 15/17 cases (88.2%). In 1 case, catheterization around a sharp turn in the basal vein of Rosenthal could not be performed. In another case, despite successful TVE, there was residual lesion which was treated 1 year later by microsurgical clipping and excision. CONCLUSION: Transvenous approaches for embolization of deep arteriovenous fistulas have become possible with modern endovascular catheter systems and liquid embolics. These lesions can be treated safely and effectively through endovascular approaches, which may spare patients the traversal of deep structures needed for microsurgical approaches to these regions. The outcomes of TVE are comparable with published outcomes of microsurgical interruption.
Subject(s)
Arteriovenous Fistula , Central Nervous System Vascular Malformations , Embolization, Therapeutic , Humans , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/therapy , Central Nervous System Vascular Malformations/pathology , Embolization, Therapeutic/adverse effects , Retrospective Studies , Treatment OutcomeABSTRACT
The history of vascular anatomy of the spinal cord and spine began in the late 19th century. With recent advances in endovascular and surgical treatment of vascular lesions of the spinal cord, understanding the vascular anatomy of the spinal cord has become more important than ever. Catheter angiography is the gold standard for diagnosing vascular lesions of the spinal cord, and the vascular architecture of the spinal cord itself is relatively simple, a repetition of basic longitudinal and axial structures. This review discusses the vascular anatomy of the spine and spinal cord from the embryological point of view, as well as an overview of typical spinal arteriovenous shunt diseases mainly depending on their locations: paraspinal, epidural, dural, perimedullary, intramedullary, filum terminale/cauda equina, craniocervical junction, and related diseases.
Subject(s)
Arteriovenous Fistula , Cauda Equina , Central Nervous System Vascular Malformations , Humans , Spinal Cord/diagnostic imaging , Spinal Cord/surgery , Cauda Equina/blood supply , Cauda Equina/pathology , Cauda Equina/surgery , Arteriovenous Fistula/diagnostic imaging , Arteriovenous Fistula/surgery , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/therapy , Magnetic Resonance ImagingABSTRACT
PURPOSE: Many variations in the origin of the posterior inferior cerebellar artery (PICA) have been reported. To our knowledge, only one case of a PICA originating from the posterior meningeal artery (PMA) has been reported. METHODS: We describe a case with a PICA that was supplied retrograde from the distal segment of the PMA, mimicking a dural arteriovenous fistula on magnetic resonance angiography (MRA). RESULTS: A 31-year-old man was admitted to our hospital with a sudden occipital headache and nausea. MRA showed a hyperplastic left PMA, continuing to an abnormal vessel that was suspicious for venous drainage. Digital subtraction angiography revealed the left PMA originated from the extradural segment of the vertebral artery and then connected to the left PICA near the torcula. The cortical segment of the PICA flowed retrograde, which appeared as venous reflux on MRA. A second PICA originated from the extradural segment of the left vertebral artery and perfused the tonsillomedullary and televelotonsillar segment of the left PICA territory. CONCLUSION: We present an anatomical variant of the PICA mimicking a dural arteriovenous fistula. Digital subtraction angiography is useful for diagnosis of the cortical segment of the PICA flowing retrograde from the distal segment of the PMA because signal intensity in MRA of retrograde flow tends to decrease and diagnosis may be difficult. During endovascular treatment and open surgery, we should note that ischemic complications may occur due to the potential anastomosing channels between cerebral and dural arteries.
