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LIVER HYDATID DISEASE




CLINICAL PRESENTATION & COMPLICATIONS
✓ Presenter : Gurmehar Singh Hundal (31)
✓ Moderators : Dr. Zahid Iqbal Mir


• Dr. Sanjay Gupta
• Initial phase of primary infection is always asymptomatic.


• Latent periods of > 50 years before symptoms arise have been reported.


• ~50 % of detected cases occur in asymptomatic patients


• Clinical presentation depends upon site, size, number, vitality, stage of
development of cyst


• Small and/or calcified cysts - asymptomatic


• Symptoms - due to mass effect, obstruction of blood or lymphatic flow,
complications such as rupture or secondary infections
• UNCOMPLICATED • COMPLICATED
• Cysts typically increase in diameter at a rate of 1-5 cm/year -
variable


• Almost any site of the body can be involved via primary inoculation/
secondary spread


Liver (2/3rd patients )


Lungs (25%)


Muscles, Bones, Kidneys, Brain, Spleen, Heart, Pancreas (small
proportion)


• Single organ involvement - 85 to 90 % (E.granulosus)


• There is presence of a single cyst in 70 % of the cases
• Majority (79.60%) - uncomplicated


• Clinical latency is judged by size of cyst, which is confirmed
intraoperatively


• Adults – rapidly develop clinical manifestations


• Children in endemic area with large cyst – “hydatid cachexia”


• Mc symptom - Pain in RUQ / epigastrium


• Mc sign – Palpable lump


• Right lobe involvement - 60 to 85 % of cases
• UNCOMPLICATED LIVER HYDATID CYST
• World J Surg.
2001;25:21-27
• •
•Pressure effects are initially vague -


✓ Nonspecific pain


✓ Cough


✓ Low grade fever


✓ Sensation of abdominal fullness.


•Significant symptoms are unusual - cyst < 10 cm diameter


•If the cysts become large –


✓Hepatomegaly +/- RUQ pain


✓Nausea and vomiting


✓Abdominal mass.


✓Chronic abdominal discomfort
• Based on study in which 1,333 consecutive patients operated for liver hydatidosis at the First
Surgical Clinic, University of Belgrade School of Medicine, during January 1963 to December
2014.
Symptoms Number Percent
Right upper quadrant


discomfort/pain
1033 77.49
Dyspepsia 506 37.40
Vomiting 294 22.05
Non specific fatigue 246 18.45
Asymptomatic 238 17.85
Weight loss 151 11.32
History of jaundice 94 7.05
History of fever 87 6.52
Allergy 11 0.82
Sign Number Percent
Palpable right upper


quadrant mass
541 40.58
Fever 97 7.27
Jaundice 59 4.42
Malnutrition 33 2.47
Pleural effusion 36 2.57
Cholangitis 15 1.12
Splenomegaly 14 1.05
Skin rash 11 0.82
Dyspnea, asthma 33 2.47
Ascites 11 0.82
Edema of extremities 6 0.45
Pancreatitis 1 0.07
• Pressure or mass effects


• Suppuration & Secondary bacterial infection


• Rupture & Bile duct communication


✓Obscure (internal) rupture


✓Free rupture


( Intraperitoneal. & Intrathoracic rupture )


✓Communicant rupture


( Intrabiliary, & Rupture into adjacent vessels, organs )
• COMPLICATED LIVER HYDATID CYST
•
•
• Pressure or mass effect on the bile ducts, portal
and hepatic veins, or the inferior vena cava


1. Cholestasis


2. Obstructive jaundice


3. Portal hypertension


4. Venous obstruction


5. Budd-Chiari syndrome.
• Cyst leak is a prerequisite for bacterial contamination and Mc
cause of infection in cystobiliary communication


• Incidence in literature: 11.0 % - 27.1 % of cases.


• The incidence of hepatic hydatid cyst superinfection is up to 24%,
in surgically managed cases.


