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\title{Simultaneous Echinococcal Superinfection in a Patient with Polycystic
Liver Disease.}



\author[1]{Ottavia Cicerone}%
\author[1]{Raffaella Lissandrin}%
\author[1]{Enrico Brunetti}%
\author[1]{Marcello Maestri}%
\affil[1]{Università degli Studi di Pavia Facoltà di Medicina e Chirurgia}%


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  \date{May 22, 2024}


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\textbf{Simultaneous Echinococcal Superinfection in a Patient with
Polycystic Liver Disease.}

Cicerone O.*, Lissandrin R.\^{}, Brunetti E.\^{}, Maestri M.*

*: Fondazione IRCCS Policlinico San Matteo, Chirurgia Generale I, Pavia

Tropicali, Pavia

\textbf{Abstract:}

Cystic echinococcosis, caused by Echinococcus granulosus eggs, is a
parasitic zoonosis that typically affects humans through accidental
ingestion. Polycystic liver disease is a condition characterized by the
presence of multiple liver cysts and is often associated with polycystic
kidney disease. Here, we present a case of a man in his 70s with a
pre-existing diagnosis of polycystic liver disease. Radiological
findings of a suspicious cyst in the S4 segment initially lacked
serological evidence of echinococcosis; however, intraoperative
confirmation revealed the presence of an echinococcal cyst. This article
aims to explore both clinical conditions and highlight the therapeutic
considerations for their management. Moreover, we discuss the
significance of this unique case, emphasizing the possibility of the
coexistence of these two pathologies.

\textbf{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).

\textbf{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).

\textbf{Discussion:}

Echinococcosis is a disease caused by infection with parasites of the
Echinococcus genus, which can form cysts primarily in the liver and
lungs. In most cases, the diagnosis of echinococcosis can be relatively
straightforward, but there are situations where the presence of other
conditions can complicate the clinical picture.

In the case described in the report, the presence of polycystic disease,
characterized by the formation of multiple cysts, masked the true nature
of the hepatic cysts for a prolonged period. However, when the patient
developed severe septic episodes, with a significant deterioration in
their general condition, sepsis made the diagnosis more complex.

The negativization of specific indicators of Echinococcus infection may
have been influenced by the presence of sepsis. In these complex
situations, a multidisciplinary approach involving radiologists,
infectious disease specialists, and surgeons is essential to reach an
accurate diagnosis. In some cases, surgical exploration and drainage of
the lesions may be necessary to confirm the presence of echinococcosis.

The possibility of concurrent Echinococcus infection should be
considered in cases where there is polycystic disease with the presence
of cysts exhibiting atypical features in the overall context.
Multidisciplinary evaluation is crucial for making appropriate decisions
regarding pharmacological therapy or invasive treatment, such as surgery
or PAIR (puncture, aspiration, injection, re-aspiration), depending on
the specific circumstances of the patient.

\textbf{Conclusion:}

This case presents a unique occurrence of echinococcal superinfection in
a background of polycystic liver disease. Typically, echinococcosis is
not observed in the context of polycystic liver disease, possibly due to
the unfavorable conditions for colonization by the worm. This previously
unreported event highlights the importance of considering echinococcosis
as a diagnostic possibility even in cases of polycystic liver disease.
It also underscores the significance of a multidisciplinary approach in
diagnosing and managing patients suspected of having a new cyst.

1. Budke CM, Carabin H, Ndimubanzi PC, Nguyen H, Rainwater E, Dickey M,
et al. A systematic review of the literature on cystic echinococcosis
frequency worldwide and its associated clinical manifestations. Am J
Trop Med Hyg. 2013;88(6):1011-27.

2. Olaizola P, Rodrigues PM, Caballero-Camino FJ, Izquierdo-Sanchez L,
Aspichueta P, Bujanda L, et al. Genetics, pathobiology and therapeutic
opportunities of polycystic liver disease. Nat Rev Gastroenterol
Hepatol. 2022;19(9):585-604.

3. Rinaldi F, Brunetti E, Neumayr A, Maestri M, Goblirsch S, Tamarozzi
F. Cystic echinococcosis of the liver: A primer for hepatologists. World
J Hepatol. 2014;6(5):293-305.

4. Bektasoglu HK, Hasbahceci M, Tasci Y, Aydogdu I, Malya FU, Kunduz E,
Dolay K. Comparison of Laparoscopic and Conventional Cystotomy/Partial
Cystectomy in Treatment of Liver Hydatidosis. Biomed Res Int.
2019;2019:1212404.

5. Pitchaimuthu M, Duxbury M. Cystic lesions of the liver-A review. Curr
Probl Surg. 2017;54(10):514-42.

6. van Aerts RMM, van de Laarschot LFM, Banales JM, Drenth JPH. Clinical
management of polycystic liver disease. J Hepatol. 2018;68(4):827-37.\selectlanguage{english}
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