HYDATID CYST OF LIVER

Dr.Anil Haripriya
 Introduction

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 is a zoonosis caused by caused by larva of the dog

tapeworm, Echinococcus granulosus, with man acting as an accidental

intermediate host.

Liver hydatidosis is characterized by progressive growth of the hydatid

cyst, which in its mature form is a fluid filled cavity, delimited by an

external dense host fibrous reaction (pericyst) and two internal parasite

derived layers (endocyst). The hydatid cyst grows slowly and remains

asysmptomatic for many years. Symptoms arise only when the cyst has

grown large enough to cause the pressure on adjacent organs or when a

complications occurs. Infection and intrabiliary rupture are the most

common complications.

Etiopathogenesis

Causative Agent Intermediate host Final host

Echinococcus granulosus Sheep, Human dog

(hydatinosus, cysticus)

Echinococcus vogeli Paca dog, fox

(Brazil)

Echinococcus multilocurlaris Rodents dog,fox,

(alveolaris)

Echinococcus oligarthrus ? Human dog, fox

In E. multilocularis infestation the germinal layer of the cyst sends out

processes Into the surrounding host tissue which in turn form fluid filled
pockets containg proto-Scolices.The germinal layer continues to spread
and multiply like a cancer,therefore It carries mortality upto 50%.

E.vogeli infestation is very rare and found occasionally in Brazil.Paca a

wild Rodent is the intermediate host and final host is the hunting or

domestic dog.

Epidemiology

It is world wide in distribution and is endemic in many countries like

Mediterranean area, the Middle East and South America. In India it is

found in the northern states.

Life Cycle

The adult form of Echinococcus granulosus resides in the small intestine

of dogs. The ova from the adult worm are shed through the canine feces

into the environment, where the intermediate host sheep and humans
ingest the eggs, in humans after entering proximal portion of the small

intestine, the larvae burrow through the mucosa, enter the portal

circulation and travel to liver. The cycle is completed when dogs eat the

carcass of animals infected with the hydatid cysts.

Pathology

A primary cyst in the liver is composed of three layers:

1. Adventitia (psuedocyst / 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. In some primary cysts laminated

membranes may eventually disintegrate and the brood capsules are freed

and grow into daughter cysts. Sometimes the germinal Epithelium

protrudes out towards the external side of the cyst, to form exogenous
daughter cysts, which if left untreated may cause recurrence.

The Hydatid cysts are slow growing approx 2 – 3 cm / year and remain

inapparent for long time.

CLINICAL FEATURES

Patients with simple or uncomplicated multivesicular or univesicular cysts

are asymptomatic. When symptoms occurs they are caused by pressure

on the adjacent organs. Abdominal pain and tenderness are the most

common complaints followed by palpable mass. Jaundice and ascites are

uncommon. With secondary infection tender hepatomegaly, chills, and

spiking temperatures occurs. Urticaria and erythema occur in cases of

generalized anaphylactic reaction. With biliary rupture the classic triad of

jaundice, biliary colic and urticaria occurs.

COMPLICATIONS OF HYDATID CYST

Intrabiliary rupture of Hydatid cyst

When ruptured in to biliary tree, hydatid cysts commonly manifest with

findings of biliary obstruction and cholangitis. The presence of dilated

common bile duct, jaundice, or both in addition to a cystic lesion of liver is

strongly suggestive of a hydatid cyst with intrabiliary rupture. This

complication can be most specifically diagnosed by ERCP or PTC.

( because of risk of intraperitoneal rupture of the hydatid cyst which may

result in peritoneal dissemination and anaphylactic reactions because of

the spillage of the highly antigenic cyst fluid PTC is contraindicated in

hydatid disease of liver). The presence of intrabiliary rupture requires

exploration and drainage of the biliary tract. During the exploration the

biliary tree is cleared of any hydatid material which is confirmed by intra-

operative choliangiography or choledochoscopy. After evacuation of

hydatid elements from the biliary tree, either side to side

choledochoduodenostomy or external T-tube drainage is done.

