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).