Diagnostic Ultrasound - Abdomen and Pelvis
Hepatic Echinococcus Cyst TERMINOLOGY Synonyms • Echinococcal or hydatid disease; echinococcosis Definitions • Infection of humans caused by larval stage of Echinococcus granulosus or Echinococcus multilocularis IMAGING General Features • Best diagnostic clue ○ Membranes ± daughter cysts in complex heterogeneous mass • Location: Right lobe > left lobe of liver • Size: Variable, average 5 cm (max to 50 cm) ○ May contain up to 15 liters of fluid • Key concepts ○ E. granulosus: Most common form of hydatid disease, unilocular form – Up to 60% of cysts are multiple ○ E. multilocularis (alveolaris): Less common but aggressive form ○ Most common sites: Liver and lungs Ultrasonographic Findings • Grayscale ultrasound ○ Variable manifestations based on stage of evolution and maturity ○ Lewall classification of hydatid lesions – Cyst with hydatid sand and no internal architecture – Ruptured cyst with detached endocyst – Cyst with matrix ± daughter cysts – Calcified mass ○ E. granulosus – Anechoic cyst with double echogenic lines separated by hypoechoic layer – Honeycombed cyst, multiple septations between daughter cysts in mother cyst – Detachment of endocyst from pericyst (partial or complete) results in varied appearances – Undulating floating membrane within cyst – "Water lily" sign: Complete detachment of membrane – "Snowstorm pattern": Anechoic cyst with internal debris, hydatid sand – Dilated IHDs due to compression by cysts ○ E. multilocularis – Single/multiple echogenic lesions – Irregular necrotic regions and microcalcifications – Ill-defined infiltrative solid masses – Tend to spread to liver hilum – Invasion of inferior vena cava and diaphragm – Evaluate lung, heart, and brain for deposits ○ US used to monitor efficacy of antihydatid therapy – Positive response findings include □ Reduction in cyst size □ Endocyst detachment □ Progressive increase in cyst echogenicity □ Mural calcification Radiographic Findings • Radiography ○ E. granulosus: Curvilinear or ring-like pericyst calcification – Seen in 20-30% of abdominal plain films ○ E. multilocularis: Microcalcifications in 50% of cases • Endoscopic retrograde cholangiopancreatography (ERCP) ○ Hydatid cyst may communicate with biliary tree – Right hepatic duct 55%; left hepatic duct 29%, common hepatic duct 9%, gallbladder 6%, common bile duct 1% CT Findings • NECT ○ E. granulosus – Large unilocular/multilocular well-defined hypodense cysts – Contains multiple peripheral daughter cysts of less density than mother cyst – Curvilinear ring-like calcification – Calcified wall: Usually indicates no active infection if completely circumferential – Dilated intrahepatic bile duct: Due to compression/rupture of cyst into bile ducts ○ E. multilocularis – Extensive, infiltrative cystic and solid masses of low density (14-40 HU) – Margins are irregular/ill defined – Amorphous type of calcification – Can simulate primary or secondary tumor • CECT ○ Enhancement of cyst wall and septations MR Findings • T1WI ○ Rim (pericyst): Hypointense (fibrous component) ○ Mother cyst (hydatid matrix) – Usually intermediate signal intensity – Rarely hyperintense: Due to reduction in water content ○ Daughter cysts: Less signal intensity than mother cyst (matrix) ○ Floating membrane: Low signal intensity ○ Calcifications: Difficult to identify on MR images – Display low signal on both T1- and T2WI • T2WI ○ Rim (pericyst): Hypointense (fibrous component) ○ 1st echo T2WI: Increased signal intensity – Mother cysts more than daughter cysts ○ Strong T2WI: Hyperintense – Mother and daughter cysts have same intensity ○ Floating membrane – Low to intermediate signal intensity • T1WI C+ ○ Enhancement of cyst wall and septations • MRCP ○ ± demonstrate communication with biliary tree Imaging Recommendations • Best imaging tool ○ US for diagnosis and follow-up http://radiologyebook.com/ Diagnoses: Liver 223
