Pure arterial malformations of the brain: A systematic review of clinical presentation, diagnosis, and management.

Background: Pure arterial malformations (PAMs) of the brain are rare vascular anomalies, typically identified incidentally and characterized by tortuous arterial loops. Their unclear etiology and management pose diagnostic and therapeutic challenges.

Methods: A systematic review of Medline, EMBASE, and Web of Science databases (1900-present) identified 15 studies involving 54 patients with PAM. Data on demographics, clinical presentations, diagnostic modalities, and treatments were extracted.

Results: The mean age was 37.2 years (range: 10-77), with a female predominance (69%). Severe headaches were the most common symptom (43%), though some cases presented with neurological deficits (20%) or incidentally (32%). Imaging modalities such as digital subtraction angiography, MRI, and CT were pivotal in diagnosis. Most cases (81%) were managed conservatively, while 19% had interventions, including clipping and embolization. Surgical management was effective in alleviating symptoms in select cases of symptom progression.

Conclusion: Our findings highlight the variability in clinical presentations ranging from benign to acute emergencies, emphasizing the challenges associated with the diagnosis and management of PAMs. This review underscores the need for larger studies that explore the etiology, treatment efficacy, and long-term patient outcomes.