2. 219
Introduction
The globally found parasite causes considerable socio-
economic consequences in endemic areas. However, in
Western industrial nations the incidence is relatively low.
Following oral intake of the cestode’s eggs (diameter
40 µm) the oncospheres are released in the upper gastro-
intestinal tract, penetrate the gut wall, enter the portal
vein, and reach the liver [1, 2, 3, 4]. The liver is infected
in about 60% of cases, the lung in about 20%, and re-
maining organs in about 20% (kidney, brain, bone, mus-
cles, and others) [5, 6, 7]. In principle, all tissues can be
affected. However, in about 80% of patients a solitary
cyst occurs in one organ. The cysts grow 1–30 mm in di-
ameter yearly [5, 8, 9]. Additional cysts (daughter cysts)
can develop inside the cysts. The host organism cuts it-
self off from the parasite by formation of the so-called
pericyst, which is a capsule of connective tissue [10].
This fact is important for surgical strategies leaving the
pericyst in situ and removing all inside the pericyst,
which is the real parasite [10]. The cysts displace healthy
tissue and organs but do not grow infiltratively or de-
structively. The incubation period differs with the loca-
tion of the hydatid and adjacent tissue and takes several
years because the metacestodes’ development is slow
[11]. Symptoms are caused by compression or displace-
ment of other structures or organs, with subsequent jaun-
dice, adverse effects on hollow organ motility, but, ex-
ceptionally, spontaneous or traumatic rupture [12, 13].
However, they are not pathognomic. Entire surgical re-
moval cures the patient. Intraoperative spillage of pro-
toscolices can cause secondary echinococcosis because
the parasite can form new cysts in all tissues. Differential
diagnosis comprises dysontogenetic cysts, abscesses,
metastasis, and occasionally alveolar echinococcosis,
which can cause necrosis of cystic character.
Diagnosis
Diagnosis is made by anamnesis, imaging procedures
(ultrasound, US; computed tomography, CT, magnetic
resonance imaging, MRI), und serology.
Anamnesis
The clinical symptoms of CE are not pathognomonic and
include abdominal pain, tenderness, jaundice, feeling of
a mass, palpable tumor, fever, feeling of exhaustion,
weight loss, nausea, cholangitis, pancreatitis, biliary sec-
ondary cirrhosis, abscess, portal hypertension, vena cava
compression, cyst rupture (anaphylaxis, shock), biliary
fistula, and hemobilia [1].
Ultrasound
US is a noninvasive, readily available, sensitive, cost-
effective imaging technique and is the diagnostic method
of choice. The US features and patterns of hydatid disease
have been defined by various reports [10, 14, 15, 16, 17,
18, 19]. US helps defining internal structure, number, and
location of the cysts and the presence of complications
[20]. The specificity of US is in the range of 90% [20]. The
World Health Organization (WHO) Working Group on
echinococcosis recommended a uniform classification
based on ultrasound-morphological criteria and viability of
the parasite [19, 21]. This should enable a stage-adapted
therapy. However, proper studies have to be initiated yet.
Computed tomography
CT yields information equivalent with that from US for
diagnosis of hydatid cyst of the liver. It gives better in-
formation about the location and the depth of the cyst in
the liver. The presence of daughter cysts and exogenous
cysts can also clearly be seen on CT [20]. Documenting
the site, size, and structure of cysts is easier than with
US [1] and enables examiner-independent discussion of
the findings. CT is essential when planning surgical
treatment, especially when planning a laparoscopic ap-
proach [20]. US and CT are mandatory for diagnosis and
localization of the cysts and should be used in combina-
tion [20]. CT is superior to US in monitoring of lesions
during chemotherapy and detection of recurrences [1].
Magnetic resonance imaging
MRI gives good structural details of the hydatid cysts.
Although it may be helpful for demonstrating the lesion
in the liver, it does not provide additional information in
hepatic lesions and is not cost-effective compared with
US and CT [22]. MRI is superior in identifying changes
in the hepatic venous system [23]. MRI may facilitate
the diagnosis in uncertain cases with noncalcified or par-
tially calcified lesions by showing the characteristic
multivesicular structure, necrotic areas, and proximity to
vascular structures [24].
Serology
Immunodiagnostic procedures are used to confirm the
cause of structures discovered by the imaging procedures.
