Dr. Zahid Iqbal Mir, MBBS, MS (General Surgery), DNB (General Surgery) has done his bachelors and masters in General Surgery from the prestigious Govt Medical College Jammu and DNB in General Surgery from NBEMS New Delhi. He is a passionate surgeon, earlier practising at Government Medical College, Jammu as Registrar in Department of General Surgery. Nowadays working as Senior Resident in Department of General Surgery, Government Medical College & Hospital, Sector 32, Chandigarh and a rising name in field of surgery. He is an enthusiastic, enigmatic and dedicated teacher as well. He is not just a resolute learner, but also an awe inspiring guiding light for his juniors, which makes him the most loveable and respected senior.

1. Presenter. :HymaBamba(33)
Moderators:Dr.ZahidIqbalMir
Dr.SanjayGupta
LIVERHYDATIDDISEASE
MANAGEMENT

2. MANAGEMENT APPROACH
ANTIPARASITICAPPROACH CYSTMANAGEMENT
SURGERY
PERCUTANEOUS
Whether the patient is treated only medically or in combination with surgery will
depend upon the clinical group (which gives an idea as to the activity of the disease),
thenumberof cysts andtheir anatomicalposition.
DEFINITIVE ADJUNCTIVE
PAIR &
MODIFICATIONS

3. ANTIPARASITICTHERAPY
üFor definitive treatment or adjunctive to surgery or
PAIR
üAlbendazole - primary drug
• poorly absorbed
• Ingested with fatty meal
• 15 mg/kg/day, divided into two doses, to maximum 400 mg
orally twice daily
üMebendazole (40-50mg/kg/day in three divided doses)
üPraziquantel (40 mg/Kg/wk)

4. DEFINITIVETREATMENT
• Initial management with drug treatment alone - CE1
and CE3a cysts (<5 cm)
Optimalduration–uncertain;1-3monthsmaybeappropriate,(up
to6months)
• Other indications:
ümultiplelivercysts <5cm
ücysts deep in liver parenchyma that are not amenable to
percutaneous treatment,
üperitonealcysts.

5. ADJUNCTIVETHERAPY
• Adjunctive to surgery and percutaneous treatment.
• Perioperative therapy reduces the risk of recurrent disease
by inactivating protoscolices and softens the cyst,
facilitating removal.
Theoptimaldurationof treatment- Uncertain
ØAt least 7 days ( WHO :4-30days) prior to surgery and
should be continued for at least 1 month (albendazole)
or3months(mebendazole)followingsurgery.
• For spontaneous cyst rupture

6. MONITORING :
CBC, LFT ( twice weekly X 3 months f/b
monthly )
Adverse effects of Albendazole
üReversiblehepatotoxicity(1to5%)
üCytopenia(<1%)
üalopecia(<1%)
üIncreased levels of aminotransferases ( 5X upper limit –
Alternativetreatment)
üAgranulocytosis
üDizziness,headache,vomiting,andrash
• Albendazole -avoided during pregnancy – risk of
potential teratogenicity

7. PERCUTANEOUSMANAGEMENT
• CE1andCE3a
• Cysts - difficult to drain or
tendtorelapseafterPAIR
• CE2andCE3bcysts
Destroy the germinal layer
with scolicidal agents —
PAIR
Evacuatingtheentire
cyst with large bore
catheter.

8. PAIR
• Less invasive (diagnosticandtherapeutic)
• Cure rate among appropriately selected patients >95 %
• Presence of daughter cysts - precludes use of PAIR for
definitive management
• dogmatically contraindicated for fear of spillage,
dissemination, and life-threatening anaphylactic reaction.
AJRAmJRoentgenol.1999;172(1):91
ActaTrop.1997;64(1-2):95
ClinInfectDis.1995;21(6):1372.

9. TECHNIQUE
• Ultrasound or CT guidance
• Puncture Aspiration (fluid evaluation for protoscolices)
Injection of the protoscolicidal agent.
Reaspiration (after at least 10 to 15 minutes)
Before injection of protoscolicidal agent, a radio opaque contrast is injected to
seecavity &CBC
Protoscolices in the reaspirated fluid should prompt repeat protoscolicidal
injection
• Adjunctive drug therapy - at least 4 hours prior to PAIR
Albendazole continued for 1 month & mebendazole for 3 months after the
procedure

10. Indications
ü Inoperable patient
ü Refusessurgery
ü For primary treatment of CE1
and CE3a , following relapse
after medical therapy or
surgery
ü Infectedcysts
ü Multiple cysts of > 5cm in
differentliversegments
ü Pregnant women ( except
earlypregnancy)
ü Children<3years
Contraindications
ü Nondrainable material or
echogenicfoci
ü Superficial cyst at risk of
rupture
ü Rupturedintoperitoneum
ü CystwithCBC
ü Inactiveorcalcifiedcyst
ü Multiplesepta/divisions
ü inaccessiblecysts
ü Mostcystsinlung,bone