• Clinical presentation – like pyogenic liver abscess


• Any structural changes in cyst causes death of parasite


• CT - modality of choice for showing infected liver hydatid cysts.
Suppuration & Secondary bacterial infection
• Aseptic necrosis of hydatid cyst - Parasite dies and cyst is filled with amorphous yellow debris
Axial CECT of a 23 year old male that presented with
fever, pain and leukocytosis
• Superficial, large, thin walled cysts located along the inferior or anterior
surface of liver are more prone


• Rupture can occur


1. Spontaneously or,


2. After a minor or blunt abdominal trauma.


3. Previously surgically managed disease (spillage of cyst fluid during
surgery)


• Secondary to these, the hydatid cyst rupture into the peritoneal cavity


• Peritoneal hydatid disease is reported in up to 13% of patients.
(Secondary peritoneal hydatidosis - peritoneal hydatid disease occupying
most of the peritoneal cavity as a multiloculated mass )


Intraperitoneal Rupture
1. Acute symptomatic rupture (1-4%)


Peritoneal irritation and acute abdominal symptoms occur


2. Anaphylactic shock


Severe circulatory collapse; masks abdominal manifestations


3. Silent rupture


Disseminated peritoneal hydatidosis; unaware of rupture


4. Herniation of laminated membranes


Dumbbell hepatoperitoneal cyst; no rupture actually; mimics ascitis


Allergic manifestation can occur if aspiration tried
Clinical presentation of intraperitoneal rupture
•
Metastatic hydatidosis : implantation of scolices in other abdominal
organs following the intraperitoneal seeding
Axial (A & B) Coronal (C) Sagittal (D) CECTs of a 75-year-old
female patient known to have a liver cyst presents with abdominal
pain and shortness of breath.
• Diaphragmatic or transdiaphragmatic thoracic involvement -
0.16 % - 16 % of cases.


• Early manifestations may include an elevated hemidiaphragm
and a sterile sympathetic pleural effusion


• Upward extension of a subdiaphragmatic cyst is usually
asymptomatic, but sometimes can cause dry cough, dyspnea,
chest pain, and toxemia


• Right hepatic lobe posterior segments and the bare area of the
liver are the common routes of hepatic hydatid
transdiaphragmatic extension
Intrathoracic rupture
• Frank intrapleural rupture with empyema (hydatopiothorax) is rare


• The pleura and adherent basal lung segments often become
inflamed and indurated


• Infection + pressure = destruction of lung parenchyma =
pneumonitis or lung abscess.


• Rupture into bronchiole causes expectoration of daughter cysts in
the sputum


• Expectoration of bile tinged sputum is a sign of bronchobiliary
fistula
•
• Surgical grades of diaphragmatic or transdiaphragmatic thoracic
involvement in hepatic hydatid disease :


• Grade 1: Firm adherence between diaphragm and cyst surface without
diaphragmatic perforation


• Grade 2: Cyst perforates the diaphragm, little invasion of the thoracic cavity


• Grade 3: Cyst perforation through the diaphragm with either cyst growth inside
the thoracic cavity or daughter vesicle formation


• Grade 4: Disease of the lung parenchyma by either cyst connection with the
bronchial tree or compression and atelectasis of the lung parenchyma


• Grade 5: Establishment of a bronchobiliary fistula
• Axial CECT of a 77-year-male patient with
transdiaphragmatic rupture of hepatic hydatid cyst
A bronchobiliary fistula seen post percutaneous
drainage (white arrow)
At point of contact - cyst wall and bile duct weakens


Increased intraductal pressure - fissures in the duct
wall.


• Enlargement of the HC
• Bile duct compression
• Bile stasis - Jaundice
•
•
Intrabiliary rupture
•
• Bile escapes through this breach,
decompressing the bile duct and
accumulating at the external side of
the laminated membrane (internal
rupture).


• This "leak" causes changes in
osmotic pressure and a further
increase in intracystic pressure that
facilitates rupture into the bile duct
(external rupture)
✓RHD – most commonly affected, LHD and confluence - less
frequently affected .


✓Overall incidence 2.6-30%


✓In 60% of the cases of hydatid cyst, complications occur due to
biliary rupture


Silent rupture - minor communications


• 80% to 90% of bile duct ruptures


• Bile leaks from eroded small ducts into the cyst, causing endogenic
vesiculation, suppuration, and eventually death of the parasite.


• Sometimes can cause unexpected postoperative bile leakage
Symptomatic rupture – major communication (fistula orifice
diameter of >5 mm )


• 3% to 10% cases


• Rupture into a large bile duct - spontaneous cure or
cholestatic jaundice with recurrent cholangitis.


