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Spinal Arteriovenous Shunts: Angioarchitecture and Historical Changes in Classification.

ABSTRACT: The purposes of this study were to review historical changes in the classification of spinal arteriovenous (AV) shunts and to propose a practical classification system. The associated literature regarding the classification of spinal AV shunts was reviewed in the angiography era between 1967 and 2015. The classification systems of spinal AV shunts and a proposed classification system were presented with neuroradiological imaging and medical illustrations. There have been seven major classification systems based on the evolution of diagnostic methods as well as treatments for spinal AV shunts: the first description of spinal AV shunts diagnosed and classified using spinal angiography in 1971; the second classification based on a case report of intradural direct perimedullary arteriovenous fistulas (AVFs) treated by microsurgery in 1987; the third classification based on a case series of intradural perimedullary AVFs treated by endovascular interventions in 1993; the fourth and fifth classification systems based on a case series of spinal AVFs and arteriovenous malformations (AVMs) treated by microsurgery or endovascular interventions in 2002; the sixth classification based on a case series of cranio-spinal dural AV shunts in 2009; and the seventh classification based on a case series of extradural AVFs treated by microsurgery and endovascular interventions in 2011. Based on historical reports, the author proposed a classification system according to the sites (dural, intradural, and extradural) and types (AVF and AVM) of AV shunts. By learning the historical background, we may obtain a clearer understanding of the complex and confusing classification system of spinal AV shunts.

SUBMITTER: Takai K

PROVIDER: S-EPMC5566708 | biostudies-other | 2017 Jul

REPOSITORIES: biostudies-other

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Project description:BackgroundDespite the latest advances in prenatal diagnosis and postnatal embolization procedures, intracranial arteriovenous shunts (AVSs) are still associated with high mortality and morbidity rates. Our aim was to evaluate the presentation and clinical course, the neurodevelopmental outcome, and the genetic findings of neonates with AVSs.MethodsIn this retrospective observational study, medical records of neonates with cerebral AVSs admitted to our hospital from January 2020 to July 2022 were revised. In particular, we evaluated neuroimaging characteristics, endovascular treatment, neurophysiological features, neurodevelopmental outcomes, and genetic findings.ResultsWe described the characteristics of 11 patients with AVSs. Ten infants (90.9%) required embolization during the first three months of life. In 5/9 infants, pathological electroencephalography findings were observed; of them, two patients presented seizures. Eight patients performed Median Nerve Somatosensory Evoked Potentials (MN-SEPs): of them, six had an impaired response. We found normal responses at Visual Evoked Potentials and Brainstem Auditory Evoked Potentials. Eight patients survived (72.7%) and were enrolled in our multidisciplinary follow-up program. Of them, 7/8 completed the Bayley-III Scales at 6 months of corrected age: none of them had cognitive and language delays; conversely, a patient had a moderate delay on the Motor scale. The remaining survivor patient developed cerebral palsy and could not undergo Bayley-III evaluation because of the severe psychomotor delay. From the genetic point of view, we found a novel pathogenic variant in the NOTCH3 gene and three additional genomic defects of uncertain pathogenicity.ConclusionWe propose SEPs as an ancillary test to discern the most vulnerable infants at the bedside, particularly to identify possible future motor impairment in follow-up. The early identification of a cognitive or motor delay is critical to intervene with personalized rehabilitation treatment and minimize future impairment promptly. Furthermore, the correct interpretation of identified genetic variants could provide useful information, but further studies are needed to investigate the role of these variants in the pathogenesis of AVSs.

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Spinal Arteriovenous Shunts: Angioarchitecture and Historical Changes in Classification.

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