Accogli S

Angelini G

Aringhieri G

Bartolozzi C.

Bemi P

Bozzi E

Cervelli R

Lorenzoni G

Pancrazi F

Scalise P

English

Archivio della Ricerca - Università di Pisa

Case 9892 Giant hepatic echinococcus cyst: a case reportLorenzoni G, Cervelli R, Scalise P, Pancrazi F, Angelini G, Aringhieri G, Accogli S, Bemi P, BozziE, Bartolozzi C.  Abdominal Imaging Section: 2012, Nov. 21 Published: 24 year(s), femalePatient:Authors' InstitutionDepartment of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy.Clinical HistoryA 24-years-old female patient from Albania with hepatomegaly, vulvar and lower limbs venousvarixes, already subjected to saphenectomy, came to our Department complaining of worsening ofchronic symptoms, such as right-hypochondriac pain, early satiety, sickness, and vomiting.Sierological exams were negative.Imaging FindingsUS-scan revealed a huge cystic formation, occupying the major part of hepatic dome, dislocatingdown and left of the whole liver, elevating the diaphragm and compressing the inferior vena cava,suprahepatic veins and intrahepatic biliary ducts (Fig.1a). The cyst appeared uniloculated, withoutinternal septations, filled by anechoic fluid and surrounded by a three-layer wall (Fig.1b):-inner layer (germinal) finely granular hyperechoic;-intermediate layer (laminated membrane) hypo-anechoic;-outer layer (adventitial) hyperechoic.MR-scan (Fig.2) defined the exact edges of the lesion (maximum diameter 23cm; involvement ofVII, VIII, part of IV, V, VI segment). Post contrastographic phases didn't show any enhancement(Fig.3).After US-guided percutaneous drainage and alcoholisation, follow-up evaluation showed signs ofhepatic abscess therefore the patient underwent urgent CT-scan with iodine ionic contrastadministration through the drainage (Fig.4a). CT-examination showed a voluminous formationoccupying right hepatic lobe (12x9cm) within layer of parenchimal-density tissue and aircomponent (Fig.4b). Cyst-wall presented inhomogeneous contrast-enhancement (Fig.4c).The patient underwent laparoscopic treatment (Video 5).DiscussionEchinococcal cyst is a parasitic disease caused by the larvae of Echinoccocus, primarlyE.Granulosum (EG), charaterised by a worldwide distribution. Mediterranean areas, Africa, SouthAmerica, the Middle East, Australia and New Zealand are interested by an endemic distribution dueto the transmission of EG by means of the dog-sheep cycle [1]. EG has a vital cycle that needs twohosts: definitive (dog) and intermediate (most commonly sheeps or rarely humans). This zoonoticinfection is transferred to humans by ingestion of contaminated food by dog feces within parasite'seggs. Liver (>65%) and lung (25%) are the organs where humans mainly develop cystic disease [2].The echinococcal cyst is an evolution of a oncosphere (a stage of asexual reproduction cycle ofEG), coming from the bowel through mesenteric veins. This formation appears like a fluid-filled,spherical, unilocular cyst that consists of an inner germinal layer of cells supported by acharacteristic acellular, laminated membrane of variable thickness. Each cyst is surrounded by ahost-produced layer of granulomatous adventitial reaction [3].The clinical manifestations are frequently variable due to site, size, and condition of the cysts. Atfirst, patients are often asymptomatic because of the slow growth of the echinococcal cyst, until itssize might cause organ dislocation and dysfunction. In the hepatic involvement common signs andsymptoms include hepatomegaly (with eventual palpable mass), right hypocondriac pain, sicknessand vomiting [3]. Lung echinococcal cysts, the second most frequent localisation, are clinicallycharaterised by fever, cough and chest pain, simulating a typical bacterial pneumonia, eventuallywith typical radiographic findings, but unresponsive to empiric antibiotic treatment [2].Furthermore, the inner content, released if a cyst ruptures, causes allergic reactions ranging frommild to fatal anaphylaxis. Dissemination of protoscolices, floating in the cystic fluid, might result inmultiple secondary echinococcosis disease [3].Diagnosis requires an index of suspicion (endemic areas), typical radiographic findings and specificserological test, which has low sensitivity in extra-hepatic cases [2].Traditional radiography allows detection of echinococcal cysts in the lung, appearing like ahyperdense mass, eventually with cyst-wall calcifications; in other sites, US, MR and CT are usefulto identify the deep-seated lesions [3], showing single or multiple uniloculated cysts (EG), withoutinternal septations, filled by fluid and surrounded by the typical three-layer wall.Treatment is complicated and carries significant risks. Therapeutic options include the use ofantihelmintic drugs, surgery and medico-surgical procedures such as PAIR (puncture, aspirate,injection of a scolicide, re-aspirate) [4].Final DiagnosisHepatic echinococcus cystDifferential Diagnosis ListCongenital liver cysts, Polycystic liver disease, Post-traumatic cysts (haematoma or biloma),Abscess, Benign mucinous biliary cystadenoma, Cystic metastases, Malignant cystic neoplasm,Benign cysts, Cavitary tubercolosis, MycosisFiguresFigure 1 USUltrasound scan shows an unilocular anechoic cyst of 23cm in diameter. