engleski

Ivemark syndrome: asplenia with kidney collecting duct cysts and polysplenia with cerebellar cyst

The newborns, one male and one female from the two different families, with Ivemark syndrome proved at autopsy are reported. One of them had asplenia and another had polysplenia. Both newborns had complex cardiac deffects with isomerisms of the lungs. The newborn with asplenia had dextrocardia, transposition of great vessels, stenosis of the pulmonary artery, common atrioventricular canal and patent ductus arteriosus. The Patient with newborn with polysplenia had a common atrium, hypoplastic left ventricle and patent ducts arteriosus. The patient with with asplenia had cystic dilated collecting ducts of the kidney and the patient with polysplenia had cerebellar cyst. These associate malformations have not been reported previously.Both cases were sporadic.

cerebellar cyst; dextrocardia; Ivemark syndrome; kidney collecting duct cysts; transposition of great vessels

nije evidentirano