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Echinococcosis: Treatment

Echinococcosis: Treatment
Literature review current through: Jan 2024.
This topic last updated: Jan 29, 2023.

INTRODUCTION — Echinococcosis is caused by infection with the tapeworm Echinococcus, which belongs to the family Taeniidae. Four species of Echinococcus cause infection in humans. E. granulosus and E. multilocularis are the most common, causing cystic echinococcosis and alveolar echinococcosis, respectively.

The two other species, E. vogeli and E. oligarthus, cause polycystic echinococcosis but have rarely been associated with human infection. Two new species have been identified: E. shiquicus in small mammals from the Tibetan plateau and E. felidis in African lions; their transmission potential to humans is not known [1].

The treatment of cystic and alveolar echinococcal infection will be reviewed here. The clinical manifestations, diagnosis, epidemiology, and control of echinococcal infection are discussed separately. (See "Echinococcosis: Clinical manifestations and diagnosis" and "Epidemiology and control of echinococcosis".)

This section was prepared or accomplished by Pedro Moro in his personal capacity. The opinions expressed in this article are the author's own and do not reflect the view of the United States Centers for Disease Control and Prevention, the Department of Health and Human Services, or the United States government.

CYSTIC ECHINOCOCCOSIS (E. GRANULOSUS)

Management approach — Treatment of echinococcosis usually involves antiparasitic therapy combined with either surgical resection of the cyst or percutaneous aspiration and instillation of scolicidal agents [1-3].

Antiparasitic therapy — Antiparasitic therapy may be used for definitive management in selected cases; it is also a useful adjunctive therapy to surgery and percutaneous treatment. Albendazole is the primary antiparasitic agent for treatment of E. granulosus [4]. Albendazole is poorly absorbed and should be ingested with food, ideally with a fatty meal to increase bioavailability (15 mg/kg/day, divided into two doses, to maximum 400 mg orally twice daily with food). In the absence of albendazole, or for patients with severe adverse reactions to albendazole, mebendazole may be used as an alternative therapy; it is less well absorbed than albendazole [4].

Definitive treatment — Initial management with drug treatment alone is appropriate for management of small World Health Organization (WHO) stage CE1 and CE3a cysts (eg, cysts with a single compartment and diameter <5 cm). Treatment should be administered without interruption [1]. The optimal duration is uncertain; one to three months may be appropriate, depending on clinical factors; up to six months may be required.

Drug treatment alone is usually not effective for treatment of cysts with diameter >5 cm or for treatment of WHO stage CE2 or CE3b cysts (which have multiple compartments) [5]. Other circumstances in which drug treatment alone may be warranted include management of multiple liver cysts <5 cm, management of cysts deep in liver parenchyma that are not amenable to percutaneous treatment, and/or peritoneal cysts [4]. WHO stage CE4 and CE5 cysts are inactive; antiparasitic treatment is not warranted in such cases.

Adjunctive treatment — Drug treatment is a useful adjunctive therapy to surgery and percutaneous treatment. Perioperative drug therapy with albendazole (or mebendazole) reduces the risk of recurrent disease by inactivating protoscolices [6-8]. It also softens the cyst, facilitating removal. The optimal duration of treatment is uncertain; in general treatment should be initiated at least four days prior to surgery (the WHO suggests 4 to 30 days preoperatively) and should be continued for at least one month (albendazole) or three months (mebendazole) following surgery [9-11].

Drug treatment may be appropriate for patients who cannot undergo definitive cyst removal via percutaneous treatment or surgery, although drug treatment alone is generally not sufficient for definitive management in such cases. Drug treatment is also appropriate following spontaneous cyst rupture to reduce the risk of secondary echinococcosis from seeding of protoscolices in the abdominal cavity for one month (albendazole) or three months (mebendazole) [9-11].

Drug selection — Albendazole is the primary antiparasitic agent for treatment of E. granulosus; availability and/or cost may be prohibitive in some regions. Mebendazole and praziquantel are less effective agents; in the absence of albendazole, or for patients with severe adverse reactions to albendazole, mebendazole may be used as an alternative therapy [4].

