OBJECTIVE Neurofibromatosis type 1 (NF1)-associated arterial aneurysms are rare and characterized by vessel fragility and heterogeneous presentation. Evidence guiding optimal management remains limited. This systematic review aimed to evaluate treatment strategies and outcomes in NF1-related arterial aneurysms. METHODS A systematic review was conducted according to PRISMA guidelines and registered in PROSPERO (CRD420251169825). PubMed, Scopus, and Web of Science were searched for studies reporting management and outcomes of NF1-associated arterial aneurysms. Case reports, case series, and observational studies were included. Data were independently extracted by two reviewers. Primary outcomes included treatment strategies, technical success, complications, and mortality. Isolated aortic aneurysms or aortic dissections were not considered the primary target of the review. However, studies reporting concomitant aortic disease and aneurysmal involvement of non-aortic arteries were retained when relevant data on NF1-associated vasculopathy and its management were available. RESULTS Eighty-six studies including 99 patients (mean age 42 years; 44.4% male) were analyzed. Aneurysms were most frequently located in cervico-vertebral (45.2%) and visceral arteries (24.0%). Multifocal disease was present in 12.1% of patients. Overall, 65.7% of patients presented with aneurysm rupture or acute hemorrhage. This high proportion should be interpreted cautiously because of the substantial publication bias inherent to a literature predominantly based on case reports and small case series. Endovascular therapy was the most frequently used approach (68.7%), followed by open surgery (19.2%), hybrid repair (5.0%), and conservative management (9.1%). Endovascular treatment showed a reported technical success rate exceeding 90%. Recanalization (6.3%), delayed thrombosis (4.2%), and new aneurysm formation (5.3%) were observed. Open repair was mainly used for anatomically complex or endovascularly inaccessible lesions. Overall mortality was 8.1%, primarily in patients presenting with rupture. CONCLUSIONS NF1-associated aneurysms require an individualized, anatomy-driven management strategy. Endovascular therapy was the most frequently reported treatment and appears to be a viable option in anatomically suitable patients, while open and hybrid techniques remain important for selected complex lesions. However, the available evidence is derived predominantly from case reports and small case series and does not permit comparative assessment of treatment strategies. Lifelong imaging surveillance is mandatory due to the risk of recurrence and new aneurysm formation.
