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Harrison's Principles of Internal Medicine, 21e

Chapter 235: Cestode Infections

A. Clinton White, Jr.; Peter F. Weller

INTRODUCTION
Cestodes, or tapeworms, are segmented flat worms. The adult worms reside in the gastrointestinal tract, but the larvae can be found in almost any
organ. Human tapeworm infections can be divided into two major clinical groups. In one group, humans are the definitive hosts, with the adult
tapeworms living in the gastrointestinal tract (Taenia saginata, Diphyllobothrium, and Dipylidium caninum). In the other, humans are intermediate
hosts, with larval­stage parasites present in the tissues; diseases in this category include echinococcosis, sparganosis, and coenurosis. Humans may be
the definitive and/or intermediate hosts for Taenia solium; both stages of Hymenolepis nana are found simultaneously in the human intestines.

The ribbon­shaped tapeworm attaches to the intestinal mucosa by means of sucking cups or hooks located on the scolex. Behind the scolex is a short,
narrow neck from which proglottids (segments) form. As proglottids mature, they are displaced further back from the neck by the formation of new,
less mature segments. The progressively elongating chain of attached proglottids, called the strobila, constitutes the bulk of the tapeworm. The length
varies among species. In some, the tapeworm may consist of more than 1000 proglottids and may be several meters long. The mature proglottids are
hermaphroditic and produce eggs, which are subsequently released. Because eggs of the different Taenia species are morphologically identical, only
morphologic differences in the scolices or proglottids enable species­level diagnosis.

Most human tapeworms require at least one intermediate host for complete larval development. After ingestion of the eggs or proglottids by an
intermediate host, the invasive larvae (oncospheres) are activated, escape the egg, and penetrate the intestinal mucosa. The oncosphere migrates to
tissues and develops into an encysted form known as a cysticercus (single scolex), a coenurus (multiple scolices), or a hydatid (cyst with daughter cysts,
each containing several protoscolices). The definitive host’s ingestion of tissues containing a cyst enables a scolex to develop into a tapeworm.

TAENIASIS SAGINATA AND TAENIASIS ASIATICA

The beef tapeworm T. saginata occurs in all countries where raw or undercooked beef is eaten. It is most prevalent in sub­Saharan African and Middle
Eastern countries. Taenia asiatica is closely related to T. saginata and is found in Asia, with pigs as intermediate hosts. The clinical manifestations and
morphology of these two species are very similar and are therefore discussed together.

Etiology and Pathogenesis

Humans are the only definitive host for the adult stage of T. saginata and T. asiatica. The tapeworms, which can reach 8 m in length with 1000–2000
proglottids, inhabit the upper jejunum. The scolex of T. saginata has four prominent suckers, whereas T. asiatica has an unarmed rostellum. Each
gravid segment has 15–30 uterine branches (in contrast to 8–12 for T. solium). The eggs are indistinguishable from those of T. solium; they measure
30–40 μm, contain the oncosphere, and have a thick brown striated shell. Eggs deposited on vegetation can live for months or years until they are
ingested by cattle or other herbivores (T. saginata) or pigs (T. asiatica). The embryo released after ingestion invades the intestinal wall and is carried to
striated muscle or viscera, where it transforms into the cysticercus. When ingested in raw or undercooked meat, the cysticercus evaginates and forms a
tapeworm in the human intestines. Over ~2 months, the adult worm matures and begins to produce eggs.

Clinical Manifestations

Patients become aware of the infection most commonly by noting passage of proglottids in their feces. The proglottids of T. saginata are motile, and
patients may experience perianal discomfort when proglottids are discharged. Mild abdominal pain or discomfort, nausea, change in appetite,
weakness, and weight loss can occur.

Diagnosis
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not found in the stool, the perianal region should be examined with use of a cellophane­tape swab (as in pinworm infection; Chap. 232).
Distinguishing T. saginata or T. asiatica from T. solium requires examination of mature proglottids or the scolex. Available serologic tests are not
Clinical Manifestations
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Patients become aware of the infection most commonly by noting passage of proglottids in their feces. The proglottids of T. saginata are motile, and
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patients may experience perianal discomfort when proglottids are discharged. Mild abdominal pain or discomfort, nausea, change in appetite,
weakness, and weight loss can occur.

Diagnosis

The diagnosis is made by the detection of eggs or proglottids in the stool. Eggs may also be present in the perianal area; thus, if proglottids or eggs are
not found in the stool, the perianal region should be examined with use of a cellophane­tape swab (as in pinworm infection; Chap. 232).
Distinguishing T. saginata or T. asiatica from T. solium requires examination of mature proglottids or the scolex. Available serologic tests are not
helpful diagnostically. Eosinophilia and elevated levels of serum IgE are usually absent.

Treatment of Taeniasis Saginata and Taeniasis Asiatica

A single dose of praziquantel (10 mg/kg) is highly effective. Niclosamide (adult dose, 2 g; 1 g for children weighing 11−34 kg) is also effective but is less
available.

Prevention

The major method of preventing infection is the adequate cooking of beef or pork viscera; exposure to temperatures as low as 56°C for 5 min will
destroy cysticerci. Refrigeration or salting for long periods or freezing at –10°C for 9 days also kills cysticerci in beef. General preventive measures
include inspection of beef and proper disposal of human feces.

TAENIASIS SOLIUM AND CYSTICERCOSIS

The pork tapeworm T. solium can cause two distinct forms of infection in humans: adult tapeworms in the intestine or larval forms in the tissues
(cysticercosis). Humans are the only definitive hosts for T. solium; pigs are the usual intermediate hosts, although other animals may harbor the larval
forms.

