Introduction. Splenic pathologies. Polysplenia syndrome. Anatomical variants 21/08/2017. Ultrasound of the forgotten organ of the abdomen: the spleen

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1 Ultrasound of the forgotten organ of the abdomen: the spleen Olivier Lucidarme Pitié Salpêtrière UPMC/Sorbonne University Paris France Malformations Introduction must look for other malformations frequently associated Indirect witness of more general diseases Specific spleen diseases Traumatism Organ the most involved in case of abdominal trauma Splenic pathologies Polysplenia syndrome Malformations must look for other malformations frequently associated Indirect witness of more general diseases Specific spleen diseases Traumatism Organ the most involved in case of abdominal trauma Polysplenia syndrome Multiple splenules + Heterotaxy syndrome or situs ambiguus or situs inversus Other characteristic features may include: congenital heart disease abnormal pulmonary venous return semi-annular pancreas / congenitally short pancreas midgut malrotation (80%) gallbladder agenesis (50%) renal agenesis intrahepatic IVC interruption with azygos/hemiazygos continuation preduodenal portal vein Accessory spleen : 40% Anatomical variants Same echogenicity of main spleen Moving with respiration = spleen differential diagnosis ADK of pancreatic tail (more hypo-echoic) Adrenal nodule (immobile) 1

2 Malformations Splenic pathologies must look for other malformations frequently associated Indirect witness of more general diseases Specific spleen diseases Traumatism Organ the most involved in case of abdominal trauma Homogeneous splenomegaly = general disease Mild Splenomegaly (< 700 g) Severe splenomegaly (> 700 g) Congestion Portal hypertension Venous thrombosis Heart failure Systemic infectious disease Infiltrative disease Acute leukemia Chronic lymphoid leukemia Thalasemia Lymphoma Sickle-cell anaemia Angiosarcoma Amyloïdosis Myeloproliferative disorders Sarcoïdosis Gaucher s disease Nymann-Pick disease Malaria Size of the spleen Normal volume of the spleen: between 100 and 265 ml (av :150 ml) Normal size 10.9 ± 1.4 cm (length) x 6.8 ± 0.71 cm (thickness) x 4.0 ± 0.45 cm (width) Splenic index : product of these 3 dimensions Must be lower than 480 cm 3 Longest oblique size of the spleen splenomegaly if > 12 cm Low interobserver reproducibility Do not allow comparisons with CT or MRI Size of the spleen The simplest: measure the true vertical height simple reproducible Comparable with CT and MRI Splenomegaly if > 10 cm Malformations Splenic pathologies must look for other malformations frequently associated Indirect witness of more general diseases Specific spleen diseases Traumatism Organ the most involved in case of abdominal trauma Invisible spleen 1) Agenesia situs ambigus, heterotaxia, isomerism, Ivemark synd 2) Ablation 3) Major atrophy Sickle-cell anaemia, thalassemia, Waquez repeated story of infarction = auto splenectomy 2

3 Invisible spleen 1) Agenesia situs ambigus, heterotaxia, isomerism, Ivemark synd 2) Ablation 3) Major atrophy Sickle-cell anaemia, thalassemia, Waquez repeated story of infarction = auto splenectomy 4) abnormal location wandering spleen Focal lesions of the spleen Cystic Easy or affordable Solid Mess Aswaniet al, JOP. J Pancreas (Online) 2015 Jan 31; 16(1): N DahiyaIndian Journal of Radiology and Imaging, Vol. 12, No. 3, July-September, 2002, Hypoechoic + wall Epidermoïde cyst Hematoma Pseudo Cysts Hypoechoic + wall Epidermoïde cyst Hematoma Pseudo Cysts Anechoic without wall Mesothelial simple Cyst Lymphangioma Peliosis 3

4 Be carreful: trap! Epidermoïde Cyst Epidermoïde Cyst 4

5 Hematoma (bike accident) Pseudo cyst Hypoechoic + wall Epidermoïde cyst Hematoma Pseudo Cysts Mesothelial simple cyst or cystic uniloculated lymphangioma ou hydatique cyst 5

