Cases reported "Aortic Diseases"

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1/54. Typical presentation of intramural aortic haemorrhage (IAH) with evidence of intimal tear at MRI and angiography.

    A typical appearance of IAH was evidenced by CT and TEE in a 56-year-old hypertensive female suspected of developping classical acute aortic dissection (AAD). Further examination with MRI and aortography showed unequivocally the presence of an intimal tear in the aortic arch. This coexistence of intimal tear has never been evidenced preoperatively in patients with IAH. This observation demonstrates at the outset that IAH is part of the spectrum of AAD.
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2/54. Tubular stenosis of the aorta with aortic fibromuscular dysplasia.

    The autopsy findings in a 2-year-old girl with severe hypertension resulting from stenosis of the lower part of the thoracic and the abdominal aorta were reviewed. The lower portion of the thoracic aorta and the abdominal aorta showed uniform narrowing down to the level of the bifurcation of iliac arteries. Histologically, the aortic wall of the stenotic site showed irregular proliferation of smooth muscle cells and collagen fibers. The elastic fibers had disappeared from outside the media. No intimal thickening and inflammatory cell infiltration were observed. These histologic changes of the aortic media in this case are apparently similar to fibromuscular dysplasia (medial hyperplasia).
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3/54. Enlargement of ulcer-like projections after repair of acute type A aortic dissection.

    We treated two cases of enlargement of ulcer-like projections in the descending thoracic aorta, which were recognized after emergency graft replacement from the ascending aorta to the aortic arch for acute type A aortic dissection. The intimal tear, which was near the left subclavian artery, was resected during the initial operation. Graft replacement of the descending thoracic aorta was successful.
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4/54. Spontaneous progression of ascending aortic intramural hematoma to Stanford type A dissection fortuitously witnessed during an MR examination.

    Aortic intramural hematoma may occur as a primary event (spontaneous dissection without intimal flap) or secondary to a penetrating atherosclerotic ulcer. The management of intramural hematoma of the ascending aorta is somewhat controversial because of limited published data, but some centers advocate early surgical intervention. We describe a patient with an intramural hematoma of the ascending aorta that progressed to a classic communicating dissection during an MR examination. This case graphically demonstrates the potential instability of patients with intramural hematoma of the ascending aorta.
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5/54. Histopathological characterization of aortic intimal sarcoma with multiple tumor emboli.

    A case of aortic intimal sarcoma with multiple tumor emboli and distal metastasis is reported. All metastasis (adrenal, spleen) were via the arteries. This case also had independent lung cancer. Macroscopically, the aortic tumor did not form a bulged mass, but had linear ulceration with abundant mural thrombi. Poorly cohesive large atypical cells were seen in the intima of the abdominal aorta without invasion into the media. Tumor cells were disseminated into the mural thrombi on the aorta and embolized its branches. In the metastatic tumor or tumor emboli of the distal artery, there were not only large atypical cells, but also the foci of spindle-shaped cells or epithelioid differentiation. Tumor cells in the aorta were immunohistochemically positive for only vimentin. Muscle-specific actin was positive focally for spindle-shaped cells of tumor emboli and metastatic tumors. Furthermore, cytokeratin-positive cells were scatteredly seen. All tumor cells were negative for factor viii and did not have a histologic or phenotypic analogy with lung cancer. The primary intimal sarcoma in the present case was of undifferentiated non-endothelial intimal stromal cell origin, and may have had multipotential for differentiation. Investigation of the metastatic site was useful for recognizing the features of this tumor.
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6/54. A case of penetrating aortic atherosclerotic ulcer with hemoptysis.

