scholarly journals Fatal Hemorrhage in Cerebral Proliferative Angiopathy

2012 ◽  
Vol 18 (3) ◽  
pp. 309-313 ◽  
Author(s):  
H. Maekawa ◽  
M. Tanaka ◽  
H. Hadeishi

Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality with several angiomorphological features that are distinct from brain arteriovenous malformations (AVMs). The natural history of CPAs indicates a lower risk for hemorrhage compared to brain AVMs. A 62-year-old woman presented with gait instability and dysarthria. MRI and angiography revealed a diffuse vascular network involving the tectum and cerebellar vermis with intermingled brain parenchyma. This lesion had no dominant feeder, high-flow arteriovenous shunt, flow-related aneurysm or highly dilated veins on angiogram. These findings were consistent with a diagnosis of CPA. During follow-up, she developed progressive gait instability and eye movement abnormalities, but no remarkable change was detected on the repeated MRI and angiography. Nine years later, she died of mesencephalic hemorrhage originating from the CPA. To the best of our knowledge, this is the first description of a patient with CPA who died as a result of the initial hemorrhage. It is important to recognize that a part of CPAs is aggressive and can be more vulnerable to critical hemorrhage.

2018 ◽  
Vol 17 (2) ◽  
pp. E67-E67 ◽  
Author(s):  
Stephan A Munich ◽  
Marie Christine Brunet ◽  
Robert M Starke ◽  
Jacques J Morcos

Abstract Pure arterial malformations are rarely-encountered intracranial lesions, often mistaken for arteriovenous malformations. A minority of these lesions may harbor associated aneurysms. In a recent series of 12 patients with pure arterial malformations, 3 patients had associated aneurysms.1 As the authors describe, the presentation and natural history of pure arterial malformations generally are considered benign. In over 85% of cases reported in the literature, they are discovered incidentally and in cases with documented follow-up the angio-architecture tends to remain stable.1  However, here we present the case of an aneurysm associated with a pure arterial malformation managed with microsurgical clipping. The patient presented to us with the development of hemorrhage within and/or around the lesion and the development of a partial CN III palsy. Using a cranio-orbital approach, we successfully performed clipping of the offending vessel in the region of the basilar apex. Critical to the successful treatment of this lesion were: the correct identification of the offending vessel, recognition of the presence of perforating arteries, and clipping of the offending artery distal to the origin of the perforating arteries. This case represents a rare instance of a symptomatic pure arterial malformation with associated aneurysm.  Verbal consent was provided by the patient for reproduction and publication of her case.


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.


Neurosurgery ◽  
1990 ◽  
Vol 26 (5) ◽  
pp. 725-735 ◽  
Author(s):  
Christopher S. Ogilvy

Abstract There have been numerous case reports and series of patients treated with partial brain irradiation, linear accelerator-based radiosurgery, gamma knife radiosurgery, and Bragg peak therapy for inoperable arteriovenous malformations (AVMs). These cases are summarized and compared. There is convincing evidence that radiation therapy does have a role in obliterating carefully chosen inoperable lesions. The changes that occur in vessel walls after radiation are reviewed. Data about x-ray and gamma radiation are mostly historical and difficult to evaluate because of the techniques of partial brain irradiation. There is a lack of data about the volume of AVM treated and the minimum dose delivered to the AVM nidus. For gamma knife, heavy particle, and linear accelerator therapy, more complete data are available. The incidence of hemorrhage during the first 2 years after treatment, when radiation-induced vascular changes are proposed to occur, is approximately 2.6% per year for gamma knife therapy, 2% per year for proton beam therapy, 2.3% per year for helium beam therapy, and 2.3% per year for linear accelerator therapy. These rates are similar to the recurrence rate for hemorrhage of 2.2 to 3% per year expected based on the natural history of untreated AVMs. If AVM obliteration after therapy is not achieved, the incidence of recurrent hemorrhage remains between 2% per year after treatment with gamma knife therapy. The incidence of hemorrhage for all patients treated was reported as 0.15% per year in one study and 20% over 8 years in a follow-up study using proton beam therapy. Mortality from hemorrhage after treatment was 0.6% after gamma knife therapy, 2.3% after helium beam therapy, and 2 to 5% after proton beam therapy. These figures for mortality are all lower than the 11% observed for the natural history of untreated AVMs. Permanent neurological deficits experienced as a complication of radiation occurred in 2 to 3% of patients treated with gamma knife therapy, 4% of patients treated with helium beam therapy, 1.7% of patients treated with proton beam therapy, and 3% of patients treated with stereotactic linear accelerator therapy. Proton beam therapy has been used for both small and large lesions. The majority of lesions in patients treated with gamma knife, helium beam, and linear accelerator therapy have been small (usually less than 3.0 cm average diameter) lesions. In these patients with small inoperable lesions treated with accurately directed fields of isocentric radiation, the greatest incidence of AVM obliteration has been observed on follow-up angiograms. Larger lesions may undergo vascular wall thickening with subsequent protection from recurrent hemorrhage, but more data are needed to support this hypothesis.


