Review Article

Diagnosis and Treatment of Human

Hydatid Disease

Taha T. Bekçi

Konya Education and Research Hospital, Abstract

Department of Pulmonary Medicine,
Konya, Turkey Echinococcosis or hydatid disease is caused by larvae of Echinococcus.
In the life cycle of E. granulosus, humans sometimes become acciden-
Eur J Gen Med 2012;9 (Suppl 1):15-20 tal intermediate hosts. Humans can orally take up the E. granulosus
Received: 30.12.2011 eggs from infected carnivore excretions by handling the animals or
egg-containing feces, plants, eating vegetables, uncooked fruits, and
Accepted: 10.01.2012
drinking water with the eggs. The hydatid disease is endemic in some
Mediterranean countries, the Middle East, the South America, the
South Africa and Oceania. Hydatid disease is seen in subjects of any
age and sex. The combination of imaging and serology usually enables
diagnosis. Clinical management of hepatic cysts includes albendazole
or mebendazole therapy in combination with either surgical resection
or the PAIR procedure.
Key words: Radiography, CT scan, cystic lung lesions, cavitary lung
lesions

Hidatik Kist Hastalığının Tanı ve Tedavisi

Özet
Ekinokkokozis veya hidatik hastalığına Ekinokok larvası neden olur.
Ekinokokus Granulozus’un yaşam döngüsünde, insan bazen tesadüfi
olarak konak olur. İnsan E.Granulozus yumurtalarını, ağız yoluyla hay-
van sekresyonlarından, enfekte olmuş, yumurta içeren dışkıdan, se-
bzelerden, pişmemiş meyvelerden, ve içme suyundan alabilir. Hidatik
hastalık bazı Ak Deniz ülkelerinde, Orta Doğuda, Güney Amerikada,
Güney Afrikada ve Okyanusta endemik olarak görülür. Görüntüleme
ve seroloji yöntemlerinin kombinasyonu tanı koymayı sağlar. Hepatik
kistleri klinik tedavisi, albendazol veya mebendazol tedavisi ile cer-
rahi olarak rezeksiyon veya PAIR prosedürünün kombinasyonunu içerir.
Anahtar kelimeler: Ekinokkokozis, Hidatik Hastalık, Endemik
Correspondence: Taha Tahir Bekci, MD.
Konya Education and Research Hospital,
Meram 42090 Konya/Turkey.
Tel: 905333787676
E-mail: doktortaha@konyaea.gov.tr

European Journal of General Medicine

Hydatid disease

INTRODUCTION •Parts of the Turkana district of Kenya - 220 cases per

100,000 persons
Cystic echinococcosis (CE) is among the most neglected
parasitic diseases. Development of new drugs and other Cystic echinococcosis causes not only illness but also
treatment modalities receives very little attention, if productivity losses in human and agricultural animal
any, and is slow (1, 2). Human cystic echinococcosis (CE), population, and it can have large societal impacts on
caused by the larval stage of the cestode Echinococcus endemic areas (9). Cystic echinococcosis is rarely fatal.
granulosus, is a cosmopolitan parasitic zoonosis, affecting Occasionally, deaths occur because of anaphylactic shock
mainly the liver (70%) and the lung (20%) of the human or cardiac tamponade in heart echinococcosis (10).
intermediate host. Clinical symptoms depend on the lo-
Parasite biology
cation, number, and size of the cysts. Until anthelminthic
chemotherapy became available (mebendazole in the Organ involvement
1970s and albendazole in the early 1980s), surgery was The liver is the most common site of infection followed
the only treatment choice (3). The prevalence of infec- by the lung in 10 to 30% of cases, and other sites (spleen,
tion varies widely among different countries and in dif- kidney, brain, bone) in about 10% cases. In one series of
ferent areas, ranging from 1 to 220 per 100,000 in certain 386 patients with pulmonary hydatid cysts, 59 patients
regions (4). (15%) had extrapulmonary cysts, of which 54 were in the
Cystic echinococcosis caused by Echinococcus granulo- liver, 4 were in the kidney, and 1 was intraperitoneal.
sus (hydatidosis or hydatid disease) Approximately 60% were located in the lower lobes, and a
predilection for the right lung was seen in 56% cases (11).
Life cycle
Although hydatid cysts may be asymptomatic, but usually
Cystic echinococcosis is a zoonotic illness caused by in- produce various symptoms due to compressive effect on
fection with Echinococcus granulosus. In the life cycle surrounding vital structures such as dyspnea, retroster-
of E. granulosus, humans sometimes become acciden- nal chest pain, cough, dysphasia, back pain, and superior
tal intermediate hosts. Humans can orally take up the vena cava syndrome (12, 13).
E. granulosus eggs from infected carnivore excretions by
Clinical features
handling the animals or egg-containing feces, plants, eat-
ing vegetables, uncooked fruits, and drinking water with The clinical features of cystic echinococcosis are highly
the eggs (5, 6). variable (Table 1). The spectrum of symptoms depends
on the following; involved organs, size of cysts and their
Epidemiology
sites within the affected organ or organs, interaction
The hydatid disease is endemic in some Mediterranean between the expanding cysts and adjacent organ struc-
countries, the Middle East, the South America, the South tures, particularly bile ducts and the vascular system of
Africa and Oceania. It is a disease of rural areas where the liver, complications caused by rupture of cysts, bacte-
farming is practiced traditionally (7). The hydatid cyst rial infection of cysts and spread of protoscolices and lar-
can occur also in non-endemic countries because of the val material into bile ducts or blood vessels, immunologic
upsurge of emigration and trade (8). Annual incidence reactions such as asthma, anaphylaxis, or membranous
rates of diagnosed human cases per 100,000 inhabitants nephropathy secondary to release of antigenic material
vary widely, from less than 1 case per 100,000 to high (9). The initial phase of primary infection is asymptom-
levels. For example, rates in the indicated regions are atic and may remain so for many years. Hydatid disease
as follows: is seen in subjects of any age and sex, although it is more
common in those aged 20–40 yrs (14, 15). The rate of
•Greece - 13 cases per 100,000 persons
growth of cysts is variable depending on the strain dif-
•Rural regions of Uruguay - 75 cases per 100,000 persons ferences and the organ involved. Typical measurements
•Rural regions of Argentina - 143 cases per 100,000 per- state that the average cyst growth is 1 cm to 1.5 cm/year
sons in Rio Negro province (9). Patients come to the clinician's attention for differ-
ent reasons, such as when a large cyst has some mechani-
•Parts of Xinjiang province of China - 197 cases per cal effect on organ function or rupture of a cyst causes
100,000 persons acute hypersensitivity reactions. The cyst may also be

