scholarly journals Cerebral proliferative angiopathy: a review / Angiopatia proliferativa cerebral: uma revisão

Author(s):  
André Freitas Nunes ◽  
Rafael Gomes dos Santos ◽  
Rodrigo Salmeron de Toledo Aguiar ◽  
José Carlos Esteves Veiga ◽  
Heitor Castelo Branco Rodrigues Alves ◽  
...  

AbstractIntroduction: Cerebral Arteriovenous Malformations (AVMs) are vascular lesions defined by arteriovenous shunting flowing through a nidus of coiled and tortuous vascular connections. Cerebral Proliferative Angiopathy (CPA) is a rare type of AVM with pathological, clinical, diagnostic features and treatment differences from a typical AVM. We aimed to summarize important features of CPA already described and identify important gaps of evidence. Methods: Data was selected by a search of PUBMED with the term “cerebral proliferative angiopathy”. The articles considered relevant were included in this review. We also screened article references and included them as needed. Discussion: CPA shows a diffuse nidus appearance, presence of transdural supply and stenosis of feeding arteries, without dominant feeders or flow-related aneurysms. There is intermingled brain between the vascular spaces. The usual clinical presentation is seizures, headaches, and progressive neurological deficits. Sound diagnosis can be made based on CT, MRI, Angiography and perfusion techniques, which are essential to treatment choices. Invasive treatment is seldom indicated, in order to preserve the normal brain parenchyma, and clinical control of symptoms, associated with a thorough follow-up, is the usual approach in most reported cases. Conclusion: Still much remains to be learned about CPA. Establishing more precise roles of various radiological assessing methods, how they affect the follow-up of patients, and safety and efficacy profiles of different treatment approaches will provide us the tools to give patients a more solid intervention and clearer follow-up.Keywords: Vascular malformations, Intracranial arteriovenous malformations, Brain diseases, Cerebrovascular disordersResumoIntrodução: As malformações arteriovenosas cerebrais (MAVs) são lesões vasculares definidas por comunicação arteriovenosa por meio de conexões vasculares tortuosas. A angiopatia cerebral proliferativa (ACP) é um tipo raro de MAV com características patológicas, clínicas, diagnósticas e de tratamento distintas das MAVs típicas. Objetivou-se resumir características importantes da ACP já descritas na literatura, e identificar lacunas importantes nas evidências. Métodos: Os dados foram selecionados através de busca na base de dados PUBMED, com o termo “cerebral proliferative angiopathy”. Os artigos considerados relevantes foram incluídos nessa revisão, assim como alguns outros artigos presentes nas referências. Discussão: A ACP apresenta-se como um nidus difuso, associada à presença de irrigação transdural, de estenose das artérias que a alimentam, sem suprimento arterial dominante ou aneurismas relacionados ao fluxo. Há parênquima cerebral normal entre os espaços vasculares. Comumente se apresenta com crises epilépticas, cefaleia e déficits neurológicos progressivos. O diagnóstico de certeza pode ser realizado através de TC, RM, angiografia e técnicas de perfusão, e é essencial para a escolha do tratamento. Abordagens invasivas são raramente indicadas a fim de preservar o parênquima cerebral normal. O controle clínico dos sintomas, associado a seguimento rigoroso, é a escolha terapêutica geralmente utilizada na maioria dos relatos. Conclusão: Ainda há muito que se aprender sobre a ACP. Estabelecer os benefícios de cada um dos diversos métodos imaginológicos, como eles influenciam o seguimento dos pacientes, e perfis de segurança e eficácia das diferentes terapêuticas fornecerá ferramentas para melhor decisão terapêutica.Descritores: Malformações vasculares, Malformações arteriovenosas intracranianas, encefalopatias, Transtornos cerebrovasculares 

Neurosurgery ◽  
2000 ◽  
Vol 47 (3) ◽  
pp. 571-577 ◽  
Author(s):  
Justin H. T. Pik ◽  
Michael K. Morgan

