Background:
Echinococcosis is a widespread zoonotic disease caused by Echinococcus larvae, with E. granulosus being the most prevalent species. The disease is commonly found in regions with agricultural and pastoral activities but has also spread globally due to increasing globalization (1). Adult echinococcus parasites reside in the small intestines of definitive hosts (e.g., dogs) and excrete eggs in their feces. Intermediate hosts, including humans, become infected by accidentally ingesting these eggs. Once ingested, the eggs migrate through the portal venous or lymphatic pathways, primarily affecting the liver (80%) or lungs (less frequently other organs such as the spleen, brain, or kidneys) (2). Echinococcal cysts consist of two layers: the endocyst, formed by the parasite, and the pericyst, representing the host’s reactive tissue. Accurate management of echinococcosis requires a multidisciplinary approach, involving clinical features, serology, and imaging, with ultrasound being the preferred diagnostic modality due to its ability to provide detailed cyst characteristics (3). Albendazole and mebendazole are the mainstay medical treatments, while surgical intervention is considered for patients with inadequate response to medical therapy or those with symptomatic or clinically significant cysts (2). Surgical options include total pericystectomy, endocystectomy with partial pericystectomy, or total endocystectomy, with the choice depending on cyst size, location, number, and patient’s overall condition (4).
Polycystic liver disease is characterized by the progressive development of multiple cysts in the liver (5). The two most common genetic origins are autosomal dominant polycystic kidney disease (ADPKD), affecting both the kidneys and liver, and autosomal dominant polycystic liver disease (ADPLD), involving only the liver. Significant advancements have been made in understanding the molecular mechanisms underlying this disease, leading to the development of promising drugs such as tolvaptan, a vasopressin V2 receptor inhibitor that slows cyst growth and preserves kidney function. Although liver function is generally preserved in polycystic liver disease, large cysts can cause compression symptoms and pain. Laparoscopic cyst fenestration is a preferred approach in such cases, although cyst recurrence is common (6).
Case Presentation :
We report the case of a man with a known diagnosis of autosomal dominant polycystic kidney disease with concurrent liver cysts. He was referred to our center due to radiological suspicion of hepatic echinococcosis, despite negative serological findings. Given the lack of response to albendazole therapy and the diagnostic suspicion of echinococcosis, a multidisciplinary team considered surgical intervention for cyst removal. Intraoperatively, the polycystic liver was observed, and fenestration of two major simple cysts in segments 8 and 5 was performed. The cyst in segment 4 was found to be abscessed, leading to an endocystectomy with partial pericystectomy, confirming the diagnosis of echinococcal cyst. The surgical cavity was irrigated with a hypertonic solution, hemostasis was achieved using argon, and the absence of biliary fistulas was confirmed. A Jackson Pratt drain was placed, and postoperative therapy included albendazole to prevent recurrence of the parasite and piperacillin-tazobactam due to the cyst abscess in segment 4. Histological examination further confirmed that the cyst in segment 4 was an echinococcal cyst, while the other two were simple cysts. The patient developed postoperative pneumonia, which was successfully resolved. The patient had a one-week postoperative hospital stay, and subsequent outpatient follow-ups during the following months showed no complications or recurrence. The patient is currently under infectious disease surveillance (Fig.1).