2009 ◽  
Vol 37 (4) ◽  
pp. 619-623 ◽  
Author(s):  
G. D. Puri ◽  
I. Sen ◽  
J. R. Bapuraj

This report describes three children, aged eight to 11 years, with high-flow cerebral arteriovenous malformations who underwent interventional neuroradiological procedures involving glue (N-butyl cyanoacrylate) embolisation under general anaesthesia. The procedure was facilitated by relative hypotension induced by esmolol infusion and intravenous adenosine boluses. To allow controlled deposition of N-butyl cyanoacrylate into the arteriovenous malformations, glue injection was synchronised with the onset of adenosine-induced brief cardiac standstill. This resulted in satisfactory obliteration of the arteriovenous malformations nidus in all cases. The haemodynamic modulations, including the adenosine-induced brief cardiac standstill, was noted to not affect the BIS values in our patients. All patients had satisfactory obliteration of their arteriovenous malformations and had good neurological outcomes at one-year follow-up.


2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S264-S266
Author(s):  
R Ungaro ◽  
R Jordan ◽  
C Yzet ◽  
P Bossuyt ◽  
F Baert ◽  
...  

Abstract Background The optimal endoscopic target in early Crohn’s disease (CD) that limits long-term disease complications is unknown. Methods We analysed medical records from patients who had follow-up data since the end of CALM. Patients with Crohn’s disease endoscopic index of severity (CDEIS) scores at the end of CALM were included. The primary outcome was a composite of major adverse outcomes reflecting CD progression: new internal fistula/abscess, stricture, perianal fistula/abscess, CD hospitalisation, or CD surgery since the end of CALM. We compared median CDEIS and per cent improvement from baseline CDEIS. Youden index analysis was used to identify optimal CDEIS cut-off score associated with CD progression. Kaplan–Meier and Cox regression methods were used to compare rates of progression by different CDEIS targets. Multivariable models were adjusted for age, prior surgery, and stricturing behaviour. Results 110 patients with median age 28 (IQR 22–38) years, disease duration 0.2 (0.1–0.5) years, and median follow up of 3.1 (1.9–4.4) years were included. Eleven per cent had a history of stricture, 5.5% history of surgery, and 52% were originally in the tight control arm of the CALM study. Median CDEIS score at end of CALM was 3 (0–5.4) and 32 (29%) patients had disease progression. Baseline median CDEIS score was similar between those with and without progression [10.9 (7.5–15.5) vs. 11.9 (8–17.5)]. Median CDEIS score at the end of CALM was higher among those with progression [1.3 (0–5.1) vs. 4.9 (3–9.1), p < 0.001)]. Patients within higher quartiles of CDEIS score had higher rates of progression over time (Figure 1). Patients without disease progression had a greater median decrease in CDEIS score from baseline to end of CALM [90% (60–100%) vs. 50% (30–80%), p < 0.001]. The optimal CDEIS score cut-off was 2 with sensitivity 84%, specificity 60% and NPV 90% for progression. Patients with CDEIS ≤ 2 had less progression over time compared with patients with > 50% improvement from baseline CDEIS (not reaching CDEIS ≤ 2) and those not meeting either endpoint (Figure 2). On adjusted analysis, CDEIS score ≤ 2 was associated with a decreased risk of progression (aHR 0.23, 95% CI 0.09–0.56). Conclusion In early CD, a CDEIS score ≤ 2 is optimal cut-off associated with a lower risk of disease progression.


