scholarly journals Cerebral Cavernous Malformations, Developmental Venous Anomaly, and Its Coexistence: A Review

2020 ◽  
Vol 83 (4) ◽  
pp. 360-368 ◽  
Author(s):  
Pretty Sara Idiculla ◽  
Dhineshreddy Gurala ◽  
Jobin Philipose ◽  
Kartikeya Rajdev ◽  
Prateek Patibandla
Keyword(s):  
English Language ◽  
Vascular Malformations ◽  
Incidental Finding ◽  
Neurological Deficits ◽  
Vascular Lesions ◽  
Cerebral Cavernous Malformations ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Cavernous Malformations ◽  
Acute Thrombosis

Background: Cerebral cavernous malformations (CCMs) are intracranial vascular malformations that can exist as a single lesion or mixed vascular lesions. The most common mixed form is the coexistence of CCM with an associated developmental venous anomaly (DVA). In this paper, we aim to give a comprehensive review of CCM, DVA, and their coexistence as mixed lesions. A PubMed search using the keywords “Cerebral cavernous malformations, Developmental venous anomaly, Mixed Cerebral cavernous malformations with Developmental venous anomaly” was done. All studies in the English language in the past 10 years were analyzed descriptively for this review. Summary: The search yielded 1,249 results for “Cerebral cavernous malformations,” 271 results for “Developmental venous anomaly,” and 5 results for “Mixed Cerebral cavernous malformations with Developmental venous anomaly.” DVA is the most common intracranial vascular malformation, followed by CCM. CCM can have a wide array of clinical presentations like hemorrhage, seizures, or focal neurological deficits or can also be an incidental finding on brain imaging. DVAs are benign lesions by nature; however, venous infarction can occur in a few patients due to acute thrombosis. Mixed CCM with DVA has a higher risk of hemorrhage. CCMs are angiographically occult lesion, and cerebral digital subtraction angiography is the gold standard for the diagnosis of DVA. Mixed lesions, on the other hand, are best diagnosed with magnetic resonance imaging, which has also been effective in detecting specific abnormalities. Asymptomatic lesions are treated through a conservative approach, while clinically symptomatic lesions need surgical management. Conclusion: Individual CCM or DVA lesions have a benign course; however, when they coexist in the same individual, the hemorrhagic risk is increased, which prompts for rapid diagnosis and treatment.

Unique enlarging cavernous malformation secondary to abnormal arteriovenous shunting through an associated developmental venous anomaly

2021 ◽  
pp. 197140092110428
Author(s):  
Nimisha Parikh ◽  
Richard Williamson ◽  
Matthew Kulzer ◽  
Albert Sohn ◽  
Warren M Chang ◽  
...  
Keyword(s):  
Unusual Case ◽  
Cavernous Malformation ◽  
Vascular Malformations ◽  
Venous Hypertension ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Arteriovenous Shunting ◽  
Cavernous Malformations ◽  
Progressive Growth

Cavernous malformations are angiographically occult vascular malformations. They are often associated with a developmental venous anomaly through poorly understood mechanisms. We present an unusual case of a gradually enlarging cavernous malformation associated with a developmental venous anomaly with arteriovenous shunting, suggesting venous hypertension or reflux as a potential cause of progressive growth.

The Juxtaposition of a Capillary Telangiectasia, Cavernous Malformation, and Developmental Venous Anomaly in the Brainstem of a Single Patient: Case Report

Neurosurgery ◽  
2001 ◽  
Vol 49 (5) ◽  
pp. 1246-1250 ◽  
Author(s):  
Richard E. Clatterbuck ◽  
İlhan Elmacı ◽  
Daniele Rigamonti
Keyword(s):  
Cavernous Malformation ◽  
Vascular Malformations ◽  
Magnetic Resonance Imaging Examination ◽  
Developmental Venous Anomaly ◽  
Venous Malformations ◽  
Venous Anomaly ◽  
Single Patient ◽  
Cavernous Malformations ◽  
Swallowing Problems ◽  
Capillary Telangiectasia

ABSTRACT OBJECTIVE AND IMPORTANCE Capillary telangiectasias, cavernous malformations, and developmental venous anomalies are all vascular malformations that occur on the capillary-venous side of the cerebral circulation. The associations of capillary telangiectasias with venous malformations, cavernous malformations with venous malformations, and capillary telangiectasias with cavernous malformations have all been described; however, the association of all three lesions in a single patient is extremely rare. CLINICAL PRESENTATION A 52 year-old Caucasian woman presented to our clinic with an extended history of confusion, distorted visual perceptions, photophobia, neck pain, swallowing problems, and poor balance. The patient's examination was remarkable for difficulty concentrating, mild rotatory nystagmus, subtle decreased sensation over the left side of the face and body, and brisk reflexes. Review of the patient's magnetic resonance imaging examination demonstrated a cavernous malformation, a capillary telangiectasia, and a developmental venous anomaly located adjacent to one another in the brainstem. INTERVENTION Given the patient's complex constellation of symptoms and relatively mild neurological findings, it was difficult to ascribe any one of them to a specific vascular malformation. Conservative management of this patient's vascular malformations was decided upon. CONCLUSION Juxtaposition of these three different vascular lesions in the brainstem of an otherwise normal individual suggests a relationship among them. Although there are several theories that link similar associations through physiological mechanisms such as venous hypertension, we propose that a developmental event disrupting local capillary-venous pattern formation is a plausible alternative.

