Spleen Flashcards
Splenule (Accessory Spleen)
What is it? Why do they form?
Do they have clinical significance?
How to Dx? If in doubt, how to confirm?
- Also called an accessory spleen, a splenule is a focus of normal splenic tissue separate from the main body of the spleen, due to the embryologic failure of fusion of the splenic anlage. The most common location is the splenic hilum.
- Although usually an incidental finding, the presence of a splenule does have significance in certain clinical settings. For instance, splenectomy for consumptive thrombocytopenia may not be curative if there is sufficient unresected accessory splenic tissue present. A splenule may be mistaken for a lymph node or mass when in an unusual location. As previously discussed, an intrapancreatic splenule may be mistaken for a hypervascular pancreatic mass.
- A splenule should follow splenic tissue on all MRI sequences. If in doubt, a Tc-99m sulfur colloid scan or a heat-damaged Tc-99m RBC scan can be confirmatory.
Polysplenia Syndrome
What is it? Relative to stomach?
Association? Prognosis?
Venous association?
- Polysplenia syndrome is a spectrum of anatomic disorders characterized by some degree of visceral heterotaxia in addition to multiple discrete foci of splenic tissue. Multiple spleens may be on the right or left, but are always on the same side as the stomach.
- Polysplenia is usually associated with severe congenital cardiac anomalies. Most patients die in early childhood, but a few may have only minor cardiac defects and may be incidentally discovered as adults.
- Polysplenia is associated with venous anomalies including interruption of the IVC with azygos or hemiazygos continuation. A less common association is a preduodenal portal vein.
Wandering Spleen
What is it?
Possible presentation?
- A wandering spleen is a normal spleen with abnormal laxity or absence of its fixed ligamentous attachments.
- Wandering spleen may present clinically as an abdominal mass or may cause acute abdominal pain secondary to torsion.
Splenic Hemangioma
What is it? Common distribution and size?
Associated with what two syndromes?
Imaging appearance?
Nuclear medicine techniques to visualize these lesions?
- Hemangioma is the most common benign splenic neoplasm. Hemangioma may be solitary or multiple, and lesions tend to be small.
- Splenic hemangiomas are associated with Kasabach-Merritt syndrome (anemia, thrombocytopenia, and consumptive coagulopathy) and Klippel-Trenaunay-Weber syndrome (cutaneous hemangiomas, varicose veins, and extremity hypertrophy). These visceral hemangiomatosis syndromes are usually associated with phleboliths.
- On CT, hemangiomas are typically iso- or hypoattenuating pre-contrast and hyperenhancing. On MR, hemangiomas are typically hyperintense on T2-weighted images and may enhance peripherally or homogeneously. However, the classic pattern of discontinuous nodular enhancement seen in hepatic hemangiomas is uncommon.
- Nuclear medicine scintigraphy with Tc-99m labeled red blood cells would show increased activity within the lesion on delayed images. In contrast, Tc-99m sulfur colloid scanning may show either increased or decreased activity.
Kasabach-Merritt Syndrome
- AKA hemangioma thrombocytopenia syndrome
- Kasabach-Merritt syndrome, also known as haemangioma thrombocytopenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumor is responsible for thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy.
- Associated with splenic hemangiomas
Klippel-Trenaunay-Weber Syndrome
- Klippel-Trénaunay-Weber syndrome (KTWS) is a syndrome combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. It is considered an angio-osteo-hypertrophic syndrome.
- Associated with splenic hemangiomas
- KTS classically comprises a triad of:
- port wine nevi
- bony or soft tissue hypertrophy of an extremity (localised gigantism)
- varicose veins or venous malformations of unusual distribution
Splenic Hamartoma
What is it? Possible association?
Imaging appearance?
- Splenic hamartoma is a rare, benign lesion composed of malformed red pulp elements. It may be associated with tuberous sclerosis.
- Splenic hamartoma is typically a well-circumscribed, iso- or hypoattenuating mass on unenhanced CT that enhances heterogeneously after contrast administration. On MR, a hamartoma is iso- to slightly hyperintense on T2-weighted images, featuring heterogeneous early enhancement and relatively homogeneous delayed enhancement.
