Эхинококковкые кисты печени: ликбез только для чайников

[Илья Пигович]

Спасибо, впечатляет !

[Митрич Гусаров]

Ну, Николай, здорово!!!

У Вас там , похоже, расстояния между мед помощью и пациентами совсем африканские

Никогда таких ужасов не видел...
Спасибо !!

[akserg]

Доброго дня коллеги!
В обсуждениях не нашел упоминания о очень важном, на мой взгляд, моменте. Лечение эхиноккокоза печени( какой бы вид операции вы бы не выбрали) есть лечение осложнения общей патологии под названием эхиноккокоз. Назначение препаратов антигильментной терапии до и полсе операции является обьязательным.

[Владимир К]

Фуясе, киста!

[dr.Viktor]

[Митрич Гусаров]

dr.Victor, малаец , слушай !!! Почти са-А-апсем как профессор

Завидую!!!

[dr.Viktor]

[ДмитрийЧ]

Здравствуйте всем. Пока не было времени до сего дня внимательно все прочитать.
Предыдущая тема очень интересна с печеночным Гуру, как и данная.
Рад всех видеть и слышать!
Спасибо за представленные клинические случаи.

[Вячеслав Дмитриевич РЫНДИН]

У меня скйчас больная с эхинококковой кистой - большой, но поверхностной...

Сижу и думаю: что же мне её сделать?

ICD-10: B67.0 Echinococcus granulosus infection of liver
1753 Partial or segmental hepatectomy R 3 598.95
1747 Drainage of liver cyst R 1 705.25

[dr.Viktor]

А, Вы напишите, где и какая ЭК=поверхностная....а, мы и посоветуем....
Ведь советовать всегда легче-:-:)))
Удачи!

[Вячеслав Дмитриевич РЫНДИН]

[dr.Viktor]

[Вячеслав Дмитриевич РЫНДИН]

dr.Viktor,
спасибо за отклик...
По случаю субботы позволю себе потрепаться за 15 до вызова на операцию...

Первое...
При всём уважении, так сказать, я терпеть не могу , когда мне представляются "профессор М"...
Неделю назад был в Претории на конференции... Там один престарелый профессор (БЕККЕР его фамилия) ушёл на пенсию (или его ушли)...
Про него один хирург сообщил:
- Во время обхода профессор Беккер как-то сказал "ПРОПАСТБ МЕЖДУ МОИ И ВАШИМИ ЗНАНИЯМИ ТАК ВЕЛИКА, ЧТО МЫ ДАЖЕ НЕ ВИДИМ ДРУГ ДРУГА.. "

Так вот, мне всё время кажется, что некоторые наши коллеги пытаются подчеркнуть высоту своего положения... Мне противно... Тем более, что я и сам дмн и публикацией у меня, возможно, поболее, чем у некоторых туточшных профессоров...

Второе...
Я закинул вопрос о гидатидной кисте в Русский Суржинет...
В конце беседы я написал:
Не расстраивайтесь , коллеги. А я вот не могу понять цель-и-логику расширения хирургической резекции печени при заболевании, которое может и должно лечиться лекарственно и, в крайнем случае, пункцией полости + введением в полость яда для убийства паразита и ВСЕ!!!!

Сегодня оперировали женщину... У меня сложилось впечатление , что непаразитарная киста печени с кровоизлиянием (жидкость внутри кисты типа содержимого желудка при медленном кровотечении) ... Никаких следов привычного паразита... Поспорили с Мацевичем: он ставит бутылку за то, что это умерший эхинококк , а я бутылку за то, что это непаразитарная киста... Будет две бутылки, пригласили бура анестезиолога с его буэрворсами ...


Там один коллега дал такой ответ...
"... Из жизни, вопрос: "Как Вы расслабляетесь?" Ответ: "А я не напрягаюсь." Не являюсь фанатичным сторонником какого-либо метода так, как история учит, что наука и в том числе медицинская бросает все усилия для доказательств постулата, а затем с неменьшим остервенением низвергает ранее доказанные догмы. По сути: 1) информации представленной МРТ не достаточно для адекватной оценки и принятия решения; 2) выбор метода лечения должен осуществляться с позиции цена-качество. 3) Ваше предложение. С учетом размеров кисты и предполагаемого миниинвазивного лечения, при условии полного убивства паразита, можно спрогнозировать длительное нахождение установленных в полость дренажей с неизвестным характером отделяемого, что увеличит как стоимость лечения и, догонит иные (резекцию, перицистэктомию) так и, увеличит время лечения. Поэтому и, понятны шуточные рекомендации коллег, что ни у кого нету уверенности в правильности выбраного пути. Лечить Вам. Искренне желаю удачи в решении вопроса. ..."
.
Я ему сегодня ответил:
Коллега, анекдот Вам из Африки...

