1. RECENT TRENDS IN DIAGNOSIS
AND MANAGEMENT OF HEPATIC
HYDATID DISEASE
Presenter: Dr. Archit Gupta
Moderator: Prof. R.S. Jhobta
Asstt.Prof. Jagdish Kumar Gupta
Dept.of Surgery IGMC Shimla
2. INTRODUCTION
• Hepatic hydatid was known to Hippocrates, who described it as
“livers full of water.”
• In present century, significant advances have been made in the
accurate diagnosis and effective treatment of hydatid disease
• After the World War 2, enormous advances were achieved in
• Surgical Techniques
• Organ Imaging Techniques and
• Immunologic Diagnosis
• The stage has been reached when the diagnosis and treatment of human
hydatid disease is at a most effective and sophisticated level resulting in
decreased morbidity and mortality rate.
3. INTRODUCTION
• Hydatid disease is a zoonosis caused by larval stage of Echinococcus
granulosus (also known as taenia echinococcus).
• The word echinococcus is of Greek origin and means “hedgehog
berry.”
• Hydatid is also of Greek origin (hudatid, hudatis) and means a “watery
vesicle
• In humans, 50-75% of the cysts occur in the liver, 25% are located in
lungs and 5-10% distribute along arterial system.
4. HISTORY
• The first case was observed in 1808 and published in 1822.
• The life cycle was first elucidated by Haubner in 1855
• The true nature of the disease was not known until the second half of the
nineteenth century.
• Successful results of chemotherapy in hepatic hydatid were reported in 1977.
• PAIR was proposed in 1986
• WHO classification of hydatid cyst was given in 2001
• WHO-IWGE guidelines 2009 proposed an image based approach for
management of hydatid cyst
5. Causative Organisms
• E. granulosus - produces unilocular cystic lesions
• E. multilocularis - causes multilocular alveolar lesions that are locally invasive
• E. vogeli - causes polycystic hydatid disease
• E. oligarthus – not much known. Causes polycystic echinococcus.
7. Pathogenesis
Primary cyst in the liver is composed of three layers:
1. Adventitia (Pseudocyst / Pericyst) –
• Compressed liver parenchyma and fibrous tissue induced by the expanding parasitic cyst.
• Pericyst acts as a mechanical support for hydatid cyst and is metabolic interface between the
host and the parasite.
2. Laminated membrane (Ectocyst) –
• Bluish white, gelatinous, about 0.5cm thick
• It is a cuticular chitinous structure without nuclei
• Acts as a barrier for bacteria and an ultrafilter for protein molecules.
3. Germinal epithelium (Endocyst) –
• Single layer of cells lining the inner aspects of the cyst and is the only living component
• Responsible for the formation of the other layers as well as the hydatid fluid and brood
capsules within the cyst
8. Hydatid Sand
• Brood capsule and freed protoscoleces are released into the fluid of the
original cyst and together with calcareous bodies form hydatid sand
• Hydatid sand is made of around 400,000 scolices /ml of fluid
Cyst Growth
• Cysts in liver grow to 1cm in first 6 months and 2-3cm annually thereafter
• Development of brood capsule from the germinal layer indicates complete
biologic development of the cyst, which occurs after 6 months of growth
9. Epidemiology
• Echinococcosis occurs worldwide and is endemic in some countries
such as Australia and the Middle East, especially in sheep farming
areas.
• In endemic areas, the annual incidence of cystic echinococcosis
ranges from 1 to 200 per 100,000 inhabitants.
• In India, hydatid is reported from practically all parts of the country
• Higher incidence is reported from Tamil Nadu and Andhra Pradesh,
particularly from Madurai district.
10. Clinical features
• Male = female (Avg age 45 yrs)
• Approx 70% located in the right liver and are solitary
• Most common segment- segment VII (27%)
• Both lobes 16% and only left lobe 17%
• Cysts are largely asymptomatic until complications occur
• Symptoms of hydatid disease may be caused by compression, obstruction, or
displacement of adjacent organs or structures
• The most common presenting symptoms are abdominal pain, dyspepsia and vomiting
• May present as obstructive Jaundice (intrahepatic biliary obstuction)
• Specially in children- chronic pain abdomen, wt loss and wasting
11. Clinical signs
• Hepatomegaly (most common)
• Palpable RUQ mass(cystic)
• Mass with Hydatid thrill (elicited by three-finger test)
• Cachexia in children
• Camellotte sign: Following intrabiliary rupture – partial collapse of the cyst wall.