Albendazole inhibits microtubules assembly, leading to impaired glucose absorption and causing glycogen depletion followed by degeneration of the endoplasmic reticulum and mitochondria of the germinal layer, leading to cell death [12]. The outcome of treatment with albendazole depends on host characteristics, cyst size, age, and location [13]. Cumulative experience with albendazole suggests that treatment leads to cyst resolution in up to 30 percent of patients, size reduction in another 30 to 50 percent, and no change in 20 to 40 percent [14-16]. A lower likelihood of response has been observed in the setting of older patient age and longer duration of infection [17].

Albendazole has a more favorable pharmacokinetic profile than mebendazole and may be given for a shorter duration of therapy [5,14,17-27]. Albendazole is usually dosed 10 to 15 mg/kg per day in two divided doses; the usual dose for adults is 400 mg twice daily. Absorption is improved by taking albendazole with a fatty meal. Mebendazole is dosed 40 to 50 mg/kg per day in three divided doses.

Albendazole is generally well tolerated. Adverse effects include reversible hepatotoxicity (1 to 5 percent), cytopenia (<1 percent), and alopecia (<1 percent). Increased levels of aminotransferases may occur as a result of drug toxicity or parasite killing. Rarely, agranulocytosis has been reported. Dizziness, headache, vomiting, and rash have also been described. These drugs should not be used in patients with significant underlying liver disease or bone marrow suppression. Laboratory monitoring including blood count and liver function tests should be checked at two-week intervals for the first three months, then monthly.

An increase in aminotransferases that is more than fivefold above the upper limit of normal should prompt discontinuation of albendazole and consideration of alternative treatment approaches such as percutaneous or surgical management. If an alternative therapeutic agent is required, praziquantel may be a reasonable alternative to albendazole.

Albendazole should be avoided during pregnancy because of potential teratogenicity; if feasible, treatment should be delayed until after delivery [1]. Data on use of albendazole in children <6 years are limited.

Praziquantel has been shown to have protoscolicidal activity, although the efficacy of praziquantel in clinical studies is variable, and, thus far, it does not have a definitive role for primary drug therapy [28-31]. Praziquantel has been used alone and in combination with albendazole [13,28,29,32-35]. Limited case series suggest the combination of albendazole and praziquantel may be superior to albendazole alone; further study is needed [36].

Cyst management

Overview of approach — Surgery has been the traditional approach for treatment of CE; subsequently, alternative approaches have been introduced and have replaced surgery as the treatment of choice in some cases [37,38].

In general, the clinical approach depends on the World Health Organization (WHO) diagnostic classification (table 1 and image 1). Stage CE1 and CE3a cysts have a single compartment; such cysts that are <5 cm may be treated with albendazole alone [1]. In settings where albendazole treatment with follow-up monitoring is not feasible, definitive management with percutaneous treatment via puncture, aspiration, injection, and reaspiration (PAIR) is an acceptable alternative approach. Stage CE1 and CE3a cysts that are >5 cm may be treated with albendazole in combination with PAIR. In situations where albendazole treatment is not feasible, percutaneous treatment with PAIR (in the absence of adjunctive drug therapy) is an acceptable alternative approach [1]. Issues related to drug therapy are discussed below. (See 'Antiparasitic therapy' above.)

Stage CE2 and CE3b cysts have many compartments that require individual puncture; patients with such cysts commonly relapse after PAIR [39]. Therefore, management of these cysts requires either modified catheterization technique (eg, non-PAIR percutaneous therapy) or surgery (with adjunctive drug therapy) [1]. The optimal choice between these approaches is uncertain and further study is needed.

Stages CE4 and CE5 are inactive cysts that may be managed with observation [39-41].

