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

Background: Echinococcosis is a parasitic disease caused by Echinococcus granulosis that most commonly affects the liver (70%) and lungs (20%). Despite rarely reported, arterial echinococcosis represents a severe and potentially life-threatening condition but management strategies 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^th, 2024 to identify relevant articles about arterial echinococcosis. Data regarding epidemiology, pathogenesis, clinical presentation, diagnostic methods, and treatment were investigated.

Results: A total of 143 papers were screened and 30 articles were included. Overall 32 cases were identified. About half of patients were female (18, 56.2%) and the mean age was 40.6 years (range 12-60 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 albendazole (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 2-72 months) only one recurrence was reported.

Conclusion: Arterial echinococcosis is an extremely rare condition that may potentially be lethal. The current review showed favorable outcomes following complete surgical excision and antiparasitic drug that currently remain the only definitive treatment.