Subject(s)
Central Nervous System Vascular Malformations , Vertebral Artery , Male , Humans , Adult , Cerebellum/blood supply , Meningeal Arteries/diagnostic imaging , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Magnetic Resonance AngiographyABSTRACT
BACKGROUND: The pressure cooker technique (PCT) was developed to enable safer and more extensive embolization of hypervascular lesions by simultaneously minimizing backflow of liquid embolic materials and thus reduce the risk of non-target embolization of adjacent healthy vessels. We report our experience in applying the PCT to cerebral arteriovenous malformations (AVMs) and dural arteriovenous fistulas (DAVFs) and additionally suggest our technical adjustments. METHODS: We retrospectively reviewed all patients suffering from AVMs or DAVFs that were treated with the PCT between 2018 and 2020 in two university hospitals. The endpoints of the study were clinical safety and the technical efficacy. The endpoints of the study were clinical safety and the technical efficacy. Clinical safety was the absence of death, major or minor symptomatic stroke, TIA and procedure-related intracranial bleeding (SAH) in the peri-procedural period until dismission. Good clinical outcome was defined as no deterioration of the modified Rankin Scale (mRS) score (comparing the pre- and post-procedural mRS evaluated by a trained neurologist at admission and dismission). The technical efficacy was considered as the complete embolization occlusion of the target vessels of the lesion documented on the final control angiogram. Long-term follow-up evaluation was not intended for this study as it was not available for all patients. RESULTS: Fifteen consecutive patients (6 women; mean age 55 y; range 20-82 y) with seven AVMs and eight DAVFs met the inclusion criteria. The primary clinical safety end point was obtained in all cases as no intraprocedural complications were encountered. All patients had a good clinical outcome with no difference between the pre- and post-mRS scores. The primary efficacy end point was reached in all cases. CONCLUSION: The PCT is a safe and effective technique in the treatment of cerebral AVMs and DAVFs. Adding an easy and fast step to the procedure, namely the injection of contrast media to test the complete obstruction by the plug, the risk of reflux seems to additionally be reduced.
Subject(s)
Central Nervous System Vascular Malformations , Embolization, Therapeutic , Intracranial Arteriovenous Malformations , Humans , Female , Middle Aged , Retrospective Studies , Treatment Outcome , Intracranial Arteriovenous Malformations/diagnostic imaging , Intracranial Arteriovenous Malformations/therapy , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/therapy , Embolization, Therapeutic/methodsABSTRACT
BACKGROUND: Craniocervical junction (CCJ) vascular abnormalities can be challenging to treat because of the surrounding density of critical neurovascular anatomy. Although most dural arteriovenous fistulas (dAVFs) are now treated with endovascular surgery, dAVFs near the CCJ are often better suited for microsurgical obliteration with precise vascular control. METHODS: We describe our microsurgical approach to treating dAVFs at the CCJ. This includes a far-lateral approach with a small incision centered over the transverse process of the atlas and circumferential skeletonization of the vertebral artery in addition to clipping the fistula to limit lesion recurrence. CONCLUSIONS: Definitive microsurgical treatment of CCJ dAVFs can be accomplished using a minimally invasive approach.
Subject(s)
Central Nervous System Vascular Malformations , Embolization, Therapeutic , Endovascular Procedures , Humans , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/surgery , Central Nervous System Vascular Malformations/pathology , Craniotomy , Spine/surgery , Vertebral Artery/surgerySubject(s)
Arteriovenous Fistula , Central Nervous System Vascular Malformations , Embolization, Therapeutic , Humans , Arteriovenous Fistula/pathology , Central Nervous System Vascular Malformations/complications , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathologyABSTRACT
BACKGROUND: Dural arteriovenous fistulas (DAVFs) of the sphenoparietal sinus or sphenoid wing region are uncommon lesions with unique and interesting angioarchitecture. Understanding appropriate anatomy and recognizing patterns provide important treatment implications. OBJECTIVE: To describe a single surgeon's experience with open surgical treatment of sphenoparietal sinus DAVFs, the surgical indications for this uncommon lesion, and the microsurgical techniques related to its treatment and to review the literature on its surgical treatment. METHODS: Consecutive cases of sphenoparietal sinus DAVF treatment conducted by a single surgeon over 24 years (1997-2020) were retrospectively reviewed. Published reports of similar cases were reviewed. RESULTS: Of 202 surgically treated DAVFs, 10 lesions in 10 patients were sphenoparietal sinus DAVFs. Four patients presented with intracranial hemorrhage, 3 with headache, and 2 with pulsatile tinnitus; 1 patient was incidentally identified as having a DAVF during treatment for a ruptured aneurysm. Most patients (7 of 10) had undergone endovascular embolization previously. Nine patients had Borden type III DAVFs and one had a Borden type II fistula. Surgery in all 10 patients resulted in angiographically confirmed fistula obliteration. Clinical outcomes at the last follow-up, measured by a modified Rankin Scale (mRS) score, were excellent in 6 patients (mRS ≤ 2) and poor in 1 patient (mRS ≥ 3); late outcomes were not available for 3 patients. CONCLUSION: Sphenoparietal sinus DAVFs are an uncommon anatomic subtype. Careful attention to angiographic detail leads to identification of the site of venous interruption and results in a high rate of surgical cure with excellent clinical outcomes.