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Ultrasound scan shows the cyst appearing uniloculated, without internal septations, filled byanechoic fluid and surrounded by a three-layer wall. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Abdomen; Liver;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Figure 2 MRAxial T1-weighted FLASH (Fast Low Angle SHot) 2D image shows an ipointense lesion. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Axial T1-weighted fatsat FLASH 2D image. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Axial T2-weighted HASTE (Half Fourier Acquisition Single Shot Turbo Spin Echo) MRimage shows the lesion with an hyperintense fluid and a simple cystic appearance, withoutinternal septa or solid portions. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Sagittal T2-weighted HASTE MR image. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Figure 3 MR T1-weighted fat-sat images with contrastThe figure shows vascular arterial phase (A); parenchimal arterial phase (B); portal phase(C); delayed phase (D). There is no contrast-enhancement of the lesion. The pericyst wall ishighlighted during delayed phase. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Cysts; Figure 4 CTSagittal CT scan after iodine ionic contrast administration through the drainage. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: CT;  Procedure: Diagnostic procedure;  Special Focus: Abscess; Axial CT scan shows a voluminous formation occupying right hepatic lobe within layer ofparenchimal-density tissue (germinal membrane) and air component. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: CT;  Procedure: Diagnostic procedure;  Special Focus: Abscess; Axial CT scan after iodine ionic contrast administration shows an inhomogeneouscontrast-enhancement of the cyst wall (maximum ticktness, 25mm, in the cranial portion). © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: CT;  Procedure: Diagnostic procedure;  Special Focus: Abscess; Figure 5 videoLaparoscopic treatment. © Department of Diagnostic and Interventional Radiology, University Hospital of Pisa, Italy. Area of Interest: Liver;  Imaging Technique: Percutaneous;  Procedure: Surgery;  Special Focus: Cysts; MeSH [C06.552.664.272]Echinococcosis, HepaticHelminth infection of the liver caused by Echinococcus granulosus or Echinococcus multilocularis. [C01.908]ZoonosesDiseases of non-human animals that may be transmitted to man or may be transmitted from man tonon-human animals. [C01.539.830.025.020.455]Liver AbscessSolitary or multiple collections of pus within the liver. It is usually associated with systemicmanifestations of toxemia and clinical signs of disease in the right upper quadrant of the abdomen.It was known in ancient times to Hippocrates and Celsus. (Bockus, Gastroenterology, 4th ed,p3288-9) [C03.335.190.396]EchinococcosisAn infection caused by the infestation of the larval form of tapeworms of the genus Echinococcus.The liver, lungs, and kidney are the most common areas of infestation. [C03]Parasitic DiseasesInfections or infestations with parasitic organisms. They are often contracted through contact withan intermediate vector, but may occur as the result of direct exposure.References[1] Ishimitsu DN, Saouaf R, Kallman C, Balzer BL (2010) Best cases from the AFIP: renal hydatiddisease Radiographics 2010 Mar 30(2):334-7[2] Ben SS, Greenberg D (2011) Pneumonia unresponsive to treatment? A case report ofintrathoracic hydatid cyst Harefuah 2011 Dec 150(12):899-901, 936[3] Moro P, Schantz PM (2009) Echinococcosis: a review International Journal of InfectiousDiseases Volume 13, Issue 2 , Pages 125-133, March 2009[4] Bristow B N, Lee S, Shafir S, Sorvillo F (2012) Human Echinococcosis Mortality in the UnitedStates, 1990-2007 PLOS: Neglected Tropical Diseases February; 6(2): e1524CitationLorenzoni G, Cervelli R, Scalise P, Pancrazi F, Angelini G, Aringhieri G, Accogli S, Bemi P, BozziE, Bartolozzi C. (2012, Nov. 21)  Giant hepatic echinococcus cyst: a case report {Online}URL: http://www.eurorad.org/case.php?id=9892

Giant hepatic echinococcus cyst: a case report

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Giant hepatic echinococcus cyst: a case report

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