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HYDATID CYST

Dr Rojan Adhikari
FCPS Resident
General Surgery
Kathmandu Model Hospital
Hydatid cyst
• Echinococcosis (hydatid disease) is a
zoonosis caused by the larval stage of
Echinococcus.

• Species: granulosus , multilocularis,


ligartus, vogeli

• cestodes (flat worms)


Epidemology
• The first case was observed in North America
in 1808 and published in 1822.
• E. granulosus is commonly seen in the
Mediterranean, South America, the Middle
East, Australia, and New Zealand, and is the
most common type of hydatid disease in
humans
• 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
Life cycle
• Definitve host- Dog and some other carnivore

• intermediate host – most commonly sheep,

• Humans are the accidental intermediate host

• The adult worm of the parasite lives in the


proximal small bowel of the definitive host
attached by hooklets to the mucosa
Life Cycle
After ingesion by intermittent host ovum
loses the protective chitinous layer and is
digested in the duodenum.
The released hexacanth embryo (oncosphere)
passes through the intestinal wall into the
portal circulation and develops into cysts
within the liver.
The definitive host eats the viscera of the
intermediate host and the cycle is completed.
Pathology

• By 21 days becomes visible with with naked eye

• Host tissue response- covers parasite in fibous tissue

• Parasite responds by forming inert chitinous material


• There are three known forms of echinococcosis in
humans:

(i) cystic echinococcosis (hydatid disease)


caused by Echinococcus granulosus,

(ii) alveolar echinococcosis (alveolar hydatid disease)


caused by Echinococcus multilocularis, and

(iii) polycystic echinococcosis caused by Echinococcus


vogeli
• Hydatid cyst
– Unilocular
– Increases size about 1 to 1.5mm/month
– Fluid is under pressure
– Liters of fluid
Pericyst
• thin, indistinct fibrous tissue layer representing
an adventitial reaction to the parasitic infection.
• acts as a mechanical support for the hydatid cyst
• metabolic interface between the host and the
parasite.
• As the cyst grows, bile ducts and blood vessels
stretch and become incorporated within this
structure, which explains the biliary and
hemorrhagic complications of cyst growth and
difficulties with resection.
• Over time, the pericyst calcifies.
The fully developed wall of the cyst

Ectocyst (laminated membrane) • Endocyst (germinative )


• is a cuticular chitinous • microscopic dimensions
structure without nuclei • responsible for the
• never grows thicker than 5 production of the
mm, regardless of cyst size. – crystal-clear hydatid
fluid
– ectocyst
– brood capsules
– scoleces
– the daughter cysts
Cyst layers and contents
• Hydatid fluid is antigenic

• This antigenicity is rarely of great clinical


significance

• Allergic reactions range from skin rash to a frank


anaphylactic reaction

• The antigenicity of hydatid fluid is the basis of


serodiagnostics
Clinical presentation
• The clinical features of hydatid liver disease
depend on the site, size, stage of
development, whether the cyst is alive or
dead, and whether the cyst is infected or not.

• Pain in the RUQ or epigastrium is the most


common symptom, whereas hepatomegaly
and a palpable mass are the most common
signs.
Clinical Presentation

SYMPTOMS Percentage
Asymptomatic 75%
Abdominal pain 20%
Dyspepsia 13 %
Fever and chills 8%
Jaundice 6%
Clinical Presentation
SIGNS
• Right upper quadrant mass
• Right upper quadrant tenderness
Suppuration and Secondary Bacterial Infection

• most frequent cause of infection is a cystobiliary


communication

• Clinically presents at pyogenic liver abscess

• An infected hydatid cyst undergoes structural changes


and the parasite dies
Pressure Effects
• grow in the direction of the least resistance

• Pressure effects appear sooner or later

• symptoms result from direct pressure or distortion


of neighboring structures or viscera.