T. solium is found worldwide in areas where pigs are raised and have access to human feces. However, it is most prevalent in Latin America, sub­
Saharan Africa, China, India, and Southeast Asia. Cysticercosis occurs in industrialized nations largely as a result of the immigration of infected
persons from endemic areas.

Etiology and Pathogenesis

The adult tapeworm generally resides in the upper jejunum. The scolex attaches by both sucking disks and two rows of hooklets. The adult worm
usually lives for a few years. The mature tapeworm, usually ~3 m in length, may have as many as 1000 proglottids, each of which produces up to 50,000
eggs. Proglottids are released and excreted into the feces, and the eggs in these proglottids are infective for both humans and animals. After ingestion
of eggs by the pig intermediate host, the invasive larvae are activated, escape the egg, penetrate the intestinal wall, and are carried via the bloodstream
to many tissues; they are most frequently identified in striated muscle of the neck, tongue, and trunk. Within 60–90 days, the encysted larval stage
develops. These cysticerci can survive for months to years. By ingesting undercooked pork containing cysticerci, humans acquire infections that lead to
intestinal tapeworms. Infections that cause human cysticercosis follow the ingestion of T. solium eggs. Transmission is usually associated with close
contact with a tapeworm carrier. The eggs are sticky and may be found under the fingernails of tapeworm carriers. Autoinfection may occur if an
individual with an egg­producing tapeworm ingests eggs derived from his or her own feces.

Clinical Manifestations

Intestinal infections with T. solium may be asymptomatic. Fecal passage of proglottids may be noted by patients. Other symptoms are infrequent.

In cysticercosis, the clinical manifestations are variable. Cysticerci can be found anywhere in the body but are most commonly detected in the brain,
cerebrospinal fluid (CSF), skeletal muscle, subcutaneous tissue, or eye. The clinical presentation of cysticercosis depends on the number and location
of cysticerci as well as on the extent of associated inflammatory responses or scarring. Neurologic manifestations are the most common (Fig. 235­1).
Seizures are associated with inflammation surrounding cysticerci in the brain parenchyma. These seizures may be generalized, focal, or Jacksonian.
Hydrocephalus results from CSF flow obstruction by cysticerci and accompanying inflammation or by CSF outflow obstruction from arachnoiditis.
Symptoms of increased intracranial pressure, including headache, nausea, vomiting, changes in vision, dizziness, ataxia, or confusion, are often
evident. Patients with hydrocephalus may develop papilledema or display altered mental status. When cysticerci develop at the base of the brain or in
the subarachnoid space, they may cause chronic meningitis or arachnoiditis, communicating hydrocephalus, hemorrhages, or strokes.
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FIGURE 235­1
Chapter 235: Cestode Infections, A. Clinton White, Jr.; Peter F. Weller Page 2 / 11
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Neurocysticercosis is caused by Taenia solium. Neurologic infection can be classified on the basis of the location and viability of the parasites.
Upper left: Parenchymal viable cysts (FLAIR MRI sequence). Upper center: Parenchymal viable cysts (postcontrast T1 MRI sequence). Upper right:
Seizures are associated with inflammation surrounding cysticerci in the brain parenchyma. These seizures may be generalized, focal, or Jacksonian.
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Hydrocephalus results from CSF flow obstruction by cysticerci and accompanying inflammation or by CSF outflow obstruction Medical University
from arachnoiditis.
Symptoms of increased intracranial pressure, including headache, nausea, vomiting, changes in vision, dizziness, ataxia, or confusion,
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evident. Patients with hydrocephalus may develop papilledema or display altered mental status. When cysticerci develop at the base of the brain or in
the subarachnoid space, they may cause chronic meningitis or arachnoiditis, communicating hydrocephalus, hemorrhages, or strokes.

FIGURE 235­1

Neurocysticercosis is caused by Taenia solium. Neurologic infection can be classified on the basis of the location and viability of the parasites.
Upper left: Parenchymal viable cysts (FLAIR MRI sequence). Upper center: Parenchymal viable cysts (postcontrast T1 MRI sequence). Upper right:
Single enhancing lesion (postcontrast T1 MRI sequence). Bottom left: Extensive basal subarachnoid neurocysticercosis in the anterior fossa (FLAIR
MRI sequence). Bottom center: Viable cyst in the fourth ventricle (FLAIR MRI sequence). Bottom right: Intraparenchymal brain calcifications
(noncontrasted CT scan). Lesions are marked with arrowheads. FLAIR, fluid­attenuated inversion recovery. (Modified with permission from A White, H
Garcia: Curr Opin Infect Dis 31:377, 2018. Lippincott Williams & Wilkins.)

Diagnosis

The diagnosis of intestinal T. solium infection is made by the detection of eggs or proglottids, as described for T. saginata. More sensitive methods,
including antigen­capture enzyme­linked immunosorbent assay (ELISA), polymerase chain reaction (PCR), and serology for tapeworm stage­specific
antigens, are currently available only as research techniques. In cysticercosis, diagnosis can be difficult. A panel of international experts recently
proposed revised diagnostic criteria (Table 235­1). Diagnostic certainty is possible only with definite demonstration of the parasite (absolute
criteria). This task can be accomplished by histologic observation of the parasite in excised tissue, by funduscopic visualization of the parasite in the
subretinal space of the eye, or by neuroimaging studies with definite evidence of a cystic lesion containing a characteristic scolex (Fig. 235­1). With
improving resolution of neuroimaging studies, the scolex can now be identified in a large proportion of cases. In other instances, a clinical diagnosis is
based on a combination of clinical presentation, radiographic studies, exposure history, and serodiagnosis.

