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Polysplenia with pulmonary arteriovenous malformations |
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| Authors: | John Papagiannis Ronald J. Kanter Eric L. Effman Philip C. Pratt Roxanne Marcille Iley B. Browning III Brenda E. Armstrong |
| Affiliation: | (1) Department of Pediatrics, Division of, Duke University Medical Center, P.O. Box 3195, 27710 Durham, North Carolina, USA;(2) Division of Pediatric Pulmonary Medicine, Duke University Medical Center, P.O. Box 3195, 27710 Durham, North Carolina, USA;(3) Division of Pediatric Radiology, Duke University Medical Center, P.O. Box 3195, 27710 Durham, North Carolina, USA;(4) Department of Pathology, Duke University Medical Center, P.O. Box 3195, 27710 Durham, North Carolina, USA |
| Abstract: | Summary A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed. |
| Keywords: | Polysplenia Pulmonary arteriovenous malformations Portosystemic anastomosis |
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