• Incomplete emptying and a persisting communication -
secondary infection.


• Anaphylaxis
• Rupture into adjacent organs
✓Into GIT – Hydatidemesis , Hydatidenterica


✓Into Urinary tract – Hydatiduria


✓Into Aorta, IVC, pericystic vessels, Heart – Embolism
Triad of symptomatic rupture


• Biliary colic


• Partial intermittent or complete ductal obstruction with
cholangitis and jaundice


• Germinative membranes in the feces.
(A) Coronal CECT and (B) Coronal MRI of a 52-year-old male patient with a history of
hepatic hydatid disease presented with abdominal pain.
• Coronal T2-weighted MR image confirming the intrahepatic and
extrahepatic biliary dilatation caused by passage and impaction of the
hydatid membranes in the common bile duct (black arrow).
Lung involvement
• 25% of cases of Hepatic Echinococcosis have lung
involvement


• 60 % of pulmonary hydatid disease affects right lung, and 50 -
60 % of cases involve lower lobes


• 20 % of patients with lung cysts also have liver cysts


• Lung : Liver involvement is higher in children than in adults
• Mc symptoms described


✓ Cough (53 - 62 %)


✓ Chest pain (49 - 91 %)


✓ Dyspnea (10 - 70 %)


✓ Hemoptysis (12 - 21 %).
• Less frequent symptoms


✓ Malaise


✓ Nausea


✓ Vomiting


✓ thoracic deformations
Involvement of other organs
• Heart - Mechanical rupture with widespread dissemination or
pericardial tamponade


• CNS – Seizures or signs of raised intracranial pressure


• Spinal cord - Cord compression


• Kidney - Hematuria or flank pain.


• Bone - Usually asymptomatic until a pathologic fracture develops


• Eye - Decreased visual acuity, blindness, and exophthalmos


•
Subcutaneous cysts
ALVEOLAR ECHINOCOCCOSIS ( E. MULTILOCULARIS)
• Mc symptoms are non specific and include malaise, weight loss, and
right upper quadrant discomfort


• Extrahepatic primary disease is very rare (1 % of cases).


• Multiorgan disease - 13 % of cases in one series in which
metacestodes involved the lungs, spleen, or brain in addition to the
liver (via hematogenous and lymphatic spread)


• Immunodeficiency may accelerate manifestations


• If left untreated, > 90 % of patients will die within 10 years of the
onset of clinical symptoms, and virtually 100 % will die by 15 years


• Lancet. 2003;362(9392):1295.
• Gastroenterol Clin North Am. 1996;25(3):655.
INVOLVEMENT OF ATYPICAL SITES
Axial T2WI (MRI) showing hyper intense, multicystic lesion with multiple
daughter cysts in relation to the right seminal vesicle
Abdominal CECT – A – Sagittal plane depicting a large multi-septate cystic lesion in the
pouch of Douglas splaying and displacing the uterus anteriorly; B – Coronal plane
depicting the hydronephrosis with dilated ureter (white arrowhead).
•
•
Presentations in Emergency
•Severe abdominal pain following minor
trauma


•Signs of peritoneal irritation (rebound
tenderness, guarding and rigidity) may
be present


•Allergic reactions


•
Skin rash


•
Anaphylactic shock if cyst ruptures
spontaneously, due to trauma or
surgery.
•
•
History
•Detailed surgical history


•History of living in or visiting
an endemic area?


•Exposure to the parasite
through the ingestion of foods
or water contaminated by the
feces of a definitive host
should be established by
asking relevant questions.
•
Examination
• Skin


• Yellowish discolouration


• Urticaria and erythema


• Spider angiomas - Due to portal
hypertension secondary to either biliary
cirrhosis or obstruction of the inferior
vena cava.


• Vital signs


• Fever - Secondary infection or
allergic reaction.


• Hypotension - anaphylaxis secondary
to a cyst leak
•
•Abdomen


• Hepatomegaly or mass


• Abdominal tenderness


•Signs of peritoneal irritation
(rebound tenderness, guarding
and rigidity) may be present


• Splenomegaly - splenic
echinococcosis or portal
hypertension.