Enlarged palpable liver due to
hydatid with positive hydatid thrill
12. CASE DEFINITIONS
Possible case
• Any patient with a clinical or epidemiological history
• Imaging findings or Serology positive for CE
Probable case.
• Any patient with the combination of clinical history, epidemiological history, imaging findings and
serology positive for CE on two tests.
Confirmed case.
• The above, plus either
(1) Demonstration of protoscoleces or their components, using direct microscopy or molecular
biology, in the cyst contents aspirated by percutaneous puncture or at surgery
(2) Changes in US appearance, e. g. detachment of the endocyst in a CE1 cyst, thus moving to a
CE3a stage, or solidification of a CE2 or CE3b, thus changing to a CE4 stage, after
administration of ABZ (at least 3 months) or spontaneous.
Brunetti et al. / Acta Tropica 114 (2010) 1–16
13. Laboratory Findings
• Routine haematological tests may reveal eosinophilia
• Casoni’s intradermal test – due to its low sensitivity and specificity and because of risk of
causing anaphylactic reactions, it is obsolete now.
• 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 titers of specific IgM and IgA antibodies are observed.
• Detection of circulating hydatid antigen in the serum is of use in monitoring after
surgery and pharmacotherapy and in prognosis.
14. Serological Tests
• Indirect Haemagglutinin Test (IHA)
• Complement Fixation Test (CFT)
• Latex Agglutination Test (LT)
• Indirect Flourescent Antiody test (IFAT)
• Immunoelectrophoresis (IEP)
• Counterimmune Elecctrophoresis (CIE)
• Double diffusion test (DD)
• ELISA
• Radioallergosorbent test (RAST)
• Basophil degranulation test (BDT)
• For an individual patient, stratergy should be initial screening with high sensitivity test like IHA or LT,
followed by confirmation with highly specific test like IEP, DD, ELISA OR RAST.
• The only serological test that has a role in monitoring progress after surgery for hydatid is CFT because it
reverts to negative within 12 months of cure.
• Recently, reports have suggested that BDT has a high sensitivity and that it becomes negative within a week
of cure.
16. Ultrasound
• Initial imaging test of choice
• Role of ultrasound in hydatid disease includes:
a. Screening in endemic areas
b. First line diagnostics
c. Interventional non operative procedures
d. Intraoperative ultrasound
e. Monitoring treatment and during follow up
• In 1984 Hassen A. Gharbi gave a classification based on ultrasound
findings of hydatid cyst, which was modified by WHO in 2001.
17. Gharbi’s Classification
• Type I : pure cystic fluid Collection
(spherical-oval, thick-walled)
• Type II : fluid Collection with
membrane separation
• Type III : Fluid collection with septa
• Type IV: heterogeneous
(hypoechoic-hyperechoic-
intermediate) pattern
• Type V: completely calcified
(Reflecting) walls
18. USG Classification (WHO-IWGE, 2001)
Group 1 : Active group –
• Cysts larger than 2 cm and fertile (CE1, CE2)
Group 2 : Transition group –
• Cysts starting to degenerate and entering a transitional stage because of host resistance or treatment,
but may contain viable protoscolices (CE3)
Group 3 : Inactive group –
• Degenerated, partially or totally calcified cysts, unlikely to contain viable protoscolices. (CE4,CE5)
18
21. CE2
- Cyst with multiple septations
giving it multivesicular
appearance or rossette
appearance or honey comb
appearance with unilocular
mother cyst
22. CE3
• Unilocular cyst with daughter
cysts with detached laminated
membranes appearing as water
lily sign
• CE3 transitional cysts may be
differentiated into
• CE3a (with detached endocyst)
• CE3b (predominantly solid with
daughter vesicles)
23. CE4
Mixed hypo and hyperechoic contents
with absent daughter cysts
These contents give an appearance of
Ball of wool sign (indicating the
degenerative nature of the cyst)
25. CECT Abdomen
• CT gives similar information to ultrasound, but more specific
information about the location and depth of cyst within the liver.
