Torcular dural sinus malformations: a grading system proposal

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ORIGINAL ARTICLE

Torcular dural sinus malformations: a grading system proposal P. Liby 1 & V. Lomachinsky 2 & B. Petrak 3 & M. Kyncl 4 & U. R. Montarroyos 2 & M. Tichy 1 Received: 13 December 2019 / Accepted: 6 March 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract 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. Keywords Pediatric dural arteriovenous malformations . Torcular dural sinus malformation . Endovascular treatment . Dural sinus thrombosis . Grading system

Introduction Torcular dural sinus malformations (tDSMs) were first described within the pediatric dural arteriovenous shunt group in 1996 [1]. A giant torcular lake replacing confluens sinuum Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00381-020-04569-8) contains supplementary material, which is available to authorized users. * P. Liby [email protected] 1

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

2

Universidade de Pernambuco (UPE), Recife, Pernambuco, Brazil

3

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

4

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

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