Rare Presentation of Echinococcal Disease: A Systematic Review on Arterial Hydatid Cyst

Background: Echinococcosis is a parasitic disease caused by Echinococcus granulosus that most commonly affects the liver (70%) and lungs (20%). Despite rarely reported, arterial echino- coccosis represents a severe and potentially life-threatening condition but management strate- gies and related outcomes have not been fully investigated. Methods: A comprehensive review of the international English literature was performed on PubMed, Scopus, and Web of Science from inception to May 30, 2024 to identify relevant arti- cles about arterial echinococcosis. Data regarding epidemiology, pathogenesis, clinical presen- tation, diagnostic methods, and treatment were investigated. Results: A total of 143 articles were screened and 30 articles were included. Overall 32 cases were identified. About half of the patients were female (18, 56.2%) and the mean age was 40.6 years (range 12e60 years). Echinococcus cyst was more commonly detected on the aorta (27, 84.3%) in association or not with other cysts on the iliofemoral axis. Five cases (15.2%) affected only peripheral arteries. Most patients presented with chronic pain due to vascular obstruction or thromboembolism, while one patient was asymptomatic. In most cases (31, 96.8%) a total surgical excision was done in association with antiparasitic treatment using alben- dazole (17, 53.1%) or mebendazole (4, 12.5%). Arterial reconstruction was required in 19 cases (59.4%) and done with prosthetic substitutes or bovine pericardium. Only one case (3%) was treated with an endovascular approach. Four patients (12.5%) died in-hospital from hemorrhagic shock (3) or respiratory failure (1). During the follow-up period (mean 14 months, range 2e72 months) only one recurrence was reported. Conclusions: Arterial echinococcosis is an extremely rare condition that may potentially be le- thal. The current review showed favorable outcomes following complete surgical excision and antiparasitic drug that currently remain the only definitive treatment.