TABLE 235­1

Revised Diagnostic Criteria for Neurocysticercosisa

1. Absolute criteria
a. Histologic demonstration of the parasite from biopsy of a brain or spinal cord lesion
b. Visualization of subretinal cysticercus
c. Conclusive demonstration of a scolex within a cystic lesion on neuroimaging studies
2. Neuroimaging criteria
a. Major neuroimaging criteria
Cystic lesions without a discernible scolex, typical small enhancing lesions, multilobulated cystic lesions in the subarachnoid space, typical
parenchymal brain calcifications
b. Confirmative neuroimaging criteria
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c. Minor neuroimaging criteria
Obstructive hydrocephalus or abnormal enhancement of basal leptomeninges
criteria). This task can be accomplished by histologic observation of the parasite in excised tissue, by funduscopic visualization of the parasite in the
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subretinal space of the eye, or by neuroimaging studies with definite evidence of a cystic lesion containing a characteristic scolex (Fig. 235­1). With
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improving resolution of neuroimaging studies, the scolex can now be identified in a large proportion of cases. In other instances, a clinical diagnosis is
based on a combination of clinical presentation, radiographic studies, exposure history, and serodiagnosis.

TABLE 235­1

Revised Diagnostic Criteria for Neurocysticercosisa

1. Absolute criteria
a. Histologic demonstration of the parasite from biopsy of a brain or spinal cord lesion
b. Visualization of subretinal cysticercus
c. Conclusive demonstration of a scolex within a cystic lesion on neuroimaging studies
2. Neuroimaging criteria
a. Major neuroimaging criteria
Cystic lesions without a discernible scolex, typical small enhancing lesions, multilobulated cystic lesions in the subarachnoid space, typical
parenchymal brain calcifications
b. Confirmative neuroimaging criteria
Resolution of cystic lesions spontaneously or after cysticidal drug therapy
Migration of ventricular cysts documented on sequential neuroimaging studies
c. Minor neuroimaging criteria
Obstructive hydrocephalus or abnormal enhancement of basal leptomeninges
3. Clinical/exposure criteria
a. Major clinical/exposure criteria
Detection of specific anticysticercal antibodies (e.g., by enzyme­linked immunoelectrotransfer blot [EITB]) or cysticercal antigens by well­
standardized immunodiagnostic tests
Cysticercosis outside the central nervous system
Evidence of a household contact with T . solium infection
b. Minor clinical/exposure criteria
Clinical manifestations suggestive of neurocysticercosis
Individuals coming from or living in an area where cysticercosis is endemic

aDiagnosis is confirmed by one absolute criterion, by two major criteria or one major and one confirmatory neuroimaging criteria plus any clinical/exposure

criterion, or by one major neuroimaging criterion plus two clinical/exposure criteria (including at least one major clinical/exposure criterion), together with the
exclusion of other pathologies producing similar neuroimaging findings. A probable diagnosis is supported by one major neuroimaging criterion plus any two
clinical/exposure criteria or by one minor neuroimaging criterion plus at least one major clinical/exposure criterion.

Source: Reproduced with permission from OH Del Brutto et al: Revised diagnostic criteria for neurocysticercosis. J Neurol Sci 372:202, 2017.

Neuroimaging findings constitute the primary major diagnostic criteria (Fig. 235­1). Major findings include cystic lesions with or without enhancement
(e.g., ring enhancement), one or more nodular calcifications (which may also have associated enhancement), focal enhancing lesions, or
multilobulated cystic lesions in the subarachnoid space. Cysticerci in the brain parenchyma are usually 5–20 mm in diameter and rounded. Cystic
lesions in the subarachnoid space or fissures may enlarge up to 6 cm in diameter and may be lobulated. For cysticerci within the subarachnoid space
or ventricles, the walls may be very thin and the cyst fluid is often isodense with CSF. Thus, obstructive hydrocephalus or enhancement of the basilar
meninges may be the only finding on CT in extraparenchymal neurocysticercosis. However, since these findings are less specific, they are considered
only minor criteria. Cysticerci in the ventricles or subarachnoid space are more readily identified by MRI, especially with three­dimensional views (e.g.,
fast imaging employing steady­state acquisition [FIESTA] or three­dimensional constructive interference in steady state [3D CISS]). CT is more sensitive
than MRI in identifying calcified lesions, whereas MRI is better for identifying cystic lesions, scolices, and enhancement. Spontaneous resolution,
resolution after therapy with albendazole, or mobile cystic lesions within the ventricles are findings that can support the diagnosis of
neurocysticercosis.

