Hydatid cysts are most commonly found in the liver and lungs, although they may also occur in other organs, bones and muscles. The cysts can increase in size to 5 – 10 cm or more and may survive for decades. Non-specific signs include loss of appetite, weight loss and weakness
Echinococcus granulosus sensu lato occurs practically worldwide, and more frequently in rural, grazing areas where dogs ingest organs from
diagnosis
epidemiology
managment
3. Case presentation
A 32-years-old woman without any history of digestive or hepatic disease.
The patient had been well until few hours before of her admission.
She was admitted to hospital complaining of right upper quadrant and
epigastrium abdominal pain, and nausea.
On routine physical examination an abdominal mass was discovered on
the right upper quadrant, and there was tenderness in the upper abdomen
Hematologic and blood chemical tests were performed:
Her haematocrit was 36.4%, with a MCV of 86.9 μm3 and a platelet count
of 162/mm3.
White-cell count of 7.0/mm3, neutrophils 79.8%, lymphocytes 7.1%,
monocytes 6.4%,
Liver tests were normal.
1
4. Case presentation
MRI of the abdomen with contrast medium revealed a low-density cystic mass
measuring 11x11x10 cm in the right lobe, with a thick, irregular wall (Figure 1
)
A cystectomy was performed by laproscopy, with the use of specific
precautions to prevent local spread of disease.
Aspiration of the contents of the cyst yielded clear, colorless fluid.
Hydatid sand containing a protoscolex of Echinococcus granulosus was seen o
n microscopical examination (unstained wet preparation, x40; Figure 2).
Serological test to Echinococcus granulosus antibodies IgG were positive.
Specific anti-parasitic treatment was given and after two months the patient is
asymptomatic.
2
Figure 1 Figure 2
6. Background
Hydatid disease is a parasitic infestation by a tapeworm
of the genus Echinococcus.
Zoonosis
Humans are accidental intermediate hosts, whereas animals
can be both intermediate hosts and definitive hosts.
In humans, 50–75% of the cysts occur in the liver,
25% are located in the lungs, and
5–10% distribute along the arterial system
4
7. pathophysiology
There are four known species of Echinococcus
Three of them are medical importance in humans:
1. E granulosus Cystic echinococcosis (CE)
2. E multilocularis Alveolar echinococcosis (AE)
3. E vogeli Polycystic Echinococcosis
E granulosus is the most common of the three
E multilocularis is rare but is the most virulent, and
E vogeli is the most rare.
5
8. Etiology
The life cycle has three developmental stages:
1. The adult tapeworm in the definitive host
2. eggs in the environment
3. metacestode in the intermediate host
Metacestodes are ingested by the definitive host.
The metacestodes mature into the tapeworm in the definitive host and, in turn,
release eggs into the environment.
The intermediate host ingests the eggs, which hatch into metacestodes, which
infest the liver, lungs, muscles, and other organs of the intermediate host.
6
9. Life cycle of
Echinococcus granulosus:
Structure of
hydatid cyst:
A) pericyst membrane (infla
mmatory host reaction)
B) Laminated layer (parasite origin)
C) Germinal layer
D) and E). Daughter cyst.
7
10. Epidemiology
No sexual predilection is recognized for hydatid cysts.
The parasite has the capability of infecting persons of all races equally
The cysts grow slowly, and a cyst is rarely diagnosed during childhood or adol
escence unless the brain is affected.
CE is a disease of younger adults, with an average age at diagnosis of 30-40
years
Alveolar echinococcosis (AE) is a disease of older adults
Echinococcosis is also unusual in northern Europe.
8
11. Epidemiology
The highly endemic areas are marked in red and include countries in the Mediterranean basin,
the Middle East, central Asia, sub-Saharan Africa, and South America.
Note that this zoonosis is present worldwide, with only a few countries free of endemic case
s.
9
13. History
The parasite load, the site, and the size of the cysts determi
ne the degree of symptoms.
Many hydatid cysts remain asymptomatic
history of living in or visiting an endemic area
symptoms can be produced by a mass effect or cyst complications
Liver: symptoms of obstructive jaundice and abdominal pain.