Sensitivity and specificity up 93.5% and 89.7%, respective-
ly, have been reported [25]. However, these data vary with
the sites of CE and the patient’s age (children may have a
low antibody response) [21]. False-positive reactions may
occur in patients with other helminthic diseases [21].
3. 220
Cyst location
Up to 80% of patients suffer an infection of a single or-
gan and harbor a solitary cyst [21]. A study of the sites
of infection in 15,289 patients showed infection of the
liver (75.2%), lung (22.4%), abdominal and pelvic cavity
(5.2%), spleen (1%), kidney (0.4%), brain (0.4%), and
other organs (≤ 0.2% each) [26]. Relative percentages
vary depending on the country, but other authors report
the same order [1, 21, 27]. In the liver most cysts are lo-
cated in the right lobe. This is in accord with the fact that
the right lobe is larger than the left one. Table 1 gives the
distribution of hydatid cysts in the liver.
Indication for treatment
When hydatid disease is diagnosed, treatment should be
instituted to prevent complications such as secondary in-
fection, rupture of the cyst to adjacent structures, rupture
with intraperitoneal dissemination of the disease, ana-
phylactic reaction, pressure on contiguous organs, and
penetration into the biliary tree causing obstructive jaun-
dice; however, most patients with hydatid cysts of the
liver are asymptomatic or have few symptoms [20, 28].
Small cysts not (yet) characteristic of CE surrounded by
liver parenchyma are an exception because they grow
slowly and cause no symptoms. A policy of “wait and
see” with planned controls is indicated [17].
Indication for medical treatment
Medical treatment
There are both temporary and permanent contraindica-
tions to surgery, depending the difficulty in reaching the
lesion, the poor condition of some patients having been
operated on several times, some patients’ refusal to un-
dergo surgery, advanced age, pregnancy, severe comor-
bidity, multiple cysts difficult to access, dead cysts either
partially or totally calcified, very small cysts, and in
some highly endemic regions long waiting lists and a
lack of adequate medical structures or experienced stuff
[21, 29].
Benzimidazoles
Mebendazole and albendazole are the drugs of choice.
Past experience is that these act principally parasitostati-
cally and not as a parasiticide because the ceasing of tak-
ing the drugs causes recurrent disease [30], which means
they cannot cure the patient [8]. However, there are case
reports on probably parasiticidal effects following long-
term chemotherapy. Hence the real effects of the benzim-
idazoles have not yet been entirely determined [31, 32,
33].
Other drugs
Preoperative treatment of abdominal hydatidosis with
praziquantel and albendazole is more effective than a
monotherapy of albendazole [34]. Praziquantel may also
be useful in cases of operative spillage, but the rationale
behind must yet be confirmed [29]. Oxfendazole has
been shown to be effective in animals with cystic hydati-
dosis and warrants a comparison with albendazole [29,
35].
Adverse reactions
The major side effects are the changes in liver enzyme
levels and bone marrow suppression [21, 29]. Some
10–20% of patients develop a self-limited temporary in-
crease in transaminase levels. Severe pancytopenia and
agranulocytosis are exceptional. Alopecia is observed in
long-term treatment with albendazole. In all cases these
Table 1 Cyst location in the liver (S segment of the liver)
Reference n Right Left Both S1 (%) S2 (%) S3 (%) S4 (%) S5 (%) S6 (%) S7 (%) S8 (%)
lobe lobe lobes
(%) (%) (%)
Röthlin et al. [106] 20 60 18 30 − − − − − − − −
Khuroo et al. [76] 50 64 36 − − − − − − − − −
Perdomo et al. [17] 105 70 30 − 0 8 11 11 12 11 31 16
Alfieri et al. [37] 65 80 20 − − − − − − − − −
Balik et al. [27] 304 78 14 8 − − − − − − − −
Milicevic [10] 818 70 14 16 − − − − 34 15 43 −
Seven et al. [107] 23 60 40 − − − − − − − − −
Ozamak et al. [62] 108 57 43 − 0 13 19 15 18 8 12 15
Haddad et al. [108] 61 77 23 − − − − − − − − −
Uravic et al. [73] 27 48 37 15 − − − − − − − −
4. events disappear once treatment with albendazole is in-
terrupted [29].
Contraindication
Contraindications include cysts that are at risk of rupture
(superficially situated, infected cysts) and calcified cysts
[29]. Patients with chronic liver diseases and bone mar-
row depression should not undergo benzimidazole treat-
ment. Early pregnancy is a contraindication, and the
treatment is only rarely indicated in later stages of preg-
nancy [29].