Infection

Suppuration of cysts takes place in 5 to 15 % of cases. The clinical picture

resembles liver abscess and urgent surgery is necessary.

Recurrence

Incidence of recurrence is estimated to range from 8.5 % to 25 %. The

causes of recurrence are peritoneal spillage and implantation during

operations. New cyst formation from exogenous vesicles attached to the

remaining pericyst after conservative treatment and reinfection.

Coexisting cholelithiasis

Cholelithiasis exists with liver hydatid in three forms: true hydatid lithiasis,

parahydatid lithiasis and accidential coincidence. In true hydatid lithiasis,

histologic examination reveals the presence in central part of the stones of

hydatid elements that constitute the lithogenic nidus. The parahydatic

lithiasis is attributed to the abnormal delay in passage of bile into

gallbladder provoked by an adjacent hydatid cyst.

INVESTIGATIONS

Routine laboratory tests are rarely abnormal occasionally eosinophilia

may be present. Serum alkaline phosphatase levels are raised in one third

of patients.

Immunological tests

Serological tests detect specific antibodies to the parasite and are the

most commonly employed tools to diagnose past and recent infection with

E. granulosus. Detection of IgG antibodies implies exposure to the

parasite, while in active infection high titres of specific IgM and IgA

antibodies are observed. Detection of circulating hydatid antigen in the

serum is of use in monitoring after surgery and pharmcotherapy and in

prognosis. ELISA is used most commonly, but alternate techniques are

counter-immuno-electrophoresis and bacterial co-agglutination. Elisa

techniques have a high sensitivity above 90% and are useful in mass

scale screening. The counter-immuno-electrophoresis has highest

specificity (100%)and high sensitivity (80 – 90%).

CASONI TEST

It has been used most frequently in the past but this cutaneous

hypersensitivity reaction using hydatid fluid is at present considered only

of historical importance. The allergen is rarely standardized and

infestation with other helminthes particularly cestodes can give a false

positive response.

Imaging techniques

Plain abdominal radiography may reveal calcification, hepatomegaly, or

indirect evidence of an hepatic SOL. (for eg. Elevated hemi diaphragm,

right lung basal collapse, and pleural effusion). A coincidental lung cyst

may be picked up on a plain skiagram.

Ultrasound – is 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.

Gharbi Classification on ultrasonographic features of Hydatid Cyst3

 Type Ultrasound Appearance

I Pure fluid Collection

II Fluid collection with a split wall

III Fluid collection with septa

IV Heterogeneous echo pattern

V Reflecting walls

Type V cysts determined by ultrasound to be calcified and have been

assumed to be dead cysts and do not require surgery.

Computed Tomographic scan - has the highest sensitivity of imaging of

the cyst (100%). It is the best mode to detect the number, size, and

location, of the cysts. It may provide 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.

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.

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) – MRI delineates the cyst

capsule better than CT scan, as a low intensity on both T1 and T2

weighted images. However CT scan is better in demonstration of mural

calcifications, cysts less than 3 cm may not show any specific features

and small peritoneal cysts may be missed.

ASPIRATION CYTOLOGY

Hydatid was considered to be a contraindication for FNAC. However, it

may be used in diagnosis of hydatidosis if radiological studies reveal a

cyst and serological tests are equivocal. Diagnostic features include

presence of laminated membrane, which gives a positive periodic acid

schiff reaction, and a diagnosis of hydatid may be presumed.

TREATMENT

The treatment of choice is surgery. The principle of hydatid surgery are 1)

Total removal of all infective components of the cysts; 2) the avoidance of

spillage of cyst contents at time of surgery; 3) management of

communication between cyst and adjacent structures; 4) management of

the residual cavity; 5) minimize risks of operation7,8.

All the surgical procedures can be divided into two large groups,

conservative group and radical group. The conservative technique

consists of aspiration of the cyst, instillation of scolicidal agents and

evacuation of the cyst contents and leaving the pericyst. The residual

pericyst is managed by marsupialization, which consists of suturing the

edges of opened pericyst with the skin, capitonnage (suture obliteration),

partial pericystectomy, omentoplasty (omentum is thought of fill residual

cavity, to assist healing of raw surfaces and to promoted resorption of

serosal fluid and macrophagic migration of septic focus)10, and suture

closure of the pericyst cavity after filling it with saline.