• Daughter cysts and exogenous cysts are also clearly visualised and
cyst volume can be estimated.
• CT is imperative for operative management especially when
laparoscopic approach is used.
26. CECT abdomen showing a large univesicular
cyst.
CECT abdomen showing a large cyst full of daughter
cysts (multivesicular, rosettelike)
27. CT scan showing hydatid cyst in left lobe of liver with
periphery showing double edge s/o lamellar membrane
CT scan showing a round lesion with water
attenuation and a ringlike pattern of calcification.
This pattern represents calcification of the pericyst
28. MRI
• MRI provides excellent structural detail of hydatid cysts and is superior to CT
in demonstrating alteration of the hepatic venous system.
• MRI and magnetic resonance cholangiopancreatography (MRCP) is suggested
in
(a) Subdiaphragmatic site of HC
(b) Disseminated disease
(c) Extra-abdominal location
(d) Complicated, symptomatic, cysts
(e) Pre-surgical evaluation and planning (liquid areas and structure of the HC).
• MRCP is an excellent noninvasive tool for investigating jaundiced patients
with liver hydatidosis
29. T2 weighted coronal MRI showing multiple daughter cysts MRCP showing large hydatid cyst with daughter cysts
communicating with common bile duct
30. ERCP
• ERCP has little value in asymptomatic patients and should be avoided
• ERCP is indicated when there is suspicion of daughter cysts in the
biliary tree causing obstructive jaundice
• Indications for endoscopic papillotomy in the preoperative period are
• when US, CT, MRCP, or ERCP detect hydatid material in the CBD
• when cholangitis has been a feature of the clinical presentation
• Critical use of ERCP and papillotomy in patients with cystobiliary
communications has reduced mortality and in-hospital stay
32. TREATMENT
Modalities of treatment of hydatid cyst include
1. Chemotherapy
2. PAIR and other percutaneous treatments
3. Open Surgery
4. Laparoscopic Surgery
• In 2009, WHO –IWGE proposed an image based approach for
management of hydatid cyst.
33. <5 cm – Albendazole
>5 cm – PAIR and
Albendazole
Surgery
Other percutaneous
treats can be used
3a 3b
<5 cm - ABZ
>5 cm – PAIR
and ABZ
Surgery
Wait and
Watch
Wait and
Watch
WHO guidelines for management of hydatid cyst, 2010
34. CHEMOTHERAPY
Albendazole – drug of choice
Mechanism of action : Metabolite of albendazole in liver, albendazole
sulfoxide is active against protoscoleces of echinococcus granulosus .
Dosage: 10 to 15 mg/kg/d, in two divided doses, with a fat rich meal
Duration of therapy: For patients being managed conservatively : to be given
for 3 months
Pre-interventional: 4 days before intervention and to be continued till 1
month after intervention.
It should be administered continuously, without the monthly treatment
interruptions (recommended in the 1980s).
