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Original research
Imaging features and prognostic factors in fetal and postnatal torcular dural sinus malformations, part II: synthesis of the literature and patient management
  1. Edward Yang1,2,
  2. Armide Storey1,3,
  3. Heather E Olson2,4,
  4. Janet Soul2,4,
  5. Judy A Estroff1,2,
  6. Cameron C Trenor5,
  7. Benjamin K Cooper1,
  8. Edward R Smith3,6,
  9. Darren B Orbach1,2,3
  1. 1 Department of Radiology, Boston Children’s Hospital, Boston, Massachusetts, USA
  2. 2 Advanced Fetal Care Center, Boston Children’s Hospital, Boston, Massachusetts, USA
  3. 3 Cerebrovascular Surgery and Interventions Center, Boston Children’s Hospital, Boston, Massachusetts, USA
  4. 4 Department of Neurology, Boston Children’s Hospital, Boston, Massachusetts, USA
  5. 5 Stroke and Cerebrovascular Center and Division of Hematology/Oncology, Boston Children’s Hospital, Boston, Massachusetts, USA
  6. 6 Department of Neurosurgery, Boston Children’s Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Darren B Orbach, Department of Radiology, Boston Children’s Hospital, Boston, MA 02115, USA; darren.orbach{at}


Background Torcular dural sinus malformations (tDSMs) are described as slow flow dural arteriovenous fistulae with frequently poor outcomes in the neuroangiographic literature, but other etiologies have been proposed in the obstetric literature, where outcomes have been more favorable.

Objective To review tDSMs reported in the literature of multiple specialties for features that support a common etiology, and to identify key prognostic factors, with an emphasis on tDSM trajectory highlighted in part I.

Methods Analysis of imaging features and clinical outcome for 77 prenatal and 22 postnatal tDSMs reported in 37 papers from the literature.

Results In addition to large venous lakes, 36% of prenatal and 96% of postnatal tDSMs had evidence of arterialization, where specifically assessed. For fetal cases, where there was an observable natural history, 97% underwent a spontaneous decrease—13% after an initial increase and only 1 case with subsequent enlargement after a decrease. Prenatal cases had 83% survival (62% with a favorable outcome) whereas postnatal cases had 59% survival (29% favorable). In addition to a postnatal diagnosis, unfavorable features included ventriculomegaly, parenchymal injury, arterialization, and need for intervention. Favorable features included decreasing tDSM size, presence of clot, and increasing clot percentage.

Conclusions Neonatal and fetal tDSMs have overlapping imaging appearances, suggesting a common etiology, where neonatal tDSMs represent those rare fetal tDSMs that do not undergo spontaneous regression and have a propensity for worse outcomes. Decrease in tDSM size is a critical observation when managing a tDSM because it is generally irreversible and associated with a favorable outcome.

  • pediatrics
  • fistula
  • vascular malformation
  • mri
  • angiography

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  • Contributors EY and AS abstracted literature cases, analyzed the outcomes by patient variables, and prepared the manuscript. DBO conceived of and supervised the study. All authors have contributed to the interpretation of the literature and drafting of the manuscript. All authors have approved the final version.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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