POLYSPLENIA WITH NORMALLY STRUCTURED HEARTS

Polysplenia is known to exist with a constellation of anomalies that exhibit bilateral left-sidedness. Other well-established features of the "left-sided" syndrome include bilateral morphologically left bronchi, lobation bilaterally characteristic of a left lung and interruption of the inferior caval vein with continuation via the azygos vein. These features are commonly associated with the presence of complex congenital heart malformations,1 and have also been noted to accompany extrahepatic biliary atresia.2 It has subsequently been argued that multiple spleens (or polysplenia) are not the best indicator of congenital heart disease (most importantly those defects associated with isomerism of the left atrial appendages) because not all patients with such isomerism have multiple spleens.3 The 10 cases presently reviewed reinforce this opinion, demonstrating that the status of the spleen is an unreliable diagnostic tool with respect to recognizable syndromes of congenital heart malformations. Each case has multiple spleens without either complex congenital heart disease or isomerism of the left atrial appendages. © 1990.