ABSTRACT OBJECTIVE To examine the results of surgery in 110 consecutive patients with arteriovenous malformations (AVMs) smaller than 3 cm in diameter. These results are compared with the published results of other microsurgical series as well as with results for patients treated with focused irradiation. METHODS From January 1989 to November 1998, 121 patients with AVMs smaller than 3 cm were treated at our institution. One hundred ten patients underwent microsurgical removal of their AVMs. The presentation, preoperative neurological status, and postoperative outcome were recorded. Follow-up was complete for all surgical cases. RESULTS Of the 110 patients, 109 (99%) had angiographically confirmed obliteration of their AVMs. Two patients (1.8%) required reoperation for residual AVM. Two (4.3%) of 46 patients with AVMs in eloquent brain areas experienced worsening of their neurological status after surgery. One (1.6%) of 64 patients was worse neurologically after removal of an AVM in a noneloquent area. CONCLUSION Microsurgical removal is a safe and effective treatment for the majority of AVMs smaller than 3 cm in diameter. Although the treatment is accompanied by a risk of acute onset of neurological deficits, this tends to be transient in the majority of cases. Furthermore, microsurgical excision of small AVMs offers patients immediate protection from the natural history of their vascular lesions.


2021 ◽  
Vol 11 ◽  
Author(s):  
Liyan Zhao ◽  
Yining Jiang ◽  
Yubo Wang ◽  
Yang Bai ◽  
Ying Sun ◽  
...  

ObjectiveSpinal epidural cavernous hemangiomas are very rare vascular lesions and are, therefore, seldom reported and easily misdiagnosed. Herein, we present a series of 9 cases with spinal epidural cavernous hemangiomas and discuss their pathogenesis, clinical characteristics, radiological findings, differential diagnosis, surgical interventions, pathological characteristics, and prognosis.MethodsWe retrospectively retrieved and analyzed the data of patients with pure epidural cavernous hemangiomas, who underwent spinal magnetic resonance imaging, and surgical intervention at the First Hospital of Jilin University, China, between January 2005 and December 2019. The data on patients’ clinical manifestations, imaging characteristics, surgical intervention, histopathological findings, and postoperative follow-up were also recorded and analyzed.ResultsIn all, 5 men and 4 women with the mean age of 61 years (range, 41–78 years) were recruited. All patients experienced a gradual onset of symptoms and a slowly progressive clinical course, and no patient presented an acute onset of symptoms. The clinical manifestations include myelopathic signs in 8 patients (88.9%) and radicular symptoms in 3 patients (33.3%). On T1-weighted imaging, 6 lesions appeared isointense (55.6%), and 4 lesions exhibited hypointense (44.4%) signals. On T2-weighted imaging, 8 lesions appeared hyperintense (88.9%), and 1 lesion was heterogeneously intense (11.1%). Following gadolinium administration, 5 lesions appeared homogeneous with significant enhancement (55.6%), 1 lesion was homogeneous and mild enhancement (11.1%), and 3 lesions were heterogeneous with mild enhancement (33.3%). All patients received early microsurgery assisted by intraoperative electrophysiologic monitoring and neuronavigation in the lateral position via the posterior midline approach. Five patients underwent total laminectomy (55.6%), and 4 underwent hemilaminectomy (44.4%). Total excision was achieved in all cases. The average follow-up period was 55.1 months (ranging 10–123 months). All patients exhibited significant clinical improvement of their neurological deficits and achieved a favorable outcome with no recorded recurrence at last follow-up.ConclusionsSpinal epidural cavernous hemangiomas are rare vascular malformations. Early surgical treatment with total resection is an optimum treatment, particularly for patients with an acute exacerbation onset. The prognosis is mostly good and depends predominantly on the severity of the preoperative status.


2014 ◽  
Vol 14 (1) ◽  
pp. 1-11 ◽  
Author(s):  
Matthew B. Potts ◽  
Sunil A. Sheth ◽  
Jonathan Louie ◽  
Matthew D. Smyth ◽  
Penny K. Sneed ◽  
...  