11. RISKS OFPAIR
• Spillage of contents
• Chemical sclerosing cholangitis
• Biliary fistula (6%)
• Local recurrence (3%)
• Bleeding and infection (4%)
• Fever and urticaria (11-13%)
• Anaphylaxis - reduced with fine needles and
catheters and advances in imaging (0.5%)
Endoscopy can be useful both before
and after PAIR to evaluate for cyst
communication with the biliary tree

12. PROTOSCOLICIDALAGENTS
AGENTS CONC. DURATION
HYPERTONIC SALINE 5% 180 min
HYPERTONIC SALINE 10% 30min
HYPERTONIC SALINE 15,20,30% 10 min
ALCOHOL 70% 10 min
POVIDONE IODINE 10% 10min
FORMALIN 10% 10min
CHLOROHEXIDINE GLUCONATE 5% 10min
CETAVALON - 5min
CEVALAX - 5min
HYDROGEN PEROXIDE 3% 10 min
Complications:
• acute toxic reaction
• anaphylaxis
• hypernatremia
• air embolism
• metabolic acidosis
• Unconsciousness
coma, and death
• peritoneal adhesions
• methemoglobinemia
• caustic sclerosing
cholangitis( formalin)

13. PAIRmodifications
Ø Complicatedcysts,Cysts withmanydaughtercysts,Largevolume
cysts
1. Pair catheterisation technique
2. D-PAI (double-puncture, aspiration, and injection) technique
3. Percutaneous evacuation of cyst content (PEVAC) technique
4. Modified catheter aspiration technique (MoCAT)
Heterogeneouspattern -in2to4months
Pseudotumor–in 4to8months
Cystobliteration-in9to12months.
AFTERPAIR
USG

14. Adjunctive therapy
The optimal duration of
treatment- Uncertain
Ø At least 7 days ( WHO :4-
30days) prior to surgery
and should be continued
for at least 1 month
(albendazole) or 3 months
(mebendazole) following
surgery.
SURGERY-indications
üComplicated cysts
üNot suitable for percutaneous treatment
(CE2 and CE3b)
üp >10 cm,
üsuperficial cyst at risk of rupture
üExtrahepatic disease (lung, bone, brain,
kidney etc.)
üPercutaneous treatment not available.
SURGERY–goldstandard

15. OPENSURGERY
Rationale for surgical management
• surgery - most effective treatment applicable to all
cysts,
• Removes parasite
• Manages CBC
• Manages the cystic cavity (conservative surgery)
• Acceptable morbidity and mortality rate
• Low recurrence rate.
CONSERVATIVE
PROCEDURE
RADICAL
PROCEDURE

16. Conservativesurgicalprocedures
Partial pericystectomy
Rationale
• All cysts can be treated
• Safely by general surgeons, HPB surgeon– not necessary
• Parenchyma is not entered
• No special equipment
• Applicable to multiple B/L cysts
• Morbidity, mortality, and recurrence rates are acceptable
• Cost effective
• T issue sparing(important in endemic areas)

17. ØGoals
üSafe & complete exposure of cyst
üSafe decompression of cyst
üSafe evacuation of cyst contents
üSterilisation of cyst
üManagement of CBC ( cystobiliary
communication) if present
üManagement of remaining cyst cavity

18. Technique
Surgicalapproach-individualized
• If feasible-removaloftheintactcyst
• If not feasible - protoscolicidal agent injected into the cyst &
surgical field should be protected with pads soaked in
protoscolicidalagentsbeforeremoval “walledoff”
Or
Cyst opened and sterilized with protoscolicidal agents, followed by
evacuationofcystcontentsandremovalofthepericystictissue.
• If spillage - peritoneum washed with hypertonic saline &
albendazole (3 to 6 months) and a brief course of
praziquantel(7days)

19. MANAGEMENTOFRESIDUALCYSTCAVITY
Partial cystopericystectomy - redundant portion of the pericyst is resected,
residual cavity is left behind
Subtotal pericystectomy - most of the pericyst is resected
A. The pericyst cavity left
open after oversewing
the rim
- small, shallow cyst
B. Simple closure of the
cyst cavity filled with
saline.
C. Introflexion (infolding)
of the rim of the pericyst
cavity

20. D. Flattening the rim of
the pericyst in shallow
cysts
E.Capitonnage F. Omentoplasty ( anterior ,
posterior )
Other procedures:
Simple cyst closure, Marsupilastion, External tube drainage, Partial
capitonnage + Omentoplasty, Roux-en Y cystojejunostomy (rare) ,
Myoplasty

22. MANAGEMENTOFCBC
• Suture (simple suture, suture with T-tube CBD drainage,
intralameral pericystectomy, and capitonnage)
• Internal drainage procedures (biliodigestive bypass,
transduodenal sphincterotomy, internal transfistular drainage +/-
transduodenal sphincteroplasty)
• External drainage procedures (bipolar drainage, cystobiliary
disconnection)
• Reconstructive procedures ( pericystojejunostomy,
intracavitary biliodigestive bypass, or bile duct repair)
• liver resection
number, type, site, size of the orifice, involved bile duct, patient status, condition of
liver & expertise of the surgeon.

