BACKGROUND: Torcular dural sinus malformations (tDSMs) are congenital complex vascular anomalies often referred as a single unit. Nevertheless, they possess distinct anatomical features, clinical diversity, and markedly different outcomes. OBJECTIVE: On the basis of our institutional experience and analysis of published data, we propose a grading system. METHODS: We have identified 44 papers to which we added our four institutional cases for a total of 126 patients. Eight predictor variables were studied. In order to assess their individual impact on mortality and possible correlations, a logistic regression model was constructed through a stepwise forward process. RESULTS: Overall mortality was 22.1%. Mortality was higher in tDSM patients diagnosed postnatally, 40.7% versus a 15.6% in prenatally found cases (p = 0.007). We divided the patients into four grades. Grade I comprised patients with no feeder evidence and possessed the best outcomes (mortality of 7.55%). Mortality rose for grades II and III defined respectively by scarce and multiple feeders. Brain damage was the defining feature of grade IV. A mortality of 75% could be observed within this grade. Grade IV was further divided into grades IVa (antenatal) and IVb (postnatal cases). Furthermore, our logistic regression model found that brain damage (OR 11.3, p < 0.001, 95% CI 2.97-42.91) and patent feeders (OR 4, p = 0.03, 95% CI 1.15-13.86) were major determinants of poor outcome (area under ROC curve of 81.44%). CONCLUSION: The grading system (tDSM-GS) streamlines classification into four different grades facilitating both diagnosis, clinical decision-making, and proper prognostication.

Department of Neurosurgery 2nd Faculty of Medicine Charles University Prague and Motol University Hospital 5 Úvalu 84 Prague 5 150 06 Prague Czech Republic

Department of Paediatric Neurology 2nd Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czech Republic

Department of Radiology 2nd Faculty of Medicine Charles University and Motol University Hospital Prague Czech Republic

Universidade de Pernambuco Recife Pernambuco Brazil

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