Слуга (пьяный в за-зю актер, которому следует сказать "Отело, к вам гонец из Пизы")
- Отело, ккккккк. Ввввам пиздец из Ганы...

Отело, задумчиво удивленно:
- Надо же - из самой Ганы...

А вот и то, что пришло в Россию из Африки ))

Percutaneous Aspiration-Injection-Reaspiration Drainage Plus Albendazole or Mebendazole for Hepatic Cystic Echinococcosis: A Meta-analysis
Raymond A. Smego Jr.1,
Sabha Bhatti1,
Amir A. Khaliq3, and
M. Asim Beg2

+ Author Affiliations

1Department of Medicine, Aga Khan University, Karachi, Pakistan
2Department of Microbiology, Aga Khan University, Karachi, Pakistan
3Department of Health Administration and Policy, University of Oklahoma Health Sciences Center, Oklahoma City

Reprints or correspondence: Dr. R. A. Smego Jr, Dept. of Medicine, University of North Dakota, School of Medicine and Health Sciences, 1919 Elm St. N, Fargo, ND 58102 (rsmego@medicine.nodak.edu).
1.
Presented in part: 40th Annual Meeting of the Infectious Diseases Society of America, Chicago, Illinois, 24–27 October 2002 (abstract 727).

Using meta-analysis methodology, we compared the clinical outcomes for 769 patients with hepatic cystic echinococcosis treated with percutaneous aspiration-injection-reaspiration (PAIR) plus albendazole or mebendazole (group 1) with 952 era-matched historical control subjects undergoing surgical intervention (group 2). The rate of clinical and parasitologic cure (P < .0001) was greater in patients receiving PAIR plus chemotherapy. Disease recurrence (P < .0001), major complications (anaphylaxis, biliary fistula, cyst infection, liver/intra-abdominal abscess, and sepsis; P < .0001), minor complications (P < .0001), and death (P < .0824) occurred more frequently among surgical control subjects. Fever (P < .002) and minor allergic reactions subjects (P < .0001) were more common among PAIR-treated subjects. The mean durations of hospital stay were 2.4 days for group 1 and 15.0 days for group 2 (P < .001). Compared with surgery, PAIR plus chemotherapy is associated with greater clinical and parasitologic efficacy; lower rates of morbidity, mortality, and disease recurrence; and shorter hospital stays.

[Митрич Гусаров]

Добрый день, коллеги...

Честно говоря, я порой сильно устаю от менторского и наставнического тона моих соотечественников...

Хочу признаться, что у меня под рукой помимо Интернета, в котором я достаточно хорошо ориентируюсь, у меня куча образованных ребят различных мест выучки и мой друг экс-советский коллега, который намедни сдал "промежуточный" экзамен на хирурга - до "финального" экзамена еще долгий путь... Просто хочу информировать несведущих , что "первичный" экзамен на хирурга здесь сдают два дня, "промежуточный" экзамен - сдают три дня... Улавливаете разницу от одногодичной ординатуры по хирургии в России и кандидатского экзамена???

Поэтому наезды местных гуру с выездом за рамки приличного для коллег разговора не имеют под собой никакой почвы...

У маня есть три источника литературы на тему "Что есть правда по лечению эхинококкоз"

