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Introduction Vein of Galen malformation (VOGM) is a congenital arteriovenous malformation of the brain’s midline venous system. VOGM account for approximately 30% of all pediatric cerebrovascular malformations. Historically, symptomatic VOGM had a high mortality rate, but with the recent advancement of early diagnostics and endovascular embolization technology, early treatment can now reduce aberrant development and offers the potential for patients to lead relatively normal lives. While endovascular interventions have drastically improved, the clinical range of VOGM presentations remains wide from asymptomatic to extensive developmental delay and impairment. Many complications are known to arise from cerebral venous hypertension caused by the increased pressure of the draining vein of the VOGM such as hydrocephalus or other forms of brain damage. The identification of clinical markers of disease severity and progress would greatly aid in the guided management of VOGM treatment. Here, we propose cortical vein tortuosity—or corkscrew sign—as a marker of cortical venous hypertension and as a predictor of ventriculomegaly in VOGM patients.Materials and Methods Consecutive 55 VOGM cases (median age 2.9 years [IQR 1.7-5.4]; 45.5% female) with complete cerebral angiograms and brain MRI available in our PACS system were reviewed and evaluated. Patients were divided into 2 groups based on the presence of venous corkscrew sign. For all MRIs obtained during the course of the staged endovascular treatment, the maximum frontal ventricle width and inner cranial skull width were measured to calculate Evans index (EI) to assess ventriculomegaly.Results Qualitative evaluation of cerebral arteriograms did not show regression or progression of corkscrew sign across age for individual patients. When comparing EI between corkscrew sign groups, VOGM patients with corkscrew signs showed a significantly higher average EI relative to patients without corkscrew sign (p = 0.048). Moreover, the presence of corkscrew sign was modestly predictive of hydrocephalus (AUC = 0.65; 95% CI: 0.54-0.78). EI between corkscrew groups did not show a significant interaction with age or gender.Conclusions Overall, our study finds that the presence of corkscrew sign in VOGM patients is associated with increased EI. This suggest that corkscrew sign indicates increased probability of ventriculomegaly in patients with VOGM and supports the use of corkscrew sign as a marker of potential developmental complications. We also found no significant association of EI with age between corkscrew sign groups which suggests that corkscrew sign indicates an increased severity of aberrant cerebrovascular state that is present at birth and remains throughout development. Based on these results, corkscrew sign could provide a promising prognostic marker of subsequent cerebrovascular development in patients with VOGM and may be an indication to work on reducing venous pressure by aggressively closing the malformation. Future work is needed to further evaluate the associations of corkscrew sign with other VOGM outcome measures such as cognitive development.Disclosures S. Allen: None. A. Devarajan: None. J. Fifi: None. T. Shigematsu: None.