World Neurosurg
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Brain arteriovenous malformations (bAVMs) are unusual vascular pathologies characterized by the abnormal aggregation of dilated arteries and veins in the brain parenchyma and for which the absence of a normal vascular structure and capillary bed leads to direct connections between the arteries and veins. Although bAVMs have long been believed to be congenital anomalies that develop during the prenatal period, more recent studies have shown that inflammation is associated with AVM genesis, growth, and rupture. ⋯ However, no definitive guidelines have been established for the management of bAVMs. We have comprehensively reviewed the current basic and clinical studies of bAVMs and discussed the contemporary status of multidisciplinary management of bAVMs.
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Rhombencephalosynapsis is a rare congenital anomaly, characterized by partial or total agenesis of the cerebellar vermis with midline fusion of the cerebellar hemispheres, dentate nuclei, and the superior cerebellar peduncles, creating the distinctive keyhole appearance of the fourth ventricle. Rhombencephalosynapsis can be isolated or can occur in association with other congenital anomalies and syndromes such as Gómez-López-Hernández syndrome (GLHS) or VACTERL: vertebral anomalies (V), anal atresia (A), cardiovascular defects (C), esophageal atresia and/or tracheoesophageal fistula (TE), and renal (R) and limb/radial (L) anomalies. Recent advances in prenatal imaging have resulted in an increasing rate of prenatal diagnosis of abnormalities of the posterior fossa including rhombencephalosynapsis. ⋯ Patients can be categorized into 4 groups: 1) rhombencephalosynapsis associated with GLHS; 2) rhombencephalosynapsis with VACTERL; 3) rhombencephalosynapsis with atypical holoprosencephaly, and 4) isolated rhomboencephalosynapsis. The etiology of rhombencephalosynapsis is unknown. Here, we discuss several hypotheses about its etiology.
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As an emerging minimally invasive endoscopic technique, unilateral biportal endoscopic spinal surgery (UBESS) has the advantages of flexibility, a wide and clear field of view, and less soft tissue damage. However, the clinical evidence is insufficient and controversy exists regarding UBESS for the treatment of lumbar spinal stenosis (LSS). In the present meta-analysis, we investigated the clinical efficacy and complications of UBESS for the treatment of LSS. ⋯ From the available clinical results and experience from reported studies, UBESS for LSS is a feasible and effective approach and a worthwhile choice for clinicians. However, the complications associated with the procedure should also be seriously considered.
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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. ⋯ 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.