Вот вам первая, а потом дам в отдельных постах еще две


Clinical manifestations and diagnosis of cystic and alveolar echinococcosis
Aujthors
Karin Leder, MBBS, FRACP, PhD, MPH, DTMH
Peter F Weller, MD, FACP
Section Editor
Peter F Weller, MD, FACP
Deputy Editor
Elinor L Baron, MD, DTMH[youtubefulllink][/youtubefulllink]
Disclosures
Last literature review version 19.3: Fri Sep 30 00:00:00 GMT 2011 | This topic last updated: Thu Jun 14 00:00:00 GMT 2007 (More)
INTRODUCTION — Echinococcal disease is caused by infection with the metacestode stage of the tapeworm Echinococcus, which belongs to the family Taeniidae. Four species of Echinococcus produce infection in humans; E. granulosus and E. multilocularis are the most common, causing cystic echinococcosis (CE) and alveolar echinococcosis (AE), respectively. The two other species, E. vogeli and E. oligarthrus, cause polycystic echinococcosis but have only rarely been associated with human infection.
The clinical manifestations and diagnosis of cystic and alveolar echinococcal infection will be reviewed here. The epidemiology, treatment and prevention of echinococcosis are discussed separately. (See "Life cycle and epidemiology of echinococcal species" and "Treatment and prevention of echinococcosis".)
CLINICAL FEATURES — The different species of Echinococcus have different geographic distributions and involve different hosts. E. granulosus and E. multilocularis also differ in their clinical presentation. (See "Life cycle and epidemiology of echinococcal species".)
Echinococcus granulosus — The initial phase of primary infection is always asymptomatic. Many infections are acquired in childhood but do not cause clinical manifestations until adulthood. Latent periods of more than 50 years before symptoms arise have been reported. While approximately 50 percent of detected cases occur in asymptomatic patients, many more cases remain undiagnosed or are found incidentally at autopsy.
The clinical presentation of E. granulosus infection depends upon the site of the cysts and their size. Small and/or calcified cysts may remain asymptomatic indefinitely. However, symptoms due to mass effect within organs, obstruction of blood or lymphatic flow, or complications such as rupture or secondary bacterial infections can result.
Cysts typically increase in diameter at a rate of one to five centimeters per year. Hydatid cysts may be found in almost any site of the body, either from primary inoculation or via secondary spread. The liver is affected in approximately two-thirds of patients, the lungs in approximately 25 percent, and other organs including the brain, muscle, kidney, bone, heart, and pancreas in a small proportion of patients. Eighty-five to 90 percent of patients with E. granulosus infection have single-organ involvement, and more than 70 percent have only one cyst (picture 1).
Liver involvement — E. granulosus infection of the liver frequently produces no symptoms. The right lobe is affected in 60 to 85 percent of cases. Significant symptoms are unusual before the cyst has reached at least 10 cm in diameter. If the cysts become large, hepatomegaly with or without associated right upper quadrant pain, nausea and vomiting can result (picture 2).
E. granulosus cysts can rupture into the biliary tree and produce biliary colic, obstructive jaundice, cholangitis, or pancreatitis. (See "Endoscopic diagnosis and management of biliary parasitosis".) Pressure or mass effects on the bile ducts, portal and hepatic veins, or on the inferior vena cava can result in cholestasis, portal hypertension, venous obstruction, or the Budd-Chiari syndrome. (See "Etiology of the Budd-Chiari syndrome".) Liver cysts can also rupture into the peritoneum causing peritonitis, or transdiaphragmatically into the pleural space or bronchial tree causing pulmonary hydatidosis or a bronchial fistula. Secondary bacterial infection of the cysts can result in liver abscesses. (See "Pyogenic liver abscess".)