Indication for surgery
Indication for surgery depend on the nature of the cyst
and the general state of the patient [20]. It is generally
accepted that surgery is indicated for noncomplicated
cysts with a peripheral location in patients who are in
good general condition [20]. Small cysts (<4 cm) located
deep in the parenchyma of liver, if noncomplicated, can
be managed conservatively [10]. This is due mainly to
the difficulty in approaching these deeply located cysts
[20]. The WHO has listed following indications for sur-
gery: large liver cysts with multiple daughter cysts, sin-
gle superficially situated liver cysts that may rupture
spontaneously or as the result of a trauma, infected cysts,
cysts communicating with the biliary tree and/or exerting
pressure on adjacent vital organs, and cysts in the lung,
brain, kidney, bones, and other organs [16].
Surgical treatment
General aspects of surgical strategy in hydatid disease
The basic principles of hydatid surgery were originally
set forth decades ago: entire elimination of the parasite,
no intraoperative spillage, and saving healthy tissue [36].
However, the technical procedure of choice is under de-
bate because appropriate randomized controlled studies
are lacking. Apart the basics, current surgical procedures
are influenced by such factors as arguments that are
merely plausible conclusions, personal experience of the
surgeon, and continuation of the “surgical school” from
which the surgeon comes. There are numerous terms in
the literature for the surgical procedures used, including
marsupialization, simple external tube drainage, simple
resection of the prominent part of the cyst, cystectomy
(partial or total), cysto-pericystectomy (open and closed,
partial and total), endocystectomy with marsupialization
or omentoplasty, capitonnage, Whipple procedure, cysto-
jejunostomy; there are also combinations of procedures,
and with or without additional use of scolicidal agents
[20, 37, 38, 39, 40, 41, 42, 43, 44]. The terms are not
well differentiated from each other, clear definitions are
rare, and substantial variation exists. This impairs the
evaluation of published data. The main point of conten-
tion is the procedure’s extent: whether pericystectomy is
always necessary, or whether procedures are sufficient
that leave behind the pericyst. Procedures evacuating the
cyst and keeping the pericyst are called “conservative”
and those removing the cyst and pericyst are referred to
as “radical”. Advocates of radical regimens claim that
postoperative complications and recurrence rates are de-
creased by this procedure because it includes facultative
exogenous cysts, which are a factor in recurrence, and
closed removal of the cyst should prevent secondary
echinococcosis [20, 45, 46]. Despite these plausible ad-
vantages many surgeons believe that these procedures,
especially liver resection, constitute overtreatment of a
benign disease [20, 47, 48, 49]. It was maintained that
the more radical the intervention, the higher the intraop-
erative risk usually is, but the lower the likelihood of a
relapse, and vice versa [16].
Surgical techniques
Liver resection
Hemihepatectomy and lobectomy are procedures in
terms of anatomical criteria including the parasite. If
used commonly, too much healthy tissue would be need-
lessly resected, and this may be thus considered as ex-
tensive if not overtreatment [41, 46, 50]. Therefore these
procedures are recommended if the parasite occupies
such anatomical regions almost entirely.
Pericystectomy
In pericystectomy the cyst and a more or less thick coat
of healthy liver parenchyma is resected. Because healthy
liver tissue is removed, the term is not clearly differenti-
ated from (atypical) liver resection. In the open-cyst
method the parasite foci is sterilized with scolicidal
agents, the contents of the cyst are evacuated, and the
pericyst is removed [44]. The closed-cyst method entails
en bloc resection of the parasite, including the pericyst
without cutting into the pericyst [44]. Throughout the op-
eration the external pericyst remains under visual obser-
vation, and the afferent blood vessels and biliary ducts
are ligated between the pericyst and the normal liver [51].
Partial/subtotal (cysto-)pericystectomy
Removal of the endocyst and partially/subtotally the
pericyst [44] belong to the so-called conservative proce-
221
5. dures. These procedures differ in the extent of the resec-
tion of the pericyst. Their technical feasibility is easier
than that of partial liver resection.