Prior exposure significantly modifies the interpretation of neuroimaging studies. Detection of specific antibodies to or antigens of T. solium are major
exposure criteria. Antibody tests using unfractionated antigens (e.g., ELISAs using crude parasite antigen) have high rates of false­positive and false­
negative results and should be avoided. An immunoblot assay using lentil lectin–purified glycoproteins is >99% specific and highly sensitive. However,
patients with single intracranial lesions or with calcifications may be seronegative. With this assay, serum samples provide greater diagnostic
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sensitivity
Chapter 235:than CSF. All Infections,
Cestode of the diagnostic antigens
A. Clinton have
White, Jr.;been
Petercloned, and assays using recombinant antigens are being developed. Antigen detection
F. Weller Page 4 / 11
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CSF may also facilitate diagnosis and patient follow­up. These assays are
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currently available commercially in Europe but not in the United States. More recently, real­time PCR has been employed for diagnosis and follow­up of
extraparenchymal disease.
neurocysticercosis.
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Prior exposure significantly modifies the interpretation of neuroimaging studies. Detection of specific antibodies to or antigens of T.
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exposure criteria. Antibody tests using unfractionated antigens (e.g., ELISAs using crude parasite antigen) have high rates of false­positive and false­
negative results and should be avoided. An immunoblot assay using lentil lectin–purified glycoproteins is >99% specific and highly sensitive. However,
patients with single intracranial lesions or with calcifications may be seronegative. With this assay, serum samples provide greater diagnostic
sensitivity than CSF. All of the diagnostic antigens have been cloned, and assays using recombinant antigens are being developed. Antigen detection
assays using monoclonal antibodies to detect parasite antigen in the blood or CSF may also facilitate diagnosis and patient follow­up. These assays are
currently available commercially in Europe but not in the United States. More recently, real­time PCR has been employed for diagnosis and follow­up of
extraparenchymal disease.

Other major clinical/exposure criteria for neurocysticercosis include the presence of cysticerci outside the central nervous system (CNS) (e.g., typical
cigar­shaped calcifications in muscle) or exposure to a tapeworm carrier or a household member infected with T. solium. Minor clinical/exposure
criteria include residence in an endemic area or clinical symptoms suggestive of neurocysticercosis (e.g., seizures or obstructive hydrocephalus).

Studies have demonstrated that clinical criteria may aid in diagnosis in selected cases. In patients from endemic areas who had single enhancing
lesions presenting with seizures, a normal physical examination, and no evidence of systemic disease (e.g., no fever, adenopathy, or chest
radiographic abnormalities), the constellation of rounded CT lesions 5–20 mm in diameter with no midline shift was almost always caused by
neurocysticercosis.

A definite or probable diagnosis is made in accordance with the criteria and combinations of criteria listed in the footnote of Table 235­1. Patients may
have CSF pleocytosis with a predominance of lymphocytes, neutrophils, or eosinophils. The protein level in CSF may be elevated; the glucose
concentration is usually normal but may be depressed.

Treatment of Taeniasis Solium and Cysticercosis

Intestinal T. solium infection is treated with a single dose of praziquantel (10 mg/kg). However, praziquantel occasionally evokes an inflammatory
response in the CNS if concomitant cryptic cysticercosis is present. Niclosamide (2 g) is also effective but is not widely available.

The initial management of neurocysticercosis should focus on symptom­based treatment of seizures or hydrocephalus. Seizures can usually be
controlled with antiepileptic treatment. If parenchymal lesions resolve without development of calcifications and patients remain free of seizures,
antiepileptic therapy can usually be discontinued after 2 years; less in patients with a single enhancing lesion. Placebo­controlled trials are clarifying
the clinical advantage of antiparasitic drugs for parenchymal neurocysticercosis. Faster resolution of neuroradiologic abnormalities has been
observed in most studies. The clinical benefits are less dramatic and consist mainly of shortening the period during which recurrent seizures occur and
decreasing the number of patients who have many recurrent seizures. For the treatment of patients with brain parenchymal cysticerci, most
authorities favor antiparasitic drugs, including albendazole (15 mg/kg per day for 8–28 days) and/or praziquantel (50–100 mg/kg daily in three divided
doses for 15–30 days). A combination of albendazole and praziquantel (50 mg/kg per day) is more effective in patients with more than two cystic
lesions. A longer course or combination therapy is needed in patients with multiple subarachnoid cysticerci. Both agents may exacerbate the
inflammatory response around the dying parasite, thereby exacerbating seizures or hydrocephalus as well. Thus, patients receiving these drugs
should be carefully monitored. High­dose glucocorticoids should be used during treatment. Because glucocorticoids induce first­pass metabolism of
praziquantel and may decrease its antiparasitic effect, cimetidine should be co­administered to inhibit praziquantel metabolism.

For patients with hydrocephalus, the emergent reduction of intracranial pressure is the mainstay of therapy. In the case of obstructive hydrocephalus,
the preferred approach is removal of the cysticercus via neurosurgery. This should be performed via neuroendoscopy when the cysticerci are in the
lateral or third ventricles. The fourth ventricular cysticerci can be approached by microdissection using an open craniotomy and a posterior approach
or, in some cases, via neuroendoscopy. However, removal of the cysticercus is not always possible. An alternative approach is initially to perform a
diverting procedure, such as ventriculoperitoneal shunting. Historically, shunts have usually failed, but failure rates may be lowered by administration
of antiparasitic drugs and glucocorticoids. For patients with subarachnoid cysts or giant cysticerci, anti­inflammatory medications such as
glucocorticoids are needed to reduce arachnoiditis and accompanying vasculitis. Most authorities recommend prolonged courses of antiparasitic
drugs as well as shunting when hydrocephalus is present. Methotrexate and, in some cases, tumor necrosis factor inhibitors can be used as steroid­
sparing agents in patients requiring prolonged therapy. In patients with diffuse cerebral edema and elevated intracranial pressure due to multiple
inflamed lesions, glucocorticoids are the mainstay of therapy, and antiparasitic drugs should be avoided. For ocular and spinal medullary lesions,
drug­induced inflammation may cause irreversible damage. Ocular disease should be managed surgically. Recent data suggest that either medical or
surgical therapy can be used for spinal disease.

