Incidental adult polysplenia with situs inversus, interrupted inferior vena cava with azygos continuation, patent ductus arteriosus, and aortic branches variations: a case report

Abstract

Polysplenia syndrome is a rare condition (about 2.5:100,000 live births) wherein a person has two or more spleens with accompanying various thoracic and abdominal organ abnormalities. Polysplenia is usually associated with situs ambiguous, while polysplenia with situs inversus is very rare. Herein, we report a rare case of incidentally found polysplenia syndrome combined with situs inversus in a 59-year-old female who also had an interrupted inferior vena cava (IVC) with azygos continuation, patent ductus arteriosus (PDA), and variations in the aortic arch branches. An aberrant left subclavian artery with Kommerell's diverticulum and a large aneurysm in the non-aberrant, proximal-right subclavian artery were also noted.

Keywords: Polysplenia syndrome; dextrocardia; inferior; situs inversus; splenic diseases; vena cava.

Figure 1

Plain chest radiography shows dextrocardia, a left-sided liver, and right-sided stomach gas contours. A cardiac pacemaker is also noted.

Figure 2

Axial (A-D) and three-dimensional (E,F) computed tomography images showing polysplenia combined with situs inversus totalis with an interrupted IVC with AzV continuation. A dilated AzV drains into the SVC. HVs form a confluence and drain directly into the right atrium via the SH-IVC. The LSCA aberrantly originates from the distal aortic arch with KD. A large aneurysm of the non-aberrant RSCA and PDA were also noted. IVC, inferior vena cava; AzV, azygos vein; SVC, superior vena cava; HVs, hepatic veins; SH, supra-hepatic; LSCA, left subclavian artery; KD, Kommerell’s diverticulum; RSCA, right subclavian artery; PDA, patent ductus arteriosus; RCCA, right common carotid artery; LCCA, left common carotid artery; S, spleens.