With biliary rupture, the classic triad of biliary colic, jaundice, and urticaria is
observed.
Lungs: chronic cough, dyspnea, pleuritic chest pain, and hemoptysis.
Brain: Headache, dizziness, and a decreased level of consciousness may sig
nify cerebral involvement.
Specific neurologic deficits may occur depending on the location of the cyst in
the brain
11
14. History
Secondary complications: due to infection or leakage of the
cyst
Minor leaks lead to increased pain and a mild allergic reaction characterized b
y flushing and urticaria.
Major rupture leads to a full-blown anaphylactic reaction
A rupture into the biliary tree can lead to obstruction by the daughter cysts,
producing cholangitis.
Rupture into the bronchi can lead to expectoration of cyst fluid.
Infection: primary or secondary mild fever to full-blown sepsi
s.
Extremity pain with or without neurologic deficit is a sign of either bone or mus
cle involvement.
12
15. Physical examination
Examination findings are nonspecific
Skin: Jaundice due to biliary obstruction, Spider angiomas due to liver cirrhosi
s, Urticaria and erythema may be seen.
Vital signs: fever due to primary or secondary infection or an allergic reaction
Hypotension is observed with anaphylaxis secondary to a cyst leak.
Lungs: decreased breath sounds ,signs of airway obstruction ,consolidation o
f the affected segment, lobule, lobe, or the whole lung.
Abdomen: abdominal tenderness most common, Hepatomegaly or a mass m
aybe felt.
Tender hepatomegaly is a sign of secondary infection of the cyst,
Ascites is rare,
Splenomegaly either due to splenic echinococcosis or PHT.
13
16. Physical examination
Extremities:
Bone involvement tenderness and, rarely, a palpable mass
.
Muscle involvement usually a palpable mass.
Peripheral nerve compression can occur, although extremely rarely.
It results in nerve-specific sensory and/or motor deficit.
Brain: nonspecific findings on neurological examination
they range from very mild to full coma and cerebral herniation.
Eyes: rare, abnormal findings include:
decreased visual acuity, blindness, and exophthalmos.
14
18. Laboratory studies
Routine blood studies are non specific.
Eosinophilia in 25% of all infected cases
Hypogammaglobinemia in 30% of all infected cases
Bilirubin and Alkaline phosphatase elevation in liver involvement .
Indirect hemagglutination test and ELISA : for detection of Anti-Echinococcus I
gG antibodies, are initial screening test of choice
Immunodiffusion and immunoelectophoresis demonstrate antibodies to antige
n 5 and provide specific confirmation of reactivity .
ELISA is useful in follow up to detect recurrence .
Casoni test : intradermal skin test , is now largely abandoned because of its lo
w sensitivity and potential for severe allergic reaction .
16
19. Imaging studies
1-Plain x-ray film
Nonspecific and non revealing
Thin rim of calcification delineating a cyst is suggestive of hydatid cyst .
Elevated diaphragm and concentric calcifications in the cyst wall on CHEST X RAYS
17
21. Imaging studies
2-Ultrasonography
Specificity- approx 90%
Internal structure,number,and location of the cysts
Diagnostic when demonstrate daughter cyst and hydatid sand
Accuracy remain operator-dependent
Usual findings :
1. Solitary cyst
2. Water lily sign
3. Daughter cysts
Gharbi US classification
of cystic echinococcosis
Water lily sign
19
22. Imaging studies
3-CT scan
Has accuracy of 98%
It is the best test for differentiation
of hydatid cyst from amebic and pyogenic cyst in liver
specific information about the location
depth of the cyst within the liver
structural details of the hydatid cyst
It shows cyst adequately but not superior to CT scan .
4-MRI
Calcified hydatid cyst
MRI of Hepatic hydatid cyst
20
23. procedures
ERCP
Is both diagnostic and therapeutic in cases of intrabiliary rapt
ure of hydatid cyst in whom sphincterotomy can be preforme
d
• ERCP showing biliary
communication with
cyst and hydatid material in
common bile duct.
