Unusual locations of hydatid disease: diagnostic and surgical management of a case series.
ABSTRACT Hydatid disease is endemic in many areas of the world, where it is an important public health problem. The aim of this study was to describe a series of patients with atypically located primary hydatid disease, accompanied by a literature review.
Six male and four female patients with atypically located hydatid cysts who presented to the Kars State Hospital between September 2004 and March 2008 were evaluated. The mean age was 42.5 years (range 17-72 years). Hydatid cysts were identified by using a combination of serology tests, ultrasonography, and computed tomography (CT).
Six of the patients underwent surgical treatment. Three patients (two with pericardial hydatid involvement and one with pancreatic involvement) were sent to a tertiary medical center for surgery, and one patient died from cardiac and renal failure two days after diagnosis.
Although this disease is seen most often in the liver and lungs, it can be found in any part of the body. Hydatid disease must be considered in the differential diagnosis of cystic lesions, especially in patients who have spent time in endemic areas.
- SourceAvailable from: Georgios D Lianos[Show abstract] [Hide abstract]
ABSTRACT: Introduction. Hydatid disease is caused by the tapeworm Echinococcus granulosus and is still a matter of public health in many regions of the world, where it is an endemic parasitic disease. Although the liver is the most involved organ, hydatidosis can be found anywhere in the human body. Rare forms of location may lead to diagnostic and therapeutic dilemmas. Case Report. Herein we report a rare case of acute abdominal pain and progressively increasing abdominal distension due to abdominal and multiple splenic echinococcosis in a 72-year-old Caucasian male. We also provide a brief review of the literature. Conclusion. Although hydatid disease is found most often in the liver and lungs, rarely any organ of the body can be involved by this zoonosis. Though rare, the possibility of unusual location of echinococcosis must always be considered by the operating surgeon, when dealing with diffuse abdominal pain in endemic areas, because any misinterpretation may result in unfavorable outcomes.Case reports in gastrointestinal medicine. 01/2012; 2012:387102.
Unusual Locations of Hydatid Disease:
Diagnostic and Surgical Management of a Case Series
Kasım C ¸ ag ˘layan,1Atilla C ¸ elik,2Ali Koc ¸,3Ali Cevat Kutluk,4Edız Altınlı,2
Aysun S ¸ims ¸ek C ¸ elik,5and Nes ¸et Ko ¨ksal2
Background: Hydatid disease is endemic in many areas of the world, where it is an important public health
problem. The aim of this study was to describe a series of patients with atypically located primary hydatid
disease, accompanied by a literature review.
Methods: Six male and four female patients with atypically located hydatid cysts who presented to the Kars State
Hospital between September 2004 and March 2008 were evaluated. The mean age was 42.5 years (range 17–72
years). Hydatid cysts were identified by using a combination of serology tests, ultrasonography, and computed
Results: Six of the patients underwent surgical treatment. Three patients (two with pericardial hydatid in-
volvement and one with pancreatic involvement) were sent to a tertiary medical center for surgery, and one
patient died from cardiac and renal failure two days after diagnosis.
Conclusions: Although this disease is seen most often in the liver and lungs, it can be found in any part of the
body. Hydatid disease must be considered in the differential diagnosis of cystic lesions, especially in patients
who have spent time in endemic areas.
. Hydatid disease is a zoonotic infection caused by Echi-
nococcus granulosus and E. multilocularis, of which the for-
mer is responsible for the majority of cases. Although the
total cumulative reported case number for E. multilocularis
was only 202 between 1980 and 1998, the estimated surgi-
cal case rate of cystic Echinococcus in Turkey is 0.87–6.6/
The parasite spreads from animals to humans through the
fecal-oral route and is endemic in the Mediterranean, the
Middle East, South America, New Zealand, Australia, South
Asia, and Turkey [2,3]. A high prevalence of parasitic
infection is found in the Middle East as well as North
The cyst can develop in almost any organ or tissue, al-
though the liver accounts for between 60% and 70% of cases
and the lungs for 5–20% [4,5]. Hydatid disease also has been
described in the spleen, kidneys, heart, brain, bone, and, in-
deed, almost every other organ or tissue in the body [1,6,7].
chinococcosis or hydatidosis is one of the major
zoonotic parasitic diseases in many areas of the world
The differing prevalence of hydatid disease in various
locations in the body arises from the life cycle of the parasite.
Theliverrepresents thefirst filterwhere organismsenter from
the intestine through the portal circulation. Most of the larvae
seed the parenchyma of the liver at this stage and continue
their life cycle in the form of cysts. However, some travel
beyond this first microvascular barrier and reach the lungs.
A small percentage of parasites (10–20%) that escape the
hepatic–pulmonary filter spread between the preparenchymal
and postparenchymal circles to any organ or tissue, probably
through precapillary anastomoses [5,8]. In 1965, Grassi pub-
lished a classification of rare forms of primary echinococcosis
based on statistical data that remains valid today: (1) In-
frequent: kidney, spleen, bone, muscle; (2) rare: brain, pan-
adrenal glands, lymphatic ganglia, peripheral nerves, eyes,
labia majora, and others .
