Congenital lobar emphysema associated with polysplenia syndrome
Annals of Saudi Medicine. 2010; 30 (6): 482-484
in En
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| ID: emr-125719
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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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Index:
IMEMR
Main subject:
Pulmonary Artery
/
Spleen
/
Syndrome
/
Vena Cava, Inferior
/
Vena Cava, Superior
/
Magnetic Resonance Imaging
/
Echocardiography
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Tomography, X-Ray Computed
/
Choristoma
/
Heart Atria
Type of study:
Case_reports
Limits:
Humans
/
Male
Language:
En
Journal:
Ann. Saudi Med.
Year:
2010