its a liver disease

1. By: Dr. Kailash Makhejani

2.  Hydatid cyst (HC), or hydatidosis, is a global parasitic
zoonosis
 Hippocrates recognized human hydatid over 2,000 years
ago
 The Arab physician, Al Rhazes, made reference to hydatid
disease of the liver in AD 900.
 Liver hydatid disease --- zoonosis --- caused by larva of the
dog tapeworm, Echinococcus granulosus, with human being
acting as an accidental intermediate host.

3. Causative agent Intermediate host Definative host
Echinococcus granulosus
(Cystic echonococcosis)
Sheep, Human dog
Echinococcus
multilocurlaris
(Alveolar echinococcosis)
Rodents ,Humans dog,fox

5.  A primary cyst in the liver is composed of three layers:

 1.Adventitia (pseudocyst / pericyst)
 consisting of compressed liver parenchyma and fibrous tissue induced by the
expanding parasitic cyst.
 2.Laminated membrane (ectocyst)
 is elastic white covering, easily separable from the adventitia.
 3.Germinal epithelium (endocyst)
 is a single layer of cells lining the inner aspects of the cyst
and is the only living component, being responsible for
the formation of the other layers as well as the hydatid
fluid and brood capsules within the cyst.

6.  Echinococcosis can involve any organ
 Organs affected by E granulosus are the
 Liver (63%)
 Lungs (25%)
 Muscles (5%)
 Bones (3%)
 Kidneys (2%)
 Brain (1%)
 Spleen (1%)
 The clinical presentation of a hydatid cyst is largely
asymptomatic until complications occur.

7.  The most common presenting symptoms are abdominal pain,
dyspepsia, and vomiting
 The most frequent sign is hepatomegaly/palpable mass
 Jaundice and fever are each present in about 8% of patients
 Bacterial superinfection of a hydatid cyst can occur and present like
a pyogenic abscess
 Rupture of the cyst into the biliary tree
 Free ruptures can result in disseminated echinococcosis and a
 potentially fatal anaphylactic reaction.

8.  Routine laboratory blood workup: Nonspecific
 Liver involvement may be reflected in an elevated
bilirubin or alkaline phosphatase level.
 Leukocytosis may suggest infection of the cyst.
 Eosinophilia is present in 25% of all persons who are
infected, while hypogammaglobinemia is present in
30%.

9.  Serodiagnostic techniques
 Indirect hemagglutination(IHA) test and the enzyme-
linked immunosorbent assay (ELISA)
 sensitivity of 80% overall (90% in hepatic echinococcosis, 40% in pulmonary
echinococcosis) and are the initial screening tests of choice.
 Immunodiffusion and immunoelectrophoresis
 demonstrate antibodies to antigen 5 and provide specific confirmation of
reactivity
 The ELISA test is useful in follow-up to detect recurrence.

10.  Plain X-RAY Films:
 Findings from plain films of the
chest, abdomen, or any other
involved site are nonspecific and
mostly non revealing. A thin rim
of calcification delineating a cyst
is suggestive of an echinococcal
cyst.

11.  Ultrasound:
 currently the primary diagnostic technique and has diagnostic accuracy of 90%.
 Findings usually seen are:
 a) Solitary Cyst –
 anechoic univesicular cyst with well defined borders and enhancement of back wall
echoes in a manner similar to simple or congenital cysts. Features are suggesting a
hydatid etiology include dependent debris (hydatid sand) moving freely with change in
position; presence of wall calcification or localized thickening in the wall corresponding
to early daughter cysts.
 b) Separation of membranes (ultrasonic water lily sign)
 due to collapse of germinal layer seen as an undulating linear collection of echoes.
 c) Daughter cysts –
 probably the most characteristic sign with cysts within a cyst, producing a cartwheel
or honeycomb cyst.
 d) Multiple cysts with normal intervening parenchyma
 (differential diagnosis are necrotic secondaries, Polycystic liver disease, abscess,
chronic hematoma and biliary cysts.
 e) Complications may be evident such as echogenic cyst in infection or
signs of biliary obstruction usually implying a biliary communication.