There are few randomized trials comparing different treatments, and the level of evidence supporting the use of one therapeutic modality over another is low. In one study of 32 patients with hepatic cysts comparing percutaneous drainage alone, albendazole alone, and percutaneous drainage in combination with albendazole, those who received combination therapy had the greatest size reduction observed ultrasonographically [42]. Subsequently, a randomized trial performed among 50 patients demonstrated that outcomes among those who underwent percutaneous drainage (in combination with albendazole) were comparable to outcomes among patients who underwent surgery (with no albendazole therapy) [43].

Surgery

Indications — Surgery is the treatment of choice for management of complicated cysts (eg, rupture cyst, cysts with biliary fistulae, cysts compressing vital structures, cysts with secondary infection or hemorrhage) [1]. Surgery is also warranted for management of cysts with many daughter vesicles that are not suitable for percutaneous treatment (eg, WHO stage CE2 and CE3b) (table 1 and image 1) [1,44]. Other indications for surgery include cyst diameter >10 cm, superficial cyst at risk of rupture due to trauma, and extrahepatic disease (lung [45,46], bone [17,47], brain [17,48,49], kidney, or other site) [4,37,40,50,51]. Surgery is also appropriate in settings where percutaneous treatment is not available.

Adjunctive drug therapy should be administered to minimize risk of secondary echinococcosis from seeding of protoscolices in the abdominal cavity in the event of fluid spillage. Albendazole is generally administered beginning one week prior to surgery and continued for at least four weeks postoperatively. Some use praziquantel in addition to albendazole, although there is no clear evidence regarding its efficacy [29,52]. (See 'Adjunctive treatment' above.)

Surgery may cure the patient, but morbidity, mortality, and relapse rates can vary widely [49]. Complications include secondary infection of cyst cavity, intraabdominal abscess, biliary fistula, sclerosing cholangitis, and spillage of cyst contents leading to secondary echinococcosis and/or anaphylaxis. Postoperative complications occur in less than 1 percent of cases; recurrent echinococcosis occurs in 2 to 25 percent of cases [40,53]. These rates depend on the location and size of the cyst and the surgeon's experience.

Techniques — The goals of surgical therapy consist of evacuating the cyst and obliterating the residual cavity [51]. Every effort should be made to avoid fluid spillage, which can lead to secondary seeding of infection and/or anaphylaxis. The safest and most effective surgical procedure is uncertain; the relative advantages of the different approaches have not been clearly established. Traditional approaches have included radical resection including pericystectomy or more conservative techniques. Laparoscopic surgery may be an alternative to open surgery in some cases.

The surgical approach must be individualized depending on the cyst features [54]. Removal of the intact cyst is preferred, if feasible [55-58]. Alternatively, the cyst can be opened and sterilized with protoscolicidal agents, followed by evacuation of cyst contents and removal of the pericystic tissue. In one study including 132 patients with liver cysts treated with a variety of surgical procedures, cyst excision and omentoplasty were associated with the most favorable clinical results and lowest complication rates; a recurrence rate of 4.5 percent was observed with 4.5 years median follow-up [59].

Every effort should be made to avoid fluid spillage, which can lead to secondary seeding of infection and/or anaphylaxis. For circumstances in which intact removal of the cyst is not feasible, a protoscolicidal agent such as 20% hypertonic saline should be injected into the cyst prior to removing the contents. In addition, the surgical field should be protected with pads soaked in protoscolicidal agents. However, protoscolicidal agents should not be used in the setting of biliary communication, to minimize the risk of sclerosing cholangitis or pancreatitis. The anatomy of biliary communication should be defined intraoperatively (radio-opaque dye may be useful if available); any biliary leaks should be repaired surgically prior to application of the protoscolicidal agent.

The most commonly used protoscolicidal agent is 20% hypertonic saline; the solution should be in contact with the germinal layer for at least 15 minutes (figure 1). Albendazole, ivermectin, and praziquantel solutions have been used as protoscolicidal agents, although their efficacy and safety require further study [60,61]. Formalin has been associated with sclerosing cholangitis and should not be used.

If spillage does occur, the peritoneum should be washed with hypertonic saline. The patient should be treated with albendazole (three to six months) and a brief course of praziquantel (seven days) should also be administered [33]. The management of anaphylaxis is described separately. (See "Anaphylaxis: Emergency treatment".)