Object Stereotactic radiosurgery (SRS) is an established treatment modality for brain arteriovenous malformations (AVMs) in children, but the optimal treatment parameters and associated treatment-related complications are not fully understood. The authors present their single-institution experience of using SRS, at a relatively low marginal dose, to treat AVMs in children for nearly 20 years; they report angiographic outcomes, posttreatment hemorrhage rates, adverse treatment-related events, and functional outcomes. Methods The authors conducted a retrospective review of 2 cohorts of children (18 years of age or younger) with AVMs treated from 1991 to 1998 and from 2000 to 2010. Results A total of 80 patients with follow-up data after SRS were identified. Mean age at SRS was 12.7 years, and 56% of patients had hemorrhage at the time of presentation. Median target volume was 3.1 cm3 (range 0.09–62.3 cm3), and median prescription marginal dose used was 17.5 Gy (range 12–20 Gy). Angiograms acquired 3 years after treatment were available for 47% of patients; AVM obliteration was achieved in 52% of patients who received a dose of 18–20 Gy and in 16% who received less than 18 Gy. At 5 years after SRS, the cumulative incidence of hemorrhage was 25% (95% CI 16%–37%). No permanent neurological deficits occurred in patients who did not experience posttreatment hemorrhage. Overall, good functional outcomes (modified Rankin Scale Scores 0–2) were observed for 78% of patients; for 66% of patients, functional status improved or remained the same as before treatment. Conclusions A low marginal dose minimizes SRS-related neurological deficits but leads to low rates of obliteration and high rates of hemorrhage. To maximize AVM obliteration and minimize posttreatment hemorrhage, the authors recommend a prescription marginal dose of 18 Gy or more. In addition, SRS-related symptoms such as headache and seizures should be considered when discussing risks and benefits of SRS for treating AVMs in children.


2014 ◽  
Vol 121 (5) ◽  
pp. 1015-1021 ◽  
Author(s):  
Chun-Po Yen ◽  
Dale Ding ◽  
Ching-Hsiao Cheng ◽  
Robert M. Starke ◽  
Mark Shaffrey ◽  
...  

Object A relatively benign natural course of unruptured cerebral arteriovenous malformations (AVMs) has recently been recognized, and the decision to treat incidentally found AVMs has been questioned. This study aims to evaluate the long-term imaging and clinical outcomes of patients with asymptomatic, incidentally discovered AVMs treated with Gamma Knife surgery (GKS). Methods Thirty-one patients, each with an incidentally diagnosed AVM, underwent GKS between 1989 and 2009. The nidus volumes ranged from 0.3 to 11.1 cm3 (median 3.2 cm3). A margin dose between 15 and 26 Gy (median 20 Gy) was used to treat the AVMs. Four patients underwent repeat GKS for still-patent AVM residuals after the initial GKS procedure. Clinical follow-up ranged from 24 to 196 months, with a mean of 78 months (median 51 months) after the initial GKS. Results Following GKS, 19 patients (61.3%) had a total AVM obliteration on angiography. In 7 patients (22.6%), no flow voids were observed on MRI but angiographic confirmation was not available. In 5 patients (16.1%), the AVMs remained patent. A small nidus volume was significantly associated with increased AVM obliteration rate. Thirteen patients (41.9%) developed radiation-induced imaging changes: 11 were asymptomatic (35.5%), 1 had only headache (3.2%), and 1 developed seizure and neurological deficits (3.2%). Two patients each had 1 hemorrhage during the latency period (116.5 risk years), yielding an annual hemorrhage rate of 1.7% before AVM obliteration. Conclusions The decision to treat asymptomatic AVMs, and if so, which treatment approach to use, remain the subject of debate. GKS as a minimally invasive procedure appears to achieve a reasonable outcome with low procedure-related morbidity. In those patients with incidental AVMs, the benefits as well as the risks of radiosurgical intervention will only be fully defined with long-term follow-up.


2013 ◽  
Vol 47 (1) ◽  
pp. 50-56 ◽  
Author(s):  
Sławomir Blamek ◽  
Dawid Larysz ◽  
Leszek Miszczyk ◽  
Adam Idasiak ◽  
Adam Rudnik ◽  
...  

