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 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.

scholarly journals Developmental Venous Anomaly with Adjacent Parenchymal Abnormality Masquerading as Glioma-A Unique Case Report

2021 ◽  
pp. 1-9
Author(s):  
Amna Alvi ◽  
Rabail Raza ◽  
Fatima Mubarak ◽  
Asma Alvi
Keyword(s):  
Vascular Malformations ◽  
Common Type ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Invasive Procedures ◽  
Unique Case ◽  
Cavernous Malformations ◽  
Underlying Pathophysiology ◽  
Cerebral Vascular Malformations ◽  
Parenchymal Abnormality

Developmental venous anomaly (DVA)/ Cerebral Venous Angioma is the most common type of cerebral vascular malformations, mostly an incidental benign finding. But there are documented associated complications like parenchymal signal abnormalities, thrombosis, cavernous malformations and parenchymal atrophy. In this report, we present a unique case of cerebral DVA with cortical changes mimicking the Glioma. Altered haemodynamics in DVA is the underlying pathophysiology for these changes. Correct MRI interpretation, by an expert neuroradiologist, can establish the diagnosis of DVA and its related changes. Therefore, it can reduce the morbidity and mortality by preventing the unnecessary invasive procedures like biopsy for diagnosis.

scholarly journals Growing Dural Sinus Malformation with Associated Developmental Venous Anomaly, Multiple Cavernomas and Facial Venous Malformation in an Infant

2002 ◽  
Vol 8 (4) ◽  
pp. 421-430 ◽  
Author(s):  
Z. Mohamed ◽  
LL Batista ◽  
M. Sachet ◽  
J. Mahadevan ◽  
H. Alvarez ◽  
...  
Keyword(s):  
Complex Disease ◽  
Venous Malformation ◽  
Venous Hypertension ◽  
Dural Sinus ◽  
Cerebral Vein ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Cavernous Malformations ◽  
Dural Sinus Malformation ◽  
The Right

This is an unusual case report of an infant, who initially presented with a facial haemangioma and was later diagnosed to have a dural sinus malformation (DSM) involving the torcula. The DSM increased in size lateralising to the right transverse sinus at three months of age. Postnatal enlargement of the dural sinus has not been described before suggesting a delay in the maturation of the dural sinus which normally would occur antenatally. There was a further association with a complex developmental venous anomaly (DVA) draining the right cerebral hemisphere into the deep cerebral vein and multiple cavernous malformations. The DVA was not clearly demonstrated at age one month but was more obvious at age three months. This would be the first reported case of DSM associated with a DVA. Increasing venous hypertension probably contributed to the poor opacification of the DVA on follow-up angiography at age six months and to the haemorrhagic changes within the cavernomas on magnetic resonance imaging (MRI). The therapeutic goal was to correct venous hypertension by partially embolising the dural shunts to remodel the cerebral vasculature and preserve the patent sinus. The treatment strategy and possible link between the complex disease entities presented in this infant are discussed. Despite these attemps, the lesion continued to grow compressing the posterior fossa structures. The infant died at nine months of age.

Cavernous Malformation Associated With Arterialized Developmental Venous Anomaly: A Case Report

Neurosurgery ◽  
2017 ◽  
Vol 80 (6) ◽  
pp. E257-E262 ◽  
Author(s):  
Kenta Nakase ◽  
Yasushi Motoyama ◽  
Tokiko Nakai ◽  
Yasuhiro Takeshima ◽  
Ichiro Nakagawa ◽  
...  
Keyword(s):  
Case Report ◽  
Clinical Presentation ◽  
Cavernous Malformation ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Outflow Obstruction ◽  
Venous Outflow ◽  
Cavernous Malformations ◽  
Surgical Specimen ◽  
Cerebellar Hemorrhage