12. Hydatid cyst of the liver on ultrasound examination. Note the multiloculated appearance of
the cyst due to the presence of multiple daughter cysts. Detached germinal membranes and
brood capsules can be seen in the more anterior daughter cyst. This multiseptated anechoic
or hypoechoic appearance on sonography is typical of a hydatid cyst.

13.  Highly sensitive (98%)
 Best to detect the number, size, and location of the cysts
 Provides clue to presence of complications such as
infection, and intrabiliary rupture
 CT features include
 sharply marginated single or multiple rounded cysts of fluid
density (3 – 30 Hounsfield units) with a thin dense rim.
 It is supported by floating membrane within the cysts on CT
scan.

15.  Angiography
 of the liver is suggestive but due to lack of specificity and availability of lesser
invasive techniques it is rarely required
 It may be required in a differential diagnosis of suspected malignancy or
vascular malformation
 Typical features include an avascular lesion with vascular displacement and a
thin peripheral halo of higher density
 Direct cholangiography (Endoscopic or percutaneous)

 May be required in suspected intrabiliary rupture and bile duct obstruction
 ERCP is also a valuable method for detecting post-operative complications
involving the biliary tree following surgical intervention.

16.  Radionuclide scan
 has largely replaced by ultrasound and CT scan. It remains
most accurate method of demonstration of a bronchobiliary
fistula.
 Immunoscintigraphy
 is an innovation using radiolabelled antibodies to antigens in
the parasite
 Magnetic resonance Imaging (MRI scan)
 Images show the cysts adequately, but MRI offers no real
advantage over CT scan.

17.  The treatment of choice is surgery
 Available Options:
 Medical
 Per-cutaneous
 Endoscopic
 Surgical

18.  CHEMOTHERAPY FOR HYDATID DISEASE OF LIVER
 The compounds in common clinical use are Mebendazole and Albendazole
 inhibit glucose uptake by the parasite and inhibit production of ATP
 Indications:
 Indicated in patients with primary liver or lung cysts that are inoperable (because of
location or medical condition)
 Patients with cysts in 2 or more organs
 Peritoneal cysts
 Contraindications:
 Early pregnancy
 Bone marrow suppression
 Chronic hepatic disease
 Large cysts with the risk of rupture
 Inactive or calcified cysts
 A relative contraindication is bone cysts because of the significantly decreased
response.

19.  Mebendazole:
 Disadvantages are that it is poorly absorbed from the gastrointestinal tract
 It is no longer used in hydatid disease
 Albendazole
 administered in a dose of 10 – 15 mg/kg/day in adults or a fixed dose of 400 mg twice
daily.
 The treatment is given in cycles of 28 days with two weeks treatment free periods
between the cycles.
 The different schedules for the treatment are:
 1. Inoperable cases - as primary treatment - 3 cycles
 2. Pre-operatively – to reduce the risk of recurrence 6 weeks continuous treatment
 3. Post-operatively to prevent recurrence in cases of intraoperative cyst spillage – 3 cycles.
 Side effects of Albendazole
 Mild abdominal pain, nausea, vomiting, pruritis, dizziness, alopecia, rash and
headache.
 Occasionally leucopoenia, eosinophillia, icterus, and mild elevation in transaminase
levels.

20.  PAIR (Puncture, Aspiration, Injection, Re-aspiration)
 Proposed in 1986 by the Tunisian team that first used it in a
prospective study
 Recent and minimally invasive therapeutic option,
 complements or replaces surgery which was long considered
as the only treatment.
 If a catheter is temporarily left in the cyst after the
procedure for drainage (D), the acronym PAIRD
should be preferred

21.  Performed using either ultrasound or CT guidance
 Involves aspiration of the contents via a special cannula
 Followed by injection of a scolicidal agent for at least 15 minutes
 Then reaspiration of the cystic contents
 This is repeated until the return is clear
 The cyst is then filled with isotonic sodium chloride solution
 Perioperative treatment with a benzimidazole is mandatory
 The cysts should be larger than 5 cm in diameter