Prevention

Measures for the

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prevention of human intestinal infections. Optimal eradication programs
in endemic areas include mass chemotherapy administered to human and porcine populations and vaccinations of pigs. A vaccine for porcine
infection is licensed in India and a few other countries.
inflamed lesions, glucocorticoids are the mainstay of therapy, and antiparasitic drugs should be avoided. For ocular and spinal medullary lesions,
drug­induced inflammation may cause irreversible damage. Ocular disease should be managed surgically. Recent data suggest that either
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Prevention

Measures for the prevention of intestinal T. solium infection consist of the application to pork of precautions similar to those described above for beef
with regard to T. saginata infection. The prevention of cysticercosis involves minimizing the opportunities for ingestion of fecally derived eggs by
means of good personal hygiene, effective fecal disposal, and treatment and prevention of human intestinal infections. Optimal eradication programs
in endemic areas include mass chemotherapy administered to human and porcine populations and vaccinations of pigs. A vaccine for porcine
infection is licensed in India and a few other countries.

ECHINOCOCCOSIS
Echinococcosis (also termed hydatidosis) is an infection caused in humans by the larval stage of Echinococcus granulosus sensu lato, E. multilocularis,
or E. vogeli. E. granulosus sensu lato parasites produce cystic hydatid disease, with unilocular cystic lesions. These infections are prevalent in most
areas where livestock is raised in association with dogs. Molecular evidence has demonstrated that E. granulosus strains belong to a range of
genotypes and several species. Currently, human cystic hydatid disease is caused by organisms formerly termed E. granulosus that are now classified
as E. granulosus sensu stricto (genotypes 1–3), E. canadensis (genotypes 6–8 and 10), and E. ortleppi (genotype 5). Other species—E. equinus
(genotype 4) and E. felidis (lion strain)—have not been identified in human infections. Some classify genotypes 6 and 7 as a separate species—E.
intermedius. E. granulosus sensu lato parasites are found on all continents, with areas of high prevalence in western China, central Asia, the Middle
East, the Mediterranean region, eastern Africa, and parts of South America. E. multilocularis, which causes multilocular alveolar lesions that are locally
invasive, is found in Alpine, sub­Arctic, or Arctic regions, including central and northern Europe; western China and central Asia; and isolated areas in
North America. E. vogeli and E. oligarthrus cause neotropical echinococcosis (formerly termed polycystic hydatid disease) and are found only in South
America.

Like other cestodes, echinococcal species have both intermediate and definitive hosts. The definitive hosts are canines that pass eggs in their feces.
After the ingestion of eggs, cysts develop in the intermediate hosts—sheep, cattle, humans, goats, camels, and horses for the E. granulosus complex
and mice and other rodents for E. multilocularis. When a dog (E. granulosus) or fox (E. multilocularis) ingests infected meat containing cysts, the life
cycle is completed. Humans are an incidental dead­end host and not part of the transmission life cycle.

Etiology

The small (5­mm­long) adult E. granulosus sensu lato worms live for 5–20 months in the jejunum of dogs. They have three proglottids: one immature,
one mature, and one gravid. The gravid segments are shed to release eggs that are morphologically similar to Taenia eggs and are extremely hardy.
After humans ingest the eggs, embryos escape from the eggs, penetrate the intestinal mucosa, enter the portal circulation, and are carried to various
organs, most commonly the liver and lungs. Larvae of E. granulosus sensu lato develop into fluid­filled unilocular hydatid cysts that consist of an
external membrane and an inner germinal layer. Daughter cysts develop from the inner aspect of the germinal layer, as do germinating cystic
structures called brood capsules. New larvae, called protoscolices, develop in large numbers within the brood capsule. The cysts expand slowly over a
period of years.

The life cycle of E. multilocularis is similar except that wild canines, such as foxes or wolves, serve as the main definitive hosts, and small rodents serve
as the intermediate hosts. The larval form of E. multilocularis, however, is quite different in that it remains in the proliferative phase, the parasite is
always multilocular, and vesicles without brood capsules or protoscolices progressively invade the host tissue by peripheral extension of processes
from the germinal layer.

Clinical Manifestations

Slowly enlarging echinococcal cysts generally remain asymptomatic until their expanding size or their space­occupying effect in an involved organ
elicits symptoms. The liver and the lungs are the most common sites of these cysts. The liver is involved in about two­thirds of E. granulosus infections
and in nearly all E. multilocularis infections. Because a period of years elapses before cysts enlarge sufficiently to cause symptoms, they may be
discovered incidentally on a routine x­ray or ultrasound study.

Patients with hepatic echinococcosis who are symptomatic most often present with abdominal pain or a palpable mass in the right upper quadrant.
Compression of a bile duct or leakage of cyst fluid into the biliary tree may mimic recurrent cholelithiasis, and biliary obstruction can result in jaundice.
Rupture of or episodic leakage from a hydatid cyst may produce fever, pruritus, urticaria, eosinophilia, or anaphylaxis. Pulmonary hydatid cysts may
rupture into the bronchial tree or pleural cavity and produce cough, salty phlegm, dyspnea, chest pain, or hemoptysis. Rupture of hydatid cysts, which
can occur spontaneously or at surgery, may lead to multifocal dissemination of protoscolices, which can form additional cysts. Other presentations are
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The larval forms of E. multilocularis characteristically present as a slowly growing hepatic tumor, with progressive destruction of the liver and
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Rupture of or episodic leakage from a hydatid cyst may produce fever, pruritus, urticaria, eosinophilia, or anaphylaxis. Pulmonary hydatid cysts may
rupture into the bronchial tree or pleural cavity and produce cough, salty phlegm, dyspnea, chest pain, or hemoptysis. Rupture of hydatid cysts, which
can occur spontaneously or at surgery, may lead to multifocal dissemination of protoscolices, which can form additional cysts. Other presentations are
due to the involvement of bone (invasion of the medullary cavity with slow bone erosion producing pathologic fractures), the CNS (space­occupying
lesions), the heart (conduction defects, pericarditis), and the pelvis (pelvic mass).