21
25. Treatment
1-Medical treatment
Two benzimidazoles
1-Albendazole: ( 10-15 mg/kg/d , 14 days intervals , for 3-6 months )
2-mebendazole: ( 40-50mg/kg/d , 3-6 months )
Praziquentel: ( 400mg/kg/week )
• Is isoquinoloine derivative It has poor effect on germinal layer so it is of
choice for prophylaxis in pre and post operative period in order to prevent
secondary implantation of spilled protoscoleces.
Indications:
1-primary liver or lung cysts that are inoperable
2-cysts in 2 or more organs
3-peritoneal cysts
23
26. Treatment
Contraindications for medical Rx:
1-Early pregnancy
2-chronic hepatic disease
3-large cyst with risk of rapture
4-inactive or calcified cyst ( relative contraindication in cases of bone cyst
because it has very low response )
5-bone marrow suppression
Monitoring :
CBC and liver enzymes adverse effects of drugs , every 2 weeks
for 3 months then every 4 weeks .
Imaging studies follow-up on the morphologic status of the cy
st.
24
27. Treatment
2-PAIR(puncture,aspiration,injection,reaspiration):
Either under US or CT scan guidance
Perioperative treatment with a benzimidazole is mandatory
(4 d prior to the procedure and for 1-3 mo after).
Steps
1-puncture with parasitological examination if available
2-aspiration of cystic fluid ( take 10-15 cc and examine for bilirubin,
if present stop procedure and if not present aspirate whole fluid)
3-injection of 95% ethanol solution or hypertonic saline ( 1/3 of aspirated fluid )
4-reaspiration of protoscolicide solution after 15 minutes)
This is repeated until the return is clear.
The cyst is then filled with isotonic sodium chloride solution.
25
28. Treatment
PAIR
FREQUENTLY USED P
ROTOSCOLICIDAL AG
ENTS:
15–20% Saline
95% Ethanol
A combination Of 30%
saline and 95% Ethanol
Mebendazole Solution
26
29. Treatment
The PAIR technique can be performed on liver, bone, and kidney
The cysts should be larger than 5 cm in diameter
Type I or II according to the Gharbi ultrasound classification of liver cysts
Performed on type III cysts as long as it is not a honeycomb cyst.
Indications:
1-inoperable patient
2-patient refusing surgery
3-relapse after surgery or chemotherapy
PAIR
27
30. Treatment
PAIR
Contraindications :
1-lung and brain cyst
2-inaccessible cyst
3-superficially located cyst
4-cyst communicating with biliary tre
5-types 3,4,5 cysts
6-early pregnancy
Complications:
1-spillage and anaphylaxis
2- Sclerosing cholangitis
3-biliary fistulas
28
31. treatment
3-surgery:
Indications:
1-Large cyst with multiple daughter cyst
2-superficially located single liver cyst
3-liver cyst with biliary communication
4-cyst in lung , brain , kidney , bone , eyes or any other organs .
5-infected cyst
Contraindications:
1-general contraindications to surgery ( extreme age , severe preexisting medica
condition )
2-multiple cyst in multiple organs
3-dead , very small cyst
4-Cyst difficult to access
29
32. Treatment
Preparation for surgery:
Give 4-6 week of albendazole tablet before surgery (800mg/day in
divided doses) in adult
Pre operative visualization of biliary tract by ERCP.
Anaesthesist warned of sudden anaphylactic shock in case of spillage.