2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Alaa Nabawi ◽  
Nader Abraham ◽  
Ayman Nabawi

Abstract Arteriovenous malformations (AVMs) are congenital vascular anomalies resulting from defects in angiogenesis. Approximately 40% of AVMs go undetected after birth and only experience the delayed clinical onset of symptoms in adulthood. AVMs are rare, representing only 1.5% of all vascular anomalies. The most common sites for the aberrant vascular nidus are the oral cavity and maxillofacial region, which represent 50% of the cases. AVMs are the most challenging and life-threatening form of vascular malformation. Exsanguination, thrombus detachment and embolization are the most hazardous operative risks. Small case series revealed a 75% recurrence rate during a 5-year follow-up, which adds another layer of complexity to their management. Large lesions in the head and neck cause deformation to the patient and present a challenge to the surgeon during their excision among vital structures and reconstruction of the 3D complex defects.


2010 ◽  
Vol 113 (4) ◽  
pp. 715-722 ◽  
Author(s):  
Ichiro Yuki ◽  
Robert H. Kim ◽  
Gary Duckwiler ◽  
Reza Jahan ◽  
Satoshi Tateshima ◽  
...  

Object High-flow fistulas associated with brain arteriovenous malformations (AVMs) pose a significant challenge to both stereotactic radiosurgery (SRS) and surgical treatment. The purpose of this study was to examine the outcomes of multimodality treatment of AVMs in association with a large arteriovenous fistula (AVF), with a special focus on endovascular embolization and its associated complications. Methods One hundred ninety-two patients harboring cerebral AVMs underwent endovascular treatment in the authors' department between 1997 and 2003. Of these, the authors selected 74 patients presenting with an AVM associated with high-flow AVF(s) for a retrospective analysis based on the findings of superselective angiography. After endovascular embolization, 32 patients underwent resection, 33 underwent either SRS or hypofractionated stereotactic radiotherapy (HSRT), and 3 underwent both surgery and SRS. Six patients underwent embolization only. Immediate and midterm treatment outcomes were analyzed. Results Fifty-seven (77%) of the 74 patients had AVMs that were Spetzler-Martin Grade III or higher. A complete resection was achieved in all 32 patients. Of patients who underwent SRS/HSRT, 13 patients (39.3%) had either complete or > 90% obliteration of the AVM, and 2 patients (6.1%) had incomplete obliteration. Fourteen patients (42.4%) with residual AVM underwent repeated radiotherapy (and remain under observation). Of the 3 patients who underwent both SRS and resection, resection was complete in 2 and incomplete in one. No follow-up was obtained in 6 patients (8.1%). An endovascular complication was observed in 4 patients (5.4%). Fistula embolization was safely performed in every patient, whereas every endovascular complication was associated with other procedures such as nidus embolization. Conclusions Endovascular occlusion of the fistulous component was successfully achieved in every patient; every endovascular complication in this series was related to other procedures such as nidus embolization. The importance of the fistula treatment should be emphasized to minimize the endovascular complications and to maximize the treatment effect when a multimodality therapy is used to treat brain AVMs with large AVF.


2014 ◽  
Vol 37 (3) ◽  
pp. E7 ◽  
Author(s):  
Isaac Josh Abecassis ◽  
David S. Xu ◽  
H. Hunt Batjer ◽  
Bernard R. Bendok

Object The authors aimed to systematically review the literature to clarify the natural history of brain arteriovenous malformations (BAVMs). Methods The authors searched PubMed for one or more of the following terms: natural history, brain arteriovenous malformations, cerebral arteriovenous malformations, and risk of rupture. They included studies that reported annual rates of hemorrhage and that included either 100 patients or 5 years of treatment-free follow-up. Results The incidence of BAVMs is 1.12–1.42 cases per 100,000 person-years; 38%–68% of new cases are first-ever hemorrhage. The overall annual rates of hemorrhage for patients with untreated BAVMs range from 2.10% to 4.12%. Consistently implicated in subsequent hemorrhage are initial hemorrhagic presentation, exclusively deep venous drainage, and deep and infrantentorial brain location. The risk for rupture seems to be increased by large nidus size and concurrent arterial aneurysms, although these factors have not been studied as thoroughly. Venous stenosis has not been implicated in increased risk for rupture. Conclusions For patients with BAVMs, although the overall risk for hemorrhage seems to be 2.10%–4.12% per year, calculating an accurate risk profile for decision making involves clinical attention and accounting for specific features of the malformation.


