Where's the spleen? Looking for the spleen and assessing its function in the syndromes of isomerism

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Abstract
Despite the close association of certain constellations of cardiac malformations with splenic status (right isomerism / asplenia, left isomerism / polysplenia), the association is not perfect. Given the risk of infection in the patient lacking splenic tissue, it seems prudent to assess the state of the spleen separately from the cardiovascular system, in order to decide on antimicrobial prophylaxis. A wide variety of tests is available to assess splenic function and/or anatomy. Counting pitted erythrocytes or Howell-Jolly bodies in the peripheral blood smear can be a good indicator of the lack of splenic function, although the former appears more sensitive and quantitative than the latter. Radionuclide spleen scans can provide both anatomic and functional information about the spleen, and may be a useful adjunctive test. Relatively poor spatial resolution, and interfering hepatic activity (even with spleen-specific scans), mitigate its usefulness in the young infant. The reliability of computed tomography and magnetic resonance imaging in detecting splenic tissue in the heterotaxy syndromes is unproven and perhaps only fair at best. They are probably most helpful as adjunctive tests. Ultrasonography offers excellent spatial resolution, portability, and lack of ionizing radiation, but is operator- and patient-dependent. Nonetheless, ultrasonography, coupled with an examination of the peripheral blood for Howell-Jolly bodies or pitted erythrocytes, is probably the set of tests with highest sensitivity and greatest convenience. Management strategies for the asplenic patient are also discussed.