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146 PART II Abdominal and Pelvic Sonography

TABLE 5.1 Causes of Splenomegaly

Splenomegaly (<18 cm)

HEMATOLOGIC

Red blood cell membrane

defects

Hemoglobinopathies

Autoimmune hemolytic

anemias

RHEUMATOLOGIC

Rheumatoid arthritis

Systemic lupus

erythematosus

Sarcoidosis

INFECTIOUS

Viruses

Bacteria

Mycobacteria

Fungi

Parasites

CONGESTIVE

Hepatic cirrhosis

Venous thromboses (hepatic,

portal, splenic)

Congestive heart failure

INFILTRATIVE

Lymphomas

Myeloproliferative neoplasms

Metastatic cancer

Amyloidosis

Gaucher disease

Niemann-Pick disease

Glycogen storage disease

Hemophagocytic syndrome

Langerhans cell histiocytosis

Massive Splenomegaly

(>18 cm)

Thalassemia major

Visceral leishmaniasis

Hyperreactive malarial

splenomegaly syndrome

Mycobacterium aviumintracellulare

complex

Lymphomas

Myeloproliferative

neoplasms

Gaucher disease

Data from Abramson JS, Chatterji M, Rahemtullah A. Case records of

the Massachusetts General Hospital. Case 39-2008. A 51-year-old woman

with splenomegaly and anemia. N Engl J Med. 2008;359(25):2707-2718 17 ;

Pozo AL, Godfrey EM, Bowles KM. Splenomegaly: investigation, diagnosis

and management. Blood Rev. 2009;23(3):105-111. 18

investigations, such as diagnostic splenic biopsy or splenectomy. 17

In selected cases, ultrasound-guided splenic biopsy of focal

abnormalities can be helpful in establishing a diagnosis, with an

acceptable complication rate and high accuracy. 24,25

Complications of splenomegaly include hypersplenism and

spontaneous splenic rupture. Spontaneous splenic rupture

typically occurs in patients with an enlarged spleen ater minimal

trauma or insigniicant events such as coughing. 26,27

Focal Abnormalities

Ultrasound can be helpful in inding and characterizing focal

splenic lesions. However, because of the overlap in the appearance

Types of Splenic Cysts

Congenital cysts

Pseudocysts

Hydatid (echinococcal) cysts

Pancreatic pseudocysts

Endothelial-lined cysts

Lymphangiomas

Cystic hemangiomas

Peliosis

Cystic metastasis a

Abscess a

Hematoma a

a Not true cysts.

of splenic lesions, it is oten not possible to make a speciic

diagnosis based on the sonographic indings alone. Splenic lesions

can be solitary or multiple, difuse, and iniltrative. Focal lesions

can be cystic, complex cystic, or solid. Further, lesions can be

categorized according to size as micronodular (<1 cm), nodular

(1-3 cm), or focal (>3 cm) masses. 28 Knowledge of past medical

history, clinical presentation, and sonographic indings is required

to provide an appropriate diferential diagnosis and guide further

management. 29 Ultimately, percutaneous biopsy or splenectomy

may be required to obtain a deinitive diagnosis.

Splenic Cysts

Splenic cysts, as with cysts elsewhere in the body, appear as

anechoic lesions with posterior acoustic enhancement. Simple

cysts are round to ovoid in shape and have a thin, sharply deined

wall. Complex cysts do not meet these criteria and may demonstrate

septations, thick walls, calciications, solid components,

or internal echoes. Occasionally, cysts can grow to a very large

size, becoming predominantly exophytic. It may then be diicult

to appreciate their splenic origin (Fig. 5.10).

he most common splenic cystic lesions are primary

congenital cysts, pseudocysts, and hydatid cysts. Uncommon

splenic cystic lesions include pancreatic pseudocysts, lymphangiomas,

hemangiomas, peliosis, hamartomas, angiosarcomas,

and cystic metastases. 30 Other lesions that can mimic cysts on

ultrasound are abscesses, lymphoma, necrotic metastases, and

hematomas. 31

In the developed world, most splenic cysts are asymptomatic

incidental indings discovered during routine imaging and

generally represent congenital cysts or pseudocysts. hese cysts

may cause symptoms when large or when present with complications

such as internal hemorrhage, infection, or rupture. Primary

congenital cysts, also called epidermoid cysts or true cysts,

are characterized by the presence of an epithelial lining on

pathologic examination. 32 hought to arise from embryonic rests

of primitive mesothelial cells within the spleen, typical epidermoid

cysts present as well-deined, thin-walled anechoic lesions that

do not change over time (Fig. 5.11A). Pseudocysts, also known

as false cysts, have no cellular lining and are presumably secondary

to trauma, infarct, or infection. 33 hese cysts are more oten

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