• Ascites (rare)


• Hydatid thrill (rare)


•Lungs


• Decreased breath sounds
over the affected area
• REFERENCES

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HYDATID CYST.02

  • 1. LIVER HYDATID DISEASE 
 
 CLINICAL PRESENTATION & COMPLICATIONS ✓ Presenter : Gurmehar Singh Hundal (31) ✓ Moderators : Dr. Zahid Iqbal Mir • Dr. Sanjay Gupta
  • 2. • Initial phase of primary infection is always asymptomatic. • Latent periods of > 50 years before symptoms arise have been reported. • ~50 % of detected cases occur in asymptomatic patients • Clinical presentation depends upon site, size, number, vitality, stage of development of cyst • Small and/or calcified cysts - asymptomatic • Symptoms - due to mass effect, obstruction of blood or lymphatic flow, complications such as rupture or secondary infections • UNCOMPLICATED • COMPLICATED
  • 3. • Cysts typically increase in diameter at a rate of 1-5 cm/year - variable • Almost any site of the body can be involved via primary inoculation/ secondary spread Liver (2/3rd patients ) Lungs (25%) Muscles, Bones, Kidneys, Brain, Spleen, Heart, Pancreas (small proportion) • Single organ involvement - 85 to 90 % (E.granulosus) • There is presence of a single cyst in 70 % of the cases
  • 4. • Majority (79.60%) - uncomplicated • Clinical latency is judged by size of cyst, which is confirmed intraoperatively • Adults – rapidly develop clinical manifestations • Children in endemic area with large cyst – “hydatid cachexia” • Mc symptom - Pain in RUQ / epigastrium • Mc sign – Palpable lump • Right lobe involvement - 60 to 85 % of cases • UNCOMPLICATED LIVER HYDATID CYST • World J Surg. 2001;25:21-27
  • 5. • • •Pressure effects are initially vague - ✓ Nonspecific pain ✓ Cough ✓ Low grade fever ✓ Sensation of abdominal fullness. •Significant symptoms are unusual - cyst < 10 cm diameter •If the cysts become large – ✓Hepatomegaly +/- RUQ pain ✓Nausea and vomiting ✓Abdominal mass. ✓Chronic abdominal discomfort
  • 6. • Based on study in which 1,333 consecutive patients operated for liver hydatidosis at the First Surgical Clinic, University of Belgrade School of Medicine, during January 1963 to December 2014. Symptoms Number Percent Right upper quadrant discomfort/pain 1033 77.49 Dyspepsia 506 37.40 Vomiting 294 22.05 Non specific fatigue 246 18.45 Asymptomatic 238 17.85 Weight loss 151 11.32 History of jaundice 94 7.05 History of fever 87 6.52 Allergy 11 0.82 Sign Number Percent Palpable right upper quadrant mass 541 40.58 Fever 97 7.27 Jaundice 59 4.42 Malnutrition 33 2.47 Pleural effusion 36 2.57 Cholangitis 15 1.12 Splenomegaly 14 1.05 Skin rash 11 0.82 Dyspnea, asthma 33 2.47 Ascites 11 0.82 Edema of extremities 6 0.45 Pancreatitis 1 0.07
  • 7. • Pressure or mass effects • Suppuration & Secondary bacterial infection • Rupture & Bile duct communication ✓Obscure (internal) rupture ✓Free rupture ( Intraperitoneal. & Intrathoracic rupture ) ✓Communicant rupture ( Intrabiliary, & Rupture into adjacent vessels, organs ) • COMPLICATED LIVER HYDATID CYST
  • 8. • • • Pressure or mass effect on the bile ducts, portal and hepatic veins, or the inferior vena cava 1. Cholestasis 2. Obstructive jaundice 3. Portal hypertension 4. Venous obstruction 5. Budd-Chiari syndrome.
  • 9. • Cyst leak is a prerequisite for bacterial contamination and Mc cause of infection in cystobiliary communication • Incidence in literature: 11.0 % - 27.1 % of cases. • The incidence of hepatic hydatid cyst superinfection is up to 24%, in surgically managed cases. • Clinical presentation – like pyogenic liver abscess • Any structural changes in cyst causes death of parasite • CT - modality of choice for showing infected liver hydatid cysts. Suppuration & Secondary bacterial infection • Aseptic necrosis of hydatid cyst - Parasite dies and cyst is filled with amorphous yellow debris
  • 10. Axial CECT of a 23 year old male that presented with fever, pain and leukocytosis