Side effects: Pancytopenia, aplastic anemia, agranulocytosis, leucopenia
35. CHEMOTHERAPY
Praziquantel
• A synthetic isoquinoline pyrazine derivative
• Increases the protoscolicidal effect of albendazole
• Dosage: 40 mg/kg once a week in combination with albendazole
36. Indications of chemotherapy
(a) Inoperable patients with primary liver cystic echinococcosis
(b) Patients with multiple cysts in two or more organs
(c) Multiple small (5 cm, CE1 and CE3) liver cysts
(d) Cysts deep in liver parenchyma
(e) Prevention and management of secondary hydatidosis
(f) Management of recurrent hydatidosis
(g) Unilocular cysts in unfit elderly patients
(h) In combination with surgery and interventional procedure
37. Contraindications of Chemotherapy
(a) Large cysts (10 cm)
(b) Cysts with multiple septa divisions (honeycomblike cysts)
(c) Cysts that are prone to rupture (superficial)
(d) Infected cysts
(e) Inactive cysts
(f) Asymptomatic calcified cysts
(g) Severe chronic hepatic disease
(h) Bone marrow depression
(i) Early pregnancy
• Diabetes is a relative contraindication
38. PAIR
•PAIR Protocol (Minimum Requirements):
1. Puncture and parasitological examination (if possible) or fast test for
antigen detection in cyst fluid
2. Aspiration of cystic fluid (10-15 cc)
Test for bilirubin in cyst fluid
If bilirubin present: →→ →→ stop procedure
If no bilirubin present: →→ →→ aspirate all cystic fluid
3. Injection of 95 % ethanol solution or hypertonic saline (1/3 of the
amount
of aspirated fluid)
4. Re aspiration of protoscolicide solution after 15 minutes
40. Indications for PAIR
• Inoperable patients
• Patients who refuse surgery
• Cysts types CL, CE1, CE3a
• Relapse after surgery
• Infected cysts
• Failure of chemotherapy,
• Multiple cysts of more than 5-cm diameter in different liver segments
• Pregnant women (chemotherapy contraindicated)
• Children less than 3 years old.
41. Contraindications for PAIR
• Inaccessible cysts
• Superficially located cysts
• Cysts with multiple septa divisions (honeycomblike cysts CE2, CE3b)
• Cysts with hyperechogenic solid patterns (CE4)
• Cysts communicating with bile ducts
• Partially or totally calcified cysts (CE5)
42. Modifications of PAIR
Complicated cysts, cysts with many daughter cysts, or large-volume
cysts are indications for PAIR modifications:
1. The PAIR-catheterization technique
2. The D-PAI (doublepuncture, aspiration, and injection) technique
3. The percutaneous evacuation of cyst content (PEVAC) technique
4. The modified catheter aspiration technique (MoCAT)
43. Surgery in Hepatic Hydatid Disease
• The classic open surgical procedures can be subdivided into two groups:
Conservative
• Tissue-sparing procedures that are limited to removing the parasite, with part or
most of the pericyst left in situ
Radical
• Resectional procedures that remove the entire pericyst, with or without entering the
cyst itself.
The choice of the surgical techniques depends on
• Type and size of the cyst
• Site
• Presence of complications
• Expertise of the surgeon.
44. Principles of Hydatid Surgery
• Total removal of all infective components of the cysts
• The avoidance of spillage of cyst contents at time of surgery
• Management of communication between cyst and adjacent
structures
• Management of the residual cavity
• Minimize risks of operation
45. Indications for open surgery
• Large cysts with multiple daughter cysts type (CE2, CE3b)
• Single liver cysts situated superficially that may rupture
• Infected cysts
• Cysts with cystobiliary communication
• Cysts exerting pressure on adjacent organs
46. Contraindications for surgery
• Patients refusing surgery
• Extreme age
• Pregnant women
• Concomitant severe diseases
• Numerous cysts
• Cysts difficult to access
• Dead cysts
• Cysts partially or totally calcified
• Very small cysts (<5 cm)
47. Conservative Technique ( Open Cystectomy)
• Safe decompression of cyst is importrant
• All cysts should be treated as if they are vital and infectious
• The entire area around the mobilized liver is packed with blue or green packs
and drapes are soaked in 15% - 20% saline.
• The point where the cyst is to be punctured is determined and a working
area, as small as possible, is delineated by additional packing.
• This is important because the high intracystic pressure makes it difficult to avoid some
leakage of cyst contents.
• The cyst is then opened and the contents are aspirated with a suction device
• Special devices have been designed for safe decompression of HCs.
• The use of “cones” (adhere to liver surface by freezing or vacuum)
• The cavity is then irrigated with a scolicidal agent
49. Aaron Cryogenic Cones
• The cryogenic cone is a funnel-shaped appliance with the narrow end chopped off
midway.
• A coiled tube is soldered to the base of the cone.
• The cone can be frozen and fixed on to the surface of the liver over a hydatid cyst by the
passage of liquid nitrogen through the tube.