Subject(s)
Cavernous Sinus , Central Nervous System Vascular Malformations , Embolization, Therapeutic , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/surgery , Embolization, Therapeutic/methods , Humans , Intracranial Hemorrhages/therapy , Retrospective StudiesABSTRACT
BACKGROUND: Dural arteriovenous fistulas (dAVFs) are often treated with stereotactic radiosurgery (SRS) to achieve complete obliteration (CO), prevent future hemorrhages, and ameliorate neurological symptoms. OBJECTIVE: To summarize outcomes after SRS for dAVFs and propose relevant practice recommendations. METHODS: Using a PICOS/PRISMA/MOOSE protocol, we included patients with dAVFs treated with SRS and data for at least one of the outcomes of the study. Relevant outcomes were CO, symptom improvement and cure, and post-SRS hemorrhage or permanent neurological deficits (PNDs). Estimated outcome effect sizes were determined using weighted random-effects meta-analyses using DerSimonian and Laird methods. To assess potential relationships between patient and lesion characteristics and clinical outcomes, mixed-effects weighted regression models were used. RESULTS: Across 21 published studies, we identified 705 patients with 721 dAVFs treated with SRS. The CO rate was 68.6% (95% CI 60.7%-76.5%) with symptom improvement and cure rates of 97.2% (95% CI 93.2%-100%) and 78.8% (95% CI 69.3%-88.2%), respectively. Estimated incidences of post-SRS hemorrhage and PNDs were 1.1% (95% CI 0.6%-1.6%) and 1.3% (95% CI 0.8%-1.8%), respectively. Noncavernous sinus (NCS) dAVFs were associated with lower CO (P = .03) and symptom cure rates (P = .001). Higher grade was also associated with lower symptom cure rates (P = .04), whereas previous embolization was associated with higher symptom cure rates (P = .01). CONCLUSION: SRS for dAVFs results in CO in the majority of patients with excellent symptom improvement rates with minimal toxicity. Patients with NCS and/or higher-grade dAVFs have poorer symptom cure rates. Combined therapy with embolization and SRS is recommended when feasible for clinically aggressive dAVFs or those refractory to embolization to maximize the likelihood of symptom cure.
Subject(s)
Central Nervous System Vascular Malformations , Embolization, Therapeutic , Intracranial Arteriovenous Malformations , Radiosurgery , Central Nervous System Vascular Malformations/pathology , Humans , Intracranial Arteriovenous Malformations/surgery , Radiosurgery/methods , Retrospective Studies , Societies , Treatment OutcomeABSTRACT
With the rapid advancements in endovascular therapy over previous decades, the treatment of spinal arteriovenous malformations (AVMs) continues to evolve. The decision to use endovascular versus surgical therapy largely depends on the type of lesion and its anatomic location. Recent studies demonstrate that endovascular treatment is effective for extradural arteriovenous fistulas (AVFs), intradural ventral (perimedullary) AVMs, and intramedullary spinal AVMs. Treatment of intradural dorsal (dural) AVFs remains largely surgical because of lower recurrence rates, although recent studies demonstrate equivocal outcomes. Extradural-intradural (juvenile) AVMs and conus AVMs remain difficult-to-treat lesions.