Hydatid cysts cause death and morbidity by their associ-
ated complications such as secondary infection; cyst rupture
1Department of General Surgery, Bozok University, Yozgat, Turkey.
2Second Department of General Surgery, Haydarpasa Numune Teaching and Research Hospital, Istanbul, Turkey.
Department of3Radiology and4Thoracic Surgery, Kars State Hospital, Kars, Turkey.
5Second Department of General Surgery, Vakif Gureba Teaching and Research Hospital, Istanbul, Turkey.
Volume 11, Number 4, 2010
ª Mary Ann Liebert, Inc.
into adjacent structures, which commonly involves intraper-
itoneal dissemination of the disease from hepatic cysts; ana-
phylactic reactions; and pressure on adjacent organs leading
to signs such as obstructive jaundice as a result of pressure on
the biliary tree .
In Turkey, the estimated rate of hydatid disease that re-
quires surgery is 0.8–2 cases per 100,000 people [3, 11]. Ac-
cording to these data, the average number of new cases of
hydatid disease in Turkey is approximately 2,000–2,500 per
year. Rarer locations of primary disease account for between
5% and 30% of cases; these instances are of interest for epi-
demiologic reasons as well as for the diagnostic problems that
sometimes lead to an unclear clinical diagnosis [8,12].
The aim of this retrospective study was to review our ex-
hydatid cysts. The clinical presentations, diagnostic work-up,
and treatment outcomes of the patients are discussed.
Patients and Methods
Between September 2004 and March 2008, 98 patients with
hydatid disease were admitted to the General Surgery Unit at
Kars State Hospital, Turkey. The medical records of these
patients were reviewed. Patients were evaluated with regard
to age, sex, symptomatology, treatment, complications, and
length of the hospital stay (LOS).
In all patients, the diagnosis was based on the history,
physical examination, laboratory tests, and radiographic ex-
aminations. Ultrasonography was performed in six patients,
and computed tomography (CT) imaging was performed for
all. After the diagnosis of extrahepatic hydatid disease was
made, all patients were evaluated for hepatic hydatidosis.
Patients who had positive or suggestive radiologic or clinical
evidence of hepatic disease were excluded from this series.
After diagnosis, all patients were treated immediately with
a anthelminthic drug (albendazole 800mg/day) for one week
before surgery and for at least four weeks postoperatively.
The mean age of the 98 patients with hydatid disease was
37.1 years (range 10–72 years), and the male-female ratio was
59:39. Hepatic and pulmonary disease was diagnosed in 88
patients in our hospital.
We found six male and four female patients with primary
hydatid disease in an unusual location. Their mean age was
42.5 years (range 17–72 years). Nine patients were examined
with the echinococcal immune hemagglutination test, and the
results were positive in all. Demographic features, along with
radiologic and serologic findings, are summarized in Table 1.
Two patients with pericardial hydatidosis were referred to
the tertiary cardiac surgery center for operative management
of their disease, and one patient with pancreatic hydatid
disease was referred to a tertiaryhospital having a specialized
hepatopancreaticobiliary surgery unit. Six patients under-
went operations by the general surgeons in our hospital.
Two male patients, who were 32 and 38 years old, pre-
sented with thoracic pain and dyspnea. Chest radiographs
and a thoracic CT scan revealed cystic disease in the right
mediastinum (Fig. 1A) and left pleural space (Fig. 1B), re-
spectively. Both patients were treated successfully by extir-
pation of the cysts with decortication.
A subcutaneous cyst had previously been diagnosed in the
right infrascapular region in a 35-year-old woman. The pa-
tient presented with pain and swelling over the right dorsal
side of her torso. Ultrasonography and a CT scan (Fig. 2)
revealed a cystic mass before the diagnosis was confirmed by
the immune hemagglutination test. This mass was removed
Another female patient who likewise was 35 years old
complained of a painful neck swelling, which was located
within the thyroid gland. An ultrasound examination re-
vealed a swelling with a cystic nodular formation, and a hy-
The nature of the disease was confirmed by a combination of
CT (Fig. 3), which revealed a hypodense mass, and serology.
This patient was treated with a right lobectomy and isth-
mectomy of the thyroid.
Splenic hydatid disease was diagnosed in a 72-year old
woman who complained of abdominal discomfort. The di-
scan, and serology. A laparotomy was performed through a
midline incision in order to explore the whole abdominal
cavity, and a splenectomy was performed to remove all of the
hydatid disease with vaccination before surgery.
One patient, who was 22 years old, was admitted to the
emergency department with symptoms of an acute abdomen.
Table 1. Demographic Features with Laboratory and Radiologic Findings
of Patients Having Atypical Locations of Echinococcal Cysts
Age/sexLocationUltrasound findingsComputed tomography findingsPreoperative IHA
Left pleural space
Hypodense cystic mass
Hypodense cystic mass
Hypodense cystic mass
Cyst in pancreas
Cyst in splenorenal
Cyst in right lobe
Hypodense cystic lesion
Hypodense cystic mass
between spleen and left kidney
Hypodense cystic lesion
Hypodense mass; thickened
Multilocular hypodense mass
Rectovesical space cyst
Splenic hypodense cystic mass
Right lobe of thyroid
F¼female, IHA¼indirect hemagglutination; M¼male; NA¼not available.