• The hydatid cyst can then be opened without any danger of spillage and seeding of
daughter cysts into the abdominal cavity
51. Management of Residual Pericyst Cavity
• Marsupialization
• Deroofing
• Omentoplasty
• Interoflexon
• Cappitonage
• Drainage of cyst
52. Techniques for the management of the residual cavity. A: Cyst with
oversewn rim left open. B: Introflexion - infolding of the rim of the
pericyst cavity. Suture does not engage the bottom of the cavity. C:
Capitonnage with drainage. There is spiral suturing from the bottom of
the cavity upward. D: Omentoplasty.
53. Radical surgical procedures
Radical surgical procedures include
• Pericystectomy
• Lobectomy
• Hepatectomy
• Radical procedures have lower rate of complications and recurrences
• Many authors consider them inappropriate, claiming that intraoperative
risks are too high for a benign disease.
Fethi Derbel, Mohamed Ben Mabrouk, et al. (2012). Hydatid Cysts of the Liver - Diagnosis, Complications and Treatment, Abdominal Surgery, Prof. Fethi Derbel (Ed.), InTech, DOI: 10.5772/48433.
54. Pericystectomy
• This procedure involves a non-anatomical resection of
cyst and surrounding compressed liver tissue.
• This is technically a 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 such as; hepatic veins or biliary ducts.
Fethi Derbel, Mohamed Ben Mabrouk, et al. (2012). Hydatid Cysts of the Liver - Diagnosis, Complications and Treatment, Abdominal Surgery, Prof. Fethi Derbel (Ed.), InTech, DOI: 10.5772/48433.
55. Rationale for Radical Procedures
• Total removal of the cyst and exocysts is associated with the lowest
recurrence rate
• Chemotherapy after radical removal of the intact cyst is unnecessary
• The use of intraoperative protoscolicidal agents is unnecessary if the
cyst is not entered
• The chance for a biliary fistula and cavity- related complications is low
• Calcified cysts can be removed
• In expert hands the mortality and morbidity rates are low.
56. Laparoscopic Surgery
• The rapid development of laparoscopic techniques has encouraged
surgeons to replicate principles of conventional hydatid surgery using
a minimally invasive approach.
• It offers a lower morbidity outcome and a shorter hospital stay
• Gives a better visual control of the cyst cavity under magnification
which allows a better detection of biliary fistula.
• Different instruments have been described to try to avoid leakage of
daughter cysts and scolices which include the Palanivelu hydatid
system and the perforator grinder aspirator apparatus.
58. The criteria to exclude laparoscopic treatment of hydatid cyst of liver
are:
Cystobiliary communication (on imaging)
Central localization of the cyst
Cysts dimension >15 cm
Number of cysts > 3
Thickened or calcified walls
Opening of bile ducts that leak bile
59. Trocar placement based on
baseball diamond concept.
Aspiration of cyst contents and injection of hypertonic saline
to create a negative balance
61. Complications of surgery
• Biliary leakage is the most frequent complication
• Although most of the external biliary fistulas close spontaneously, they may be persistent in 4%-27.5% of
the cases.
• Endoscopic sphincterotomy is performed after a 3-weeks in patients with low-output fistulas
• Can be performed earlier in patients with high-output fistulas.
• Infection of the residual cavity
• More frequent when the pericyst is thick and calcified.
• Needs reoperation or percutaneous drainage under CT-scan guidance.
• Mortality:
• 0.9 -3.6 %.
• Recurrence rate
• Varies with type of surgery
• Up to 11.3 % within 5 years.
62. ?Best for management of residual cyst cavity
• According to the RCT by Dziri et al omentoplasty alone leads to fewer
complications.
Dziri C, Haouet K, Fingerhut A. Treatment of hydatid cyst of the liver: where is the evidence? World J Surg
2004; 28:731-736
63. RADICAL OR CONSERVATIVE SURGICAL
TREATMENT?
