Incidental Detection of Adult Polysplenia Syndrome With Situs Inversus Totalis, Interrupted Inferior Vena Cava, and Bronchiectasis

A 32-year-old male was referred to our hospital for evaluation of exertional dyspnea and cough. Chest x-ray depicted reticular opacities in left middle and lower lung zones. Interestingly, there was dextrocardia, right-sided aortic arch, and gastric bubble beneath the right hemidiaphragm.

Contrast-enhanced computed tomography done for further evaluation revealed his liver on the left side and stomach on the right, suggesting abdominal situs inversus (Figure 1 A, B). There were multiple spleens on the right side of the upper abdomen. On lung window setting, the left bronchus was eparterial and right bronchus was hyparterial, suggesting bronchial situs inversus. There was dextrocardia with atrial and ventricular situs inversus (Figure 1). However, there was atrioventricular and ventriculoarterial concordance. Furthermore, the systemic drainage was anomalous. The intrahepatic portion of the inferior vena cava (IVC) was interrupted with azygous continuation. Hepatic veins were draining directly into the right atrium (Figure 2). Aortic arch was right sided with mirror image branching. The patient had no other cardiac abnormality. There was cystic bronchiectasis involving bilateral lungs. Noncontrast scan of the paranasal sinuses showed no evidence of sinusitis (Figure 3). The patient is presently in good medical condition with no significant symptoms.

Figure 1

Figure 2

Figure 3

Polysplenia syndrome results when multiple (usually two to six) similar-sized splenic masses are present along the greater curvature of the stomach. It is a rare congenital disorder generally diagnosed in early childhood and usually associated with a gamut of cardiovascular anomalies, often situs ambiguous and interrupted IVC with azygous continuation. A majority of patients die by age 5 due to severe cardiovascular anomalies.¹ The gastrointestinal abnormalities associated with polysplenia include double ureters, renal agenesis, and short pancreas. Incidental detection of polysplenia associated with situs inversus totalis in an adult is rarely reported in literature.^1,2 “Heterotaxy” refers to a wide spectrum of anomalies involving dysmorphism of thoracoabdominal organs across the right-left axis of the body. Polysplenia is observed in left isomerism along with bilateral bilobed bronchi and bilateral morphologic left atria. However, in the present case, there was polysplenia without isomerism. It is critical to be aware of the various cardiovascular anomalies associated with polysplenia syndrome that will have clinical implications if a patient must undergo catheterization, bypass surgery, IVC filter placement, and temporary pacing through transfemoral approach.