  • 11. • Superficial, large, thin walled cysts located along the inferior or anterior surface of liver are more prone • Rupture can occur 1. Spontaneously or, 2. After a minor or blunt abdominal trauma. 3. Previously surgically managed disease (spillage of cyst fluid during surgery) • Secondary to these, the hydatid cyst rupture into the peritoneal cavity • Peritoneal hydatid disease is reported in up to 13% of patients. (Secondary peritoneal hydatidosis - peritoneal hydatid disease occupying most of the peritoneal cavity as a multiloculated mass ) Intraperitoneal Rupture
  • 12. 1. Acute symptomatic rupture (1-4%) Peritoneal irritation and acute abdominal symptoms occur 2. Anaphylactic shock Severe circulatory collapse; masks abdominal manifestations 3. Silent rupture Disseminated peritoneal hydatidosis; unaware of rupture 4. Herniation of laminated membranes Dumbbell hepatoperitoneal cyst; no rupture actually; mimics ascitis Allergic manifestation can occur if aspiration tried Clinical presentation of intraperitoneal rupture • Metastatic hydatidosis : implantation of scolices in other abdominal organs following the intraperitoneal seeding
  • 13. Axial (A & B) Coronal (C) Sagittal (D) CECTs of a 75-year-old female patient known to have a liver cyst presents with abdominal pain and shortness of breath.
  • 14. • Diaphragmatic or transdiaphragmatic thoracic involvement - 0.16 % - 16 % of cases. • Early manifestations may include an elevated hemidiaphragm and a sterile sympathetic pleural effusion • Upward extension of a subdiaphragmatic cyst is usually asymptomatic, but sometimes can cause dry cough, dyspnea, chest pain, and toxemia • Right hepatic lobe posterior segments and the bare area of the liver are the common routes of hepatic hydatid transdiaphragmatic extension Intrathoracic rupture
  • 15. • Frank intrapleural rupture with empyema (hydatopiothorax) is rare • The pleura and adherent basal lung segments often become inflamed and indurated • Infection + pressure = destruction of lung parenchyma = pneumonitis or lung abscess. • Rupture into bronchiole causes expectoration of daughter cysts in the sputum • Expectoration of bile tinged sputum is a sign of bronchobiliary fistula
  • 16. • • Surgical grades of diaphragmatic or transdiaphragmatic thoracic involvement in hepatic hydatid disease : • Grade 1: Firm adherence between diaphragm and cyst surface without diaphragmatic perforation • Grade 2: Cyst perforates the diaphragm, little invasion of the thoracic cavity • Grade 3: Cyst perforation through the diaphragm with either cyst growth inside the thoracic cavity or daughter vesicle formation • Grade 4: Disease of the lung parenchyma by either cyst connection with the bronchial tree or compression and atelectasis of the lung parenchyma • Grade 5: Establishment of a bronchobiliary fistula
  • 17. • Axial CECT of a 77-year-male patient with transdiaphragmatic rupture of hepatic hydatid cyst
  • 18. A bronchobiliary fistula seen post percutaneous drainage (white arrow)
  • 19. At point of contact - cyst wall and bile duct weakens Increased intraductal pressure - fissures in the duct wall. • Enlargement of the HC • Bile duct compression • Bile stasis - Jaundice • • Intrabiliary rupture •
  • 20. • Bile escapes through this breach, decompressing the bile duct and accumulating at the external side of the laminated membrane (internal rupture). • This "leak" causes changes in osmotic pressure and a further increase in intracystic pressure that facilitates rupture into the bile duct (external rupture)
  • 21. ✓RHD – most commonly affected, LHD and confluence - less frequently affected . ✓Overall incidence 2.6-30% ✓In 60% of the cases of hydatid cyst, complications occur due to biliary rupture Silent rupture - minor communications • 80% to 90% of bile duct ruptures • Bile leaks from eroded small ducts into the cyst, causing endogenic vesiculation, suppuration, and eventually death of the parasite. • Sometimes can cause unexpected postoperative bile leakage
