Congenital lobar emphysema associated with polysplenia syndrome
| Autor: | Choh Naseer, Choh Suhil, Jehangir Majid, Naikoo Bashir |
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| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: | |
| Zdroj: | Annals of Saudi Medicine, Vol 30, Iss 6, Pp 482-484 (2010) |
| Druh dokumentu: | article |
| ISSN: | 0256-4947 0975-4466 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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