Laparoscopic pericystectomy + aumentopexy
Open surgery paricystectomy + aumentopexy
30
33. prevention
Because human infection with Echinococcus results from fecal-
oral contamination, prevention requires the following step
s:
•Education on proper hygiene
•Regular treatment of infected dogs to reduce worm load
•Prevention of dogs from eating viscera organs of intermediate
hosts (shep….etc)
•Avoidance of unnecessary contact with infected dogs
•Regulation of livestock butchering
31
For men, 38.3 to 48.6 percent. For women, 35.5 to 44.9 percent
80–100 fl
Life cycle of Echinococcus granulosus. The adult Echinococcus granulosus (3 to 6 mm long) resides in the small bowel of the definitive hosts, dogs or other canids. Gravid proglottids release eggs that are passed in the feces. After ingestion by a suitable intermediate host (under natural conditions: sheep, goat, swine, cattle, horses, camel), the egg hatches in the small bowel and releases an oncosphere that penetrates the intestinal wall and migrates through the circulatory system into various organs, especially the liver and lungs. In these organs, the oncosphere develops into a cyst that enlarges gradually, producing protoscolices and daughter cysts that fill the cyst interior. The definitive host becomes infected by ingesting the cyst-containing organs of the infected intermediate host. After ingestion, the protoscolices evaginate, attach to the intestinal mucosa, and develop into adult stages in 32 to 80 days. The same life cycle occurs with E. multilocularis (1.2 to 3.7 mm), with the following differences: the definitive hosts are foxes, and to a lesser extent dogs, cats, coyotes, and wolves; the intermediate host are small rodents; and larval growth (in the liver) remains in the proliferative stage indefinitely, resulting in invasion of the surrounding tissues. With E. vogeli (up to 5.6 mm long), the definitive hosts are bush dogs and dogs; the intermediate hosts are rodents; and the larval stage (in the liver, lungs, and other organs) develops both externally and internally, resulting in multiple vesicles. E. oligarthrus (up to 2.9 mm long) has a life cycle that involves wild felids as definitive hosts and rodents as intermediate hosts. Humans become infected by ingesting eggs, with resulting release of oncospheres in the intestine and the development of cysts in various organs. (Redrawn from data from the Centers for Disease Control and Prevention, http://www.dpd.cdc.gov/dpdx/html/Echinococcosis.htm.)
Many hydatid cysts remain asymptomatic, even into advanced age.
The parasite load, the site, and the size of the cysts determine the degree of symptoms.
A history of living in or visiting an endemic area must be established.
In cystic echinococcosis (CE), symptoms can be produced by a mass effect or cyst complications. Symptoms due to the pressure effect of the cyst usually take a long time to manifest, except when they occur in the brain or the eyes.
In the liver, the pressure effect of the cyst can produce symptoms of obstructive jaundice and abdominal pain. With biliary rupture, the classic triad of biliary colic, jaundice, and urticaria is observed. Passage of hydatid membranes in the emesis (hydatid emesia) and passage of membranes in the stools (hydatid enterica) may occur rarely.
Involvement of the lungs produces chronic cough, dyspnea, pleuritic chest pain, and hemoptysis. Expectoration of cyst membranes and fluid is observed with intrabronchial rupture.
Headache, dizziness, and a decreased level of consciousness may signify cerebral involvement. Specific neurologic deficits may occur depending on the location of the cyst in the brain.
Minor leaks lead to increased pain and a mild allergic reaction characterized by flushing and urticaria. Major rupture leads to a full-blown anaphylactic reaction, which is fatal if not treated promptly. A rupture into the biliary tree can lead to obstruction by the daughter cysts, producing cholangitis. Rupture into the bronchi can lead to expectoration of cyst fluid.
Infection of the cyst can occur either as a primary infection or as a secondary infection following an episode of a leak into the biliary tree, a cystobiliary fistula. Symptoms range from mild fever to full-blown sepsis.
Extremity pain with or without neurologic deficit is a sign of either bone or muscle involvement.
In alveolar echinococcosis (AE), the liver is the primary site of infection, and it closely mimics cirrhosis or carcinoma. Symptomatology is that of progressive liver dysfunction that ultimately leads to liver failure. The progression can occur over weeks, months, or years. Distant metastasis is possible, and involvement of other organs (eg, lung, brain, bone) can occur in as many as 13% of the patients.
Physical examination findings in patients with echinococcosis are nonspecific. The findings are related to the effect of the cyst on the anatomy or the function of the affected organ(s) and to an acute allergic reaction
Skin
Jaundice could be a sign of biliary obstruction. Spider angiomas are a sign of cirrhosis of the liver. Urticaria and erythema may be seen.
