The Turkish Journal of Pediatrics 2011 , Vol 53 , Num 1
Cardiovascular Findings İn A Boy With Arterial Tortuosity Syndrome: Case Report And Review Of The Literature
1Department of Pediatric Cardiology, Ankara Children’s Hematology and Oncology Hospital, and Departments of 2Pediatric Cardiology, and 3Radiology, Ankara University Faculty of Medicine, Ankara, Turkey Ekici F, Uçar T, Fitöz S, Atalay S, Tutar E. Cardiovascular findings in a boy with arterial tortuosity syndrome: case report and review of the literature. Turk J Pediatr 2011; 53: 104-107.

Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue disorder. Herein, we describe a five-year-old boy. He had hyperextensible skin, atypical facial features and inguinal hernia. We present his vascular imaging studies of kinking and tortuosity of the aorta and bilateral multiple peripheral pulmonary artery stenoses determined by conventional and magnetic resonance angiographic (MRA) examination. ATS must be considered in patients with connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints and other organs. Vascular imaging studies, especially MRA, are useful in the screening of these vascular abnormalities. Keywords : arterial tortuosity syndrome, cardiovascular findings, magnetic resonance angiography, children.

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