Management of the cyst cavity
After pericystectomy and conservative procedures there
are several methods that deal with the remaining cavity
in the liver. (a) The cavity can remain open with or with-
out drainage, leaving the volume of the cavity as it is
[52]. Oversewing the cyst edges prevents bleeding. (b)
Small, noncalcified, or infected cysts can be filled up
with saline and closed by a suture [10, 53, 54]. (c) Mar-
supialization drains the residual cyst cavity (which usu-
ally remains open) externally, leaving the pericyst intact
[44]. The cyst edges are sewn to the peritoneum/abdomi-
nal wall. This procedure has been abandoned because the
associated morbidity is unacceptable today. (d) Capiton-
nage [53, 55] and introflexion [48, 50, 52, 56] reduce the
size of the cyst cavity by adapting the cyst walls to each
other or enrolling the edges of the liver parenchyma into
the cavity. Capitonnage is not possible if the volume of
the cyst is large, or if the walls are calcified and rigid
[10]. The risk of capitonnage is to injure hepatic veins
and bile ducts running close to the cyst wall [10]. (e)
Omentoplasty fills the cavity by a pediculated part of the
greater omentum. The reported advantages include re-
sorption of fluid, reduced risk of infection and bile fistu-
lation, acceleration of wound healing, and hemostatic
properties [10, 12, 48, 48, 50, 55, 56, 57, 58, 59, 60, 61].
A prospective study observed omentoplasty to be associ-
ated with significantly fewer postoperative complica-
tions (6%) than external drainage (23%) [62]. Another
study confirmed the benefits [63]. A multicenter, pro-
spective randomized trial found the rate of deep abdomi-
nal complications after surgery for hydatid disease of the
liver (partial and total pericystectomy) to be significantly
reduced by omentoplasty (10% vs. 23%) [63].
Procedures to prevent secondary echinococcosis
(a) It is common practice to wall off the abdominal cavi-
ty immediately next to the cyst, using disinfectant-
soaked towels to reduce the risk of contamination [10,
20, 40]. If spillage occurs, infective material can be de-
stroyed [40]. (b) The use of a cone as described by
Aarons and Kune [64] and Saidi and Nazarian [65] can
prevent spillage of the cyst content and simplifies the
disinfection of the cyst cavity (see also [36, 54, 66]). The
liver must be mobilized so far that the cone can be cen-
tered on the apex of the cyst. The cyst is opened through
the cone, the parasite sucked off entirely, and the remain-
ing cavity disinfected [36, 54, 65, 66]. Saidi and Nazari-
an et al. [65] recommend freezing the cone to the liver
surface. However, the warm cyst fluid can thaw the seal
and lead to leakage [64]. Therefore Aarons and Kune
[64] use negative pressure to fix the cone, which is sim-
pler than using a cryogenic device, and a suction unit is
available in every theater. The use of a cone, however, is
not mandatory. (c) In viable cysts the pressure can be as
high as 75 cm H2O [67, 68]. Puncture of the cyst and as-
piration of a relatively small volume of the fluid reduces
this pressure and concurrently the risk of running out of
the fluid and spillage.
Decision making
The objectives of surgery are (a) inactivating infectious
material (scolices and germinative membrane), (b) pre-
venting contamination (spillage), (c) eliminating all via-
ble elements (endocyst), and (d) managing the residual
cavity. These criteria are no longer discussed in the liter-
ature but are cited relatively often and are apparently ob-
vious and accepted. The only subject that continues to be
discussed is whether radical or conservative techniques
are superior. Some authors report lower recurrence rates
with pericystectomy than with conservative procedures
(2% vs. 16%) [45, 69, 70]. However, the validity of these
studies is limited, and the same authors could not recom-
mend pericystectomy generally as the standard proce-
dure because such factors must be considered as the
number of cysts, size, relation to bile ducts and blood
vessels, extrahepatic disease, age, and general condition
[45]. Pericystectomy should include the so-called exoge-
nous cysts. However, pericystectomy is not free of recur-
rence [46, 71, 72]. Some authors have found no differ-
ence in lethality and morbidity between the surgical
techniques. Uravic et al. [73] have noted that radical pro-
cedures are too aggressive for a benign disease and rec-
ommend conservative approaches, having published fa-
vorable results.