Lung involvement — Pulmonary involvement with E. granulosus can lead to a variety of symptoms, including chronic cough (sometimes with accompanying hemoptysis or evacuation of cyst material), chest pain, pleuritis or dyspnea. Rupture of a cyst into a bronchus may lead to hemoptysis, respiratory distress, and asthma-like symptoms [1]. If cysts rupture into the pleural space, a pleural effusion or empyema may develop. Lung abscesses can also occur. (picture 3)
Approximately 60 percent of pulmonary hydatid disease affects the right lung and 50 to 60 percent involve the lower lobes [2]. Multiple cysts are common. Approximately 20 percent of patients with lung cysts also have liver cysts [3].
Other organs — Involvement of organs outside of the liver or lung are unusual but can lead to significant morbidity and mortality:
• Infection of the heart can result in mechanical rupture with widespread dissemination or pericardial tamponade.
• Central nervous system involvement can lead to seizures or signs of raised intracranial pressure; infection of the spinal cord can result in spinal cord compression.
• Cysts in the kidney can cause hematuria or flank pain [4]. Immune complex-mediated disease, glomerulonephritis leading to the nephrotic syndrome, and secondary amyloidosis have also been described [5,6].
• Bone cysts are usually asymptomatic until a pathologic fracture develops; the spine, pelvis and long bones are most frequently affected.
• Ocular cysts also occur.
Cyst rupture — Fever and acute hypersensitivity reactions including anaphylaxis, may be the principal manifestations of cyst rupture. Hypersensitivity reactions are related to the release of antigenic material and secondary immunologic reactions. (See "Anaphylaxis: Rapid recognition and treatment".)
Outcome of infection — The outcome of infection varies with the stage of the disease.
One study reported on the long-term outcome of 33 patients with asymptomatic liver hydatid cysts [7]. The natural history of the infection was variable. Fifteen percent of patients had undergone surgery 10 to 12 years after the initial diagnosis, while the remaining 85 percent had not. Seventy-five percent of the patients who did not have an operation remained asymptomatic; 57 percent did not show a change in the size of the cyst by imaging.
Calcification, which usually requires five to 10 years to develop, occurs most commonly with hepatic cysts but rarely with pulmonary or bone cysts. Total calcification of the cyst wall suggests that the cyst may be nonviable.
Echinococcus multilocularis — E. multilocularis infections are less likely to be asymptomatic, although the clinical manifestations are frequently nonspecific. The most common presenting complaints include malaise, weight loss, and right upper quadrant discomfort due to hepatomegaly. Cholestatic jaundice, cholangitis, portal hypertension and the Budd-Chiari syndrome can also occur.
Extrahepatic primary disease is very rare (1 percent of cases). Thirteen percent of cases present as multiorgan disease where metacestodes involve the lungs, spleen, or brain in addition to the liver [8]. Immunodeficiency, such as HIV or transplantation, may accelerate the manifestations of alveolar echinococcosis [9]. WHO has proposed a staging system for alveolar echinococcosis.
If left untreated, more than 90 percent of patients will die within 10 years of the onset of clinical symptoms, and virtually 100 percent by 15 years [10]. Since treatment with albendazole has been introduced, the prognosis has improved considerably. Of 117 patients from France who underwent long term follow-up, the actuarial survival rate was 88 percent [9].