Laparoscopic surgery has been described for treatment of Echinococcus, although no randomized trials comparing laparoscopy with open procedures have been performed [37]. Laparoscopy may be associated with increased risk of spillage due to elevated intraabdominal pressures caused by pneumoperitoneum [37]. Laparoscopy is most likely to be successful in the setting of anteriorly located hepatic cysts [62]. Exclusion criteria for laparoscopy include deep intraparenchymal cysts, posterior cysts situated close to the vena cava, and presence of more than three cysts with calcified walls [37,63-66].

Surgical approaches for management of lung cysts include lobectomy, wedge resection, pericystectomy, intact endocystectomy, and capitonnage. In a study of 842 patients followed for 3 to 20 years, a recurrence rate of 1.9 percent was noted after intact endocystectomy [67].

Complications — Postoperative complications may include:

Obstructive jaundice (often attributable to echinococcal remnants in the biliary tree)

Development of an external biliary fistula occurs in up to 2 percent of patients, typically two to four weeks postoperatively)

Sphincter of Oddi stenosis, bile duct stricture, or sclerosing cholangitis − These are late complications most commonly observed in patients who underwent cyst sterilization with formalin

Management of these complications is discussed below. (See 'Patients with biliary complications' below.)

Percutaneous management — There are two categories of percutaneous techniques. The first approach aims to destroy the germinal layer with scolicidal agents (figure 1). This is done via the PAIR technique [68]. PAIR is usually effective for definitive treatment of cysts that do not have daughter cysts (eg, WHO stage CE1 and CE3a).

The second approach consists of evacuating the entire cyst with a large-bore catheter. This is generally done for management of cysts that are difficult to drain or tend to relapse after PAIR, such as WHO stage CE2 and CE3b cysts (which may contain daughter cysts). Establishing whether daughter cysts are present is important for guiding treatment, since the presence of daughter cysts reduces the likelihood of successful definitive treatment with PAIR.

Percutaneous treatment is associated with risk for anaphylaxis; in one review of treatment for nearly 6000 cysts, anaphylaxis occurred in 1.6 percent of patients [69]. Albendazole should be administered for at least one month following percutaneous treatment [44]. (See 'Adjunctive treatment' above.)

PAIR procedure — PAIR is less invasive than surgery and can be both a diagnostic and therapeutic procedure [70]. The cure rate with PAIR for appropriately selected cysts is >95 percent [71-73]. Among 231 liver cysts in 163 Italian patients treated with PAIR (ethanol was the scolicidal agent), one relapse was observed after four years of follow-up; this was successfully treated with repeat PAIR [72]. Establishing whether daughter cysts are present is important for guiding treatment, since the presence of daughter cysts generally precludes use of PAIR for definitive management.

PAIR may be used for primary treatment of WHO stage CE1 and CE3a cysts (eg, cysts that do not have daughter cysts), for treatment following relapse of CE1 or CE3a cysts managed previously with medical therapy alone, or for treatment following relapse after surgery (in the absence of daughter cysts). PAIR has been used successfully for management of cysts in the liver and other abdominal sites (eg, abdominal cavity, spleen, kidney), although many experts would approach these lesions surgically. Cysts in extra-abdominal sites such as lung or bone are less amenable to management with PAIR.

PAIR is performed under ultrasound or computed tomography (CT) guidance. Following cyst puncture, aspirated fluid should be evaluated histopathologically for presence of protoscolices. Following injection of the protoscolicidal agent, reaspiration should be performed after at least 10 to 15 minutes. The presence of protoscolices in the reaspirated fluid should prompt repeat protoscolicidal injection. Follow-up after PAIR is discussed below. (See 'Follow-up' below.)

Adjunctive drug therapy with albendazole or mebendazole should be administered at least four hours prior to PAIR. Albendazole should be continued for one month after the procedure; mebendazole should be continued for three months after the procedure [1,4,53]. Issues related to drug therapy are discussed below. (See 'Antiparasitic therapy' above.)

