Original ArticleManagement of Arteriovenous Malformations Associated with Developmental Venous Anomalies: A Literature Review and Report of 2 Cases
Introduction
The commonly recognized subgroups of cerebrovascular malformations include arteriovenous malformation (AVM), capillary telangiectasia, cavernous malformation, and developmental venous anomaly (DVA).1 The estimated incidence of AVM in population-based studies is generally approximately 1 in 100,000.2 AVM occurs when a collection of arteries and veins forms without an intervening capillary bed. This results in a high-pressure shunting of blood, thought to be responsible for the symptomatic presentation of hemorrhage at an annual rate of 2%–3%.2, 3, 4, 5
In comparison, a DVA usually appears as a collection of radiating veins converging on a large and centrally located draining vein. This is classically described as having a fan-shaped “caput medusae” appearance, although this is not always seen.6 Histologically, the DVA consists of a collection of thickened and hyalinized veins with little smooth muscle and elastic tissue.1 DVA is the most common cerebrovascular abnormality, with an overall incidence of 2%–4%,7, 8, 9, 10, 11 much more common than AVMs. However, DVAs, usually discovered incidentally, rarely if ever bleed, and the reported annual bleeding rate of 0.2%–0.3% is likely due to bleeding from coexisting cavernous malformations.12, 13
Despite their contrasting properties, the convergence of AVM and DVA presents a tenuous hemodynamic balance that warrants neurosurgical intervention. Presumably, when the DVA's low resistance drainage system receives a high-pressure inflow, the bleeding risk exceeds that associated with either entity alone. Perhaps the additional strain on the hemodynamic balance from increased inflow leads to the commonly reported symptoms of coexisting AVMs and DVAs, including headache, seizures, and neurologic deficits.14
Given the ongoing need to understand the clinical manifestations and management of these colocalizing cerebrovascular malformations, we present 2 cases of coexisting AVM and DVA from our institutions. In 1984, Huang et al.15 reported the first case of a coexisting AVM and DVA. To aid in the treatment of future cases, we also review the decision making and outcomes reported in the literature for this rare transitional malformation.
Section snippets
Materials and Methods
We identified cases of coexisting AVM and DVA from a locally held institutional database at Stanford University. In addition, M.Z. and I.D.C. conducted a literature search for cases published between 1980 and 2016 using the PubMed database to identify case reports of this unique entity. The following terms were used: arteriovenous malformation and developmental venous anomaly, AVM and DVA, venous angioma and AVM, venous malformation and AVM, and associated DVA. We also reviewed the references
Case 1
A 29-year-old female who had sustained an intracerebral hemorrhage presented with an episode of right-sided headache and partial left homonymous hemianopsia. Computed tomography scan and magnetic resonance imaging (MRI) of the brain demonstrated rupture of the right parieto-occipital AVM component. Angiography demonstrated a 3.5-cm Spetzler–Martin grade IV mixed AVM fed from the right anterior cerebral artery (ACA), middle cerebral artery (MCA), and posterior cerebral artery (PCA), with primary
Discussion
As shown in our summary of the published series of coexisting AVMs and DVAs, 68% (95% confidence interval, 49%–88%) of the reported cases presented with hemorrhage, often with associated motor deficits, highlighting the high-risk nature of these lesions. This hemorrhagic prevalence is higher than the 38% identified in the traditional AVM population.27 The reported transient neurologic deficits, seizures, or loss of consciousness in the remaining patients further demonstrates the maladaptive
Conclusions
Although rare, AVMs and DVAs can coexist. The finding of a DVA in the proximity of a vascular lesion warrants consideration for catheter angiography, because this does not necessarily imply that the lesion is a cavernous malformation. Patients with coexisting AVMs and DVAs tend to have hemorrhagic presentations, likely associated with the delicate hemodynamic balance between the malformations. In contrast to traditional AVM management, here it is important to preserve the draining vein via the
Acknowledgments
We thank Cindy Samos and Christine Plant for their help with the preparation of this manuscript.
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Conflict of interest statement: This study was supported in part by funding from Bernard and Ronni Lacroute and from the William Randolph Hearst Foundation (to G.K.S.). The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.