• An enlarging cyst
– atrophy of surrounding hepatocytes
– fibrosis
– compensatory hypertrophy of the remaining liver
parenchyma
– replaces an entire liver lobe
• Serious consequence of cyst enlargement is cyst
Rupture

• Three types of cyst rupture have been addressed:


– obscure
– free
– communicant rupture
Obscure (Internal) Rupture
Injury or penetration of bile between pericyst and ectocyst

Ruptue of ectocyst

Protoscolesces occupies spaces

Develops 100s of daughter cyst

Unilocular  multilocular
Within yellow fluid of gelatin like amorphous mass inside
pericyst
Free Rupture

In free rupture, the hydatid contents disseminates throughout the


peritoneal or pleural cavity
Intraperitoneal Rupture
• Hydatid cyst grows in the direction of the least resistance

• superficial portion of the pericyst is stretched, thinned out

• cyst irregularly shaped, fibrous whitish structure


protruding from normal liver parenchyma

• Cysts reaching the anterior and inferior part of the liver


continue to grow, protruding into the abdominal cavity

•high intracystic pressure causes rupture


of both univesicular and multivesicular
cysts
Clinical presentations of
intraperitoneal rupture
(i) In acute symptomatic rupture,
– peritoneal irritation and acute abdominal symptoms occur
– The incidence is about 1% to 4%.

(ii) In anaphylactic shock


– rupture precipitates severe circulatory collapse, which
may be fatal mask the abdominal manifestations

(iii) In silent rupture, the patient presents with disseminated


abdominal hydatidosis, unaware when the rupture occurred
• Intraperitoneal rupture is a life-threatening complication that
results in secondary echinococcosis

• •Multiple cysts develop throughout the peritoneal cavity


causing
– intestinal obstruction,
– gross abdominal distention,
– ascites,
– and cachexia after years of the rupture.

• This is the secondary, smaller life cycle for the parasite,


occurring only in the intermediate host.
Intrathoracic Rupture
• Elevated hemidiaphragm and a sterile pleural effusion
can be the first signs of liver hydatid disease
• Upward extension of a subdiaphragmatic cyst is usually
asymptomatic, although it can cause dry cough,
dyspnea, chest pain, and toxemia
• The pleura and adherent basal lung segments often
become inflamed and indurated

• Frank intrapleural rupture with empyema


(hydatopiothorax) is rare

• pneumonitis or lung abscess


• The hydatid cyst may erode into a bronchiole and the contents
can be evacuated

• Rupture into bronchiole daughter cysts in the sputum

• Ocassionally a bronchobiliary fistula will arise


Expectoration of bile-tinged sputum

• The incidence of diaphragmatic or transdiaphragmatic


thoracic involvement by hydatid cysts in the dome of the liver
is rare
Communicant Rupture

Hydatid cysts can rupture into physiologic


channels (e.g., biliary, blood vessels) or
adjacent organs (e.g., digestive tract)
• In silent rupture, bile leaks from eroded small ducts into the
cyst, causing
– endogenic vesiculation
– suppuration
– eventually death of the parasite

• Such cysts are filled with bile-stained fluid, although no


visible bile duct communications can be seen.

• Probably 80% to 90% of hydatid cyst bile duct ruptures are of


the silent type.
• A triad of symptoms characterizes rupture into the bile
ducts:

I. biliary colic

II. partial intermittent or complete ductal obstruction


with cholangitis and jaundice

III. germinative membranes in the feces.


Investigation
• Casoni or intradermal test

• Indirect hemagglutination test and enzyme-linked


immunosorbent assay are the most widely used
methods for detection of anti-Echinococcus antibodies
(immunoglobulin G [IgG]).

• These tests give false positive results in cases of


schistosomiasis and nematode infestations that is why
they are not specific for diagnosing hydatidosis.
Lab
• Eosinophilia - 35%

• Bilirubin >2 mg/Dl - 20%

3
• WBC count <10,000/mm - 10%
Investigation
X-ray
• Limited value

• In endemic areas, elevation of the right hemidiaphragm in an


otherwise healthy, asymptomatic patient is highly indicative of
liver hydatidosis

• Sometimes streaklike or round calcification of a senile hydatid


cyst.
Ultrasound Imaging

• comparatively cheap, noninvasive, enables


interventional procedures
• Pathognomonic USG diagnostic features are

I. unmistakable daughter cysts (rosettes) within the main cyst


cavity

II. detachment of the membrane of the cyst (double-contoured


membrane)

III. agglomeration of daughter cysts in the dependent portion of a


hydatid cyst

IV. calcification of the cyst wall


• Based on USG signs, Hassen Gharbi in 1981 classified
liver hydatid cysts into five types

