Cases reported "Aortic Arch Syndromes"

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1/6. Aortic pseudoaneurysm complicating Takayasu disease: CT appearance.

    The CT findings of a patient with takayasu arteritis and an ascending aortic pseudoaneurysm are presented. Computed tomography demonstrated the nature, location, and extent of the aortic lesion and revealed the ominous finding of a related large hematoma indicating prior aortic rupture.
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2/6. Cutaneous manifestations of Takayasu's arteritis. A clinicopathologic correlation.

    Takayasu's arteritis is a chronic, granulomatous, large-vessel arteriopathy of unknown cause. We retrospectively reviewed the medical records of 38 patients with Takayasu's arteritis and identified 21 with cutaneous findings. Seven patients had lesions that were related to their systemic vasculitis. We found a Churg-Strauss granuloma, a pyodermatous leg ulcer, and inflammatory leg nodules in these patients. biopsy specimens from three patients with presumed "erythema nodosum" did not support the clinical diagnosis but did show arteritis. In patients with Takayasu's arteritis, small-vessel inflammation, and other inflammatory lesions may be present, in addition to large-vessel disease. Histopathologic study is necessary to categorize the nature of inflammatory leg nodules of these patients.
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3/6. Anti-factor ix circulating anticoagulant and immune thrombocytopenia in a case of Takayasu's arteritis.

    A 38-year-old male with proven Takayasu's arteritis presented, in a routine investigation, with thrombocytopenia, prolonged whole blood clotting time and activated partial thromboplastin time. Further studies demonstrated low levels of factor ix caused by a circulating anticoagulant. Immunological studies revealed an IgG (with kappa chains predominance) nature of this. Corticoid therapy decreased but did not suppress the anticoagulant activity. Since similar coagulation abnormalities have been described in collagen diseases, this observation is in support of this etiology to be considered in Takayasu's arteritis.
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4/6. Direct involvement of intracerebral arteries in Takayasu's arteritis.

    A man aged 20 with sudden onset of headaches and rapidly evolving spastic quadriparesis died within 3 weeks. Pathologic examination revealed intensive inflammation confined mainly to the adventitia of the aorta, the internal carotids, and all the major intracranial arteries. The gross changes and the lymphocytic and plasma cell nature of the inflammatory infiltrates were typical of Takayasu's arteritis. thrombosis of the right internal carotid artery resulted in ischemic necrosis of the ipsilateral hemisphere. Quite recent thrombus occluded the left internal carotid artery. This seems to be the first case report on histologically proven extensive direct involvement of intracranial vessels in Takayasu's disease. The differential diagnostic aspects of the case are also briefly discussed.
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5/6. Anatomic conundrum in a case of complete transposition of the aorta and pulmonary trunk.

    An unusual cord-like structure was found on postmortem examination of the heart of a 30-year-old woman who had complete transposition of the great arteries. This extended from the posterior aspect of the right side of the base of the aorta to the posterior wall of the distal part of the arch immediately proximal to the insertion of the arterial ligament. The nature and origin of this is discussed.
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6/6. Retinal ischemia in aortic arch atheromatous disease.

    Retinal ischemia is often caused by emboli arising from the cardiac chambers or the common carotid artery bifurcation; the latter are often composed of cholesterol. However, in many patients no lesions are identified after evaluation of these sources of emboli. Two patients were observed who had retinal ischemia and emboli originating from aortic atheromatous plaques that were visualized by transesophageal echocardiography. Cardiac, carotid, and intracranial sources of emboli were excluded. The embolic nature of retinal ischemia was further corroborated by the presence of microembolic signals during transcranial Doppler insonation of the middle cerebral artery on the side ipsilateral to the symptomatic retina. In patients with Hollenhorst plaques the aortic arch can be a potential source of emboli. Transesophageal echocardiography should be considered in these patients when the initial evaluation does not identify a cardiac or carotid lesion.
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