Congenital lobar emphysema associated with polysplenia syndrome
Annals of Saudi Medicine. 2010; 30 (6): 482-484
em Inglês
| IMEMR
| ID: emr-125719
ABSTRACT
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
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Índice:
IMEMR (Mediterrâneo Oriental)
Assunto principal:
Artéria Pulmonar
/
Baço
/
Síndrome
/
Veia Cava Inferior
/
Veia Cava Superior
/
Imageamento por Ressonância Magnética
/
Ecocardiografia
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Tomografia Computadorizada por Raios X
/
Coristoma
/
Átrios do Coração
Tipo de estudo:
Relato de Casos
Limite:
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Ann. Saudi Med.
Ano de publicação:
2010
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