scholarly journals Developmental timing of CCM2 loss influences cerebral cavernous malformations in mice

2011 ◽  
Vol 208 (9) ◽  
pp. 1835-1847 ◽  
Author(s):  
Gwénola Boulday ◽  
Noemi Rudini ◽  
Luigi Maddaluno ◽  
Anne Blécon ◽  
Minh Arnould ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Vascular Malformations ◽  
Vascular Lesions ◽  
Developmental Timing ◽  
Cerebral Cavernous Malformations ◽  
Loss Of Function ◽  
Cavernous Malformations ◽  
The Central Nervous System ◽  
Dominant Condition

Cerebral cavernous malformations (CCM) are vascular malformations of the central nervous system (CNS) that lead to cerebral hemorrhages. Familial CCM occurs as an autosomal dominant condition caused by loss-of-function mutations in one of the three CCM genes. Constitutive or tissue-specific ablation of any of the Ccm genes in mice previously established the crucial role of Ccm gene expression in endothelial cells for proper angiogenesis. However, embryonic lethality precluded the development of relevant CCM mouse models. Here, we show that endothelial-specific Ccm2 deletion at postnatal day 1 (P1) in mice results in vascular lesions mimicking human CCM lesions. Consistent with CCM1/3 involvement in the same human disease, deletion of Ccm1/3 at P1 in mice results in similar CCM lesions. The lesions are located in the cerebellum and the retina, two organs undergoing intense postnatal angiogenesis. Despite a pan-endothelial Ccm2 deletion, CCM lesions are restricted to the venous bed. Notably, the consequences of Ccm2 loss depend on the developmental timing of Ccm2 ablation. This work provides a highly penetrant and relevant CCM mouse model.

scholarly journals De novo formation of cerebral cavernous malformation adjacent to existing developmental venous anomaly – an effect of change in venous pressure associated with management of a complex dural arterio-venous fistula

2016 ◽  
Vol 29 (6) ◽  
pp. 458-464 ◽  
Author(s):  
Hariprakash Chakravarthy ◽  
Tzu-Kang Lin ◽  
Yao-Liang Chen ◽  
Yi-Ming Wu ◽  
Chin-Hua Yeh ◽  
...  
Keyword(s):  
Case Report ◽  
Temporal Lobe ◽  
De Novo ◽  
Venous Pressure ◽  
Cerebral Cavernous Malformations ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Cavernous Malformations ◽  
Developmental Venous Anomalies ◽  
Venous Fistula

This is a case report of de novo development of two cerebral cavernous malformations adjacent to existing developmental venous anomalies. The development of cavernomas was noted over a follow-up period of 10 years. These developments happened during the course of staged endovascular management of a complex dural arterio-venous fistula along the right sphenoid wing. The patient presented with a proptosis secondary to lympho-haemangiomatous lesion of the fronto-orbital region and a high-flow right sphenoid wing dural arterio-venous fistula. During the initial period of conservative management of the dural arterio-venous fistula, he developed de novo cavernous malformations in the left mesial temporal lobe adjacent to a developmental venous anomaly in the temporal lobe, and along with this there was engorgement of deep veins related to another existing developmental venous anomaly in the brainstem. Later during the course of endovascular treatment of the dural arterio-venous fistula, a large brainstem cavernoma developed adjacent to the brainstem developmental venous anomaly. This case report discusses the cause-effect relationship of venous pressure changes related to management of dural arterio-venous fistula and de novo formation of cerebral cavernous malformations adjacent to existing developmental venous anomalies.

Effective surgical treatment of cerebral cavernous malformations: a multicenter study of 79 pediatric patients

2011 ◽  
Vol 8 (5) ◽  
pp. 522-525 ◽  
Author(s):  
Michael Hugelshofer ◽  
Nicola Acciarri ◽  
Ulrich Sure ◽  
Dimitrios Georgiadis ◽  
Ralf W. Baumgartner ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Refractory Epilepsy ◽  
Epileptic Seizures ◽  
Neurological Deficits ◽  
Vascular Lesions ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Persistent Symptoms ◽  
The Mean ◽  
The Brain

