%0 Journal Article
%T Congenital lobar emphysema associated with polysplenia syndrome
%A Choh Naseer
%A Choh Suhil
%A Jehangir Majid
%A Naikoo Bashir
%J Annals of Saudi Medicine
%D 2010
%I King Faisal Specialist Hospital and Research Centre
%X Polysplenia, or left isomerism, is a rare heterotaxy syndrome characterized by bilateral bi-lobed lungs, bilateral pulmonary atria, a symmetrical midline liver, and multiple aberrant splenic nodules. We report a case of polysplenia associated with congenital lobar emphysema apart from other typical anomalies. Such an association has not been previously reported. The patient was a young male with progressive exertional breathlessness referred for high resolution CT of the lungs. CT, MRI and echocardiography revealed (in addition to congenital lobar emphysema of right lung) a hemiazygos continuation of the inferior vena cava, a persistent left superior vena cava, multiple splenunculi in the right hypochondrium, midline liver, bilateral bilobed lungs, a large pulmonary artery (suggestive of severe pulmonary artery hypertension) and a large VSD-a typical constellation of findings described in polysplenia syndrome.
%U http://www.saudiannals.net/article.asp?issn=0256-4947;year=2010;volume=30;issue=6;spage=482;epage=484;aulast=Choh