The larval forms of E. multilocularis characteristically present as a slowly growing hepatic tumor, with progressive destruction of the liver and
extension into vital structures. Clinical symptoms develop decades after initial infection. Patients commonly report upper­quadrant and epigastric
pain. Liver enlargement and obstructive jaundice may be apparent. The lesions may infiltrate adjoining organs (e.g., diaphragm, kidneys, or lungs) or
may metastasize to the spleen, lungs, or brain.

Diagnosis

Radiographic and related imaging studies are important in detecting and evaluating echinococcal cysts. Plain x­rays will define pulmonary cysts of E.
granulosus—usually as rounded masses of uniform density—but may miss cysts in other organs unless there is cyst wall calcification (as occurs in the
liver). MRI, CT, and ultrasound reveal well­defined cysts with thick or thin walls. Imaging methods may reveal a fluid layer of different density, termed
hydatid sand, that contains protoscolices. However, the most pathognomonic finding, if demonstrable, is that of daughter cysts within the larger cyst.
This finding, like eggshell or mural calcification on CT, is indicative of E. granulosus infection and helps to distinguish the cyst from carcinomas,
bacterial or amebic liver abscesses, or hemangiomas. In contrast, ultrasound or CT of alveolar hydatid cysts reveals indistinct solid masses with central
necrosis and plaquelike calcifications.

A specific diagnosis of cystic hydatid disease can be made by the examination of aspirated fluids for protoscolices or hooklets, but diagnostic
aspiration is not usually recommended because of the potential risk of fluid leakage resulting in either dissemination of infection or anaphylactic
reactions. Serodiagnostic assays can be useful, although a negative test does not exclude the diagnosis of echinococcosis. Cysts in the liver elicit
positive antibody responses in ~90% of cases, whereas up to 50% of individuals with cysts in the lungs are seronegative. Detection of antibody to
specific echinococcal antigens by immunoblotting has the highest degree of specificity.

Treatment of Echinococcosis

Therapy for cystic echinococcosis is based on considerations of the size, location, and manifestations of cysts and the overall health of the patient.
Surgery has traditionally been the principal definitive method of treatment. Currently, ultrasound staging is recommended for cystic echinococcosis
(Fig. 235­2). Small CL, CE1, and CE3 lesions may respond to chemotherapy with albendazole. For CE1 lesions and uncomplicated CE3 lesions, PAIR
(percutaneous aspiration, infusion of scolicidal agents, and reaspiration) is now recommended instead of surgery. PAIR is contraindicated for
superficially located cysts (because of the risk of rupture) and for cysts communicating with the biliary tree. For prophylaxis of secondary peritoneal
echinococcosis due to inadvertent spillage of fluid during PAIR, the administration of albendazole (15 mg/kg daily in two divided doses) should be
initiated at least 2 days before the procedure and continued for at least 4 weeks afterward. Ultrasound­ or CT­guided aspiration allows confirmation of
the diagnosis by demonstration of protoscolices or hooks in the aspirate. After aspiration, contrast material should be injected to detect occult
communications with the biliary tract. Alternatively, the fluid should be checked for bile staining visually and by dipstick. If no bile is found and no
communication is visualized, the contrast material is reaspirated, with subsequent infusion of scolicidal agents (usually 95% ethanol; alternatively,
hypertonic saline). This approach, when implemented by a skilled practitioner, yields rates of cure and relapse equivalent to those following surgery,
with less perioperative morbidity and shorter hospitalization. In experienced hands, some CE2 lesions can be treated by modified catheter drainage.
Daughter cysts within the primary cyst may need to be punctured separately.

FIGURE 235­2

Management of cystic hydatid disease caused by Echinococcus granulosus should be based on viability of the parasite, which can be
estimated from radiographic appearance. Staging is done by imaging studies including ultrasound, CT, or MRI and includes lesions classified as active,
transitional, and inactive. Active cysts include types CL (with a cystic lesion and no visible cyst wall), CE1 (with a visible cyst wall and internal echoes
[snowflake sign]), and CE2 (with a visible cyst wall and internal septation). Transitional cysts may have detached laminar membranes (CE3a) or may be
partially collapsed (CE3b). Inactive cysts include types CE4 (a nonhomogeneous mass) and CE5 (a cyst with a thick calcified wall).

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estimated from radiographic appearance. Staging is done by imaging studies including ultrasound, CT, or MRI and includes lesions classified as active,
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transitional, and inactive. Active cysts include types CL (with a cystic lesion and no visible cyst wall), CE1 (with a visible cyst wall and internal echoes
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[snowflake sign]), and CE2 (with a visible cyst wall and internal septation). Transitional cysts may have detached laminar membranes (CE3a) or may be
partially collapsed (CE3b). Inactive cysts include types CE4 (a nonhomogeneous mass) and CE5 (a cyst with a thick calcified wall).