Reported mortality rates are generally the overall re-
sults of all methods employed in the respective series,
and it is very difficult to analyze the figures according to
individual surgical techniques unless the authors specify
their techniques [46]. In contrast to acceptable mortality,
morbidity rates following hydatid cyst surgery are gener-
ally high. The reasons are infection (wound, cavity, and
intra-abdominal abscesses), pulmonary complications,
fever, biliary fistula, hemorrhage, and sclerosing cholan-
gitis) [46]. Mentes [46] considered cavity management
as the crucial step. While authors using simple drainage
or marsupialization more often report morbidity rates
above 50%, Mentes [46] and authors favoring cavity
management techniques or resective methods [47, 74] re-
ported morbidity rates between 8.4% and 32.8%. Ta-
bles 2 and 3 list results of the surgical treatment of cystic
echinococcosis. Radical procedures seem superior to
conservative methods in lethality (1.2% vs. 2%), morbid-
222
8. 225
Table 4 Results of laparoscopic procedures to treat cystic echino-
coccosis [Morbmorbidity, Mort mortaliy,Rec recurrence, Add ad-
ditional measures (delayed staged procedures or percutaneous
drainage),na not available, n number of patients observed, m num-
ber of patients followed up]
ity (11.7% vs. 23%), and recurrence rates (2.0% vs.
10.4%). However, as with other meta-analyses, this one
is also of only limited validity.
Reasons for the limited validity of published reports
There are numerous factors that limit the validity of
studies: (a) No study compares radical vs. conservative
procedures prospectively. (b) The natural course of hyda-
tid disease is not considered, and stage dependent treat-
ment has not been established. (c) Only limited numbers
of patients are examined. (d) Although radical and con-
servative procedures are differentiated, substantial varia-
tion exists within these categories. (e) The effect of scol-
icidal agents is not evaluated, causing uncertainty with
their use (some authors regard them as crucial others
abandon them) [46, 75]. If the agents prove effective,
their use affects the outcome and biases the study disre-
garding this factor. To our knowledge, no prospective
studies have compared the clinical effect of scolicidal
agents. (f) Benzimidazoles are administered before and
after surgery or both. Since these the substances are ef-
fective, this biases the results. (g) While the rates on le-
thality are relatively certain (although the causal rela-
tionship to an intervention can be obscured), conclusions
regarding morbidity vary enormously. For instance,
some authors regard temporary fever (<48 h) without ob-
vious consequences as a complication, while others do
not regard occurrences as a complication unless they
prolong hospital stay [76]. Such subjective assessment of
events biases the results. (h) Recurrence due to inade-
quate initial surgical treatment must be differentiated
from persistence of infection and reinfection [12]. How-
ever, this differentiation is difficult if possible at all. (i)
Nonstandardized procedures make interpretation of the
authors’ conclusion difficult. Considering all of these
factors even meta-analysis (Tables 2, 3) should be re-
garded as indicative only.
Lethality of procedures for recurrent disease
Operative lethality increases with the number of opera-
tions. Saimot [29] reported a series with lethality rates of
0.9–3.6% for the first operation, 6% for the second, and
20% for the third.
Laparoscopic approach
Total excision, unroofing, evacuation, and obliteration of
the cyst cavity have been performed [39, 77, 78, 79]. Ad-
vantages include noninvasiveness, shorter hospital stay,
and reduced wound complications [20]. The cyst cavity
can be examined in more detail. The laparoscopic ap-
proach appears more cost effective, but randomized con-
trolled studies should be performed before definite con-
clusions can be drawn. Disadvantages include: limited
area of manipulation, difficulty in controlling spillage
during puncture, difficulty in aspirating the thick, degen-
erated cyst contents (Gharbi III, IV) [20]. The presence
and location of complications may render this method
difficult in practice [20]. Proper selection of patients for
the laparoscopic approach is important. Centrally located
cysts carry a high risk of bleeding and should be treated
by conventional open methods. Because of the difficulty
in evacuating type IV cysts (Gharbi) laparoscopic ap-
proach should not be preferred and should be used cau-
tiously in type III cysts (Gharbi) [20].