• A comparative retrospective study of 242 patients described significantly higher
morbidity and recurrence rates in patients who underwent conservative surgery
(11% vs 3%; 24% vs 3%)
Aydin et al , J Gastroenterol 2008
• Randomized study involving 32 patients, compared radical surgery and
conservative surgery. The authors concluded that conservative surgery leads to a
significantly higher early recurrence rate (P = 0.045) compared to radical surgery,
as well as a higher rate of complications in the residual cyst cavity (P = 0.011)
Yüksel O, J Gastrointest Surg 2008
64. Complications of hydatid cyst of the liver:
• Echinococcal cysts of the liver can cause complications in about 40% of cases.
• The most common complications in order of frequency are
• Infection
• Intrabiliary rupture of hydatid cyst
• The rupture in the thorax
• The rupture in the peritoneum
Fethi Derbel, Mohamed Ben Mabrouk, et al. (2012). Hydatid Cysts of the Liver - Diagnosis, Complications and Treatment, Abdominal Surgery, Prof. Fethi Derbel (Ed.), InTech, DOI: 10.5772/48433.
65. Treatment of hydatid cysts rupture into the biliary
tracts
• There are two different clinical settings associated with intrabiliary rupture
• Frank intrabiliary rupture - the cyst content drains to biliary tract and causes cholestatic jaundice
• Simple communication - simple communications can cause post-operative biliary fistulae.
• If the diameter of communication is larger than 5 mm, cystic content migration into the biliary tract
will occur in 65% of the cases.
• Vesicles, debris and purulent materials may be found in the biliary ducts.
• Surgery must be done early.
• Delay can cause suppurative cholangitis, septicemia and liver abscess formation.
66. Treatment of hydatid cysts rupture into the
biliary tracts
• In bile leakage cases, peroperative cholangiography can be done
• The injection of radiopaque solution or methylene blue is helpful to diagnose intrabiliary
rupture or to see the orifice.
67. • The treatment of the cysto-biliary communication is based on several techniques:
Suture of the communication
• Simple suture
• Suture with T-tube CBD drainage
Internal drainage procedures
• Biliodigestive bypass
• Transduodenal sphincterotomy
• Internal transfistular drainage with or without transduodenal sphincteroplasty
External drainage procedures
Reconstructive procedures
• Pericystojejunostomy
• Intracavitary biliodigestive bypass
• Bile duct repair
Liver resection
68. Post-operative cholangiography in a patient treated for hydatid cyst with large bilio-
cystic fistula treated with partial cystectomy and T tube drainage.
69. Follow Up
• Chemotherapy:
• Postoperative treatment with benzimidazoles for 1 month who have
undergone cystectomy or PAIR successfully.
• Continued for 3-6 months for patients, incompletely resected cyst,
spillage during surgery.
70. Follow Up
• Laboratory tests:
• Patients on benzimidazoles should have a CBC count and liver enzyme
evaluation performed at biweekly intervals for 3 months and 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.
• Imaging: Ultrasonography or CT scan at the same intervals as the
laboratory tests or as clinically indicated
71. Prevention
• Public education about the life cycle and transmission of the disease
• Washing hands after contact with canines
• Eliminating the consumption of vegetables grown at ground level from the diet
• Stopping the practice of feeding entrails of slaughtered animals to dogs
72. Conclusion
• Hydatid disease remains a continuous public health problem in endemic countries.
• The liver is the most common site for hydatid disease, followed by the lungs (15%), the
spleen (5%), and other organs (5%).
• Diagnosis of liver hydatid disease is made with Ultrasonography and computed
tomography.
• Surgery combined with medical treatment by albendazole is effective in the eradication of
hepatic hydatid disease and in the prevention of local recurrences.
• Although surgery is the recommended treatment for liver hydatid disease, percutaneous
treatment has been introduced as an alternative to surgery.
• PAIR is a valuable alternative to surgery. It is safe and efficient in selected patients
73. Sources
• WHO-IWGE Guidelines on management of Cystic Echinococcosis, 2009
• Maingot Abdominal Operations, 9th & 12th Edition
• Mastery of Surgery, 6th Edition
• Art of Laparoscopic Surgery by C. Palanivelu, 1st Edition
• Bailey & Love’s Short Practice of Surgery, 26th Edition
The cyst is punctured with a laparoscopic needle and hypertonic saline is put and aspirated from the cyst. This is done to replace the cyst contents with hypertonic saline.