Congenital True (Epithelial) Splenic Cyst
What is it? Interesting lab findings?
Compare to pseudocyst
- A congenital true cyst is defined as having an epithelial lining. Interestingly, a splenic epithelial cyst may cause elevation of tumor markers including CA19-9, CA125, and CEA, despite its completely benign nature.
- Unlike a post-traumatic pseudocyst, a true cyst may have septations, but mural calcification is uncommon.
Post-Traumatic Pseudocyst
What is it?
Compare to true (epithelial) splenic cyst
Imaging?
- A post-traumatic pseudocyst is the end result of the evolution of a splenic hematoma.
- Unlike a true epithelial splenic cyst, the periphery of a pseudocyst is not cellular but made of fibrotic tissue.
- On imaging, a post-traumatic pseudocyst appears as a well-circumscribed, fluid density lesion, with no peripheral enhancement.
- In contrast to a true cyst, septations are uncommon but there may be mural calcification.
Intrasplenic Pancreatic Pseudocyst
When do you see this?
More similar to a true epithelial splenic cyst or post-traumatic pseudocyst?
Rare reported complication?
- A post-pancreatitis pseudocyst involving the tail of the pancreas may extend into the spleen. There is almost always a history of pancreatitis.
- Unlike a true congenital cyst, an epithelial lining is lacking and histology more closely resembles a post-traumatic pseudocyst.
- Splenic rupture has been reported in some cases of intrasplenic post-pancreatitis pseudocysts.
Splenic Lymphangioma
What is it?
Classic appearance?
- Splenic lymphangioma is a rare, benign neoplasm usually diagnosed in childhood, which may be solitary or multiple.
- Lymphangioma features a classic imaging appearance of a multilocular cystic structure with thin septations. Post-contrast images may show septal enhancement.
Sarcoidosis involving the spleen
Most common presentation and often association?
Other possible (less common) appearance?
This appearance is generally indistinguishable from what other entity?
- Sarcoidosis is a systemic disease of unknown etiology characterized histologically by multiple nodules composed of noncaseating granulomas.
- When sarcoidosis involves the spleen, splenomegaly is the most common presentation, often associated with hepatomegaly and lymphadenopathy.
- Less commonly, sarcoidosis may involve the spleen in a multinodular pattern with numerous hypoattenuating 1-3 cm lesions demonstrating essentially no enhancement.
- These nodules are formed by coalescent sarcoid granulomas and have low signal on all MRI sequences.
- Sarcoid nodules are most conspicuous on T2-weighted images and early-phase post-contrast T1-weighted images. On the post-contrast images, the non-enhancing nodules will stand out against the avidly enhancing splenic parenchyma.
- Imaging appearance is generally indistinguishable from splenic lymphoma.
Splenic Inflammatory Pseudotumor
What is it?
Presentation?
Typical imaging appearance?
- Splenic inflammatory pseudotumor is a rare focal collection of immune cells and associated inflammatory exudate, of unclear etiology.
- Patients often have constitutional symptoms including fever and malaise.
- Inflammatory pseudotumor has a variable and nonspecific imaging appearance, but a typical presentation is of a well-circumscribed, heterogeneously enhancing mass.
Splenic Pyogenic Abscess
Most commonly seen in what population?
A solitary abscess is more likely caused by what vs multifocal abscesses?
Characteristic US finding?
Treatment?
- Splenic bacterial abscesses are uncommon and usually seen in immunocompromised patients. A solitary abscess is much more likely to be bacterial. In contrast, multifocal small abscesses are more likely to be fungal.
- On CT, a bacterial abscess usually has an irregular, enhancing wall. Gas is not usually seen, but is highly specific for a bacterial abscess when present.
- A characteristic ultrasound finding is the wheel within a wheel or bull’s-eye appearance, which describes concentric hyperechoic and hypoechoic rings surrounding the abscess.
- Treatment is CT- or ultrasound-guided percutaneous drainage in addition to antibiotics.