Technical progress in laparoscopic equipment is rap-
id. A perforator-grinder and aspirator apparatus grinds
Reference n Follow-up Conversion Add Rec Morb Mort
Length m % n % n % n/m % n % n %
Manterola et al. [113] 8 30 mo 8 100 0 − 0 − 0 − 0 − 0 −
(mn; 23–44)
Bickel et al. [28] 31 49 mo na − 1/31 3 6/31 19 0/na − 5/31 16 1 3
(mn; 9–97)
Seven et al. [107] 30 17 mo 11 37 7/30 23 0 − 1/11 9 5/30 17 0 −
(mn; 3–72)
Khoury et al. [114] 12 12 mo na − 1/12 8 0 − 1/na − 1/12 8 0 −
Saglam [39] 6 6–20 mo 6 100 1/6 17 0 − 0 − 2/6 33 0 −
Yücel et al. [79] 2 17–19 mo 2 100 0 − 0 − 0 − 0 − 0 −
Alper et al. [78] 22 2–17 mo 16 100 6/22 27 1/16 6 0 − 4/16 25 0 −
Total 111 − − − 16/111 14.4 7/105 6.7 1/43 2.3 17/105 16.2 1/111 0.9
9. the parasitic tissue and promises more effective evacua-
tion of the cyst elements [39, 78]. These instruments are
certainly effective in preventing spillage of the cyst con-
tents into the abdominal cavity [20]. This apparatus, with
the help of a retractable rotary blade, punctures the cyst
through a narrow hole, and with a propeller grinds and
aspirates the cyst elements. Use of a trocar with an um-
brella-shaped locking mechanism enables the surgeon to
suspend the cyst wall against the abdominal wall. It
should be remembered that this method can be used on
the surface of the liver. The surgiport and the transparent
cannula for improved access are other examples of tech-
nical advances [80, 81]. There are no prospective ran-
domized clinical trials comparing laparoscopic treatment
with conventional open treatment or percutaneous drain-
age [20]. It is still too early to report on recurrence rates.
Initial experiences of selected cases are summarized in
Table 4.
Scolicidal agents
The substances whose use have been reported include
polyvinylpyrrolidone-iodine (PVP) 10%, a mixture of
cetrimide 1.5% and chlorhexidine 0.15% (Savlon 10%),
ethylalcohol 95%, hydrogen peroxide 3%, silver nitrate
0.5%, formalin 2%, and saline 30% as well dilutions of
these substances [20, 36, 40, 42, 52, 82].
In vitro activity
In vitro effectiveness of the scolicidal agents have been
confirmed by using the flame cell activity and dye up-
take [40]. Effective solutions are at least 20% saline, at
least 95% ethylalcohol, at least 1.5% hydrogen peroxide,
at least 5% PVP, and a mixture of at least 0.015% cet-
rimide and 0.0015% chlorhexidine [40].
In vivo activity
Gökce et al. [82] performed animal experiments and in-
oclulated viable scolices intraperitoneally. Subsequent ir-
rigation of the abdominal cavity with 1% PVP reduced
the incidence of peritoneal cysts from 90.9% (control) to
8.7%. The effect was caused by mechanical lavage (re-
duction in the concentration and wash out) and chemical
destruction of the scolices [82].
Limited evidence-based knowledge
Some authors regard disinfection of the cyst before
opening as a crucial step and demonstrate inactivation of
the scolices in up to 92% of cases with a cetrimide-
chlorhexidine combination [83]. Other authors empha-
size that the lack of objective evidence about the efficacy
of the practice of injecting scolicidal agents into the hy-
datid cysts before opening, and the presence of toxicity
associated with the scolicidal agents have led surgeons to
abandon this step in the operative management of hyda-
tid cysts [40, 46, 84, 85, 86]. Several publications ex-
press uncertainty about the efficacy of scolicidal agents.
A prospective study evaluating the efficacy of omento-
plasty left the decision whether to use scolicidal agents
or not to sterilize the cysts to the discretion of the sur-
geon [63]. Varying recommendations have been pub-
lished, including “it is the primary responsibility of the
surgeon to select the proper scolicidal agent” [20], “for
years we have preferred hypertonic saline (3–15%)—we
have not encountered any significant complications sec-
ondary to the use of these solutions” [20], and “inactiva-
tion of the scolices with a scolicidal agent prior to open-
ing or removing a cyst is strongly recommended” [87].
Although the respective authors have complete confi-
dence in their own reports, the difference in opinions re-
flects the fact that definitive knowledge and scientific
foundation in this issue are lacking. However, if the cysts
do not communicate to the bile duct system, it seems ap-
propriate to disinfect the cyst cavities after complete
evacuation, but proper studies are lacking. The WHO re-
gards as questionable the use of scolicidal drugs for in-
traoperative killing of infectious material, as there is no
ideal agent that is both effective and safe [16]. Ethanol
(70–95%), hypertonic saline (15–20%), and cetrimide
solution (0.5%) have been judged as substances with a
relatively low risk [8, 21]. For optimal efficacy the sub-
stances should be left in the cyst cavity for at least
15 min [8]. More experimental studies and clinical ob-
servations are urgently needed to evaluate the efficacy
and safety of protoscolicides [21].