DIAGNOSIS — The combination of imaging and serology usually make the diagnosis of both cystic and alveolar echinococcosis [11], although serologic assays are more sensitive and specific for E. multilocularis compared to E. granulosus infection. The following discussion primarily deals with the more common E. granulosus infection, except as indicated. Specific discussion related to the diagnosis of alveolar echinococcosis follows below (see 'Echinococcus multilocularis' above).
Routine laboratory tests — Nonspecific leukopenia or thrombocytopenia, mild eosinophilia, and nonspecific liver function abnormalities may be detected, but are not diagnostic. Fewer than 15 percent of cases have eosinophilia, which generally occurs only if there is leakage of antigenic material.
Imaging — Computed tomography (CT) scanning, magnetic resonance imaging (MRI), and ultrasound are used to detect hydatid cysts and to evaluate their characteristics. Ultrasound is employed most widely because it is easy to perform and less expensive. Portable ultrasound machines are frequently used for screening patients in communities in which E. granulosus infection is endemic, sometimes with confirmatory serologic testing to maximize the diagnostic yield [12]. However, both CT and MRI may provide more detail and greater specificity. The number of cysts, the presence or absence of daughter cysts, rupture, calcification and the exact location within the organ can be established and can help to guide optimal management. (See "Treatment and prevention of echinococcosis".)
Plain radiography may reveal calcification within a cyst, but cannot detect uncalcified cysts and is not the imaging technique of choice.
Ultrasound — Ultrasound has a sensitivity of approximately 90 to 95 percent [13,14]. The most common appearance on ultrasound is an anechoic smooth, round cyst, which can be difficult to distinguish from a benign cyst.
When the liver cyst contains membranes, mixed echoes will appear that can be confused with an abscess or neoplasm. When daughter cysts are present, characteristic internal septation results. Pulmonary cysts may be single or multiple, usually do not calcify, rarely lead to daughter cyst formation, and may contain air if the cyst has ruptured.
The term "hydatid sand," reflects a complex image which consists predominantly of hooklets and scolexes from the protoscolices. This finding may be visible when shifting the patient's position during imaging. When ultrasound reveals infoldings of the inner cyst wall, separation of the hydatid membrane from the wall of the cyst, or hydatid sand, a diagnosis of hydatid disease is probable [15].
Ultrasound allows for the classification of the cyst(s) by biologic activity, which may influence the choice of treatment; these categories are: active, transitional, or inactive. Characteristics on ultrasound that are suggestive of an inactive lesion include a collapsing, flattened elliptical cyst (corresponds to low pressure within the cyst), detachment of the germinal layer from the cyst wall ("water lily sign"), coarse echoes within the cyst, and calcification of the cyst wall [16,17]. Cysts with a calcified rim may have an "eggshell" appearance.
Several other classification systems are based upon ultrasound appearance (table 1).
• One is the Gharbi classification, which divides cysts into five types [18]. Type I cysts consist of pure fluid; type II have a fluid collection with a split wall; type III cysts contain daughter cysts (with or without degenerated solid material); type IV have a heterogeneous echo pattern; and type V have a calcified wall [18].
• A newer classification proposed by the World Health Organization (WHO) characterizes cysts by type, size (small cysts <5.0 cm; medium cysts 5 to 10 cm; large cysts >10 cm), and decade of life in which they were first diagnosed by ultrasound [19]. This scheme is detailed on the worldwide web at http://www.medicalweb.it/aumi/echinonet/.
Classification of echinococcal cysts
Gharbi classification
WHO classification
Grouping
Type I
Type CE 1
Group 1- Active group:
Cysts developing and are usually fertile
Type II
Type CE 2
 