Vital signs
Fever could be a sign of primary or secondary infection or an allergic reaction. Hypotension is observed with anaphylaxis secondary to a cyst leak.
Lungs
Decreased breath sounds over the affected area are signs of airway obstruction with consolidation of the affected segment, lobule, lobe, or the whole lung.
Abdomen
The most common sign is abdominal tenderness. Hepatomegaly may be present or a mass may be felt. Tender hepatomegaly is a sign of secondary infection of the cyst, especially when coupled with fever and chills. Ascites is rare.
Splenomegaly can be the result of either splenic echinococcosis or portal hypertension.
Extremities
Bone involvement can result in tenderness over the affected area and, rarely, a palpable mass. Muscle involvement is usually characterized by a palpable mass.
Peripheral nerve compression can occur, although extremely rarely. It results in nerve-specific sensory and/or motor deficit.
Brain
Findings from the neurologic examination are nonspecific and depend on the area of the brain involved. They range from very mild to full coma and cerebral herniation.
Eyes
Ocular involvement is rare. Abnormal findings from the ophthalmologic examination include decreased visual acuity, blindness, and exophthalmos.
Pulmonary HD in a 45-year-old woman who was coughing and had abundant watery sputum 2 weeks previously. At the time of her imaging examination, she also had a high-grade fever. (a, b) Posteroanterior (a) and lateral (b) chest radiographs show a large cystic lesion with thin walls (arrows) in the middle region of the right lung and associated airspace disease (A), which is consistent with acute bacterial pneumonia. The wavy soft-tissue opacity (arrowheads) occupying the base of the cyst is consistent with detached endocystic membranes, or the water lily sign.
Usual findings : 1. Solitary cyst – features suggestive include dependant debris (hydatid sand) moving freely with change in position; presence of wall calcification 2. Water lily sign – separation of membranes due to collapse of germinal layer 3. Daughter cysts – most charecteristic sign with cyst in a cyst – cart wheel / honeycomb cyst
Procedures Endoscopic retrograde cholangiopancereatography (ERCP) Is both diagnostic and therapeutic in cases of intrabiliary rapture of hydatid cyst in whom sphincterotomy can be preformed .
1
Monitoring: Monitor patients for adverse effects of agents every 2 weeks with a complete blood cell (CBC) count and liver enzyme evaluation for the first 3 months and then every 4 weeks. Monitoring albendazole and mebendazole serum levels is desirable, but few laboratories are capable of performing this measurement. Imaging studies are required for follow-up on the morphologic status of the cyst.
Outcome from medical treatment of CE: Response rates in 1000 treated patients showed that 30% had cyst disappearance (cure), 30%-50% had a decrease in the size of the cyst (improvement), and 20%-40% had no changes. Also, younger adults responded better than older adults.
PAIR ( puncture,aspiration,injection,reaspiration ) Performed using US or CT scan . Performed on liver , bone and kidney cysts . Steps 1-prophylaxis with Albendazole 2-puncture with parasitological examination if available 3-aspiration of cystic fluid ( take 10-15 cc and examine for bilirubin , if present stop procedure and if not present aspirate whole fluid ) 4-injection of 95% ethanol solution or hypertonic saline ( 1/3 of aspirated fluid ) 5-reaspiration of protoscolicide solution after 15 minutes )
The PAIR technique can be performed on liver, bone, and kidney cysts but should not be performed on lung and brain cysts. The cysts should be larger than 5 cm in diameter and type I or II according to the Gharbi ultrasound classification of liver cysts (ie, type I is purely cystic; type II is purely cystic plus hydatid sand; type III has the membrane undulating in the cystic cavity; and type IV has peripheral or diffuse distribution of coarse echoes in a complex and heterogeneous mass). PAIR can be performed on type III cysts as long as it is not a honeycomb cyst.
Preparation
Give 4-6 week of albendazole tablet before surgery (800mg/day in divided doses) in adult
Pre operative visualization of biliary tract by ERCP.
Anaesthesist warned of sudden anaphylactic shock in case of spillage.
Laparoscopic pericystectomy + aumentopexy
Open surgery pericystectomy + aumentopexy or cappitonage