Further study is needed regarding the optimal approach, including use of needle versus catheter, quantity of protoscolicidal agent, and duration of follow-up required.

PAIR should not be performed in the following circumstances [40]:

Cyst with nondrainable solid material or echogenic foci

Superficial cyst at risk of rupture into the abdominal cavity

Cyst that has ruptured into the peritoneum

Cyst with biliary communication

Inactive or calcified cyst

Risks of PAIR include spillage of cyst contents into the peritoneum (which can lead to secondary echinococcosis, urticaria, and/or anaphylaxis), chemical sclerosing cholangitis, biliary fistula (6 percent), local recurrence (3 percent), and bleeding and infection (4 percent) [73-76]. Fever and urticaria occur in 11 to 13 percent of cases [73,74]; the risk of anaphylaxis is 0.5 percent and has been reduced with development of fine needles and catheters and advances in imaging techniques [74].

Endoscopy can be useful both before and after PAIR to evaluate for cyst communication with the biliary tree [72,77]. (See 'Patients with biliary complications' below.)

Modified catheterization techniques — Modified catheterization techniques are used to remove the entire endocyst and daughter cysts from the cyst cavity using large-bore catheters and cutting devices together with an aspiration apparatus [1]. This approach (in combination with albendazole if available) may be appropriate for cysts that are difficult to drain via PAIR and for management of WHO stage CE2 and CE3b cysts, which have many compartments so commonly relapse after PAIR [39,78]. The alternative management for such cysts is surgery. The optimal choice between these approaches is uncertain. Experience with modified catheterization techniques and data regarding outcomes are limited; further study is needed.

Patients with biliary complications — Patients with biliary complications of echinococcosis may warrant endoscopic retrograde cholangiopancreatography (ERCP) [79-81]:

Obstructive jaundice or cholangitis − Patients with obstructive jaundice or cholangitis (either prior to or following surgery) may warrant sphincterotomy, followed by removal of cysts and membranes with the help of a basket or an occlusion balloon [82]. In addition, hydatid sand and small daughter cysts may be flushed from the bile duct via saline irrigation. In the setting of cholangitis, a nasobiliary drain may be placed, followed by extraction of hydatid cysts and membranes (with or without sphincterotomy) [83]. The drained bile can be examined for hydatid hooklets or membranes. (See "Echinococcosis: Clinical manifestations and diagnosis", section on 'Cyst aspiration or biopsy'.)

External biliary fistula − Management consists of external biliary fistula consists of endoscopic biliary stenting for approximately four to six weeks [84]; sphincterotomy may also be effective [85].

Sphincter of Oddi stenosis − Management of sphincter of Oddi stenosis consists of sphincterotomy [86].

Bile duct stricture – For most patients with a biliary stricture associated with cholestasis, management consists of ERCP with balloon dilation and placement of a temporary biliary stent [87-89]. Several endoscopic sessions may be required to achieve stricture resolution. Management of other benign biliary strictures is discussed separately. (See "Endoscopic management of postcholecystectomy biliary complications", section on 'Biliary stricture'.)

Follow-up — Cystic echinococcosis can relapse years after treatment. Evaluating the success of therapy can is difficult since the natural history of infection can be highly variable. Cysts may continue growing (1 to 50 mm per year), persist with no change, rupture spontaneously, or resolve completely.

The optimal approach to monitoring is uncertain and must be individualized according to patient characteristics and available resources [90]. Follow-up usually consists of ultrasound or other imaging (CT or magnetic resonance imaging [MRI]) at three- to six-month intervals until the findings are stable, followed by yearly monitoring. Follow-up for up to five years is usually warranted to evaluate for recurrence; in some cases, three years may be sufficient if radiographic findings are stable at 12, 24, and 36 months [44].

Ultrasonography — Ultrasonography may be useful in assessing the response to therapy but has limitations. Ultrasound findings that appear to correlate with effective therapy include [42,91]:

Complete cyst disappearance

Reduction in cyst size and volume

Increase in proportion of solid component of cyst

Thickening and irregularity of the cyst wall

Within multivesicular cysts, reduction in size and/or number of daughter cysts

Ultrasound findings that appear to correlate with relapse include development of new cysts, increase in cyst size or volume, and increase in liquid component of the cyst [1,92].