Subject(s)
Arteriovenous Fistula , Arteriovenous Malformations , Central Nervous System Vascular Malformations , Arteriovenous Fistula/surgery , Arteriovenous Malformations/diagnostic imaging , Arteriovenous Malformations/surgery , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/surgery , Humans , Spinal Cord/pathology , Spinal Cord/surgeryABSTRACT
Dural arteriovenous fistulas represent a distinct direct pathological connection between dural arterial feeders of the meninges to cortical veins or sinuses. Vascular supply of the meninges is provided by a series of named arteries-anterior, middle, and posterior meningeal arteries, with the tentorium provided by the artery of Bernasconi and Cassinari (anterior-medial) and the artery of Davidoff and Schechter (posterior-medial). This case is the first report in the literature of a lateral distal posterior cerebral artery supplying the meninges and contributing dural feeders to a Cognard type III/Borden type III dural arteriovenous fistula.
Subject(s)
Central Nervous System Vascular Malformations/pathology , Meningeal Arteries/abnormalities , Meninges/blood supply , Central Nervous System Vascular Malformations/surgery , Embolization, Therapeutic , Endovascular Procedures , Female , Humans , Meningeal Arteries/surgery , Middle AgedABSTRACT
OBJECTIVE: Cranial dural arteriovenous fistulas (dAVFs) are rare lesions, hampering efforts to understand them and improve their care. To address this challenge, investigators with an established record of dAVF investigation formed an international, multicenter consortium aimed at better elucidating dAVF pathophysiology, imaging characteristics, natural history, and patient outcomes. This report describes the design of the Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) and includes characterization of the 1077-patient cohort. METHODS: Potential collaborators with established interest in the field were identified via systematic review of the literature. To ensure uniformity of data collection, a quality control process was instituted. Data were retrospectively obtained. RESULTS: CONDOR comprises 14 centers in the United States, the United Kingdom, the Netherlands, and Japan that have pooled their data from 1077 dAVF patients seen between 1990 and 2017. The cohort includes 359 patients (33%) with Borden type I dAVFs, 175 (16%) with Borden type II fistulas, and 529 (49%) with Borden type III fistulas. Overall, 852 patients (79%) presented with fistula-related symptoms: 427 (40%) presented with nonaggressive symptoms such as tinnitus or orbital phenomena, 258 (24%) presented with intracranial hemorrhage, and 167 (16%) presented with nonhemorrhagic neurological deficits. A smaller proportion (224 patients, 21%), whose dAVFs were discovered incidentally, were asymptomatic. Many patients (85%, 911/1077) underwent treatment via endovascular embolization (55%, 587/1077), surgery (10%, 103/1077), radiosurgery (3%, 36/1077), or multimodal therapy (17%, 184/1077). The overall angiographic cure rate was 83% (758/911 treated), and treatment-related permanent neurological morbidity was 2% (27/1467 total procedures). The median time from diagnosis to follow-up was 380 days (IQR 120-1038.5 days). CONCLUSIONS: With more than 1000 patients, the CONDOR registry represents the largest registry of cranial dAVF patient data in the world. These unique, well-annotated data will enable multiple future analyses to be performed to better understand dAVFs and their management.
Subject(s)
Central Nervous System Vascular Malformations , Embolization, Therapeutic , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/therapy , Cohort Studies , Embolization, Therapeutic/methods , Humans , Retrospective Studies , Treatment OutcomeABSTRACT
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.
Subject(s)
Arteriovenous Fistula/pathology , Arteriovenous Fistula/therapy , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/therapy , Spinal Cord/blood supply , Adult , Aged , Embolization, Therapeutic/methods , Female , Humans , Male , Microsurgery/methods , Middle Aged , Retrospective Studies , Spinal Cord/pathology , Vascular Surgical Procedures/methodsABSTRACT
Preeclampsia, a hypertensive disorder of pregnancy, complicates up to 10 % of all pregnancies and increases the risk for perinatal stroke in offspring. The mechanism of this increase is unknown, but may involve vascular dysfunction. The goal of this study was to evaluate the effect of experimental preeclampsia (ePE) on cerebrovascular function in offspring to eludciate a possible mechanism for this association. Dams were fed a high cholesterol diet beginning on day 7 of gestation to induce experimental preeclampsia. Middle cerebral arteries (MCA) and the Vein of Galen (VoG) were isolated from pups from ePE dams and compared to pups from normal pregnant (NP) dams at postnatal days 16, 23, and 30 and studied pressurized in an arteriograph chamber. Markers of inflammation and oxidative stress were measured in serum. Our results suggest altered structure and function in both MCA and VoG of ePE pups. We also found evidence of systemic inflammation and oxidative stress in ePE pups. These findings provide a potential link between preeclampsia and the occurrence or severity of perinatal stroke.