Intracystic injections of scolicidal agents used in the past are

formaldehyde solution, cetrimide solution 0.5%, hypertonic saline solution,

0.5% silver nitrate solution, and hydrogen peroxide solution. The

arguments against the use of conventional intracystic solutions are:

1. In a large univesicular cyst, dilution of the scolicidal solution is

unpredictable and impairs its efficacy.

2. If cyst communicates with the biliary system, it can lead to serious

complications like sclerosing cholangitis and acute pancreatitis, have

been reported with use of formaldehyde and hypertonic saline. Air

embolism has been reported with the use of hydrogen peroxide.

3. Cetrimide solution produces severe adhesions formation.

The best choice is silver nitrate solution 0.5% which has been reported to

be safe and efficacious.

The conservative surgical procedures are easy to perform but the

postoperative complications and duration of hospital stay are not

satisfactory.
Radical surgical procedures include cystectomy, pericystectomy,

lobectomy and hepatectomy Radical procedures have lower rate of

complications and recurrences but many authors consider them

inappropriate, claiming that intraoperative risks are too high for a benign

disease.

Cystectomy – The procedure of choice is cystectomy. The procedure

involves removal of hydatid cyst, comprising laminar layer, germinal layer

and cyst contents i.e. daughter cysts and brood capsules. No attempt is

made to remove the pericyst. The procedure is simple to perform and has

low recurrence rates.

Pericystectomy – this procedure involves non-anatomical resection of

cyst and surrounding compressed liver tissue. This is technically more

difficult procedure than cystectomy and can be associated with

considerable blood loss; it can also be hazardous in the case of large and

complicated cysts when the cyst distorts vital anatomical structures.

Hepatic resections – is the only surgical therapy for E. multilocularis as

the disease is infiltrative and disease margin is ill defined. The arguments

against hepatic resection as a primary modality of treatment are that

outside of dedicated liver units there is considerable morbidity and

mortality from resection of what is essentially a benign condition and also

distortion of anatomy makes surgery more difficult.

LAPAROSCOPIC MANAGEMENT OF HYDATID CYSTS

A special instrument has been developed for the removal of the hydatid

cyst with the laparoscope called the perforator-grinder-aspirator

apparatus. The instrument penetrates the cyst, grinds the particulate

matter and sucks it all out. The advantage of this instrument over that of

conventional suction apparatus is that it does not gets blocked by the

daughter cysts and laminated membranes. Vacuum obliteration of cavity

is carried out with application of – 250 mbar of negative pressure, which

obliterates the cystic cavity by clinging to the opposing cyst walls9.

COMPLICATIONS OF SURGERY

Biliary leakage is the most frequent postoperative complication following

surgery for hydatid 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 a mortality

rate of 0.9 to 3.6 % and recurrence up to 11.3 % within 5 years.

Operations carry a progressively higher mortality – increasing from 6 %

after second to 20% after third1.

PERCUTANEOUS DRAINAGE OF HYDATID CYST

Puncture of hydatid cysts have been discouraged in the past due to the

potential risk of Anaphylactic shock and peritoneal dissemination.

However, in the recent years percutaneous drainage has been used

successfully to treat the hepatic hydatid cysts. Khuroo et al from India

reported 88% disappearance of cysts with percutaneous drainage which

was preceded by Albendazole therapy (10 mg/kg body weight) for 8

weeks5,6.

ENDOSCOPIC MANAGEMENT OF HYDATID CYST

The ERCP is effective in diagnosing biliary tree involvement from the cyst.

The Endoscopic management is useful in presence of intrabiliary rupture,

which requires exploration and drainage of the biliary tract and also after

surgery in 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.

