title: A case of variant scimitar syndrome.
abstract: Scimitar syndrome is one of the large congenital pulmonary venolobar syndromes and is defined as hypogenetic lung associated with partial anomalous pulmonary venous return. We report the case of a 25-year-old man with complex and exceptional variant scimitar syndrome. A chest CT scan with three-dimensional (3-D) reconstruction led us to identify hypoplastic right lung with homolateral hemidiaphragm agenesis and hypogenetic right pulmonary artery. There was a large and sinuous systemic arterial supply and anomalous venous return directed into the left atrium (venous return being usually directed into the right atrium or inferior vena cava in scimitar syndrome). Hyperoxia test showed no shunt. This variant scimitar syndrome has been previously reported and anomalous venous return called "meandering" pulmonary vein. Diagnosis and management of these patients with complex congenital anomalies are difficult because of their exceptional condition. Chest CT scan with 3-D reconstruction offers an accurate noninvasive diagnosis.
authors.affiliation: Service de Chirurgie Thoracique, Hôpital Hôtel-Dieu, Paris, France. Electronic address: le_ant@hotmail.fr.
authors.forename: Antoine
authors.initials: A
authors.lastname: Legras
authors.affiliation: Service de Radiologie, Hôpital Hôtel-Dieu, Paris, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Guinet
authors.affiliation: Service de Chirurgie Thoracique, Hôpital Hôtel-Dieu, Paris, France.
authors.forename: Marco
authors.initials: M
authors.lastname: Alifano
authors.affiliation: Service de Chirurgie Thoracique, Hôpital Hôtel-Dieu, Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Lepilliez
authors.affiliation: Service de Chirurgie Thoracique, Hôpital Hôtel-Dieu, Paris, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Régnard
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D006325:Heart Atria / Q000002:abnormalities / Q000000981:diagnostic imaging*; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods*; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging*; D011667:Pulmonary Veins / Q000002:abnormalities / Q000000981:diagnostic imaging*; D012587:Scimitar Syndrome / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed; D014682:Vena Cava, Inferior / Q000002:abnormalities / Q000000981:diagnostic imaging*