Hepatic Echinococcus Cyst 224 Diagnoses: Liver DIFFERENTIAL DIAGNOSIS Hemorrhagic or Infected Cyst • Complex cystic heterogeneous mass • Septations, fluid-levels, and mural nodularity • Calcification may or may not be seen Complex Pyogenic Abscess • "Cluster of grapes": Confluent complex cystic lesions "Cystic" Metastases • e.g., cystadenocarcinoma of pancreas or ovary • May present with debris, mural nodularity, rim enhancement Biliary Cystadenocarcinoma • Rare, multiseptated water density cystic mass • No surrounding inflammatory changes PATHOLOGY General Features • Etiology ○ Caused by larval stage of Echinococcus tapeworm – E. granulosus and E. multilocularis • Carried by sheep, transmitted to humans by dog or fox ○ Humans are incidental hosts • Larvae → portal vein → liver (75%) • E. granulosus ○ Develop into hydatid stage (4-5 days) within liver ○ Hydatid cysts grow to 1 cm during first 6 months, 2-3 cm annually • E. multilocularis ○ Larvae proliferate and penetrate surrounding tissue ○ Cause diffuse and infiltrative granulomatous reaction, simulating malignancy ○ Necrosis → cavitation → calcification Microscopic Features • Cyst fluid content: Antigenic, pale yellow, neutral pH • Endocyst: Gives rise to daughter vesicles/brood capsule, which may detach, form sediment, or produce daughter cysts • Ectocyst: Acellular substance secreted by parasite • Pericyst: Host response forming layer of granulation/fibrous tissue • Diagnosis ○ Serologic tests positive in more than 80% of cases ○ Percutaneous aspiration of cyst fluid – Danger of peritoneal spill and anaphylactic reaction Demographics • Age ○ Hydatid disease usually acquired in childhood ○ Not diagnosed until 30-40 years of age • Gender: M = F • Epidemiology ○ E. granulosus: Mediterranean region, Africa, South America, Australia, and New Zealand ○ E. multilocularis: France, Germany, Austria, USSR, Japan, Alaska, and Canada Natural History & Prognosis • Complications ○ Compression/infection or rupture into biliary tree ○ Rupture into peritoneal or pleural cavity ○ Spread of lesions to lungs, heart, brain, and bone • Prognosis ○ E. granulosus: Good ○ E. multilocularis: Fatal in 10-15 years untreated Treatment • E. granulosus ○ Medical: Albendazole/mebendazole ○ Direct injection of scolicidal agents ○ PAIR procedure: Puncture, aspiration, injection, respiration ○ Surgical: Segmental or lobar hepatectomy • E. multilocularis ○ Partial hepatectomy/hepatectomy + liver transplant DIAGNOSTIC CHECKLIST Consider • Rule out other complex or septate cystic liver masses ○ Biliary cystadenoma, pyogenic liver abscess, cystic metastases, and hemorrhagic or infected cyst ○ E. multilocularis imaging and clinical behavior simulates solid malignant neoplasm Image Interpretation Pearls • Daughter cysts can float freely within mother cyst ○ Altering patient's position may change position of daughter cysts CLINICAL ISSUES Presentation SELECTED REFERENCES • Most common signs/symptoms ○ Cysts: Initially asymptomatic – Symptomatic when size ↑/infected/ruptured ○ Pain, fever, jaundice, hepatomegaly ○ Allergic reaction; portal hypertension • Clinical profile ○ Middle-aged patient with right upper quadrant pain, palpable mass, jaundice • Lab data ○ Eosinophilia; ↑ serologic titers ○ ± ↑ alkaline phosphatase/gamma-glutamyl transpeptidase (GGTP) http://radiologyebook.com/ 1. Qian LJ et al: Spectrum of multilocular cystic hepatic lesions: CT and MR imaging findings with pathologic correlation. Radiographics. 33(5):1419-33, 2013 2. Li Q et al: Echinococcal cysts of the liver and spleen: complex hepatic and splenic cystic lesions. Ultrasound Q. 28(3):205-7, 2012 3. Marrone G et al: Multidisciplinary imaging of liver hydatidosis. World J Gastroenterol. 18(13):1438-47, 2012 4. Brunetti E et al: Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop. 114(1):1-16, 2010 5. Pedrosa I et al: Hydatid disease: radiologic and pathologic features and complications. Radiographics. 20(3):795-817, 2000
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Hepatic Echinococcus Cyst<br />
224<br />
Diagnoses: Liver<br />
DIFFERENTIAL DIAGNOSIS<br />
Hemorrhagic or Infected Cyst<br />
• Complex cystic heterogeneous mass<br />
• Septations, fluid-levels, <strong>and</strong> mural nodularity<br />
• Calcification may or may not be seen<br />
Complex Pyogenic Abscess<br />
• "Cluster of grapes": Confluent complex cystic lesions<br />
"Cystic" Metastases<br />
• e.g., cystadenocarcinoma of pancreas or ovary<br />
• May present with debris, mural nodularity, rim<br />
enhancement<br />
Biliary Cystadenocarcinoma<br />
• Rare, multiseptated water density cystic mass<br />
• No surrounding inflammatory changes<br />
PATHOLOGY<br />
General Features<br />