Type III
Type CE 3
Group 2- Transition group:
Cysts starting to degenerate, but usually still contain viable protoscoleces
Type IV
Type CE 4
Group 3- Inactive group:
Degenerated or partially/totally calcified cysts, very unlikely to contain protoscolices
Type V
Type CE 5
 
• WHO classification for cystic echinococcosis due to E. granulosis.
• Adapted from: WHO Informal Working Group. Acta Trop 2003; 85:253.
 
 
Computed tomography — Many reports suggest that CT has a higher overall sensitivity than ultrasound, with sensitivity rates of 95 to 100 percent [13,14,20]. CT is the best mode for determining the number, size, and anatomic location of the cysts, and is also better than ultrasound in detecting extrahepatic cysts. CT may also be used for monitoring lesions during therapy and to detect recurrences (picture 1) [21].
CT may be superior to ultrasound in determining complications such as infection and intrabiliary rupture [22]. In one study, ultrasound performed better than CT in the investigation of the cyst wall, hydatid sand, daughter cysts, and splitting of the cyst wall, while CT was superior in detecting gas and minute calcifications within the cysts, in attenuation measurement, and in anatomic mapping [17,23].
Magnetic resonance imaging — MRI has no major advantage over CT for abdominal or pulmonary hydatid cysts, except in defining changes in the intra- and extrahepatic venous system [24]. Thus MRI is usually not required and in most instances is not cost effective [25-27]. However, it may delineate the cyst capsule better than CT and may be better at diagnosing complications, particularly for cysts with infection or biliary communication.
Both MRI and CT are useful in diagnosing echinococcal infection in other sites, such as in the brain [28].
Other — Other imaging techniques such as cholangiography may be indicated to diagnose biliary involvement, particularly in patients with cholestatic jaundice. ERCP is frequently performed in patients with liver cysts prior to intervention. (See "Endoscopic diagnosis and management of biliary parasitosis".)
Serology — Immunodiagnosis is useful for primary diagnosis and for follow-up after treatment [8,29,30]. Detection of circulating E. granulosus antigens in serum is less sensitive than antibody detection, which remains the method of choice [8]. As a general rule, serologic testing for alveolar echinococcosis is more reliable than for cystic echinococcosis. (See 'Echinococcus multilocularis' above.)
Conventional routine immunodiagnostic tests are usually based upon the use of crude antigens, such as hydatid fluid or protoscolex extracts. Additional tests using recombinant or purified species-specific antigens may complement the serological diagnosis [31].
False positive and false negative results — Serologic testing produces both many false positive and false negative results.
• False positive reactions are more likely in the presence of other helminth infections (such as Taenia saginata, Taenia solium, and particularly neurocysticercosis), cancer, and immune disorders.
• False negative results occur with varying frequency depending upon the site of the lesion and the cyst's integrity and viability. Antigen-antibody complexes that "mop" up all antibodies may lead to false negative reactions. Thus, a negative serologic test generally does not rule out echinococcosis.
Children and pregnant women more frequently have negative serology than other patient populations [2].
Site of involvement — There is no consistent correlation between the number or size of cysts and serologic results [32]. However, cysts in the liver more commonly elicit an antibody response than cysts in the lung. Overall, approximately 85 to 95 percent of liver cysts and 65 percent of lung cysts will be associated with positive serology, although this varies with the specific serologic test used and cyst activity [33].
Brain, eye, and splenic cysts often do not produce detectable antibodies, whereas bone cysts frequently are associated with positive serology. Serology is less likely to be positive with cysts at any site if the cysts are intact, calcified, or nonviable.
Serologic methods — The complement fixation test was the first immunologic method used for serodiagnosis of hydatid disease, but a number of other techniques currently are employed, including:
• Indirect hemagglutination (IHA)
• Indirect immunofluorescence
• Latex agglutination
• Double diffusion immunoelectrophoresis
• Counter-current immunoelectrophoresis (CIEP)
• Radioimmunoassay (RIA)
• Enzyme-linked immunosorbent assay (ELISA)
• Enzyme-linked immunoelectrotransfer blots (EITB)
• Enzyme-linked immunoelectrodiffusion assay (ELIEDA)
• Time-resolved fluoroimmunoassay (TR-FLA)
• Immunoblot
The methods most frequently employed for initial screening tests (using crude antigens) are IHA and ELISA. Confirmatory tests using specific antigens can then be performed, such as arc-5 immunoelectrophoresis and immunoblotting [34].
Comparisons among tests — Lack of standardization of assays contributes to discrepant results reported by the various laboratories. The sensitivity and specificity of a number of the serologic tests have been compared (table 2). The data suggest that ELISA is the most sensitive and specific of the available assays as illustrated below [32-37]:
• One study of 79 patients with surgically confirmed pulmonary hydatidosis demonstrated that IgG ELISA was the most sensitive (84 percent), followed by IgM ELISA (62 percent), passive hemagglutination (61 percent), latex agglutination (58 percent), immunoelectrophoresis (51 percent), and specific IgE ELISA (44 percent). The specificity of all tests was 98 to 100 percent. Specific IgG ELISA had the highest negative predictive value (93 percent) [32].
• Another study compared eight serologic tests in 131 patients with E. granulosus [35]. IgG ELISA was the most sensitive (94 percent) and specific (99 percent) for the majority of cyst locations.
• Another report contrasted six different serologic tests for the diagnosis of cystic hydatid disease in 243 surgically confirmed cases [36]. The two ELISA tests gave identical results, with a sensitivity of 89 percent for liver cysts and 78 percent for lung cysts. In the 39 patients with false negative results, the use of immunoblotting only increased the yield by eight percent; ELIEDA did not identify any additional cases.
Simple, heat-stable, inexpensive tests, such as hydatid antigen dot immunoassays, are often used for field testing and population screening [38]. The dot-ELISA has a reported sensitivity of 88 to 96 percent and a specificity of 90 to 98 percent [39-41].
Sensitivity of serologic tests for echinococcosis at different sites
Site of lesion
Sensitivity of serologic tests
Liver
IgG ELISA: 80-90 percent
 
