1/30. Primary polyarteritis nodosa presenting as acute symmetric quadriplegia.We report a case of peripheral neuropathy presenting as acute symmetric areflexic quadriplegia in the setting of a well-defined clinical, histopathologic, and angiographic diagnosis of classic polyarteritis nodosa. While it is usually easy to recognize the typical clinical presentation of necrotizing angiopathy-induced peripheral neuropathy as a mononeuritis multiplex or a distal polyneuropathy in association with a collagen vascular disease, clinicians must be equally sensitive to a number of more challenging possibilities. Acute quadriplegia similar to that seen in guillain-barre syndrome can be secondary to primary classic polyarteritis nodosa and the former may be the chief or even the sole manifestation of the latter.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/30. Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.- - - - - - - - - - ranking = 9keywords = vascular disease (Clic here for more details about this article) |
3/30. Nontraumatic clostridial myonecrosis.We describe three cases of nontraumatic clostridial myonecrosis seen at the Victorian Institute of forensic medicine. Nontraumatic clostridial myonecrosis is an uncommon and often fatal condition that requires immediate institution of appropriate medical and surgical therapy. It is most commonly caused by clostridium perfringens and clostridium septicum and is associated with gastrointestinal and hematologic malignancies, diabetes mellitus, and peripheral vascular disease. The clinical features include a rapidly evolving acute illness with severe pain, marked tachycardia, and brawny discoloration of the skin with bullae formation and crepitus, followed by hypotension and acute renal failure. Features at autopsy include reddish brown skin discoloration with bullae formation and necrotic skeletal muscle. Radiographs may be of use prior to the postmortem in detecting gas within the soft tissues. Gram stain and microbiologic culture are important in establishing a definitive diagnosis; although the major factors in suggesting the diagnosis are the recognition of the typical clinical history and macroscopic autopsy findings.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/30. A fatal case of craniofacial necrotizing fasciitis.A case of fatal craniofacial necrotizing fasciitis is described in a 72-year-old diabetic woman and management is discussed. Progressive infection of the eyelids occurred with involvement of the right side of the face. Computed tomography revealed soft tissue swelling. Antibiotic treatment was started and debridement performed; histopathology showed acute inflammation and thrombosis of the epidermis and dermis. Despite treatment, scepticemia occurred, resulting in death less than 48 h after presentation. At this time extensive necrosis had developed in the superficial fascia with undermining and gangrene of surrounding tissues. streptococcus and staphylococcus were the pathogens involved. Poor prognosis in similar patients has been associated with extensive infection, involvement of the lower face and neck, delayed treatment, advanced age, diabetes and vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/30. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/30. Vascular occlusions in the eye from cardiac myxomas.Vascular occlusion in the eyes from cardiac myxomas was diagnosed in two cases, belatedly in one and not until after death in the other. Suspicion of a myxomatous origin should be aroused by the combination of unexplained retinal (or choroidal) vascular disease occurring with multifocal neurological symptoms and with systemic symptoms suggesting atypical subacute bacterial endocarditis. The first patient had unilateral retinal artery occlusion by embolic material believed to have been myxomatous. Removal of the cardiac myxoma resulted in disappearance of this material (although the eye remained blind). The second patient who had had evidence of retinal artery occlusion in the clinical course of her multisystemic disease was found at autopsy to have extensive myxomatous involvement of the posterior ciliary arteries and of the choroidal arteries of both eyes and of the retinal artery in one eye.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/30. Two cases of penile necrosis in diabetics with end-stage renal failure.Distal penile necrosis associated with renal failure is a rare entity; only a few cases have been reported in the literature. Penile necrosis can frequently be a difficult management problem, the etiology of which is infectious, traumatic, or vascular. Physiological abnormalities are usually found in association with this condition, including diabetes, hyperparathyroidism, and peripheral vascular disease. Penile necrosis is a poor prognostic factor associated with high morbidity. We report two cases of this condition, presenting the clinical and pathophysiological background.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/30. fatal outcome in Eisenmenger syndrome.AIM: To assess correlations between fatal outcome and histologic findings of pulmonary vascular disease in different situations of Eisenmenger syndrome, either during the natural course or early-late after surgery. MATERIAL AND methods: The clinical follow-up and fatal outcome of 20 patients affected by Eisenmenger syndrome were investigated. In addition to the pathologic report and gross reexamination of the heart, the lung tissue was studied by histology. patients were divided into three groups: 6 non-operated patients who died during the natural course (Group 1), 11 patients who underwent correction of the congenital defect and died in the perioperative period (Group 2), and 3 patients who died late after surgery (Group 3). RESULTS: In Group 1, five patients (83%) died of cardiac arrest a few days after the onset of hypoxic attacks; in four patients histology showed Grade IV pulmonary vascular disease with diffuse fibrinoid necrosis in the distal pulmonary arterial vasculature. In Group 2, nine patients (82%) died on the first or second postoperative day after a refractory pulmonary hypertensive crisis, with histologic evidence in three patients of fibrinoid necrosis of the distal pulmonary small arteries and arterioles. In Group 3, two patients (67%) died suddenly, 6 and 18 years after cardiac surgery, following onset of dyspnea and cardiogenic shock; autopsy showed aneurysmal dilatation of the pulmonary artery with massive thrombosis in the setting of Grades III-IV pulmonary vascular disease without fibrinoid necrosis. CONCLUSION: fatal outcome in Eisenmenger syndrome, either in the natural course or after refractory hypertensive attacks post surgery, is frequently associated with fibrinoid necrosis of the small pulmonary arteries and arterioles.- - - - - - - - - - ranking = 3keywords = vascular disease (Clic here for more details about this article) |
9/30. Digital necrosis related to carboplatin and gemcitabine therapy in systemic sclerosis.We present a woman with scleroderma who developed multiple ischemic digits after chemotherapy for lung cancer. The ischemia started during treatment with carboplatin and gemcitabine and required amputation of the affected digits. A review of the literature shows that thrombotic episodes coinciding with chemotherapy are not uncommon, though venous thrombosis occurs more frequently than arterial. Scleroderma patients are at particular risk for digital infarction because of their underlying vascular disease and associated Raynaud's phenomenon. This case illustrates the risk of severe digital ischemia and digital loss in patients with scleroderma during chemotherapy with carboplatin and gemcitabine.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/30. Medial hemisoleus muscle flap: a reliable flap for soft tissue reconstruction of the middle-third tibial wound.Although the medial hemisoleus muscle has been considered a local reconstructive option for soft-tissue reconstruction of the middle-third tibial wound, its reliability and usefulness has been debated among plastic surgeons. This study reports one surgeon's experience with medial hemisoleus muscle flaps and shows their application for soft tissue reconstruction of middle-third tibial wounds. Over the past 2 years, 10 patients underwent soft tissue reconstruction of a middle-third tibial wound with a medial hemisoleus muscle flap and skin graft. The flap was elevated with emphasis on the preservation of as many perforators to the flap as possible while allowing adequate arch rotation of the flap to cover the exposed tibia and/or hardware. All patients were followed for up to 2 years. There was no total flap loss, and limb salvage was achieved in every patient in this series. Only two patients with peripheral vascular disease developed small partial but insignificant distal flap necrosis and were treated with debridement and flap advancement. The tibial wounds of all patients subsequently healed with excellent cosmetic outcome during follow-up. The results from this study indicate that the medial hemisoleus muscle flap is reliable for soft-tissue reconstruction of a middle-third tibial wound. Meticulous flap elevation with the preservation of perforators to the flap is the key for such success.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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