Improving long-term outcomes in pediatric torcular dural sinus malformations with embolization and anticoagulation: a retrospective review of The Hospital for Sick Children experience

Abstract

OBJECTIVE Torcular dural sinus malformations (tDSMs) are rare pediatric cerebrovascular malformations characterized by giant venous lakes localized to the midline confluence of sinuses. Historical clinical outcomes of patients with these lesions were poor, though better prognoses have been reported in the more recent literature. Long-term outcomes in children with tDSMs are uncertain and require further characterization. The goal of this study was to review a cohort of tDSM patients with an emphasis on long-term outcomes and to describe the treatment strategy. METHODS This study is a single-center retrospective review of a prospectively maintained data bank including patients referred to and cared for at The Hospital for Sick Children for tDSM from January 1996 to March 2019. Each patient’s clinical, radiological, and demographic information, as well as their mother’s demographic information, was collected for review. RESULTS Ten patients with tDSM, with a mean follow-up of 58 months, were included in the study. Diagnoses were made antenatally in 8 patients, and among those cases, 4 families opted for either elective termination (n = 1) or no further care following delivery (n = 3). Of the 6 patients treated, 5 had a favorable long-term neurological outcome, and follow-up imaging demonstrated a decrease or stability in the size of the tDSM over time. Staged embolization was performed in 3 patients, and anticoagulation was utilized in 5 treated patients. CONCLUSIONS The authors add to a growing body of literature indicating that clinical outcomes in tDSM may not be as poor as initially perceived. Greater awareness of the lesion’s natural history and pathophysiology, advancing endovascular techniques, and individualized anticoagulation regimens may lead to continued improvement in outcomes.

Anticoagulation helps shrink giant venous lakes and arteriovenous fistulas in dural sinus malformation

“…Despite the high risk of cerebral hemorrhagic complications from the malformation or from our interventions, we did not observe any complications on anticoagulation treatment, as also reported by Ku et al 8. In addition, the target for embolization was the AV junction to prevent the high risk of developing collateral arteries and rapidly reconstituting the AV flow.…”

“…Nevertheless, partial embolization as well as sinovenous thrombosis can lead to an uncontrolled neoangiogenic reaction, which can worsen the already difficult to manage situation. However, recent publications7 have shown some improvement in the outcome of these patients compared with earlier published results, especially when combining embolization with anticoagulation 8…”

Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review

“…However, with advancements in prenatal diagnosis and neuroendovascular interventions, the outcome has become more favorable. Ku et al [5] reported 83.3% of favorable outcome in torcular DSM with treatment. Yang et al…”

The Case of the Rare Malformation and Rare Variant: An Infant with a Self-Embolized Torcular Dural Sinus Malformation and a Concomitant Prothrombin Variant

“…Grade IV is thought to carry the highest mortality at a rate of 75%, 2 although recent reports indicate that the mortality rate for tDSMs may not be as high as originally suspected. 9 Other congenital vascular abnormalities, like vein of Galen malformations, can look similar to tDSMs on prenatal imaging, leading to challenges in early diagnosis. The differential diagnoses of prenatal brain abnormalities may also include subdural fluid collections, pial malformations, tumors, and cysts.…”

“…Grade IV is thought to carry the highest mortality at a rate of 75%, 2 although recent reports indicate that the mortality rate for tDSMs may not be as high as originally suspected. 9…”