Adverse effects
No product is free of adverse effects. These include scle-
rosing cholangitis (caustic damage to the epithelium of
communicating bile ducts) [40, 88, 89], low efficacy
[40], air embolism [90], anaphylactic shock [45, 91],
PVP storage disease [40], renal shutdown [40], sterile
peritonitis [40], sclerosing serositis and chemical perito-
nitis [40, 92], metabolic acidosis, and methemoglobine-
mia [86, 93, 94].
Perioperative chemotherapy
Intention
Perioperative chemotherapy offers the prospect of possi-
bly preventing recurrent disease, but more clinical trials
226
10. 227
are required to establish whether pre- or postoperative
chemotherapy does actually prevent recurrence [29, 95].
Medical treatment may reduce the operative risks or
even the need for further operations and hence the length
of hospitalization [29].
Chemotherapy prior to surgery
Albendazole given in mice at 15 mg/kg for 96 h prior to
experimental intraperitoneal administration of viable
protoscolices reduced the rate of peritoneal hydatidosis
from 81.8% to 11.1% [87]. Similar results have been re-
ported with mebendazole at a dose of 40–65 mg/kg 96 h
prior to inoculation of scolices in rats (cyst development
decreased to 11%, compared to 80% in untreated con-
trols) [96]. Therefore the preoperative administration of
benzimidazoles can reduce the risk of secondary hydati-
dosis due to intraoperative spillage of viable material.
Another issue is the long-term preoperative use of benz-
imidazoles as described by Morris [97] and Davidson et
al. [98]. Their intention is to achieve a sterile cyst at the
time of operation, and they reported the loss of viability
in most or one-half of the patients treated for 1 month.
Preoperative treatment with benzimidazoles has been re-
ported to soften the cysts, thereby reducing intracystic
pressure and simplifying their removal [16, 29]. It has
also been shown to reduce the risk of secondary echino-
coccosis and recurrence [99]. The required duration of
such treatment has not been definitively determined, but
it is not less than 3 months [21, 99].
Chemotherapy following surgery
A significant reduction in the number of peritoneal cysts
was seen in gerbils treated with albendazole 10 mg/kg
for 1 week after intraperitoneal injection of protoscolices
[100]. Several other studies have reported similar data
[5]. Therefore the WHO recommends postoperative che-
motherapy in cases in which spillage of protoscolices
may have occurred for at least 1 month (albendazole) or
3 months (mebendazole) [21]. There are no published
data on the added benefit of postoperative albendazole in
patients undergoing a complete surgical cure [29]. Cur-
rently it is recommended only when there is cyst spillage
at surgery, partial cyst removal, or biliary rupture. There
is as yet no formal consensus, as the efficacy and safety
of some of the methods require further evaluation before
we can establish comprehensive guidelines for the medi-
cal treatment of hydatidosis.
Alternative procedures
An alternative procedure for treating CE is by the combi-
nation of puncture, aspiration, injection, reaspiration
(“PAIR”). The cyst is punctured transcutaneously under
US guidance and the parasite killed by repetitive aspira-
tion of cyst fluid und injection of scolicidal agents. This
method is practicable for cysts surrounded by a sufficient
coat of liver parenchyma, not or only those that are less
septated, and contain no or only a few daughter cysts
(WHO type CE1, CE2 [CE3]) [8]. Selection must be
made by US [14]. Skilled physicians have reported good
results [8, 101, 102]. Despite these results and some pro-
gress in medical treatment, surgery is still the treatment
of choice for uncomplicated hydatid disease of the liver
[48, 76, 103]. Additional alternative methods comprise
percutaneous evacuation (PEVAC) and radiofrequency
thermal ablation [104, 105]. However, only initial expe-
rience has been gained in these options.
Prospects
Advances in US for CE have revealed a correlation be-
tween the parasitic lesions’ reflex pattern and the natural
course of the disease. Patients with inactive and unprob-
lematic disease can thus be identified and benefit from
avoidance of surgery. Prospective studies considering
stratification of the patients according to the US criteria
of the WHO should yield in more comparable results.
More evidence-based data are needed to establish a ratio-
nal decision tree covering all patients suffering from hy-
datid disease.
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Introduction
The globally found parasite causes considerable socio-
economic consequences in endemic areas. However, in
Western industrial nations the incidence is relatively low.