Surgery remains the treatment of choice for complicated cystic echinococcosis (e.g., cysts communicating with the biliary tract), for most thoracic and
intracranial cysts, and for areas where PAIR is not possible. For liver cysts, the preferred surgical approach is total cystectomy, in which the entire cyst
and the surrounding fibrous tissue are removed. Recent studies demonstrate that many cysts can be safely removed by laparoscopic or robotic
surgery. The risks posed by leakage of fluid during surgery or PAIR include anaphylaxis and dissemination of infectious protoscolices. The latter
complication has been minimized by careful attention to the prevention of spillage of the cyst. Infusion of scolicidal agents is no longer recommended
because of problems with hypernatremia, intoxication, or sclerosing cholangitis. Albendazole, which is active against Echinococcus, should be
administered adjunctively, beginning several days before resection of the liver and continuing for several weeks for E. granulosus. Praziquantel (50
mg/kg daily for 2 weeks or weekly throughout the duration of albendazole) may hasten the death of the protoscolices. Medical therapy with
albendazole alone for 12 weeks to 6 months results in cure in ~30% of cases and in improvement in another 50%. In many instances of treatment
failure, E. granulosus infections are subsequently treated successfully with PAIR or additional courses of medical therapy. Response to treatment is
best assessed by serial imaging studies, with attention to cyst size and consistency. Some cysts may not demonstrate complete radiologic resolution
even though no viable protoscolices are present. Some of these cysts with partial radiologic resolution (e.g., CE4 or CE5) can be managed with
observation only.

Surgical resection remains the treatment of choice for E. multilocularis infection. Complete removal of the parasite continues to offer the best chance
for cure. Ongoing therapy with albendazole for at least 2 years after presumptively curative surgery is recommended. Positron emission tomography
can be used to follow disease activity. Most cases are diagnosed at a stage at which complete resection is not possible; in these cases, albendazole
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with careful monitoring. In some cases, liver transplantation has been used because of the size of the
Chapter
necessary liver resection. However,A.continuous
235: Cestode Infections, Clinton White, Jr.; Peter F. Weller
immunosuppression Page 8 / 11
favors the proliferation of E. multilocularis larvae and reinfection of the
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transplant. Thus, indefinite treatment with albendazole is required.

Prevention
even though no viable protoscolices are present. Some of these cysts with partial radiologic resolution (e.g., CE4 or CE5) can be managed with
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observation only.
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Surgical resection remains the treatment of choice for E. multilocularis infection. Complete removal of the parasite continues to offer the best chance
for cure. Ongoing therapy with albendazole for at least 2 years after presumptively curative surgery is recommended. Positron emission tomography
can be used to follow disease activity. Most cases are diagnosed at a stage at which complete resection is not possible; in these cases, albendazole
treatment should be continued indefinitely, with careful monitoring. In some cases, liver transplantation has been used because of the size of the
necessary liver resection. However, continuous immunosuppression favors the proliferation of E. multilocularis larvae and reinfection of the
transplant. Thus, indefinite treatment with albendazole is required.

Prevention

In endemic areas, echinococcosis can be prevented by administering praziquantel to infected dogs, by denying dogs access to viscera from infected
animals, or by vaccinating sheep. Limiting the number of stray dogs is helpful in reducing the prevalence of infection among humans. In Europe, E.
multilocularis infection has been associated with gardening; gloves should be used when working with soil. Praziquantel­impregnated bait has also
been used to treat tapeworms in wild canines.

HYMENOLEPIASIS NANA
Infection with H. nana, the dwarf tapeworm, is the most common of all the cestode infections. H. nana is endemic in both temperate and tropical
regions of the world. Infection is spread by fecal/oral contamination.

Etiology and Pathogenesis

H. nana is the only cestode of humans that does not require an intermediate host. Both the larval and adult phases of the life cycle take place in the
same person. The adult—the smallest tapeworm parasitizing humans—is ~2 cm long and dwells in the proximal ileum. Proglottids, which are small and
rarely seen in the stool, release spherical eggs 30–44 μm in diameter, each of which contains an oncosphere with six hooklets. The eggs are
immediately infective and are unable to survive for >10 days in the external environment. When the egg is ingested by a new host, the oncosphere is
freed and penetrates the intestinal villi, becoming a cysticercoid larva. Larvae migrate back into the intestinal lumen, attach to the mucosa, and mature
into adult worms over 10–12 days. Eggs may also hatch before passing into the stool, causing internal autoinfection with increasing numbers of
intestinal worms. Although the life span of adult H. nana worms is only ~4–10 weeks, the autoinfection cycle perpetuates the infection.

Clinical Manifestations

H. nana infection, even with many intestinal worms, is usually asymptomatic. Heavy infection may be associated with diarrhea, abdominal pain, and
weight loss.

Diagnosis

Infection is diagnosed by the finding of eggs in the stool.

Treatment of Hymenolepiasis Nana

Praziquantel (25 mg/kg once) is the treatment of choice because it acts against both the adult worms and the cysticercoids in the intestinal villi.
Nitazoxanide (500 mg bid for 3 days) may be used as an alternative.

Prevention

Good personal hygiene and improved sanitation can eradicate the disease. Epidemics have been controlled by mass chemotherapy coupled with
improved hygiene.

HYMENOLEPIASIS DIMINUTA
Hymenolepis diminuta, a cestode of rodents, occasionally infects small children, who ingest the larvae in uncooked cereal foods contaminated by fleas
and other insects in which larvae develop. Infection is usually asymptomatic and is diagnosed by the detection of eggs in the stool. Treatment with
praziquantel results in cure in most cases.

DIPHYLLOBOTHRIASIS
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Dibothriocephalus nihonkaiensis) are found in the lakes, rivers, and deltas of the Northern Hemisphere, central Africa, and South America.
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Hymenolepis diminuta, a cestode of rodents, occasionally infects small children, who ingest the larvae in uncooked cereal foods contaminated by fleas
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and other insects in which larvae develop. Infection is usually asymptomatic and is diagnosed by the detection of eggs in the stool. Treatment with
praziquantel results in cure in most cases.

