突然死を来した若年発症急性大動脈解離の 1 例

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1 症例報告 突然死を来した若年発症急性大動脈解離の 1 例 要旨 16 X 12 3 X 20cm cystic medial necrosis Marfan Marfan Marfan. 2011; 22: はじめに 1) Ehlers-Danlos Marfan Marfan 2) 1 The juvenile acute aortic dissection which caused sudden death: a case report 症例 JJAAM. 2011; 22:

2 突然死を来した若年発症急性大動脈解離の 1 例 ,225g X mg X Fig ml Fig. 1. Chest plain roentgenography. After trans-oral intubation, right lung field permeability deteriorated and the mediastinum shifted to the left. computed tomography CT CT CT Fig cm 90.5kg Fig. 3 entry. 2011; 22:

3 前田 代元, 他 a b Fig. 2. Computed tomography (CT). a: Chest plain CT. After right chest drainage, a large hemothorax was observed in the right cavumthoracis. The mediastinal space had also shifted markedly to the left. b: Abdominal plain CT. The aorta showed remarkable collapse (arrow). No hemorrhagic lesions were observed in the abdominal cavity. Fig. 3. Pathologic findings: macroscopic findings. We found an aortic dissection measuring approximately 20 cm in length on the tail side from the bifurcation of the left subclavian artery ( ). We identified an adventitia injury ( ) approximately 8cm distal from the bifurcation of the left subclavian artery ( ). The massive hemothorax was confirmed in the right cavumthoracis. 20cm 8cm 5cm Fig. 4 foamy cell Fig. 4a cystic medial necrosis Fig. 4b Stanford B DeBakey IIIb 考察 JJAAM. 2011; 22:

4 突然死を来した若年発症急性大動脈解離の 1 例 a b Fig. 4. Pathological findings of the aorta: microscopic findings. a: Low-power field. The tunica intima was hyperplastic with fibrous irregularities. The tunica media had aberrations (arrow). b: High-power field. The tunica media showed lacerated elastic fibers and cystic medial necrosis (arrow). Marfan X 12 3) 12 X 4,5) 6) Marfan Ehlers-Danlos 1,7) Marfan Marfan Marfan. 2011; 22:

5 前田 代元, 他 20% 2,8) 結 語 Marfan 1 Marfan 9) Marfan TGF- 9) Marfan Marfan 文献 1) Nienaber CA, Eagle KA: Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. Circulation. 2003; 108: ), : ; 48: ) Bossone E, Rampoldi V, Nienaber CA, et al: Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. Am J Cardiol. 2002; 89: ) Hagan PG, Nienaber CA, Isselbacher EM, et al: The international registry of acute aortic dissection (IRAD): new insights into an old disease. JAMA. 2000; 283: ) Earnest F 4th, Muhm JR, Sheedy PF 2nd: Roentgenographic findings in thoracic aortic dissection. Mayo Clin Proc. 1979; 54: ) Schnitker MA, Bayer CA: Dissecting aneurysm of the aorta in young individuals,particularly in association with pregnancy. Ann Intern Med. 1944; 20: ), : ; ; 19: ) Schlatmann TJ, Becker AE: Pathogenesis of dissecting aneurysm of aorta. Comparative histopathologic study of significance of medial changes. Am J Cardior. 1977; 39: ) Dean JC: Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet. 2007; 15: JJAAM. 2011; 22:

6 突然死を来した若年発症急性大動脈解離の 1 例 ABSTRACT The juvenile acute aortic dissection which caused sudden death: a case report Daigen Maeda, Terutake Yonemori, Hiroaki Higashioka Department of Critical Care Medical Center, Shizuoka Saiseikai General Hospital Acute aortic dissection can lead to sudden death. We present the clinical course of a 16-year-old male whose mother had been surgically treated for acute aortic dissection. He experienced sudden back pain and was examined at a clinic. Three days later, he developed sudden convulsive seizures while taking a bath and lapsed into cardio-pulmonary arrest. He was transported to our hospital by ambulance. Chest roentogenography revealed a massive right-sided hemothorax and chest drainage was thus initiated. We continued aggressive resuscitation, but there was no return of spontaneous circulation. The same day, we performed autopsy imaging and pathological anatomic evaluations. We found an aortic dissection, approximately 20 cm in length, on the tail side from the bifurcation of the left subclavian artery, and massive hemothorax was confirmed in the right cavumthoracis. The tunica media of the aorta showed cystic medial necrosis on histological examination. Acute aortic dissection at a young age is often associated with congenital diseases such as Marfan s syndrome. However, no sign of any congenital disease was detected in this case. When an aortic dissection develops in one family member, periodic follow-up of blood relatives is considered to be very important. (JJAAM. 2011; 22: ) Keywords: juvenile sudden death, congenital connective tissue disease, cystic medial necrosis Received on March 11, 2011 (11-026). 2011; 22: