title: Long tortuous aorta in a child with Larsen syndrome.
abstract: A three-year-old boy with unusually flattened facies, multiple joint dislocations and eye abnormalities suggesting the presence of Larsen syndrome presented with a broad mediastinum on routine chest radiography. Computed tomography revealed a huge dilated tortuous aortic arch. Transthoracic echocardiography demonstrated aortic valve dilation, aneurysmal dilation of the ascending aorta with a high aortic arch and a kink in the arch at the isthmus level. Aortography showed a dilated elongated aorta with two acute curves, one at the ductal level and the second at the diaphragmatic level. These aortic lesions may have prognostic significance for the future risk of rupture or dissection. The present article reports the presence of a tortuous aorta in a child with Larsen syndrome.
authors.affiliation: Gaziantep University Faculty of Medicine, Gaziantep, Turkey. osmanbaspinar@hotmail.com
authors.forename: Osman
authors.initials: O
authors.lastname: Baspinar
authors.forename: Metin
authors.initials: M
authors.lastname: Kilinc
authors.forename: Ayse
authors.initials: A
authors.lastname: Balat
authors.forename: Mehmet Adnan
authors.initials: MA
authors.lastname: Celkan
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Coskun
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D001013:Aorta, Thoracic / Q000002:abnormalities*; D001014:Aortic Aneurysm / Q000209:etiology / Q000517:prevention & control; D001019:Aortic Rupture / Q000209:etiology / Q000517:prevention & control; D001027:Aortography; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D004452:Echocardiography; D005124:Eye Abnormalities / Q000235:genetics; D005145:Face / Q000002:abnormalities*; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D004204:Joint Dislocations / Q000235:genetics; D008297:Male; D011379:Prognosis; D012307:Risk Factors; D013577:Syndrome; D014057:Tomography, X-Ray Computed
chemical_list: D000319:Adrenergic beta-Antagonists