6 Multiloculated cystic lymphangioma Splenic peliosis Factors involved in peliosis are malignancy, tuberculosis, acquired immune deficiency, diabetes, drugs and parasites Above image from: Youssef Ghazzawi, MD, and ImadAbsah, MD. Visceral Leishmaniaas Unusual Cause of Splenic Peliosisin United States. Mayo Clinic College of Medicine, Rochester, MN. ACG Case Rep J 2013;1(1): Focal lesions of the spleen Hypoechoic + wall Epidermoïde cyst Hematoma Pseudo Cysts Anechoic without wall Mesothelial simple Cyst Lymphangioma Peliosis Cystic Easy or affordable Solid Mess Solid lesions Solid lesions hypoechoic hyperechoic Bénin hypoechoic hyperechoic Bénin Cysts Hemangiomas Cysts Hemangiomas littoral cell hemangioma littoral cell hemangioma Accute bleeding Complicated Cysts Accute bleeding Complicated Cysts Acute infarction Abcess Acute infarction Abcess Hemangiomas/ hamartomas Hematomas Hemangiomas/ hamartomas Hematomas Lymphangiomas Subacute or old infartion Lymphangiomas Subacute or old infartion SANT (sclerosis angiomatous transformation) Extramedullary Hematopoïesis Calcifications Gamna-Gandy bodies Peliosis Malin SANT (sclerosis angiomatous transformation) Extramedullary Hematopoïesis Calcifications Gamna-Gandy bodies Peliosis Malin Metastases Metastases Metastases Metastases Lymphomas Kaposi Sarcoma Lymphomas Kaposi Sarcoma 6

7 Neoplasic context No neoplasic context: unifocal lesion Hemangioma Poumon Cancer du sein Mélanome No neoplasic context: unifocal lesion Hemangioma or Hamartoma Hemangioma or hamartoma Hemangioma Benign tumor the most frequent unique or multiple Cavernous hemangioma = hypoechoic, with cystic areas or completely cystic Capillary hemangioma = either isoechoic, or hyperechoic ronded or irregular whith sharp limits Hemangioma or hamartoma Hamartoma hypervascularized Composed of normal tissue of the spleen (mainly white and red pulp) but with anarchic organization Unifocal in 90% homogeneous, slightly hypoechoic No neoplasic context: unifocal lesion Hemangioma or Hamartoma 7

8 Hemangioma or hamartoma Extramedulary hematopoiesis If lesions are multiple Malignant lesions lymphoma, metastases, Kaposi sarcoma Benign lesions Littoral cell hemangioma Sarcoïdosis, granulomatosis Infectious disease: Multiple micro abscesses... Lymphoma Most frequent malignant lesion Hodgkin or non-hodgkin lymphomas Spleen involved in 30 to 40% Splenomegaly = 50% (diffuse & infiltrative involvement, no focal lesion seen) Polylobulated multifocal lesions : 50% Micronodulary involvement (miliary like)(high frequency probe) macronodules hypoechoic, or quasi anechoic, mimicking cysts Borders of lesions are ill defined (in contrary to true cysts) Lymphoma (Hodgkin) Lymphoma (NHL) Lymphoma (NHL) 8

9 Littoral cell hemangioma Littoral cell hemangioma Rare benign disease of the spleen From the cells of the wall of the red pulp capillaries Specific to the spleen multiples nodules hyperechoic in an enlarged spleen If no oncologic context: Follow-up stable If oncologic context : Metastases must be considered until proof of contrary biopsie if spleen involvement is isolated Sarcoïdosis es Aboveimage from: Thomas Vancauwenberghe, AnnemiekSnoeckx, Dirk Vanbeckevoort, Steven Dymarkowski, Filip M Vanhoenacker: Imaging of the spleen: whatthe clinician needs to know Singapore Med J 2015; 56(3): Hyperechoic foci Gamna Gandy bodies Gamna Gandy bodies Portal hypertension +++ Sickle-cell anaemia ++ Hemolysis hémochromatosis Scar Old Micro-abcess Cysticercosis Calcified granuloma Pneumocystosis +++ Sarcoïdosis Tuberculosis histoplasmosis 9

10 Painful spleen = possible infarction due to acute venous obstruction Acute pancreatitis Pancreas adenocarcinoma (body and tail) hypercoagulability, drepanocytosis Traumatism Sickle-cell anaemia Spleen traumatism lacerations Intraparenchymal hematomas sub-capsular hematomas Sensitivity of US about 69% Echogenicity of fresh blood similar to spleen parenchyma Intra splenic vessel distribution in color Doppler Empty signal area FAST US must rather focus of free liquid in the abdomen CEUS improves ++ the detection of parenchymal injuries Spleen infaction due to Sickle-cell anaemia after Mountain hiking Gracias Olivier.lucidarme@aphp.fr 10