22.  Patients with:
 Non-echoic lesion ≥ 5 cm in diameter (TYPE 1)
 Cysts with detachment of membranes (TYPE2) and/or with daughter cysts (TYPE 3)
 Multiple cysts if accessible to puncture
 Infected cysts
 Also
 Pregnant women
 Children >3 years old
 Patients who fail to respond to chemotherapy alone
 Patients in whom surgery is contraindicated
 Patient who refuse surgery
 Patients who relapse after surgery

23.  Non-cooperative patients and inaccessible or risky location
of the cyst in the liver
 Cyst in spine, brain and/or heart
 Inactive or calcified lesion
 Cysts communicating with the biliary tree
 Cysts open into the abdominal cavity, bronchi and urinary
tract

24.  Minimal invasiveness
 Reduced risk compared with surgery
 Confirmation of diagnosis
 Removal of large numbers of protoscolices with the aspirated cyst fluid
 Improved efficacy of chemotherapy given before and after puncture
(probably because of an increased penetration of antihelminthic drugs into
cysts re-filling with hydatid fluid )
 Reduced hospitalization time
 Cost of the puncture and chemotherapy usually less than that of surgery or
chemotherapy alone

25.  Same risks as any puncture ( haemorrhage, mechanical lesions of other tissues,
infections )
 Anaphylactic shock or other allergic reactions
 Secondary echinococcosis caused by spillage
 Chemical ( sclerosing ) cholangitis if cysts communicate with the biliary tree
 Sudden intracystic decompression, thus leading to biliary fistulas
 Persistence of satellite daughter cysts
 Systemic toxicity of alcohol or hypertonic saline in case of large cysts (total
volume injected must be carefully calculated)

28.  ERCP
 Effective in diagnosing biliary tree involvement from the cyst
 Useful in presence of intrabiliary rupture, which requires
exploration and drainage of the biliary tract
 Also useful in post surgerical cases with presence of residual
hydatid material (membranes and daughter cyst) left in biliary tree
 During the endoscopic exploration the biliary tree is cleared of any
hydatid material with a balloon catheter or a dormia basket
 The endoscopic sphinterotomy is also performed to facilitate
drainage of the common bile duct.

29.  Indications:
 Large liver cysts with multiple daughter cysts
 Superficially located single liver cysts that may rupture (traumatically or spontaneously)
 Liver cysts with biliary tree communication or pressure effects on vital organs or structures
 Infected cysts
 Contraindications:
 General contraindications to surgical procedures (eg, extremes of age, pregnancy, severe
preexisting medical conditions)
 Multiple cysts in multiple organs
 Cysts that are difficult to access
 Dead cysts
 Calcified cysts
 Very small cysts

30.  Biliary leakage is the most frequent postoperative complication
following surgery for hydatid cyst of liver
 It has been reported to occur in about 50% of cases because of the
small-undetected communication between the cyst and the bile ducts
 The surgical management of hydatid disease of liver carries
 Mortality rate of 0.9 to 3.6 %
 Recurrence up to 11.3 % within 5 years
 Multiple operations carry a progressively higher mortality – increasing from 6 %
after second to 20% after third.

31.  Chemotherapy
 Postoperative treatment with benzimidazoles is continued for 1
month in patients who have undergone complete resection or PAIR
successfully
 Laboratory tests
 Patients on benzimidazoles should have a
 CBC count and liver enzyme evaluation performed at biweekly
intervals for 3 months
 Then every 4 weeks to monitor for toxicity
 ELISA or indirect hemagglutination tests are usually performed at 3-,
6-, 12-, and 24-month intervals as screening for recurrence of resected
disease or aggravation of existing disease
 Imaging:
 Ultrasound and/or CT scan are used in follow-up at the same
intervals as the laboratory tests or as clinically indicated.