  • 22. Symptomatic rupture – major communication (fistula orifice diameter of >5 mm ) • 3% to 10% cases • Rupture into a large bile duct - spontaneous cure or cholestatic jaundice with recurrent cholangitis. • Incomplete emptying and a persisting communication - secondary infection. • Anaphylaxis
  • 23. • Rupture into adjacent organs ✓Into GIT – Hydatidemesis , Hydatidenterica ✓Into Urinary tract – Hydatiduria ✓Into Aorta, IVC, pericystic vessels, Heart – Embolism Triad of symptomatic rupture • Biliary colic • Partial intermittent or complete ductal obstruction with cholangitis and jaundice • Germinative membranes in the feces.
  • 24. (A) Coronal CECT and (B) Coronal MRI of a 52-year-old male patient with a history of hepatic hydatid disease presented with abdominal pain.
  • 25. • Coronal T2-weighted MR image confirming the intrahepatic and extrahepatic biliary dilatation caused by passage and impaction of the hydatid membranes in the common bile duct (black arrow).
  • 26. Lung involvement • 25% of cases of Hepatic Echinococcosis have lung involvement • 60 % of pulmonary hydatid disease affects right lung, and 50 - 60 % of cases involve lower lobes • 20 % of patients with lung cysts also have liver cysts • Lung : Liver involvement is higher in children than in adults • Mc symptoms described ✓ Cough (53 - 62 %) ✓ Chest pain (49 - 91 %) ✓ Dyspnea (10 - 70 %) ✓ Hemoptysis (12 - 21 %). • Less frequent symptoms ✓ Malaise ✓ Nausea ✓ Vomiting ✓ thoracic deformations
  • 27. Involvement of other organs • Heart - Mechanical rupture with widespread dissemination or pericardial tamponade • CNS – Seizures or signs of raised intracranial pressure • Spinal cord - Cord compression • Kidney - Hematuria or flank pain. • Bone - Usually asymptomatic until a pathologic fracture develops • Eye - Decreased visual acuity, blindness, and exophthalmos • Subcutaneous cysts
  • 28. ALVEOLAR ECHINOCOCCOSIS ( E. MULTILOCULARIS) • Mc symptoms are non specific and include malaise, weight loss, and right upper quadrant discomfort • Extrahepatic primary disease is very rare (1 % of cases). • Multiorgan disease - 13 % of cases in one series in which metacestodes involved the lungs, spleen, or brain in addition to the liver (via hematogenous and lymphatic spread) • Immunodeficiency may accelerate manifestations • If left untreated, > 90 % of patients will die within 10 years of the onset of clinical symptoms, and virtually 100 % will die by 15 years • Lancet. 2003;362(9392):1295. • Gastroenterol Clin North Am. 1996;25(3):655.
  • 30. Axial T2WI (MRI) showing hyper intense, multicystic lesion with multiple daughter cysts in relation to the right seminal vesicle
  • 31.
  • 32. Abdominal CECT – A – Sagittal plane depicting a large multi-septate cystic lesion in the pouch of Douglas splaying and displacing the uterus anteriorly; B – Coronal plane depicting the hydronephrosis with dilated ureter (white arrowhead).
  • 33.
  • 34.
  • 35. • • Presentations in Emergency •Severe abdominal pain following minor trauma •Signs of peritoneal irritation (rebound tenderness, guarding and rigidity) may be present •Allergic reactions • Skin rash • Anaphylactic shock if cyst ruptures spontaneously, due to trauma or surgery.
  • 36. • • History •Detailed surgical history •History of living in or visiting an endemic area? •Exposure to the parasite through the ingestion of foods or water contaminated by the feces of a definitive host should be established by asking relevant questions.
  • 37. • Examination • Skin • Yellowish discolouration • Urticaria and erythema • Spider angiomas - Due to portal hypertension secondary to either biliary cirrhosis or obstruction of the inferior vena cava. • Vital signs • Fever - Secondary infection or allergic reaction. • Hypotension - anaphylaxis secondary to a cyst leak
  • 38. • •Abdomen • Hepatomegaly or mass • Abdominal tenderness •Signs of peritoneal irritation (rebound tenderness, guarding and rigidity) may be present • Splenomegaly - splenic echinococcosis or portal hypertension. • Ascites (rare) • Hydatid thrill (rare) •Lungs • Decreased breath sounds over the affected area