Carotid cavernous fistula in Sturge-Weber syndrome: A unique case and literature review of associated vascular malformations

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder associated with various intracranial abnormalities, such as leptomeningeal angiomatosis, the characteristic facial port wine stain (PWS), among others. However, several non-classic ones, such as non-traumatic carotid cavernous fistulas (CCF), represent not only life-threatening but commonly unrecognized abnormalities, posing a significant risk to these patients. Objective: We present a comprehensive analysis of current literature to describe vascular anomalies associated with SWS and present a case of a CCF in a patient with SWS. Methods: A case with an unusual concurrent finding of CCF and SWS is presented following CARE guidelines. Additionally, we conducted a literature review using MEDLINE, Scopus, and Web of Science databases focusing exclusively on vascular anomalies associated with SWS. Results: Our review identified 243 cases describing vascular anomalies in patients with SWS, with a minor number of cases describing a cavernous sinus anomaly. Conclusions: The co-occurrence of SWS and CCF appears to be underreported. Although SWS is associated with multiple vascular anomalies, current evidence supports a predisposing vascular environment rather than a direct causal relationship with CCF. Although other vascular anomalies are significantly associated with SWS, there is still a research gap with ample area of opportunity to define their clinical impact and the most appropriate management.