Abstract Background. The treatment of large arteriovenous malformations (AVMs) or AVMs involving eloquent regions of the brain remains a challenge. For inoperable lesions, observation, volume-staged radiosurgery or hypofractionated stereotactic radiotherapy (HFSRT) are proposed. The aim of our study was to assess the safety and efficiency of HFSRT for large AVMs located in eloquent areas of the brain. Materials and methods. An analysis of records of 49 patients irradiated for cerebral AVMs with a mean dose of 19.9 Gy (12-28 Gy) delivered in 2-4 fractions with planned gap (at least one week) between fractions. Actuarial obliteration rates and annual bleeding hazard were calculated using Kaplan-Meier survival analysis and life tables. Results. Annual bleeding hazard rates were 4.5% and 1.6% after one and two years of the follow-up, respectively. Actuarial total obliteration rates were 7%, 11%, and 21% and total response rate (total and partial obliterations) 22%, 41%, and 55% after one, two and three years of the follow-up, respectively. There was a trend towards larger total obliteration rate in patients irradiated with fraction dose ≥ 8 Gy and total dose > 21 Gy for lesions of volume ≤ 8.18 cm3 which was not observed in case of partial obliterations. Conclusions. HFSRT results with relatively low obliteration rate but is not associated with a significant risk of permanent neurological deficits if both total and fraction doses are adjusted to size and location of the lesion. Predictive factors for total and partial obliterations can be different; this observation, however, is not firmly supported and requires further studies.


2005 ◽  
Vol 13 (1) ◽  
pp. 23-26 ◽  
Author(s):  
Jh Phillips ◽  
Cl Tang ◽  
D Armstrong ◽  
T De Chalain ◽  
R Zuker

Due to the rarity of arteriovenous malformations (AVMs), there is a paucity of information on the outcomes of various treatments. Presently, the mainstays of treatment of an AVM are embolization, surgical excision or a combination of both. A retrospective study of 26 patients with AVMs treated at the Hospital for Sick Children, Toronto, Ontario between 1985 and 1995 was performed. The treatment strategies and patient outcomes were compared in terms of efficacy, complications and the response of patients and their families to their overall treatment. The overall findings showed that embolization alone was effective in controlling symptoms, but may be associated with an increased AVM size. A partial excision of an AVM does not appear to exacerbate recurrence, as has been previously reported. In fact, even after what appears to be a complete excision of the AVM, recurrence may still occur. Complications from a surgical excision of the AVM are more frequent but less devastating than complications from embolization of the lesion. The overall patient or parent satisfaction with treatment was high with respect to improvement in outcome. It is expected that with an increasing understanding of vascular malformations, and the evolution of interventional radiological techniques, complications will decrease and results as a whole will improve.


2000 ◽  
Vol 6 (3) ◽  
pp. 177-183 ◽  
Author(s):  
M. Al-Yamany ◽  
K. G. terBrugge ◽  
R. Willinsky ◽  
W. Montanera ◽  
M. Tymianski ◽  
...  

Large arteriovenous malformations (AVMs) located in eloquent areas of the brain are generally considered incurable because of the high morbidity and mortality associated with their treatment. When these patients develop a progressive neurological deficit they in time often become severely disabled. This report presents the results of palliative embolisation in this subgroup of patients. Analysis of our data-base of 714 patients with known brain AVMs revealed 17 patients who presented with progressive neurological deficit and who underwent palliative embolisation as the therapeutic modality of choice for management of their AVM. One patient was excluded due to lack of follow-up and two were excluded because they later received radiation therapy. Following embolisation 43% had improvement of their neurological deficit, 50% stabilized and 7% continued to deteriorate and these clinical results persisted for an average of more than 2 years follow-up. Transient neurological morbidity associated with embolisation treatment was 7% and there was no permanent morbidity and no mortality. Palliative embolisation of brain AVMs presenting with progressive neurological deficits arrested deterioration in more than 90% of patients and was associated with low morbidity and no mortality.


1999 ◽  
Vol 5 (1) ◽  
pp. 27-34 ◽  
Author(s):  
Y. Matsumaru ◽  
S. Pongpech ◽  
J. Laothamas ◽  
H. Alvarez ◽  
G. Rodesch ◽  
...  

We describe 19 (16.0%) multiple vascular malformations (AVMs) in 119 spinal cord arteriovenous malformations (SCAVMs). The associated lesions were eight vertebral vascular malformations, two cutaneous, four limbs, four radicular AVMs, three bifocal SCAVMs; one patient had a bifocal cord lesion associated with vertebral and limb localisations. Various syndromic associations were seen: nine Cobb, two Klippel-Trenaunay-Weber, one Parkes Weber. An additional subgroup of unclassified associations is constituted by seven cases with bifocal intradural uni or multimetamerk lesions. In our SCAVMs series, the incidence of multiple vascular lesions is high, in particular multifocal intradural malformations. Metameric distribution is the most frequent type of multiplicity. Identification of the myelomeric level involved in SCAVM allows segmental link between various lesions of mesodermal or neural crest origin to be discussed.