Abstract BACKGROUND AND IMPORTANCE: Formation of cavernous malformations (CMs) has been recognized to be associated with developmental venous anomaly (DVA) by many authors. Hemodynamic stress due to venous outflow restriction could be hypothesized as a cause. On the other hand, a rare subgroup of DVA with an arterial component has been reported as likely to hemorrhage or be symptomatic. Cases of arterialized DVAs reported previously have not been associated with the presence of CM. CLINICAL PRESENTATION: We present herein a case report of arterialized DVA in the brainstem with repeated cerebellar hemorrhage. The 49-year-old patient was treated with surgical evacuation of hematoma. A surgical specimen from the hematoma cavity demonstrated CMs on histological examination. CONCLUSION: To the best of our knowledge, this represents the first report of CM associated with an arterialized DVA. In addition to venous congestion due to outflow obstruction, bleeding from the arterial component of the DVA might be considered as a cause of CM formation.

De Novo Cavernous Malformation Associated with a Pre-existing Developmental Venous Anomaly

2019 ◽  
Vol 30 (1) ◽  
pp. 181-184
Author(s):  
Daniel García-Pérez ◽  
Irene Panero ◽  
Alfonso Lagares ◽  
Pedro González
Keyword(s):  
De Novo ◽  
Cavernous Malformation ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly

scholarly journals Morbidity After Symptomatic Hemorrhage of Cerebral Cavernous Malformation

Stroke ◽  
2020 ◽  
Vol 51 (10) ◽  
pp. 2997-3006
Author(s):  
Li Ma ◽  
Shuo Zhang ◽  
Zongze Li ◽  
Chun-Xue Wu ◽  
Zhaozhao Wang ◽  
...  
Keyword(s):  
Calibration Curve ◽  
Odds Ratio ◽  
Adjusted Odds Ratio ◽  
Cavernous Malformation ◽  
Cerebral Cavernous Malformation ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Discriminative Ability ◽  
Increased Risk

Background and Purpose: Symptomatic hemorrhage contributes to an increased risk of repeated bleeding and morbidity in cerebral cavernous malformation (CCM). A better understanding of morbidity after CCM hemorrhage would be helpful to identify patients of higher risk for unfavorable outcome and tailor individualized management. Methods: We identified 282 consecutive patients who referred to our institute from 2014 to 2018 for CCM with symptomatic hemorrhage and had an untreated follow-up period over 6 months after the first hemorrhage. The morbidity after hemorrhage was described in CCM of different features. Nomogram to predict morbidity was formulated based on the multivariable model of risk factors. The predictive accuracy and discriminative ability of nomogram were determined with concordance index (C-index) and calibration curve, and further validated in an independent CCM cohort of a prospective multicenter study from 2019 to 2020. Results: The overall morbidity of CCM was 26.2% after a mean follow-up of 1.9 years (range 0.5–3.5 years) since the first hemorrhage. The morbidity during untreated follow-up was associated with hemorrhage ictus (adjusted odds ratio per ictus increase, 4.17 [95% CI, 1.86–9.33]), modified Rankin Scale score at initial hemorrhage (adjusted odds ratio per point increase, 2.57 [95% CI, 1.82–3.63]), brainstem location (adjusted odds ratio, 2.93 [95% CI, 1.28–6.68]), and associated developmental venous anomaly (adjusted odds ratio, 2.21 [95% CI, 1.01–4.83]). Subgroup analysis revealed similar findings in brainstem and non-brainstem CCM. Nomogram was contracted based on these features. The calibration curve showed good agreement between nomogram prediction and actual observation. The C-index of nomogram predicting morbidity was 0.83 (95% CI, 0.77–0.88). In validation cohort, the nomogram maintained the discriminative ability (C-index, 0.87 [95% CI, 0.78–0.96]). Conclusions: Multiple symptomatic hemorrhages, initial neurological function after hemorrhage, brainstem location, and associated developmental venous anomaly were associated with morbidity of CCM hemorrhage. The nomogram represented a practical approach to provide individualized risk assessment for CCM patients. Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT04076449.

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