Diagnostic Accuracy of SWAN in the Diagnosis of Low-Flow Brain Vascular Malformations in Childhood

Purpose The main objective of this study is to demonstrate the diagnostic accuracy of susceptibility-weighted angiography (SWAN) in the diagnosis of slow-flow cerebral vascular malformations, especially developmental venous anomaly (DVA). We also aimed to determine the prevalence of DVAs identified by SWAN at 1.5 T. Methods We retrospectively evaluated 1,760 axial SWAN images for the diagnosis of low-flow vascular anomaly. Among them were 305 patients who underwent contrast-enhanced examination due to different indications. Postcontrast images were analyzed by different radiologists who were blinded to patients. The presence of DVA and other features such as location, length, depth, and direction of drainage vein was evaluated. Results Twenty-six patients with DVA had both SWAN and postcontrast images. There were four false-negative patients with SWAN. The sensitivity of the SWAN sequence was 84.6%. In addition, totally 77 DVA (4.36%), 2 capillary telangiectasia (0.11%), and 2 cavernous malformations (0.11%) were detected in 1,760 patients. Conclusion SWAN is an effective method for the diagnosis of developmental venous anomalies and other low-flow cerebral vascular malformations. Especially in the pediatric age, susceptibility-weighted imaging sequences are useful to limit contrast use.

Cerebral Arteriovenous Malformations in Pediatric Patients with Hereditary Hemorrhagic Telangiectasia: Re-evaluating Appearance, Bleeding Risk, and Treatment Necessity in a Selective Meta-analysis

Pediatric patients suffering from cerebral nidal arteriovenous malformations are a unique population due to the rare occurrence of the disease. Diagnosis of hereditary hemorrhagic telangiectasia (HHT) in children is a rare event and mainly restricted to coincidental detection from screening of possibly afflicted family members. In patients with HHT, it is well known that the incidence of cerebral vascular malformations (CVMs) is higher than expected when compared with the nonafflicted population. Even though CVMs comprise a variety of different distinct anatomical and radiographic entities (e.g., capillary telangiectasia, nidal arteriovenous malformation [AVM], cavernous malformation, dural or pial as well as mixed fistula, and vein of Galen malformation), they are mostly summarized and analyzed all in one category due to the low number of individual cases identified in single centers. Nevertheless, the best treatment algorithm will likely vary significantly between different CVMs as does the clinical presentation and the natural course of the CVM. It is therefore the objective of this article to focus on nidal AVMs in pediatric patients suffering from HHT. To this end, we performed a systematic selective literature research to estimate incidence, clinical and radiological appearance, as well as classification according to established grading system, and to evaluate the necessity of treatment of these lesions in light of their respective outcomes. Our line of arguments explains why we recommend to follow these lesions expectantly and suggest to keep pediatric patients under surveillance with sequential scans until they reach adulthood.

Do Direct Bypasses Really Resolve Capillary Vessels in Moyamoya Disease? Delayed Hemorrhage Potentially Relevant to Direct Bypasses: A Pathological Investigation

<b><i>Objective:</i></b> Moyamoya disease is a chronic but progressive obliterative cerebrovascular disease of bilateral internal carotid arteries (ICAs) causing hemorrhagic or ischemic cerebral strokes. Surgical revascularization has the potential for resolving the capillary vessels, but the effect on the occlusive ICA and the moyamoya vessels after a direct bypass remains unclear. <b><i>Patient:</i></b> A 2-year-old girl with a history of repeated transient ischemic attacks and direct bypasses but demonstrating improvement and associated anomaly is reported. A year and a half later, after a bilateral revascularization, an intracerebral capsulized hematoma growth was identified, and it was removed surgically. Neovascularization including many microvessels similar to capillary telangiectasia were identified by pathological investigation despite the reduction of moyamoya vessels on the repeated angiograms after the revascularization surgeries. In the present case, proliferation of capillary vessels was clearly confirmed by direct bypasses. <b><i>Conclusion:</i></b> There is no doubt that direct bypasses prevent further ischemic stroke by improving cerebral blood flow. However, they may result in failure in reducing the load of moyamoya vessels, albeit decreasing the potential risk of hemorrhagic strokes.

Cavernomata and angiographically occult lesions

Cerebral cavernous malformations (CCMs), known in addition as cavernomata or cavernous (haem)angiomata, are vascular malformations consisting of abnormally enlarged capillary cavities without intervening parenchyma (see section on capillary telangiectasia, this chapter) or arterial feeders with venous drainage (see arteriovenous malformations, Chapter 50). About 10–20% of CCMs are associated with a developmental venous anomaly (DVA), which is an extreme variation of the normal venous anatomy. CCMs can occur as result of an underlying genetic disorder, and genetic analysis is recommended in patients with a positive family history or multiple lesions. A conservative approach for asymptomatic CCMs seems justified in the large majority of cases and follow-up imaging is only needed if patients become symptomatic. Indications for surgical treatment are multiples haemorrhages, CCMs with mass effect causing symptoms, and patients with intractable epilepsy with an identified epileptogenic focus. Stereotactic radiosurgery can be considered in exceptional cases with a very high surgical risk.