Neurosurgery ◽  
1990 ◽  
Vol 27 (6) ◽  
pp. 892-900 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
Robert J. Coffey ◽  
David J. Bissonette ◽  
John C. Flickinger

Abstract Stereotactic radiosurgery has been shown to treat successfully angiographically demonstrated arteriovenous malformations of the brain. Angiographic obliteration has represented cure and eliminated the risk of future hemorrhage. The role of radiosurgery in the treatment of angiographically occult vascular malformations (AOVMs) has been less well defined. In the initial 32 months of operation of the 201-source cobalt-60 gamma knife at the University of Pittsburgh, 24 patients meeting strict criteria for high-risk AOVMs were treated. Radiosurgery was used conservatively; each patient had sustained two or more hemorrhages and had a magnetic resonance imaging-defined AOVM located in a region of the brain where microsurgical removal was judged to pose an excessive risk. Venous angiomas were excluded by performance of high-resolution subtraction angiography in each patient. Fifteen malformations were in the medulla, pons, and/or mesencephalon, and 5 were located in the thalamus or basal ganglia. Follow-up ranged from 4 to 24 months. Nineteen patients either improved or remained clinically stable and did not hemorrhage again during the follow-up interval. One patient suffered another hemorrhage 7 months after radiosurgery. Five patients experienced temporary worsening of pre-existing neurological deficits that suggested delayed radiation injury. Magnetic resonance imaging demonstrated signal changes and edema surrounding the radiosurgical target. Dose-volume guidelines for avoiding complications were constructed. Our initial experience indicates that stereotactic radiosurgery can be performed safely in patients with small, well-circumscribed AOVMs located in deep, critical, or relatively inaccessible cerebral locations. Because cerebral angiography is not useful in following patients with AOVMs, long-term magnetic resonance imaging and clinical studies will be necessary to determine whether the natural history of such lesions is changed by radiosurgery.


Neurosurgery ◽  
2007 ◽  
Vol 61 (2) ◽  
pp. 244-254 ◽  
Author(s):  
Werner Weber ◽  
Bernhard Kis ◽  
Ralf Siekmann ◽  
Paul Jans ◽  
Rudolf Laumer ◽  
...  

Abstract OBJECTIVE Preoperative embolization in accordance with multimodal therapies for cerebral arteriovenous malformations (AVMs) is generally the first step in treatment and may result in complete obliteration. The Onyx liquid embolic system (Micro Therapeutics, Inc., Irvine, CA) may offer advantages for intranidal targeted embolization and microsurgical resection. We present our experience in the combined treatment of intracranial AVMs using Onyx embolization and neurosurgical resection. METHODS We treated a total of 47 patients for compact intracranial AVMs that were located in the frontal or frontoparietal area (16 patients); temporal, temporoparietal, or temporo-occipital regions (12 patients); parietal or parieto-occipital areas (8 patients); occipital regions (8 patients); had basal ganglia involvement (2 patients); and was cerebellar (1 patient). The Spetzler-Martin grading scale values were as follows: 25 patients were Grades I or II, 10 patients were Grade III, and 12 patients were Grades IV or V. Twenty-three AVMs were located in eloquent brain regions. RESULTS After we performed final embolizations, the mean nidus reduction was 84%. Seven patients had new, nondisabling neurological deficits, and four patients had new, disabling neurological deficits after embolization. Periprocedurally, five vessel perforations and four stuck microcatheters were encountered without clinical deficits. In two patients, delayed hemorrhage after embolization occurred with good clinical outcome. We completely resected 46 AVMs; in one patient, we detected an AVM on postoperative angiography. The mean operative time was 4.7 hours, and the mean blood loss was 455 mL. Clinical status worsened postoperatively in 14 patients. Angiographic and clinical follow-up examinations were available for 42 patients (89%); the average follow-up period was 13 months. We found no relapse of arteriovenous shunt. Fourteen patients improved clinically after discharge. Of the 42 patients followed up, 23 individuals had no neurological deficit, 16 had a nondisabling deficit, and three had a disabling deficit. CONCLUSION Preoperative use of the Onyx liquid embolic system in cerebral AVM treatment allows profound occlusion by targeted embolization and provides a basis for safe neurosurgical resection.


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