title: Ivemark syndrome-a rare entity with specific anatomical features.
abstract: Ivemark syndrome (IS) is a rare embryological disorder which results from failure of development of the left-right asymmetry of organs. It is often associated with cardiac and other organ abnormalities, which are the usual causes of death in early neonatal life. We report a 3 months old girl with IS with dextrocardia, transposition of the great vessels, atrio-ventricular connection, total anomalous pulmonary venous drainage, a right atrial and right pulmonary isomerism, a midline liver, a midline gallbladder, asplenia, intestinal malrotation and vena cava anomalies. To our knowledge, complete right heterotaxia syndrome has been rarely described in literature. Lateralization defects such as situs inversus, asplenia or polysplenia due to defective left-right axis development are considered as defects of the primary developmental field. Therefore, additional malformations in IS can be synchronic defects in the primary developmental field rather than causally independent malformations.
authors.forename: Adrian
authors.initials: A
authors.lastname: Hrusca
authors.forename: Andreea Liana
authors.initials: AL
authors.lastname: Rachisan
authors.forename: Bogdan
authors.initials: B
authors.lastname: Lucian
authors.forename: Simona
authors.initials: S
authors.lastname: Oprita
authors.forename: Simona
authors.initials: S
authors.lastname: Manole
authors.forename: Simona
authors.initials: S
authors.lastname: Cainap
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D003914:Dextrocardia / Q000000981:diagnostic imaging; D005260:Female; D006322:Heart Aneurysm / Q000175:diagnosis; D059446:Heterotaxy Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D014057:Tomography, X-Ray Computed; D014188:Transposition of Great Vessels / Q000000981:diagnostic imaging; D014683:Vena Cava, Superior / Q000002:abnormalities