IgE ELISA: 82-92 percent
 
Latex agglutination: 65-75 percent
 
Hemagglutination: 80-90 percent
 
Immunoblot (using antigen 5 and/or a B-rich fraction): 80-90 percent
 
Enzyme-linked immunotransfer blot: 80 percent
Lung
IgG ELISA: 60-85 percent
 
IgE ELISA: 45-70 percent
 
Latex agglutination: 50-70 percent
 
Hemagglutination: 50-70 percent
 
Immunoblot (using antigen 5 and/or a B-rich fraction): 55-70 percent
 
Enzyme-linked immunotransfer blot: 55 percent
 
Antigens for serologic testing — Antigen 5 and antigen B are the two major antigens of E. granulosus utilized in serologic testing.
• Antigen 5 is a major parasite antigen found on the inner aspect of the germinal layer, brood capsule, and protoscolices. Only a few studies have assessed the value of tests based on recombinant antigen 5, which has relatively low specificity, although it is used quite extensively for diagnosis in clinical practice [34].
• Antigen B is a highly immunogenic polymeric lipoprotein. Studies have showed that antigen B shows a high degree of genetic variability [42]. It offers greater specificity than detection of antigen 5; however, neither antigen is specific for E. granulosus per se despite a very high specificity for echinococcal infection [43,44].
When these antigens are used in ELISA assays, the sensitivity is 60 to 90 percent (depending upon the site of the cyst, its viability etc), and the specificity is usually approximately 90 percent [45]. When used in immunoblot and gel diffusion assays, the sensitivity is approximately 90 percent with a specificity of 97 to 100 percent [44,46]. One study showed that the immunoblot with the antigen-B rich fraction was positive in 92 percent of patients with E. granulosus, but was also positive in 79 percent of patients with E. multilocularis [47]. No cross-reactivity was observed with sera from patients with other parasitic diseases, malignancies, or healthy controls.
Problems with serology — In addition to features of the cyst and of the host that lead to variable serologic responses, a variety of issues involved with serologic testing influence the rates of false positive and false negative reactions.
• There is a lack of standardization among different laboratories for some of these assays.
• One study showed that ELISA using antigen B had a sensitivity of 63 percent, whereas immunoblotting using the same antigen had a sensitivity of 80 percent [48].
• Different methods of antigen isolation and purification influence results.
Serologic diagnosis is often optimized by using a combination of tests or sequential testing [49]. Tests employing a number of recombinant antigens are being evaluated to improve the sensitivity and specificity of the commercially available serologic tests. A highly sensitive assay, usually an ELISA or indirect hemagglutination test, is commonly used as an initial screen, followed by a highly specific immunoblot or gel diffusion assay for confirmation. Testing for specific antibodies, such as specific IgG1 or IgG4 rather than total IgG, may improve specificity [48,50-52].
Antigen assays — A variety of purified or recombinant antigens have been used for immunodiagnosis. Demonstration of antigens in cystic fluid or serum can also be used for diagnosis of primary infection or relapse [49,53,54]. However, as many as one-half of patients with echinococcal cysts do not have circulating antigens.
Studies using latex agglutination or a dot-ELISA to detect echinococcal antigens from cyst fluid and have shown excellent sensitivity and specificity [55-57]. However, detection of hydatid antigen in urine specimens using CIEP is much less sensitive (40 to 50 percent) and false positives have occurred [58]. Antigen assays and tests for circulating immune complexes are generally not widely available, but may become useful secondary tests in the future [59].
A novel recombinant antigen for immunodiagnosis of cystic echinococcus was investigated in a study of 413 patients with echinococcosis, 172 patients with neurocysticercosis, 241 patients with other infections, and 70 healthy controls [60]. Testing of serum samples yielded an overall sensitivity and specificity of 92 and 96 percent, respectively. This assay is not commercially available.
Cyst aspiration or biopsy — Percutaneous aspiration or biopsy may be required to confirm the diagnosis by demonstrating the presence of protoscolices, hooklets, or hydatid membranes. Active cysts reveal clear watery fluid containing scolices and have elevated pressure, whereas inactive cysts exhibit cloudy fluid without detectable scolices and do not have elevated pressure [16]. Protoscolices or degenerated hooklets can sometimes be demonstrated in sputum or bronchial washings. A variety of staining methods to detect parasitic material can be used.
Percutaneous aspiration of liver cyst contents is associated with very low rates of complications, but this method of diagnosis is generally reserved for situations when other diagnostic methods are inconclusive because of the potential for anaphylaxis and secondary spread of the infection [61-64]. If aspiration is required, it should be performed under ultrasound or CT guidance; complications can be minimized by concurrent administration of benzimidazole therapy. Increasingly, cyst puncture also is performed as a therapeutic measure. (See "Treatment and prevention of echinococcosis".)
Polymerase chain reaction — Currently, PCR techniques are only being used for research purposes, but they may have a role in diagnosis and in species determination in the future. DNA probes using Southern hybridization tests are also being developed [65].
E. multilocularis — Nonspecific leukopenia or thrombocytopenia, mild eosinophilia, and nonspecific liver function abnormalities may be detected, but are not diagnostic. Hypergammaglobulinemia and elevated serum IgE levels are present in more than 50 percent of cases.
