Diagnostic Ultrasound - Abdomen and Pelvis

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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

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

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