Neurosurgery ◽  
2017 ◽  
Vol 82 (1) ◽  
pp. 35-47 ◽  
Author(s):  
Ali Tayebi Meybodi ◽  
Helen Kim ◽  
Jeffrey Nelson ◽  
Steven W Hetts ◽  
Timo Krings ◽  
...  

Abstract BACKGROUND Cerebral arteriovenous malformations (AVMs) are common in patients with hereditary hemorrhagic telangiectasia (HHT). However, due to the rarity of HHT and little published evidence of outcomes from management of brain AVMs in this disease, current international HHT guidelines recommend an individualized approach. Specifically, the outcomes for surgical vs nonsurgical management of these lesions have not been reported to date. OBJECTIVE To report long-term outcomes of surgical resection of brain AVMs in HHT patients compared to outcomes in nonsurgically treated patients. METHODS From the database of the Brain Vascular Malformation Consortium HHT project, 19 patients with 20 resected AVMs (group 1) and 22 patients with 33 AVMs who received nonsurgical treatment (group 2) were studied. The groups were retrospectively reviewed for changes in functional status (modified Rankin Scale score) during the follow-up period. RESULTS During the follow-up period, 9% of patients in group 1 suffered from worsening of functional status, whereas this figure was 16% for group 2 (P > .05). Functional outcomes were not statistically different between the 2 groups at the latest follow-up (P > .05). CONCLUSION HHT patients treated surgically for brain AVMs appear to have long-term functional outcomes comparable to nonsurgical (including observational) therapy with fewer unfavorable outcomes. It is therefore reasonable to consider surgical resection as a management option in the multidisciplinary team's individualized treatment strategy for HHT patients with brain AVMs.


2021 ◽  
Vol 10 (10) ◽  
pp. 2186
Author(s):  
Myung Ji Kim ◽  
Kyung Won Chang ◽  
So Hee Park ◽  
Won Seok Chang ◽  
Jong Hee Chang ◽  
...  

We evaluated for possible predictors of radiation-induced changes (RICs) after gamma knife radiosurgery (GKRS) for arteriovenous malformations (AVMs). We identified the nidal component within AVMs to analyze the correlation between the volume of brain parenchyma within the 50% isodose line (IDL) and RICs. We retrospectively reviewed patients with AVMs who underwent a single-session of GKRS at our institution between 2007 and 2017 with at least a 2-year minimum follow-up. Follow-up magnetic resonance images were evaluated for newly developed T2 signal changes and the proportions of nidus and intervening parenchyma were quantified. A total of 180 AVM patients (98 males and 82 females) with a median age of 34 years were included in the present study. The overall obliteration rate was 67.8%. The median target volume was 3.65 cc. The median nidus and parenchyma volumes within the 50% IDL were 1.54 cc and 2.41 cc, respectively. RICs were identified in 79 of the 180 patients (43.9%). AVMs associated with previous hemorrhages showed a significant inverse correlation with RICs. In a multivariate analysis, RICs were associated with a higher proportion of brain parenchyma within the 50% IDL (hazard ratio (HR) 169.033; p < 0.001) and inversely correlated with the proportion of nidus volume within the 50% IDL (HR 0.006; p < 0.001). Our study identified that a greater proportion of brain tissue between the nidus within the 50% IDL was significantly correlated with RICs. Nidus angioarchitectural complexity and the absence of a prior hemorrhage were also associated with RICs. The identification of possible predictors of RICs could facilitate radiosurgical planning and treatment decisions as well as the planning of appropriate follow-up after GKRS; this could minimize the risk of RICs, which would be particularly beneficial for the treatment of incidentally found asymptomatic AVMs.


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