Object Cerebral cavernous malformations (CCMs) are common vascular lesions in the brain, affecting approximately 0.5% of the population and representing 10%–20% of all cerebral vascular lesions. One-quarter of all CCMs affect pediatric patients, and CCMs are reported as one of the main causes of brain hemorrhage in this age group. Symptoms include epileptic seizures, headache, and focal neurological deficits. Patients with symptomatic CCMs can be treated either conservatively or with resection if lesions cause medically refractory epilepsy or other persistent symptoms. Methods The authors retrospectively analyzed 79 pediatric patients (41 boys and 38 girls) from 3 different centers, who were surgically treated for their symptomatic CCMs between 1974 and 2004. The mean age of the children at first manifestation was 9.7 years, and the mean age at operation was 11.3 years. The main goal was to compare the clinical outcomes with respect to the location of the lesion of children who preoperatively suffered from epileptic seizures. Results Of these patients, 77.3% were seizure free (Engel Class I) after the resection of the CCM. Significant differences in the outcome between children who harbored CCMs at different locations were not found. Conclusions Resection seems to be the favorable treatment of symptomatic CCMs not only in adults but also in children.

scholarly journals The pathogenetic features of cerebral cavernous malformations: a comprehensive review with therapeutic implications

2010 ◽  
Vol 29 (3) ◽  
pp. E2 ◽  
Author(s):  
Khaled M. Krisht ◽  
Kevin J. Whitehead ◽  
Toba Niazi ◽  
William T. Couldwell
Keyword(s):  
Neurological Deficits ◽  
Therapeutic Modality ◽  
Vascular Lesions ◽  
Cerebral Cavernous Malformations ◽  
Mice Model ◽  
Genetic Studies ◽  
Cavernous Malformations ◽  
Therapeutic Implications ◽  
Model Studies ◽  
Shed Light

Cerebral cavernous malformations (CCMs) are common vascular lesions of the CNS that may lead to seizures, focal neurological deficits, and fatal hemorrhagic stroke. Human genetic studies have identified 3 genes associated with CCM, and biochemical and molecular studies in mice have elucidated signaling pathways with important therapeutic implications. In this review, the authors shed light on the 3 discovered CCM genes as well as their protein products, with particular emphasis on their signal transduction pathways and their interaction with one another. Close focus is directed at mice model studies involving the Ccm2 gene product signaling pathway, revealing an important role for the use of simvastatin or other RhoA inhibitors as a therapeutic modality in the treatment of CCM. The remaining challenges to creating a more faithful CCM animal model as well as future clinical and research implications are reviewed.

Venous-predominant parenchymal arteriovenous malformation: a rare subtype with a venous drainage pattern mimicking developmental venous anomaly

2008 ◽  
Vol 108 (6) ◽  
pp. 1142-1147 ◽  
Author(s):  
So-Hyang Im ◽  
Moon Hee Han ◽  
Bae Ju Kwon ◽  
Jung Yong Ahn ◽  
Cheolkyu Jung ◽  
...  
Keyword(s):  
Arteriovenous Malformation ◽  
Vascular Malformation ◽  
Vascular Malformations ◽  
Gamma Knife Radiosurgery ◽  
Vascular Lesions ◽  
Arterial Phase ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Arteriovenous Shunts

Object Considerable confusion exists in the literature regarding the classification of cerebrovascular malformations and their clinical significance. One example is provided by the atypical developmental venous anomaly (DVA) with arteriovenous shunt, because it remains controversial whether these lesions should be classified as DVAs or as atypical cases of other subtypes of cerebrovascular malformations. The purpose of this study was to clarify the classification of these challenging vascular lesions in an effort to suggest an appropriate diagnosis and management strategy. Methods The authors present a series of 15 patients with intracranial vascular malformations that were angiographically classified as atypical DVAs with arteriovenous shunts. This type of vascular malformation shows a fine arterial blush without a distinct nidus and early filling of dilated medullary veins that drain these arterial components during the arterial phase on angiography. Those prominent medullary veins converge toward an enlarged main draining vein, which together form the caput medusae appearance of a typical DVA. Results Based on clinical, angiographic, surgical, and histological findings, the authors propose classifying these vascular malformations as a subtype of an arteriovenous malformation (AVM), rather than as a variant of DVA or as a combined vascular malformation. Conclusions Correct recognition of this AVM subtype is required for its proper management, and its clinical behavior appears to follow that of a typical AVM. Gamma Knife radiosurgery appears to be a good alternative to resection, although long-term follow-up results require verification.

scholarly journals Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Nikolaos Mouchtouris ◽  
Nohra Chalouhi ◽  
Ameet Chitale ◽  
Robert M. Starke ◽  
Stavropoula I. Tjoumakaris ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Vascular Malformations ◽  
Imaging Techniques ◽  
Neurological Deficits ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Microsurgical Resection ◽  
Different Characteristics ◽  
The Brain ◽  
Methods Of Treatment

Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment—microsurgical resection, stereotactic radiosurgery, and conservative management—depending on the lesion characteristics.

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