DIPHYLLOBOTHRIASIS
Dibothriocephalus latus (formerly Diphyllobothrium latum) and other diphyllobothriid species (including Adenocephlus pacificus and
Dibothriocephalus nihonkaiensis) are found in the lakes, rivers, and deltas of the Northern Hemisphere, central Africa, and South America.

Etiology and Pathogenesis

The adult worm—the longest tapeworm (up to 25 m)—attaches to the ileal and occasionally to the jejunal mucosa by its suckers, which are located on
its elongated scolex. The adult worm has 3000–4000 proglottids, which release ~1 million eggs daily into the feces. If an egg reaches water, it hatches
and releases a free­swimming embryo that can be eaten by small freshwater crustaceans (Cyclops or Diaptomus species). After an infected crustacean
containing a developed procercoid is swallowed by a fish, the larva migrates into the fish’s flesh and grows into a sparganum, or plerocercoid larva.
Humans acquire the infection by ingesting infected raw or smoked fish. Within 3–5 weeks, the tapeworm matures into an adult in the human intestine.

Clinical Manifestations

Most Diphyllobothrium infections are asymptomatic, although manifestations may include transient abdominal discomfort, diarrhea, vomiting,
weakness, and weight loss. Occasionally, infection can cause acute abdominal pain and intestinal obstruction; in rare cases, cholangitis or cholecystitis
may be produced by migrating proglottids.

Because the D. latum tapeworm absorbs large quantities of vitamin B12 and interferes with ileal B12 absorption, vitamin B12 deficiency can develop that
uncommonly causes a megaloblastic anemia resembling pernicious anemia and may result in neurologic sequelae.

Diagnosis

The diagnosis is made readily by the detection of the characteristic eggs in the stool. The eggs possess a single shell with an operculum at one end and
a knob at the other. Mild to moderate eosinophilia may be detected.

Treatment of Diphyllobothriasis

Praziquantel (5–10 mg/kg once) is highly effective. Parenteral vitamin B12 should be given if B12 deficiency is manifest.

Prevention

Infection can be prevented by heating fish to 54°C for 5 min or by freezing it at –18°C for 24 h. Placing fish in brine with a high salt concentration for long
periods kills the eggs.

DIPYLIDIASIS
Dipylidium caninum, a common tapeworm of dogs and cats, may accidentally infect humans. Dogs, cats, and occasionally humans become infected by
ingesting fleas harboring cysticercoids. Children are more likely to become infected than adults. Most infections are asymptomatic, but passage of
segments in the stool or vague abdominal symptoms may occur. The diagnosis is made by the detection of proglottids or ova in the stool. As in D. latus
infection, therapy consists of praziquantel. Prevention requires anthelminthic treatment and flea control for pet dogs or cats.

SPARGANOSIS
Humans can be infected by the sparganum, or plerocercoid larva, of a diphyllobothriid tapeworm of the genus Spirometra. Infection can be acquired
by the consumption of water containing infected Cyclops; by the ingestion of infected snakes, birds, or mammals; or by the application of infected flesh
as poultices. The worm migrates slowly in tissues, and infection commonly presents as a subcutaneous swelling. Periorbital tissues can be involved,
and ocular sparganosis may destroy the eye. Surgical excision is used to treat localized sparganosis.

COENUROSIS
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This rare infection of humans by the larval stage (coenurus) of the dog tapeworm Taenia multiceps or T. serialis results in a space­occupying cystic
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lesion.
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excision of the lesion. Chemotherapeutic agents generally are not effective.
by the consumption of water containing infected Cyclops; by the ingestion of infected snakes, birds, or mammals; or by the application of infected flesh
as poultices. The worm migrates slowly in tissues, and infection commonly presents as a subcutaneous swelling. Periorbital tissues can
International be involved,
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COENUROSIS
This rare infection of humans by the larval stage (coenurus) of the dog tapeworm Taenia multiceps or T. serialis results in a space­occupying cystic
lesion. As in cysticercosis, involvement of the CNS and subcutaneous tissue is most common. Both definitive diagnosis and treatment require surgical
excision of the lesion. Chemotherapeutic agents generally are not effective.

FURTHER READING

Brunetti E et al: Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop 114:1, 2010. [PubMed:
19931502]

Del Brutto OH et al: Revised diagnostic criteria for neurocysticercosis. J Neurol Sci 372:202, 2017. [PubMed: 28017213]

Kern P et al: The echinococcoses: Diagnosis, clinical management and burden of disease. Adv Parasitol 96:259, 2017. [PubMed: 28212790]

Nash TE et al: Natural history of treated subarachnoid neurocysticercosis. Am J Trop Med Hyg 102:78, 2020. [PubMed: 31642423]

Scholz T et al: Update on the human broad tapeworm (genus Diphyllobothrium ), including clinical relevance. Clin Microbiol Rev 22:146, 2009.
[PubMed: 19136438]

Wen H et al: Echinococcosis: Advances in the 21st century. Clin Microbiol Rev 32:e00075, 2019. [PubMed: 30760475]

White AC Jr et al: Diagnosis and treatment of neurocysticercosis: 2017 clinical practice guidelines by the Infectious Diseases Society of America (IDSA)
and the American Society of Tropical Medicine and Hygiene (ASTMH). Clin Infect Dis 66:1159, 2018. [PubMed: 29617787]

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