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From Embryology


J Neurosurg. 2015 Oct;123(4):872-90. doi: 10.3171/2014.12.JNS141249. Epub 2015 Jul 31.

Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis

Yan J1, Wen J2, Gopaul R1, Zhang CY1, Xiao SW1.


OBJECT There have been many multidisciplinary approaches to the treatment of vein of Galen malformations. Endovascular embolization is the first option for treatment. However, the effects of the treatment remain controversial. The aim of this study is to assess the efficacy and safety of endovascular embolization to treat patients with vein of Galen malformations. METHODS This paper includes a retrospective analysis of a sample of 667 patients who underwent endovascular embolization to treat vein of Galen malformations. The data were obtained through a literature search of PubMed databases. The authors also evaluate the efficacy and safety of the treatment. Mortality within the follow-up period is analyzed. Pooled estimates of proportions with corresponding 95% CIs were calculated using raw (i.e., untransformed) proportions (PRAW). RESULTS In the 34 studies evaluated, neonates accounted for 44% of the sample (95% CI 31%-57%; I(2) = 92.5%), infants accounted for 41% (95% CI 30%-51%; I(2) = 83.3%), and children and adults accounted for 12% (95% CI 7%-16%; I(2) = 52.9%). The meta-analysis revealed that complete occlusion was performed in 57% (95% CI 48%-65%; I(2) = 68.2%) of cases, with partial occlusion in 43% (95% CI 34%-51%; I(2) = 70.7%). The pooled proportion of patients showing a good outcome was 68% (95% CI 61%-76%; I(2) = 77.8%), while 31% showed a poor outcome (95% CI 24%-38%; I(2) = 75.6%). The proportional meta-analysis showed that postembolization mortality and complications were reported in 10% (95% CI 8%-12%; I(2) = 42.8%) and 37% (95% CI 29%-45%; I(2) = 79.1%), respectively. Complications included cerebral hemorrhage, cerebral ischemia, hydrocephalus, leg ischemia, and vessel perforation. CONCLUSIONS The successful treatment of vein of Galen malformations remains a complex therapeutic challenge. The authors' analysis of clinical history and research literature suggests that vein of Galen malformations treated with endovascular embolization can result in an acceptable mortality rate, complications, and good clinical outcome. Future large-scale, multicenter, randomized trials are necessary to confirm these findings. KEYWORDS: DSA = digital subtraction angiography; VGAM = vein of Galen aneurysmal malformation; complications; endovascular embolization; meta-analysis; outcome; vascular disorders; vascular malformations; vein of Galen malformation

PMID 26230476

Radiological and clinical features of vein of Galen malformations

J Neurointerv Surg. 2015 Jun;7(6):443-8. doi: 10.1136/neurintsurg-2013-011005. Epub 2014 Apr 30.

Chow ML1, Cooke DL2, Fullerton HJ3, Amans MR2, Narvid J2, Dowd CF2, Higashida RT2, Halbach VV2, Hetts SW2.


BACKGROUND: Vein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized. OBJECTIVE: To examine the clinical and radiological features of VOGMs and the predictors of outcome in patients. METHODS: We retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ(2) analyses. RESULTS: Forty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at <10 days of age and elective embolization after 1 year of age. Patients commonly presented with hydrocephalus (65.9%) and congestive heart failure (61.0%). Mixed-type (31.7%) VOGM was more common in our cohort than purely mural (29.3%) or choroidal (26.8%) types. The most common feeding arteries were the choroidal and posterior cerebral arteries. Transarterial embolization with coils was the most common technique used to treat VOGMs at our institution. Functional outcome was normal or only mildly disabled in 50% of the cases at last follow-up (median=3 years, range=0-23 years). Younger age at first diagnosis, congestive heart failure, and seizures were predictive of adverse clinical outcome. The survival rate in our sample was 78.0% and complete thrombosis of the VOGM was achieved in 62.5% of patients. CONCLUSIONS: VOGMs continue to be challenging to treat and manage. Nonetheless, endovascular approaches to treatment are continuing to be refined and improved, with increasing success. The neurodevelopmental outcomes of affected children whose VOGMs are treated may be good in many cases. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to KEYWORDS: Angiography; Arteriovenous Malformation; Intervention; Paediatrics

PMID 24789593

A Functional Perspective on the Embryology and Anatomy of the Cerebral Blood Supply

J Stroke. 2015 May;17(2):144-58. doi: 10.5853/jos.2015.17.2.144. Epub 2015 May 29.

Menshawi K1, Mohr JP1, Gutierrez J1.


The anatomy of the arterial system supplying blood to the brain can influence the development of arterial disease such as aneurysms, dolichoectasia and atherosclerosis. As the arteries supplying blood to the brain develop during embryogenesis, variation in their anatomy may occur and this variation may influence the development of arterial disease. Angiogenesis, which occurs mainly by sprouting of parent arteries, is the first stage at which variations can occur. At day 24 of embryological life, the internal carotid artery is the first artery to form and it provides all the blood required by the primitive brain. As the occipital region, brain stem and cerebellum enlarge; the internal carotid supply becomes insufficient, triggering the development of the posterior circulation. At this stage, the posterior circulation consists of a primitive mesh of arterial networks that originate from projection of penetrators from the distal carotid artery and more proximally from carotid-vertebrobasilar anastomoses. These anastomoses regress when the basilar artery and the vertebral arteries become independent from the internal carotid artery, but their persistence is not uncommon in adults (e.g., persistent trigeminal artery). Other common remnants of embryological development include fenestration or duplication (most commonly of the basilar artery), hypoplasia (typically of the posterior communicating artery) or agenesis (typically of the anterior communicating artery). Learning more about the hemodynamic consequence that these variants may have on the brain territories they supply may help understand better the underlying physiopathology of cerebral arterial remodeling and stroke in patients with these variants. KEYWORDS: Arterial variants; Cerebral arteries; Circle of willis; Embryology; Remodeling; Stroke

PMID 26060802

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


Semin Ultrasound CT MR. 2011 Jun;32(3):252-63. doi: 10.1053/j.sult.2011.02.001. Cerebral venous development in relation to developmental venous anomalies and Vein of Galen aneurysmal malformations. Pearl M1, Gregg L, Gandhi D. Author information Abstract Cerebrovascular venous development and intracranial vascular malformations are extensive topics for which volumes of text may be devoted. However, a basic knowledge of the embryology of cerebral venous system and venous architecture is essential for understanding of cerebral vascular malformations. The aim of this work is to provide the reader with a brief overview of the development of the cranial venous anatomy. We will highlight the superficial and deep venous systems with special attention to developmental venous anomalies and vein of Galen aneurysmal malformations. Copyright © 2011 Elsevier Inc. All rights reserved. PMID 21596280