Imaging — Diagnosis is often made by imaging techniques, frequently in conjunction with serodiagnosis. On ultrasound or CT, the lesions usually have an irregular contour with no well-defined wall, central necrosis, and irregular intralesional and wall calcifications. They may be difficult to distinguish from a tumor, but the patient's overall condition is usually better than would be expected for a malignancy.
Obstruction of the inferior vena cava or of the portal venous system may be evident, which may be more easily appreciated on MRI. Lung, brain, and bone lesions may also be detected.
Serology — Serology can also be helpful in the diagnosis of E. multilocularis infection, and immunodiagnostic tests are more reliable than for E. granulosus infections with sensitivity and specificity rates of 95 to 100 percent [66]. Serology usually remains positive indefinitely in patients receiving chemotherapy, yet may become negative within a few years following complete surgical resection [67]. Clinical recurrence is often associated with rising serologic titers. IgG1 and IgG4 antibodies are the most sensitive isotypes for monitoring the success of therapy [68]. (See "Treatment and prevention of echinococcosis".)
For the serodiagnosis of alveolar echinococcosis, a specific E. multilocularis antigen such as the affinity purified Em2 antigen from AE metacestodes is often used. The Em2-ELISA can discriminate between E. granulosus and E. multilocularis in 95 percent of cases. This test has also been shown to be useful in monitoring response to surgery, since it often becomes negative within four years of surgery and then becomes positive again with a recurrence [66,69,70].
An Em2plus-ELISA assay uses additional species-specific antigens to improve its diagnostic value. The Em2plus-ELISA has a 97 percent sensitivity and 99 percent specificity and is also useful for monitoring recurrence following surgical resection [65,71,72].
ELISA and immunoblot studies using an 18-kD antigen (Em 18) for AE protoscolices have shown this antigen to be sensitive and highly species-specific (95 percent specificity) [47,68,73-76]. It also reliably differentiates between active and inactive AE, and is useful for follow up of patients on treatment [68,72,77,78].
Other tests — Open biopsy may be required in seronegative patients. In the future, it is likely that PCR will also be used and may allow earlier detection of infection [79].
Reliable data are lacking about the meaning of changes on imaging and metacestode viability. One report suggested that fluorodeoxyglucose positron emission tomography (FDG-PET) may be able to distinguish between "hot" metabolically active lesions and nonenhancing, metabolically inactive lesions [80]. While this modality, and others (magnetic resonance spectrometry) may become useful tools for assessing response to therapy and detecting relapses, they are expensive and not yet widely available [31].
SUMMARY AND RECOMMENDATIONS
Clinical manifestations — The clinical features of infection due to E. granulosus and E. multilocularis differ from each other.
• The initial phase of primary infection with E. granulosus is always asymptomatic and may remain so for many years. Subsequent clinical features and complications of E. granulosus infection depend upon the site of the cysts and their size. The liver is affected in approximately two-thirds of patients, the lungs in approximately 25 percent; the vast majority of patients have single-organ involvement, and more than 70 percent have only one cyst. The long-term outcome is variable and many patients remain asymptomatic. (See 'Echinococcus granulosus' above.)
• E. multilocularis infections are less likely to be asymptomatic, although the clinical manifestations are frequently nonspecific. The most common presenting complaints include right upper quadrant discomfort due to hepatomegaly, malaise, and weight loss. If left untreated, more than 90 percent of patients will die within 10 years of the onset of clinical symptoms. (See 'Echinococcus multilocularis' above.)
Diagnosis — A combination of imaging and serology usually make the diagnosis of both cystic and alveolar echinococcosis, although serologic assays are more sensitive and specific for E. multilocularis compared to E. granulosus infection. The diagnosis is typically made by ultrasound imaging in combination with serologic testing with ELISA.
• When ultrasound reveals infoldings of the inner cyst wall, separation of the hydatid membrane from the wall of the cyst, or hydatid sand, a diagnosis of hydatid disease is probable. Serologic testing can confirm the diagnosis. (See 'Ultrasound' above.)
• For E. multilocularis, lesions on ultrasound may have an irregular contour and may be difficult to differentiate from tumor. In these cases, the patient may appear well; however we recommend that serologic testing be pursued for diagnostic confirmation. (See 'E. multilocularis' above.)
• The likelihood of a positive serology depends on cyst location and viability. Patients with cysts in the liver are more likely to be seropositive than patients with cysts in the lung. Serologic assays are less likely to be positive if the cyst is calcified or nonviable. In these situations, diagnostic judgment may need to take into account the limitations of serologic testing. (See 'Site of involvement' above.)
• Percutaneous aspiration or biopsy should be reserved for situations when other diagnostic methods are inconclusive because of the potential for anaphylaxis and secondary spread of the infection. If aspiration is required, it should be performed under ultrasound or CT guidance. We suggest concurrent administration of benzimidazole therapy to decrease the risk of complications. (See 'Cyst aspiration or biopsy' above.)
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