23. Radicalsurgicalprocedures
• Open /closed cystectomy
• Total pericystectomy
• Near total cystectomy
• Sub-adventitial cystectomy
• Completion & total cystectopericystectomy
• Liver resection (anatomical / non-anatomical)
Rationale
• totalremovalofthecystandexocysts–lowestrecurrence
• Chemotherapy- unnecessary
• Biliaryfistulaandcavity-relatedcomplications- low
• Calcifiedcystscanberemoved
• Experthands- mortalityandmorbidity(low)

24. Hepatectomy - the en bloc resection of the
echinococcal cyst along with part of the normal
liver parenchyma.
Total cystectomy - resection of the
adventitial layer , laminated layer, & germinal
layer
Sub-total cystectomy - partial resection of
the adventitial layer & total resection of
laminated layer, & germinal layer
Schematicstructureoftheechinococcalcystanddifferentapproachesforsurgicalremoval

25. LAPROSCOPICSURGERY
Ø Laparoscopic marsupialisation of the
cyst (de-roofing) - removal of cyst
containing the endocyst along with
daughtercysts
Ø Cysto pericystectomy removing entire
cystintact(small,superficial,inleftlobe)
ü increasedriskofspillage(pneumoperitoneum)
ü successfulinthesettingofanteriorlylocatedcysts
Ø Exclusioncriteria
• Deepintraparenchymalcysts
• Posterior cysts (close to vena
cava)
• >3cyst
• Calcifiedwalls

27. WHO STAGE SIZE
PREFERRED
TREATMENT
ALTERNATE
TREATMENT
STAGE CE1
<5cm ALBENDAZOLE ALONE PAIR
>5cm ALBENDAZOLE + PAIR PAIR
STAGE CE2 Any
ALBENDAZOLE + EITHER
MODIFIED
CATHETERISATION OR
SURGERY
MODIFIED
CATHETERISATION
STAGE CE3a
<5cm ALBENDAZOLE ALONE PAIR
>5cm ALBENDAZOLE + PAIR PAIR
STAGE CE3b Any
ALBENDAZOLE + EITHER
MODIFIED
CATHETERISATION OR
SURGERY
MODIFIED
CATHETERISATION
STAGE CE4 Any OBSERVATION -
STAGE CE5 Any OBSERVATION -
Summary - CE

28. AE
Stratify according to FDG-PET/ MRI Findings
Metabolically active AE (MAAE)
Complete resection of AE lesion
Possible Impossible
Surgery +ABZ
Metabolically Inactive (MIAE)
Wait and watch
Signs of activity No signs of activity
ABZ
Yearly Follow up
PET-CT
Serology negative
for 2 years
Stop ABZ
Vascular
complications
ABZ
Biliary
complications
Bacterial
infection
Surgery ERCP and stenting Drainage

29. POSTOPERATIVECOMPLICATIONS
• Obstructive jaundice (echinococcal remnants in the biliary
tree)
• Development of an external biliary fistula (2-4 weeks
postop.)
• Sphincter of Oddi stenosis
• bile duct stricture
• sclerosing cholangitis (formalin)
• secondary infection of cyst cavity
• intraabdominal abscess
• spillage of cyst contents leading to secondary
echinococcosis and/or anaphylaxis.

30. Patientswithbiliarycomplications- ERCP
• Obstructive jaundice - Sphincterotomy - removal of
cysts and membranes
• Cholangitis - Nasobiliary drain - extraction of hydatid
cysts and membranes (with or without sphincterotomy)
• External biliary fistula - endoscopic biliary stenting ( 4-
6 weeks) ; sphincterotomy
• Sphincter of Oddi stenosis - sphincterotomy.
• Bile duct stricture - ERCP with balloon dilation and
placement of a temporary biliary stent

31. OUTCOME
Varies with stage of disease
1. Cysts may continue growing (1 to 50 mm per year)
2. persist with no change,
3. rupture spontaneously,
4. resolve completely.
5. Calcification
•usually 5 to 10 years to develop
•most commonly with hepatic cysts (rarely with pulmonary or
bone cysts)
•Total calcification of cyst wall- cyst may be nonviable.

32. FOLLOWUP
• Cystic echinococcosis can relapse years after treatment.
• Must be individualized according to patient and available
resources.
• Ultrasound or other imaging (CT or MRI) at 3-6 month intervals
until stable, followed by yearly
• Follow up up to 5 years , if radiographic findings are stable at
12, 24, and 36 months – upto 3 years
IMAGING

33. Effective therapy
•Complete disappearance
•Reduction in size and volume
•Increase in proportion of solid component
•Thickening and irregularity of the wall
•In multivesicular cysts, reduction in size / no. of daughter
cysts
Relapse
Development of new cysts,
Increase in cyst size or volume
Increase in liquid component of the cyst.

34. SEROLOGY
• Increase in titre in the majority within the first 3
months after surgery
• Specific IgG ELISA - most sensitive measure of
response to treatment
• All serologic tests - decreasing titers from 3 months
after surgery in patients without relapse.

35. REFERENCES