Abstract
Central nervous system (CNS) cystic echinococcosis (CE) is a rare disease caused by Echinococcus which especially exists in agricultural endemic areas. This condition is more frequent in the pediatric and adolescent population and it can be associated with hydatid cyst in other localizations. A literature search was conducted up to 28 April 2021 by two independent reviewers. This study focused on clinical, imaging, therapeutic features as well as on complications of CNS CE among children and adults. Fifteen studies from seven different countries were included. A significant prevalence in males (ranging from 51% to 100%) was observed with an average rate of 60%. CNS CE was mainly single and located in the parietal region. The predominant symptoms were those of increased intracranial pressure. All patients were treated surgically, and the most common post-surgical complication was cyst rupture (prevalence: [3.3–37.5%], average: 12.9%). Furthermore, the outcomes were worse amongst patients with cyst ruptures and multiple recurrences. The average proportion of deaths due to CNS CE was 5.6% (range: 2.5–14.8%). This review highlights the necessity of including CNS CE in the differential diagnosis of patients with cystic lesions, especially in those residing in endemic areas. Early gross total cyst removal is the mainstay of treatment yielding the best outcomes.