16 Eur J Gen Med 2012;9(Suppl 1):15-20

 Taha Bekçi

discovered accidentally during radiographic examination, Table 1. Clinical future of hydatid diseases
body scanning, surgery, or for other clinical reasons (16). Liver
Physical findings are hepatomegaly, a palpable mass if on Hepatomegaly
Jaundice
the surface of the liver or other organs, and abdominal
Biliary colic–like symptoms
distention. If cysts in the lung rupture into the bronchi, Cholangitis
intense cough may develop, followed by vomiting of hy- Pancreatitis
Liver abscess
datid material and cystic membranes (17).
Portal hypertension
Ascites
Diagnosis
Inferior vena cava compression or thrombosis
The combination of imaging and serology usually enables Budd-Chiari syndrome
Cyst rupture, peritoneal spread, and peritonitis
diagnosis. The standard diagnostic approach for cystic Hemobilia
echinococcosis involves imaging techniques, predomi- Biliary fistula (skin, bronchial system, gastrointestinal tract)
nantly ultrasonography, computed tomography (CT), X-ray Lungs
Tumor of chest
examinations, and confirmation by detection of specific Chest pain
serum antibodies by immunodiagnostic tests. Enzyme- Chronic cough, expectoration, and dyspnea
linked immunosorbent assay (ELISA) test using hydatid Pneumothorax
Eosinophilic pneumonitis
cyst fluid has a high sensitivity (>95%) but its specificity Pleural effusion
is often unsatisfactory. Finding a cyst using ultrasound, Parasitic lung embolism
X-ray, or CT is typically expected in Echinococcus infec- Hemoptysis
Biliptysis
tion (18). Heart
Tumor
Laboratory and special investigations Pericardial effusion
Embolism
Serological tests are commonly employed to supplement
Breast - Masses that must be differentiated from neoplasms
the radiological data in the diagnosis of hydatid cyst. The Spine - Mass with neurologic symptoms
current gold standard serology test for echinococcosis de- Brain - Mass with neurologic symptoms.
tects IgG antibodies to hydatid cyst fluid-derive native or
recombinant antigen B subunits. This is performed using
ELISA or immunoblot formats (19). The lipoproteins an- bronchioles, air enters the potential space between peri-
tigen B (AgB) and antigen 5 (Ag5) are the major compo- cyst and endocyst, and appears as a thin, lucent crescent
nents of hydatid cyst fluid and are the most widely used (crescent or meniscus sign) (11). When the endocyst com-
antigens in current assays for immunodiagnosis of cystic pletely separates, it collapses internally and can be seen
echinococcosis (20). General consensus states that the floating freely on the cyst fluid (“water-lily” or “iceberg”
ELISA test with crude hydatid cyst fluid has a high sensi- sign) (11). Other classically described CT appearances are
tivity of 95%; however, its specificity is low at 61.7% (21). Cumbo sign (air-fluid level in the endocyst capped with
Imaging air between the pericyst and endocyst) and “signet ring”
sign (bleb of air dissecting into the wall of the cyst) (25).
The most valuable diagnostic method in pulmonary hyda- CT scanning can elucidate the cystic nature of the lung
tid disease is the plain chest radiograph (22). mass and provide accurate localisation for planning of
Radiography surgical treatment of complicated cysts (26).