Ultrasound lacks sensitivity for determination of cyst viability; in one study including 55 patients, no ultrasonographic changes were observed among 41 percent of nonviable cysts surgically removed following albendazole therapy [9].

On CT or MRI, degenerating cysts are often surrounded by a dense halo.

Serology — The optimal serologic test for monitoring patients on treatment for hydatid disease is uncertain. Frequently, serologic titers fall one to two years following successful surgery and rise again in the setting of recurrence. However, antibodies may remain elevated even many years after successful cyst removal [13,93].

There are some antigens that appear promising for use of serology in monitoring following treatment [94,95]. One study evaluated six serologic tests for postoperative monitoring of pulmonary hydatid disease in 79 surgically confirmed cases [96]. Latex agglutination, passive hemagglutination, immunoelectrophoresis, and specific immunoglobulin (Ig)E, IgM, and IgG enzyme-linked immunosorbent assay (ELISA) tests were used. All tests showed an increase in titer in the majority of patients within the first three months after surgery, likely as a result of antigen release during cyst manipulation. Specific IgG ELISA was the most sensitive measure of response to treatment (84 percent); specific IgE ELISA was the least sensitive (44 percent). All serologic tests showed decreasing antibody titers from three months after surgery in patients without relapse; immunoelectrophoresis, specific IgE ELISA, and specific IgM ELISA tests became negative at two years. Specific IgG ELISA was the slowest to become negative and was still positive five years after surgery in 17 percent of cases. Patients who relapsed showed either persistently high (early relapse) or initial decrease and subsequent increase (late relapse) in antibody titers in all serologic tests.

Other studies have suggested that less sensitive tests such as immunoelectrophoresis and IgM/IgE ELISA become negative earlier after successful treatment than more sensitive tests such as hemagglutination, latex agglutination, or specific IgG ELISA. The more sensitive tests may be positive in >40 percent of patients 4 to 5 years after surgery and can remain positive for more than 10 years.

Outcome — 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 [97]. The natural history of infection was variable. Approximately 15 percent of patients had undergone surgery 10 to 12 years after the initial diagnosis. Among patients who did not undergo surgery, 75 percent remained asymptomatic; 57 percent did not have a change in the size of the cyst by imaging.

Calcification usually requires 5 to 10 years to develop and 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.

ALVEOLAR ECHINOCOCCOSIS (E. MULTILOCULARIS) — The treatment of alveolar echinococcosis (AE) due to E. multilocularis is generally less effective than the treatment for cystic echinococcosis (CE) due to E. granulosus.

In general, the approach to treatment of AE consists of surgery (table 2 and table 3) [98]. Infected tissues should be removed as completely as possible, which requires complete excision of parasitic tissue and may also warrant radical resection of host tissue. The feasibility of radical resection depends on the site of the lesion, presence of metastases, patient comorbidities, and available surgical expertise.

The benefit of routine preoperative albendazole administration is not known [4]. Postoperatively, albendazole (10 to 15 mg/kg per day in two divided doses; usual adult dose 400 mg twice daily) should be administered to reduce the likelihood of relapse, even in cases of apparent cure [44]. Alternative agents such as mebendazole, praziquantel, nitazoxanide, and amphotericin are less effective than albendazole [99,100].

The optimal duration of albendazole is uncertain; in general, at least two years of therapy is advisable in conjunction with at least 10 years of follow-up monitoring for recurrence [15]. Local complications may develop that warrant intervention such as stenting, drainage of necrotic liver lesions, or endoscopic sclerosing of esophageal varices [4].

The role of palliative surgery is limited; in some cases, it may reduce the burden of infection and increase the benefit of adjunctive drug therapy [101].