I. pure fluid collection


II. fluid collection with a split wall
III. fluid collection with septa
IV. heterogeneous appearance, and
V. reflecting thick walls
CT
• CT gives similar information to ultrasound,
more specific information about the location
and depth of the cyst within the liver.
• Daughter cysts and exogenous cysts are also
clearly visualized, and the volume of the cyst
can be estimated.
• CT is imperative for operative management,
especially when a laparoscopic approach is
utilized.
MRI & ERCP
• MRI provides structural details of the hydatid
cyst, but adds little more than ultrasound or
CT, and is more expensive.
• Endoscopic retrograde cholangio
pancreatography (ERCP) may show
communication between the cysts and bile
ducts and can be used to drain the biliary tree
before surgery.
Treatment

• Medical, surgical, and percutaneous approaches


may be part of the treatment.

Small cysts (<4 cm) located deep in the


parenchyma of the liver, if uncomplicated, can be
managed conservatively.
• Basic principles of treatment are

(1)eradication of the parasite within the cyst,

(2)protection of the host against spillage of


scoleces, and

(3)management of complications
Anti helmenthics
• Medical therapy for echinococcosis is limited to the
benzimidazoles (mebendazole and albendazole)
• used alone is only 30% successful.
• Albendazole is readily absorbed from the intestine and
metabolized by the liver to an active form.
Mebendazole is poorly absorbed and is inactivated by
the liver.

• 28-day course may be repeated, after 14 days without


treatment to a total of 3 treatment cycles
PAIR technique (percutaneous
aspiration, injection and re-aspiration)
The most frequently utilized protoscolecidal agents
1. 15–20% saline,
2. 95% ethanol,
3. Mebendazole
4. 3% H2O2
5. Betadine
6. Silver nitrate
7. Formalin

Combination is used
Contraindicated in pregnancy, cyst communicating to
billary tree and calcified cyst
PAIR technique
Complication of PAIR
• Spillage and anaphylaxis,
• Recurrence
• Mechanical damage to other tissue
• Bilary fistula
• Hemorrhage
• Infection
Surgery
• Surgery is still the treatment of choice for
uncomplicated hydatid disease of the liver.

• The objectives of surgical treatment are to:


(1) inactivate the scoleces,
(2) prevent spillage of cyst contents,
(3) eliminate all viable elements of the cyst, and
(4) manage the residual cavity of the cyst.
Surgery
Indication
• Large liver cysts with multiple daughter cysts
• Superficially located single liver cysts that may
rupture
• Liver cyst with biliary tree communication or
pressure effects on vital organs or structures
• Infected cysts
• Cysts in lungs, brain, kidneys, eyes, bones
Surgical Procedure
• Early on, surgical management of hydatid cysts
via cyst evacuation resulted in a high rate of
peritoneal implantation.
• This problem prompted the use of scolecidal
agents for injection into the cyst and for use in
the surrounding peritoneum.
• The cyst is usually then aspirated through
close suction
Surgical Procedures
• The cyst is then unroofed which then can be
followed by

Conservative
• Marsupialisation
• Capittonage
• Partial Pericystectomy

This can be followed by omentoplasty


Surgical Procedure
• Radical Pericystectomy: cyst and surrounding
compressed liver tissue

• Hepatic Resection: lobectomy or partial


hepatectomy with entire cyst

• Laparoscopic approach
57 articles were selected for final analysis: one meta-analysis, 9
randomized clinical trials, 5 non-randomized comparative prospective
studies, 7 non-comparative prospective studies, and 34 retrospective
studies
Conclusion
• Antihelminthic treatment alone is not the ideal treatment
for liver hydatid cysts.
• More studies in the literature support the effectiveness of
radical treatment compared with conservative treatment.
• Conservative surgery with omentoplasty is effective in
preventing postoperative complications.
• A laparoscopic approach is safe in some situations.
• Percutaneous drainage with albendazole therapy is a safe
and effective alternative treatment for hydatid cysts of the
liver.
• Radical surgery with pre- and post-operative administration
of albendazole is the best treatment option for liver hydatid
cysts due to low recurrence and complication rates.
References

• Mastery of surgery

• Sabiston text book of surgery

• UpTodate online

• Centre of Disease Control and Prevention

• Parasitology “K D Chatterjee”
Thank you

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