The cysts are most commonly seen as spherical, homog- Ultrasonography

enous masses with smooth borders surrounded by normal Various classifications exist of the ultrasonographic pic-
lung tissue (11). Occasional appearances are the pres- ture in cystic echinococcosis, the most widely used still
ence of accompanying pleural effusion (3%), empty cyst being the one proposed by Gharbi in the early 1980s (7).
cavities appearing as bullae (1%), and calcification (0.7%) In 2003, the World Health Organization Informal Working
(23). An intact cyst is filled with clear fluid. Debris within Group on Echinococcosis (WHO-IWGE) proposed a stan-
the fluid may occasionally be seen on ultrasound; this dardized ultrasound classification based on the active-
is formed of hooklets and scolices, and is referred to as transitional-inactive status of the cyst as suggested by
hydatid sand (24). As the cyst enlarges and erodes into its sonographic appearance (27). CE1 and CE2 are active

Eur J Gen Med 2012;9(Suppl 1):15-20 17

Hydatid disease

Figure 1. Cystic lesion with visible in right middle Figure 2. Cystic lesion in left lower lobe
zone

cysts containing viable protoscolices. CE3 has been sub- and small- to medium-sized centrally located cysts can
divided into CE3a (detached endocyst) and CE3b (pre- be excised without sacrificing lung parenchyma. Standard
dominantly solid with daughter cysts). This subdivision radical procedures are wedge resection of lung paren-
is supported by a recent work that used high-field 1 H chyma of less than one segment, and for liver and lung
magnetic resonance spectroscopy to evaluate ex vivo cysts, segmentectomy and lobectomy. Conservative pro-
the metabolic profiles of cyst contents (28). The mag- cedures aim at sterilization and evacuation of cyst con-
netic resonance signal characteristics of a hydatid cyst tent, including the hydatid membrane (hydatidectomy),
may differ depending on the developmental phase, i.e. and partial removal of the cyst. The evacuation and the
whether it is uni- or multilocular and whether the cyst is hydatidectomy consists of puncture of the cyst and aspi-
viable, infected or dead. Information regarding reactive ration of part of the content, to permit introduction of
changes in the host tissue, capsule and signal intensity of the scolicidal agent, and total aspiration thereafter (30).
parent and daughter cysts is also obtained. On magnetic
Percutaneous treatment
resonance imaging (MRI), cysts show low signal intensity
on T1-weighted images and high signal intensity on T2- Historically, the first percutaneous treatment used was
weighted images (29). to puncture the cyst, aspirate cyst fluid, inject a scoli-
cidal agent, and re-aspirate the cyst content (PAIR). The
Management
classic PAIR technique is widely known (33, 34). Khuroo
CE is difficult to treat and, even more so, to cure for a and others found PAIR combined with peri-intervention-
number of reasons. The disease is complex and dynamic al benzimidazole derivatives to be as effective as open
with an evolving phase and quietly growing cysts (30). surgical drainage with fewer complications and less cost
Clinical management of hepatic cysts includes albenda- (35). A single-center report from Turkey, experience com-
zole or mebendazole therapy in combination with either paring surgery, laparoscopic surgery, and percutaneous
surgical resection or the PAIR procedure. Larger cysts (di- treatments in 355 patients over a period of 10 years and
ameter >10 cm) preferably undergo surgical resection(31, concluded that PAIR is an effective and safe option (36).
32). Safety and efficacy of percutaneous treatments is also
related to the anatomical site of the cyst. Percutaneous
Surgery
treatment is mostly used in liver and extrahepatic ab-
Surgical procedures range from simple puncture and aspi- dominal cysts, including peritoneal (30).
ration of cyst content to partial resection of the affected
Other Percuteanous Treatment
organ. The most commonly used procedures can be divid-
ed in conservative and radical. Radical procedures aim at Two types of approaches are currently in use: the cath-
complete removal of the cyst with or without hepatic or erization technique and the modified catherization tech-
lung resection. Peripherally located lung cysts of any size niques, in particular PEVAC (percutaneous evacuation)

18 Eur J Gen Med 2012;9(Suppl 1):15-20

 Taha Bekçi

MoCaT (modified catheterization technique), and DMFT 12 patients. Acta Cardiol 2005;60(1):39-41.
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