CHEMOTHERAPY FOR HYDATID DISEASE OF LIVER

The compounds in clinical use are the benzimidazole derivatives

(mebendazole and albendazole), which inhibit the uptake of glucose by

the parasite and inhibit production of adenosine triphosphate,

isoquinolone compounds (praziquantel) and immunostimulatory

compounds: isoprinosine and trans-2- phenoxycyclohexonol ethers

Mebendazole

was the first drug to show any activity against hydatid cysts. It inhibits

glucose uptake in susceptible parasites resulting in depletion of the worms

energy sources and slow death. Its disadvantages are that it is poorly

absorbed from the gastrointestinal tract. Although progressively higher

doses for long periods have been given in an attempt to boost plasma

concentrations, it has resulted in a plethora of side effects like prolonged

fever, major liver disturbance, bone marrow depression and

glomerulonephritis. It is no longer used in hydatid disease.

Albendazole

The principal metabolite, albendazole sulfoxide has antihelminthic activity

over a half-life of 8.5 hours. A dose of 10 mg/kg/day achieves an intra cyst

concentration in excess of 100 ng/ml, which is within the effective

scolicidal range. Albendazole is 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.

Cure is defined as disappearance of the cyst, improvement is defined as

> 25% reduction is size of cyst, membrane separation and appearance of

calcification and deterioration as an increase in cyst size. As reported in

study by Horton et al2 on 253 patients, cure rate was 32%, improvement

was seen in 43%, 21% had no response and 1.5% patients showed

increase in size of cyst.

Side effects of Albendazole therapy are: mild abdominal pain, nausea,

vomiting, pruritis, dizziness, alopecia, rash and headache. Occasionally

leucopoenia, eosinophillia, icterus, and mild elevation in transaminase

levels is seen.

Praziquantel

increases the permeability of plasma membrane to calcium ions resulting

in rapid loss and extreme contraction and paralysis of worms. Oral dose

of 50 mg/kg/day for upto two weeks shows rapid scolicidal activity. Side

effects are mild headache, dizziness, drowsiness, abdominal pain, and

nausea. WHO has recommended the use of praziquantel preoperatively

to achieve the sterilization of the cysts or postoperatively in cases of cyst

rupture and spillage.

Immunostimulatory compound

Isoprinosine is an immunomodulatory drug, which appears to act via

cytolytic effects on the cellular elements of the germinal layer, While the

persisting superficial structures prevent the dissemination of viable cells.

The drug has shown efficacy against E. granulosus and E. multilocularis

in an animal model.

References
1. Kumar A, Lal BK, Chattopadhay TK. Hydatid disease of liver – Non-surgical options: J
Assoc Physicians India 1993; Vol. 41.

2. Morris DL, Taylor DH, Optimal timing of postoperative albendazole therapy

prophylaxis. Ann Trop Med 1988; 82: 65–66.

3. Gharbi HA, Hassine W, Brauner MW: Ultrasound examination of hydatid cyst liver,
Radiology 1981; 139:459-463.

4. Menegelli UG, Martinelli LC, Angeles M. Polycystic hydatid disease (Echinococcus

vogeli) clinical, laboratory and morphological findings in nine Brazilian patients. J
Hepatology, 1992; 14:203-210.

5. Khuroo MS, Waini NA, Javid G, Khan BA. Percuatneous drainage compared with
surgery for Hepatic Hydatid Cysts. N Eng J Med 1997; 13:337–400.

6. Palez V, Kugler C, Correa D, Carpio MD. PAIR as percutaneous treatment of hydatid

liver cysts. Acta Tropica 2000; 75:197–202.

7. Agoglu M, DavidsonBR. A rational approach to the terminology of hydatid disease of

liver. J .Infection 1992; 24:1–6.

8. Magistrelli P, Masetti r, Coppola R, Messia A. Surgical treatment of hydatid disease of

liver: a 20 year experience. Arch Surg 1991; 126:518–523.

9. Saglam A. Laparoscopic treatment of liver hydatid cysts. Surg Lap Endosc 1996; 6:16–
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10. Dizri C, Paquet JC, Hay JM. Omentoplasty in the prevention of Deep abdominal
complications after surgery for hydatid disease of liver: a multicenter, prospective
randomized trial. J Am Coll Surg 1999; 188:281– 289.