The aim of this study is to report a case of polysplenia syndrome observed in an 1 1/2 yr. old male patient who came for CT thorax for preoperative evaluation of congenital heart disease. There was partial anomalous pulmonary venous drainage – right lung was draining into right atrium and left lung into left atrium. The IVC was interrupted and venous return was via the continuation of enlarged azygos vein, which was running adjacent to the aorta and to its right side. The bronchial and vascular anatomy of the right lung was identical to that of the left lung, i.e, bilateral bilobed lungs with bilateral hyparterial bronchi - a variant condition called left thoracic isomerism. In the abdomen splenule was present. The pathogenesis is not completely clear, but it is thought to be caused by an abnormality of embryonic curvature with a possible genetic basis.
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