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​Infectious Disease and Microbiology – Echinococcosis


Echinococcosis, also known as hydatid disease, is a parasitic infection caused by species of the genus Echinococcus. The most important species include Echinococcus granulosus, which causes cystic echinococcosis (CE), Echinococcus multilocularis, which causes alveolar echinococcosis (AE), and Echinococcus vogeli, which causes polycystic echinococcosis. These infections are characterized by the formation of cysts in various organs, most commonly the liver and lungs.


The disease is endemic in many parts of the world. Cystic echinococcosis is prevalent in Central Asia, South America, and North Africa, while alveolar echinococcosis is increasingly reported in Central Europe and is highly endemic in parts of China. Polycystic echinococcosis is rare and primarily found in Latin America. Transmission is closely associated with rural environments and animal husbandry practices. Humans acquire infection through contact with infected definitive hosts, typically dogs in CE and foxes or wild canids in AE, or through ingestion of food or water contaminated with parasite eggs.


Risk factors include close contact with dogs or other canines, exposure to livestock such as sheep, poor hygiene, and residence in endemic rural areas. Inadequate slaughterhouse practices and allowing dogs access to infected animal viscera perpetuate the parasite’s lifecycle. Preventive measures focus on improved hygiene, control of stray dog populations, proper disposal of animal remains, and vaccination of livestock.


After ingestion, parasite eggs hatch in the intestine, and larvae penetrate the intestinal wall to enter the bloodstream. They are carried to target organs, most commonly the liver (about 65%) and lungs (about 20%), where they develop into slowly growing cysts. These cysts may produce daughter cysts and can enlarge over time, causing pressure effects. In alveolar echinococcosis, lesions are typically infiltrative and destructive, resembling malignant tumors such as hepatocellular carcinoma and often confined to the liver.


Clinically, cystic echinococcosis is often asymptomatic and discovered incidentally. Symptoms arise due to mass effect depending on the organ involved. Complications include cyst rupture, which may lead to anaphylactic shock, dissemination of infection, or communication with adjacent structures such as the biliary tract or bronchial tree. Physical examination findings are often minimal, though hepatomegaly or a palpable mass may be present.


Diagnosis relies primarily on imaging and serology. Ultrasound is the initial modality of choice, particularly for hepatic cysts, and may reveal characteristic features such as daughter cysts, multilocular appearance, or specific signs like the “water lily” sign. CT and MRI provide further characterization. Serologic testing, typically using ELISA, supports the diagnosis, although sensitivity varies. Eosinophilia may be present but is not consistent. Cyst puncture is generally contraindicated due to the risk of anaphylaxis and parasite dissemination.


Treatment depends on the type and stage of disease. Surgical removal of cysts is the mainstay of treatment for cystic echinococcosis and is often curative. Albendazole is the primary pharmacologic therapy and is used in inoperable cases, disseminated disease, or as adjunctive therapy before and after surgery. Mebendazole is less effective, while praziquantel may be used as an adjunct in selected cases. A less invasive approach, known as PAIR (puncture, aspiration, injection, reaspiration), is used in selected cases and has shown promising results when combined with antiparasitic therapy.


Alveolar echinococcosis requires more aggressive management, often involving radical surgery combined with long-term albendazole therapy, though outcomes are less favorable compared to cystic disease. Follow-up is mainly performed using imaging to monitor cyst progression or recurrence.


Prognosis is generally excellent for cystic echinococcosis with appropriate treatment, whereas alveolar echinococcosis carries a higher mortality if untreated. Complications include cyst rupture with anaphylaxis, secondary infection, dissemination of the parasite, and in AE, progressive tissue destruction with metastatic-like spread.
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