Subject(s)
Central Nervous System Vascular Malformations , Cerebral Veins , Middle Cerebral Artery , Pre-Eclampsia , Stroke , Animals , Animals, Newborn , Biomarkers/blood , Central Nervous System Vascular Malformations/blood , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/physiopathology , Cerebral Veins/pathology , Cerebral Veins/physiopathology , Disease Models, Animal , Female , Middle Cerebral Artery/pathology , Middle Cerebral Artery/physiopathology , Oxidative Stress , Pre-Eclampsia/metabolism , Pre-Eclampsia/physiopathology , Pregnancy , Rats , Risk Factors , Stroke/etiology , Stroke/physiopathologyABSTRACT
Cerebral cavernous malformations (CCMs) are vascular lesions that affect predominantly microvasculature in the brain and spinal cord. CCM can occur either in sporadic or familial form, characterized by autosomal dominant inheritance and development of multiple lesions throughout the patient's life. Three genes associated with CCM are known: CCM1/KRIT1 (krev interaction trapped 1), CCM2/MGC4607 (encoding a protein named malcavernin), and CCM3/PDCD10 (programmed cell death 10). All the mutations identified in these genes cause a loss of function and compromise the protein functions needed for maintaining the vascular barrier integrity. Loss of function of CCM proteins causes molecular disorganization and dysfunction of endothelial adherens junctions. In this review, we provide an overall vision of the CCM pathology, starting with the genetic bases of the disease, describing the role of the proteins, until we reach the cellular level. Thus, we summarize the genetics of CCM, providing a description of CCM genes and mutation features, provided an updated knowledge of the CCM protein structure and function, and discuss the molecular mechanisms through which CCM proteins may act within endothelial cells, particularly in endothelial barrier maintenance/regulation and in cellular signaling.
Subject(s)
Apoptosis Regulatory Proteins/genetics , Brain/blood supply , Carrier Proteins/genetics , Central Nervous System Vascular Malformations/genetics , Endothelial Cells/metabolism , KRIT1 Protein/genetics , Membrane Proteins/genetics , Mutation , Proto-Oncogene Proteins/genetics , Apoptosis Regulatory Proteins/metabolism , Carrier Proteins/metabolism , Central Nervous System Vascular Malformations/metabolism , Central Nervous System Vascular Malformations/pathology , Endothelial Cells/pathology , Genetic Predisposition to Disease , Humans , KRIT1 Protein/metabolism , Membrane Proteins/metabolism , Phenotype , Proto-Oncogene Proteins/metabolism , Signal TransductionABSTRACT
Asymptomatic dural arteriovenous fistulas (DAVFs) with cortical venous reflux (CVR) are now more commonly encountered. However, patients with an incidental onset may have a less aggressive clinical course. It is desirable to explore methods and indicators to predict the clinical outcomes. This study investigates whether the relative signal intensity (rSI) of the draining vessels on the time-of-flight magnetic resonance angiography is related to clinical behavior in patients with intracranial DAVFs. We retrospectively reviewed 36 intracranial DAVFs. The patients were categorized as those with either aggressive-presentation or non-aggressive-presentation (n = 16 and 20, respectively). The rSIs of the shunt points, affected sinuses, and veins with CVR were compared between the two groups. The two groups were not significantly different in terms of rSIs of the shunt points and affected sinuses (p = 0.37 and 0.41, respectively). However, a significant positive correlation was observed in the rSI of the veins with CVR between the aggressive and non-aggressive behavior groups (p < 0.0001). The rSI of the veins with CVR could serve as a reliable indicator of aggressive behavior in intracranial DAVFs, and its optimal cutoff value was 1.63 with high sensitivity and specificity for predicting aggressive behavior (area under the curve, 0.909).