Congenital lobar emphysema associated with polysplenia syndrome.
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.
Choh
Naseer A
NA
Shri Maharaja Hari Singh Hospital, Kashmir, India.
Choh
Suhil A
SA
Jehangir
Majid
M
Naikoo
Bashir A
BA
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D000328:Adult; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging; D006801:Humans; D008099:Liver / Q000002:abnormalities / Q000000981:diagnostic imaging; D008168:Lung / Q000002:abnormalities / Q000000981:diagnostic imaging; D008297:Male; D011656:Pulmonary Emphysema / Q000151:congenital / Q000000981:diagnostic imaging; D011859:Radiography; D013154:Spleen / Q000002:abnormalities*; D014463:Ultrasonography; D014682:Vena Cava, Inferior / Q000002:abnormalities / Q000000981:diagnostic imaging