Sinus Pericranii: Systematic Review with a Case Illustration

Sinus pericranii (SP) is a rare vascular malformation characterized by abnormal communication between intracranial and extracranial venous systems. Often asymptomatic and presenting as a soft, nonpulsatile scalp mass, SP can be mistaken for other scalp anomalies, such as scalp arteriovenous malformations (AVMs), due to overlapping clinical features. This case of a 4-year-old girl successfully treated for a giant SP is reported to illustrate the findings of a comprehensive systematic literature review. This review was conducted according to the "Preferred Reporting Items for Systematic Reviews" guidelines. Relevant studies (1985-2025) reporting patients with SP were identified from PubMed/MEDLINE databases. A total of 65 studies reporting 82 SP patients were included in this study. The median age was 8 years, ranging from 4 months to 72 years, with a male predominance of 61.0% ( n  = 50). No contributing factors were reported in most cases (67.1%, n  = 55). However, craniosynostosis and recent trauma were reported as contributing factors to the development of SP in 7.3% ( n  = 6) and 18.3% ( n  = 15), respectively. Frontal (37.8%, n  = 31), parietal (32.9%, n  = 27), and occipital (13.4%, n  = 11) were the most reported locations of SP. The median follow-up duration was 24 months (3-96). This is the first systematic review of the SP, where the authors highlight, by data synthesis, the clinical presentation, dural sinus involvement, management, and outcome of this rare but challenging pathology. Without management guidelines, its treatment choice is still based on the surgeon's experience. This reality calls for action for an evidence-based study.