    A 69-year old Japanese woman with hypertension was admitted because of continuous back pain and recurrent hemoptysis. Radiographic findings showed an enhanced irregular mass, at the aortic arch fed by the tracheal artery, which implied both a penetrating aortic atherosclerotic ulcer and lung cancer. Diagnostic surgery revealed no evidence of cancer but did reveal a rupture of the intima at the distal part of the aortic arch. It is assumed that the transmural oozing occurred after development of the penetrating aortic ulcer, which formed an extra-aortic hematoma and caused surrounding inflammation, and led to tracheal artery feeding. The intramural hematoma might have weakened vascular wall tension from the aorta, and formed an oozing extra-aortic hematoma instead of an acute rupture.
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7/54. Intimal angiosarcoma of the aorta with tumour embolisation causing mesenteric ischaemia. Report of a case diagnosed using CD31 immunohistochemistry in an intestinal resection specimen.

    Primary intimal angiosarcomas of the aorta (i.e. mostly intraluminal sarcomas with evidence of endothelial differentiation) are extraordinarily rare. We report a case in which the diagnosis was accurately made using immunohistochemistry in an intestinal resection specimen and confirmed during autopsy. The patient was a 64-year-old woman with mesenteric ischaemia and a "thrombus" in the abdominal aorta. Two segments of the ileum and the right colon were surgically removed. Histological examination showed multiple tumour emboli in small arteries of the submucosa, serosa and mesentery. The highly atypical cells comprising these emboli were positive immunohistochemically with antibodies to ulex Europaeus, von willebrand factor and CD31 and negative for CD34. During post-mortem examination, the intraaortic mass was located around the orifices of the coeliac and the superior mesenteric arteries, and gross tumour thrombi were found in the left renal and splenic arteries. This case emphasises the need for a wide panel of immunohistochemical antibodies when tumour emboli of unknown origin are under study.
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8/54. Subadventitial hematoma of a patent ductus arteriosus and thrombus formation within the aorta secondary to cardiac catheterization.

    An 18-month-old boy with patent ductus arteriosus (PDA) underwent surgical closure of PDA. cardiac catheterization determined the PDA was not suitable for transcatheter closure. After the chest was opened, subadventitial hematoma was seen on the aortic end of the PDA. Incision of the aorta revealed a thrombus secondary to intimal laceration. The thrombus was extracted and the PDA was closed using division technique. Because no similar report was found in English-language literature, the technique and the surgical strategy are discussed.
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9/54. Mild thickening of the aortic wall: subtle intramural hematoma?

    Recently published criteria for the diagnosis of intramural hematoma (IMH) of the ascending aorta by transesophageal echocardiogram (TEE) include the presence of a crescent-shaped thickening of the aortic wall of more than 7 mm with an echolucent space. We report a patient whose clinical presentation suggested aortic dissection but whose TEE failed to meet the conventional criteria for such a diagnosis. TEE showed a uniform intimal thickening suspicious of IMH but less than 7 mm in thickness and with no echofree space. One week later, the patient developed frank aortic dissection. CONCLUSION: IMH can present with a subtle echo appearance. The diagnosis should not be totally discounted in the absence of a 7-mm crescentic wall thickness and an echofree space if the clinical presentation is suggestive of dissection.
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10/54. Surgical endovascular stent grafting for a ruptured penetrating atherosclerotic ulcer of the aortic arch.

    Penetrating atherosclerotic ulcer (PAU) is defined as an atherosclerotic lesion in which an ulceration occurs in the diseased aortic intima leading to disruption of the internal elastic lamina. It may cause a pseudoaneurysm formation or transmural aortic rupture. We describe a patient in whom a ruptured PAU in the distal aortic arch was treated successfully by a surgical endovascular stent graft. Through a median sternotomy and under deep hypothermic circulatory arrest, the aorta was transected between the left common carotid and subclavian arteries. A dacron prosthetic graft with self-expanding original Z type stents attached inside of the distal half was inserted through the aortotomy to exclude the PAU. By performing the procedure through a median sternotomy, we could eliminate dissection around the ruptured aortic wall. The stented graft was secured safely in the thoracic aorta to exclude the ruptured ulceration located distal to the left subclavian artery. PAU should be recognized widely as a distinct cardiovascular surgical problem which may lead to intramural hematoma with or without dissection or rupture.
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