Following oral intake of the cestode’s eggs (diameter
40 µm) the oncospheres are released in the upper gastro-
intestinal tract, penetrate the gut wall, enter the portal
vein, and reach the liver [1, 2, 3, 4]. The liver is infected
in about 60% of cases, the lung in about 20%, and re-
maining organs in about 20% (kidney, brain, bone, mus-
cles, and others) [5, 6, 7]. In principle, all tissues can be
affected. However, in about 80% of patients a solitary
cyst occurs in one organ. The cysts grow 1–30 mm in di-
ameter yearly [5, 8, 9]. Additional cysts (daughter cysts)
can develop inside the cysts. The host organism cuts it-
self off from the parasite by formation of the so-called
pericyst, which is a capsule of connective tissue [10].
This fact is important for surgical strategies leaving the
pericyst in situ and removing all inside the pericyst,
which is the real parasite [10]. The cysts displace healthy
tissue and organs but do not grow infiltratively or de-
structively. The incubation period differs with the loca-
tion of the hydatid and adjacent tissue and takes several
years because the metacestodes’ development is slow
[11]. Symptoms are caused by compression or displace-
ment of other structures or organs, with subsequent jaun-
dice, adverse effects on hollow organ motility, but, ex-
ceptionally, spontaneous or traumatic rupture [12, 13].
However, they are not pathognomic. Entire surgical re-
moval cures the patient. Intraoperative spillage of pro-
toscolices can cause secondary echinococcosis because
the parasite can form new cysts in all tissues. Differential
diagnosis comprises dysontogenetic cysts, abscesses,
metastasis, and occasionally alveolar echinococcosis,
which can cause necrosis of cystic character.
Diagnosis
Diagnosis is made by anamnesis, imaging procedures
(ultrasound, US; computed tomography, CT, magnetic
resonance imaging, MRI), und serology.
Anamnesis
The clinical symptoms of CE are not pathognomonic and
include abdominal pain, tenderness, jaundice, feeling of
a mass, palpable tumor, fever, feeling of exhaustion,
weight loss, nausea, cholangitis, pancreatitis, biliary sec-
ondary cirrhosis, abscess, portal hypertension, vena cava
compression, cyst rupture (anaphylaxis, shock), biliary
fistula, and hemobilia [1].
Ultrasound
US is a noninvasive, readily available, sensitive, cost-
effective imaging technique and is the diagnostic method
of choice. The US features and patterns of hydatid disease
have been defined by various reports [10, 14, 15, 16, 17,
18, 19]. US helps defining internal structure, number, and
location of the cysts and the presence of complications
[20]. The specificity of US is in the range of 90% [20]. The
World Health Organization (WHO) Working Group on
echinococcosis recommended a uniform classification
based on ultrasound-morphological criteria and viability of
the parasite [19, 21]. This should enable a stage-adapted
therapy. However, proper studies have to be initiated yet.
Computed tomography
CT yields information equivalent with that from US for
diagnosis of hydatid cyst of the liver. It gives better in-
formation about the location and the depth of the cyst in
the liver. The presence of daughter cysts and exogenous
cysts can also clearly be seen on CT [20]. Documenting
the site, size, and structure of cysts is easier than with
US [1] and enables examiner-independent discussion of
the findings. CT is essential when planning surgical
treatment, especially when planning a laparoscopic ap-
proach [20]. US and CT are mandatory for diagnosis and
localization of the cysts and should be used in combina-
tion [20]. CT is superior to US in monitoring of lesions
during chemotherapy and detection of recurrences [1].
Magnetic resonance imaging
MRI gives good structural details of the hydatid cysts.
Although it may be helpful for demonstrating the lesion
in the liver, it does not provide additional information in
hepatic lesions and is not cost-effective compared with
US and CT [22]. MRI is superior in identifying changes
in the hepatic venous system [23]. MRI may facilitate
the diagnosis in uncertain cases with noncalcified or par-
tially calcified lesions by showing the characteristic
multivesicular structure, necrotic areas, and proximity to
vascular structures [24].
Serology
Immunodiagnostic procedures are used to confirm the
cause of structures discovered by the imaging procedures.
Sensitivity and specificity up 93.5% and 89.7%, respective-
ly, have been reported [25]. However, these data vary with
the sites of CE and the patient’s age (children may have a
low antibody response) [21]. False-positive reactions may
occur in patients with other helminthic diseases [21].](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)