Background Even for the most common dural sinus malformation (DSM), the torcular DSM (tDSM), generalizable statements about etiology and prognosis are difficult because neurosurgeons/neuroradiologists and obstetrical imagers have focused on different patient age groups, have reported different outcomes, and have offered differing pathophysiologic explanations.
Objective To examine the imaging features and outcomes of a local cohort of tDSMs across fetal–neonatal life for commonalities.
Methods Review of imaging and clinical outcome for a local cohort of 12 tDSM patients (9 fetal, 3 postnatal).
Results All 12 tDSMs had similar imaging characteristics, including enlargement of the torcular and intraluminal thrombus early on, later evolving to peripheral scar tissue after treatment or spontaneous regression. Spontaneous decrease in size of the tDSM was observed in 6 prenatal and 1 postnatal case, and this decrease appeared to be irreversible once it occurred. One of 9 prenatal tDSMs was demonstrated to have arteriovenous fistulae in utero, while 2 of 3 postnatal diagnoses had arteriovenous fisutlae. All 6 prenatal tDSM diagnoses followed to term and all 3 postnatal diagnoses had a grossly normal neurologic outcome after a median of 12 months of age.
Conclusions Prenatal and postnatal tDSMs have overlapping imaging features suggesting a common etiology, and involution of a tDSM is a key imaging biomarker for a favorable outcome. While there is reason for concern with postnatally diagnosed tDSMs, good outcomes may still be achieved across the fetal–neonatal age spectrum of presentations. These findings are generalized in part II of this article.
- dural arteriovenous fistula
- dural sinus malformation
- fetal vascular malformation
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Contributors EY and AS assembled the patient cohort, analyzed the patient data, and wrote the manuscript. EY, AS, and DBO reviewed the imaging data. HEO, JS, JAE, CCT, BC, ERS, and DBO identified the relevant patients and provided clinical information. DBO conceived of and supervised the study. All authors have contributed to the drafting of the manuscript and have approved the final version.
Competing interests None declared.
Ethics approval The study was approved by the Boston Children’s Hospital institutional review board.
Provenance and peer review Not commissioned; externally peer reviewed.