Ex vivo liver resection and autotransplantation (ELRA) has shown promising outcomes in treating end-stage hepatic AE. One study found that long-term overall survival was more favorable among patients with AE who underwent ELRA than among patients who did not undergo surgery (five-year survival 82 versus 19 percent) [102].

In cases that are not amenable to definitive surgery, albendazole should be administered indefinitely to suppress progression of infection [103]. In such cases, albendazole is not curative but can improve quality of life and prolong survival. Survival rates at 15 years of 53 to 80 percent have been observed with palliative albendazole in the absence of surgery (versus 15 year mortality rate of 100 percent without treatment) [104-110]. Approximately half of patients respond with regression or lesion stabilization.

As a last resort, liver transplantation may be a consideration [111,112].

SUMMARY AND RECOMMENDATIONS

Management options for cystic echinococcosis (CE) include surgery, percutaneous management, drug therapy, and observation. In general, clinical approach depends on the World Health Organization (WHO) diagnostic classification (table 1 and image 1). There are few randomized trials comparing different treatments, and the level of evidence supporting the use of one therapeutic modality over another is low. (See 'Cystic echinococcosis (E. granulosus)' above.)

WHO stage CE1 and CE3a cysts have a single compartment; such cysts <5 cm may be treated with albendazole alone. In settings where albendazole treatment with follow-up monitoring is not feasible, definitive management with percutaneous treatment via puncture, aspiration, injection, and reaspiration (PAIR) is an acceptable alternative approach. (See 'Management approach' above.)

WHO stage CE1 and CE3a cysts that are >5 cm may be treated with PAIR and adjunctive albendazole. In situations where albendazole treatment is not feasible, percutaneous treatment with PAIR (in the absence of adjunctive drug therapy) is an acceptable alternative approach. (See 'Management approach' above.)

WHO stage CE2 and CE3b cysts have many compartments that require individual puncture; patients with such cysts commonly relapse after PAIR. Therefore, management of these cysts requires either modified catheterization technique (eg, non-PAIR percutaneous therapy) or surgery with adjunctive albendazole. (See 'Management approach' above.)

WHO stage CE4 and CE5 cysts are inactive; antiparasitic treatment is not warranted in such cases. (See 'Management approach' above.)

Every effort should be made to avoid spillage of cyst contents, which can lead to secondary seeding of infection and/or anaphylaxis. For circumstances in which intact removal of the cyst is not feasible, a protoscolicidal agent should be injected into the cyst prior to removing the contents. (See 'Techniques' above.)

We recommend albendazole for drug treatment of Echinococcus granulosus (Grade 1B); in the absence of albendazole, mebendazole may be used as an alternative therapy. Albendazole is dosed 10 to 15 mg/kg per day in two divided doses; the usual dose for adults is 400 mg twice daily, taken with food. (See 'Antiparasitic therapy' above.)

The optimal duration of definitive and adjunctive drug therapy is uncertain. Drug therapy for definitive treatment generally consists of one to three months; up to six months may be required. Drug therapy for adjunctive therapy consists of 4 to 30 days preoperatively and at least 4 weeks postoperatively. Albendazole should be administered continuously, without interruption. (See 'Antiparasitic therapy' above.)

CE can relapse years after treatment. The optimal approach to monitoring is uncertain and must be individualized. Follow-up for up to five years is warranted to evaluate for recurrence; three years may be sufficient if there is no recurrence on imaging studies at 12, 24, and 36 months. (See 'Follow-up' above.)

In general, the approach to treatment of alveolar echinococcosis consists of surgery (table 2 and table 3). Infected tissues should be removed as completely as possible, which requires complete excision of parasitic tissue and may also warrant radical resection of host tissue. In general, at least two years of adjunctive drug therapy is advisable in conjunction with at least 10 years of follow-up monitoring for recurrence. (See 'Alveolar echinococcosis (E. multilocularis)' above.)

ACKNOWLEDGMENT — The editorial staff at UpToDate, Inc. would like to acknowledge Dr. Karin Leder and Dr. Peter Weller, who contributed to an earlier version of this topic review.

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Topic 5671 Version 21.0

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