• Etiology<br />
○ Caused by larval stage of Echinococcus tapeworm<br />
– E. granulosus <strong>and</strong> E. multilocularis<br />
• Carried by sheep, transmitted to humans by dog or fox<br />
○ Humans are incidental hosts<br />
• Larvae → portal vein → liver (75%)<br />
• E. granulosus<br />
○ Develop into hydatid stage (4-5 days) within liver<br />
○ Hydatid cysts grow to 1 cm during first 6 months, 2-3 cm<br />
annually<br />
• E. multilocularis<br />
○ Larvae proliferate <strong>and</strong> penetrate surrounding tissue<br />
○ Cause diffuse <strong>and</strong> infiltrative granulomatous reaction,<br />
simulating malignancy<br />
○ Necrosis → cavitation → calcification<br />
Microscopic Features<br />
• Cyst fluid content: Antigenic, pale yellow, neutral pH<br />
• Endocyst: Gives rise to daughter vesicles/brood capsule,<br />
which may detach, form sediment, or produce daughter<br />
cysts<br />
• Ectocyst: Acellular substance secreted by parasite<br />
• Pericyst: Host response forming layer of<br />
granulation/fibrous tissue<br />
• Diagnosis<br />
○ Serologic tests positive in more than 80% of cases<br />
○ Percutaneous aspiration of cyst fluid<br />
– Danger of peritoneal spill <strong>and</strong> anaphylactic reaction<br />
Demographics<br />
• Age<br />
○ Hydatid disease usually acquired in childhood<br />
○ Not diagnosed until 30-40 years of age<br />
• Gender: M = F<br />
• Epidemiology<br />
○ E. granulosus: Mediterranean region, Africa, South<br />
America, Australia, <strong>and</strong> New Zeal<strong>and</strong><br />
○ E. multilocularis: France, Germany, Austria, USSR, Japan,<br />
Alaska, <strong>and</strong> Canada<br />
Natural History & Prognosis<br />
• Complications<br />
○ Compression/infection or rupture into biliary tree<br />
○ Rupture into peritoneal or pleural cavity<br />
○ Spread of lesions to lungs, heart, brain, <strong>and</strong> bone<br />
• Prognosis<br />
○ E. granulosus: Good<br />
○ E. multilocularis: Fatal in 10-15 years untreated<br />
Treatment<br />
• E. granulosus<br />
○ Medical: Albendazole/mebendazole<br />
○ Direct injection of scolicidal agents<br />
○ PAIR procedure: Puncture, aspiration, injection,<br />
respiration<br />
○ Surgical: Segmental or lobar hepatectomy<br />
• E. multilocularis<br />
○ Partial hepatectomy/hepatectomy + liver transplant<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• Rule out other complex or septate cystic liver masses<br />
○ Biliary cystadenoma, pyogenic liver abscess, cystic<br />
metastases, <strong>and</strong> hemorrhagic or infected cyst<br />
○ E. multilocularis imaging <strong>and</strong> clinical behavior simulates<br />
solid malignant neoplasm<br />
Image Interpretation Pearls<br />
• Daughter cysts can float freely within mother cyst<br />
○ Altering patient's position may change position of<br />
daughter cysts<br />
CLINICAL ISSUES<br />
Presentation<br />
SELECTED REFERENCES<br />
• Most common signs/symptoms<br />
○ Cysts: Initially asymptomatic<br />
– Symptomatic when size ↑/infected/ruptured<br />
○ Pain, fever, jaundice, hepatomegaly<br />
○ Allergic reaction; portal hypertension<br />
• Clinical profile<br />
○ Middle-aged patient with right upper quadrant pain,<br />
palpable mass, jaundice<br />
• Lab data<br />
○ Eosinophilia; ↑ serologic titers<br />
○ ± ↑ alkaline phosphatase/gamma-glutamyl<br />
transpeptidase (GGTP)<br />
http://radiologyebook.com/<br />
1. Qian LJ et al: Spectrum of multilocular cystic hepatic lesions: CT <strong>and</strong> MR<br />
imaging findings with pathologic correlation. Radiographics. 33(5):1419-33,<br />
2013<br />
2. Li Q et al: Echinococcal cysts of the liver <strong>and</strong> spleen: complex hepatic <strong>and</strong><br />
splenic cystic lesions. <strong>Ultrasound</strong> Q. 28(3):205-7, 2012<br />
3. Marrone G et al: Multidisciplinary imaging of liver hydatidosis. World J<br />
Gastroenterol. 18(13):1438-47, 2012<br />
4. Brunetti E et al: Expert consensus for the diagnosis <strong>and</strong> treatment of cystic<br />
<strong>and</strong> alveolar echinococcosis in humans. Acta Trop. 114(1):1-16, 2010<br />
5. Pedrosa I et al: Hydatid disease: radiologic <strong>and</strong> pathologic features <strong>and</strong><br />
complications. Radiographics. 20(3):795-817, 2000