American Board of Pathology (ABPath) Practice Test 2025 - Free Pathology Exam Practice Questions and Study Guide

Asplenia, or the absence of the spleen, is notably associated with a range of congenital anomalies, particularly involving the inferior vena cava (IVC) and congenital heart defects. This association can be understood through the concept of heterotaxy syndrome, which includes asplenia and forms of bilateral left-sidedness.

In patients with asplenia, the absence of the spleen often correlates with structural anomalies in the major venous and arterial systems, especially in the IVC. These patients frequently present with congenital heart defects, which result from improper embryonic development leading to the abnormal placement of organs and vessels. Such abnormalities create a distinct clinical picture characterized by various cardiovascular issues as well as venous anomalies.

While polysplenia is also associated with heterotaxy, it is typically linked to a different set of structural anomalies, primarily involving the presence of multiple spleens rather than the absence of one. Splenomegaly refers to an enlargement of the spleen and does not have the same associations with IVC or congenital heart anomalies. Splenectomy, the surgical removal of the spleen, although it may seem relevant, is an acquired condition rather than a congenital anomaly.

Understanding these details highlights why asplenia is the correct