Genetics and Emerging Therapies for Brain Arteriovenous Malformations.
World Neurosurg
; 159: 327-337, 2022 Mar.
Article
en En
| MEDLINE
| ID: mdl-35255632
ABSTRACT
Brain arteriovenous malformations (AVMs) are characterized by a high-pressure, low-resistance vascular nidus created by direct shunting of blood from feeding arteries into arterialized veins, bypassing intervening capillaries. AVMs pose a risk of spontaneous rupture because the vessel walls are continuously exposed to increased shear stress and abnormal flow phenomena, which lead to vessel wall inflammation and distinct morphologic changes. The annual rupture rate is estimated at 2%, and once an AVM ruptures, the risk of rerupture increases 5-fold. The ability of AVMs to grow, regress, recur, and undergo remodeling shows their dynamic nature. Identifying the underlying cellular and molecular pathways of AVMs not only helps us understand their natural physiology but also allows us to directly block vital pathways, thus preventing AVM development and progression. Management of AVMs is challenging and often necessitates a multidisciplinary approach, including neurosurgical, endovascular, and radiosurgical expertise. Because many of these procedures are invasive, carry a risk of inciting hemorrhage, or are controversial, the demand for pharmacologic treatment options is increasing. In this review, we introduce novel findings of cellular and molecular AVM physiology and highlight key signaling mediators that are potential targets for AVM treatment. Furthermore, we give an overview of syndromes associated with hereditary and nonhereditary AVM formation and discuss causative genetic alterations.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Malformaciones Arteriovenosas
/
Malformaciones Arteriovenosas Intracraneales
/
Radiocirugia
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Malformaciones del Sistema Nervioso
Tipo de estudio:
Prognostic_studies
Idioma:
En
Revista:
World Neurosurg
Asunto de la revista:
NEUROCIRURGIA
Año:
2022
Tipo del documento:
Article