Ascending Aortic Aneurysm in a Child With Fibulin-4 Deficiency

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Presentation transcript:

Ascending Aortic Aneurysm in a Child With Fibulin-4 Deficiency Makoto Hibino, MD, Yoshimasa Sakai, MD, Wataru Kato, MD, Keisuke Tanaka, MD, Kazuyoshi Tajima, MD, Takehiko Yokoyama, MD, Mitsuji Iwasa, MD, Hiroko Morisaki, MD, Toyonori Tsuzuki, MD, Akihiko Usui, MD The Annals of Thoracic Surgery Volume 105, Issue 2, Pages e59-e61 (February 2018) DOI: 10.1016/j.athoracsur.2017.08.041 Copyright © 2018 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 A transthoracic echocardiographic view showing a fluttering structure in the ascending aorta on both (A) long- and (B) short-axis views (arrowhead). A contrast-enhanced computed tomographic view showing enlargement of both the (C) ascending and (D) descending aorta with tortuosity in the descending and thoracoabdominal aorta. (Asc = ascending aorta; LV = left ventricle.) The Annals of Thoracic Surgery 2018 105, e59-e61DOI: (10.1016/j.athoracsur.2017.08.041) Copyright © 2018 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 (A) An operative picture of the ascending aortic aneurysm with the expanded thin aortic wall with poor vasa vasorum formation (white arrowhead). (B) A pathologic examination of the resected aortic specimen by Elastica van Gieson staining showed diffuse severe disruption of elastic fiber in the media (yellow arrowheads) and sporadic degeneration with histiocyte and mucopolysaccharide accumulation in the intima and media (yellow arrows). (C) In the skin specimen, disruption of elastic fiber was also identified in the dermis (white arrows). The Annals of Thoracic Surgery 2018 105, e59-e61DOI: (10.1016/j.athoracsur.2017.08.041) Copyright © 2018 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 A postoperative contrast-enhanced computed tomographic view showing excellent repair of the ascending aorta without dilatation of the descending aorta (A) during the hospital stay and (B) at 1 year after operation. The Annals of Thoracic Surgery 2018 105, e59-e61DOI: (10.1016/j.athoracsur.2017.08.041) Copyright © 2018 The Society of Thoracic Surgeons Terms and Conditions