350C ¸AG˘LAYAN ET AL.
He also had pelvic pain with guarding and rebound tender-
ness. Ultrasonography and CT revealed a cyst in the recto-
vesical space. The immune hemagglutination test was not
performed in this patient, as an emergency laparotomy was
necessary, and the cyst was removed intact.
A 62-year old man complained of abdominal pain, and
ultrasonography and CT scanning revealed a hypodense
cystic mass between the spleen and the left kidney. The im-
mune hemagglutination test was positive. Unfortunately, this
patient died of cardiac and renal failure on the second day
patient developed unilateral atelectasis, and the other had a
surgical site infection. Both of these complications were trea-
ted conservatively with antibiotics and mucolytics (Table 2).
The mean LOS of the patients undergoing surgery at our
center was 4.8 days (range 2–7 days).
Hydatid disease is an infection caused predominantly by
E. granulosus and has been well known since the time of
Hippocrates [1, 2,13]. House pets are the primary host for the
organism, with cows, sheep, horses, and sometimes human
beings being intermediate hosts [3,7]. The hydatid organism
spreads tohumans viaother intermediate hostseither directly
or through ingestion of water and vegetables that have been
contaminated with infected fecal material. Infection occurs
mainly during childhood, but is asymptomatic until adult-
Infection with E. granulosus usually is asymptomatic
until the size of the cysts increases sufficiently to have an
effect. Between 38% and 60% of infected patients are
asymptomatic [6,14]. Hydatid disease usually is found in the
liver (75%), lungs (5.8–15%), spleen, kidneys, and other
intra-abdominal and retroperitoneal organs, but cysts can be
present in any part of the body [1,15,16]. The disease gen-
erally is diagnosed incidentally by radiologic examinations
in patients without relevant clinical symptoms, and patients
usually are middle aged at diagnosis [1,5]. Hydatid disease
is rarely seen within soft tissues, and this condition causes
difficulties for accurate and timely diagnosis [1, 16]. When
the disease is diagnosed, the patient must be evaluated
systemically [5, 7].
Hydatid disease has no specific symptoms or signs and
varies according to the location of the cysts and the organ
involved [1,6,14]. When cysts are found in the liver, a hepatic
mass, biliary obstruction, and abdominal pain are most often
seen. Pulmonary disease can present as dyspnea, chest pain,
cough, and hemoptysis. If the cyst ruptures, it can result in
fever, urticaria, eosinophilia, or anaphylactic shock .
astinal space. (B) Cyst in left pleural space.
Hydatid cysts in the chest. (A) Cyst in right medi-
Hydatid cyst in right infrascapular region.
Hydatid cyst in right lobe of thyroid.
UNUSUAL LOCATIONS OF HYDATID DISEASE351
The indirect hemagglutination test is positive in approxi-
mately 85% of patients. The Casoni test is positive in >90% of
patients but can yield false-positive results after surgery or
cyst degeneration . The sensitivity and specificity of all the
serologic tests are low, but these tests remain useful in
screening for the disease [1,7].
The basis of the management of hydatid disease is surgery
[6,7,16]. There are three layers within the cyst, and scolices are
produced from the membrane of the germinative layer. The
gold standard of surgery is the removal of the germinative
membrane of the cyst and closure or reduction in the size of
the pericystic cavity [10,13]. The surgeon must be careful to
prevent seeding of the surrounding organ and tissues by
scolices during removal of the germinative membrane [6,7].
For scolex inactivation, various agents such as hypertonic
saline, hydrogen peroxide,polyvinyl iodine, and silvernitrate
are used preoperatively . To use these agents, the cystic
cavity must first be aspirated and left for 15 min before the
scolecidal agent is injected . In our study, 20% hypertonic
saline was used as a scolecidal agent.
The operations performed in this series are described in
Table 2. To decrease the recurrence rate and prevention of
contamination, chemotherapeutic agents were used during
In our cases, albendazole 800mg/day was given preopera-
tively and postoperatively. Also, it has been reported that
medical therapy can be used in cases that are inoperable or in
patients who cannot withstand the insult of surgery [7,18].
in any organ or tissue and should be considered in the diag-
nosis of a cystic space-occupying lesion in patients from
countries where hydatid disease is endemic . The optimal
disease. When this is impossible because of the location of the
cyst or other factors, a partial cystectomy, irrigation with
out as necessary suboptimal treatments.
Author Disclosure Statement
None of the authors has any financial conflict of interest.
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Left pleural spacePain, dyspneaExtirpation with
Total excisionSurgical site
352C ¸AG˘LAYAN ET AL.
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Address correspondence to:
Dr. Kasım C ¸ag ˘layan
Department of General Surgery
UNUSUAL LOCATIONS OF HYDATID DISEASE 353