11/609. Coats' disease of the retina (unilateral retinal telangiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal angiogenesis. Coats' disease is characterized by abnormal retinal vascular development (so-called 'retinal telangiectasis') which results in massive intraretinal and subretinal lipid accumulation (exudative retinal detachment). The classical form of Coats' disease is almost invariably isolated, unilateral and seen in males. A female with a unilateral variant of Coats' disease gave birth to a son affected by Norrie disease. Both carried a missense mutation within the NDP gene on chromosome Xp11.2. Subsequently analysis of the retinas of nine enucleated eyes from males with Coats' disease demonstrated in one a somatic mutation in the NDP gene which was not present within non-retinal tissue. We suggest that Coats' telangiectasis is secondary to somatic mutation in the NDP gene which results in a deficiency of norrin (the protein product of the NDP gene) within the developing retina. This supports recent observations that the protein is critical for normal retinal vasculogenesis. ( info) |
12/609. Renal involvement of thrombotic thrombocytopenic purpura: special reference to the glomeruloid structures. We report the case of a 9-year-old girl with biopsy-proven renal thrombotic microangiopathy in thrombotic thrombocytopenic purpura (TTP), with particular reference to the glomeruloid structures. The renal biopsy sample from this TTP patient revealed platelet thrombus deposition, a glomeruloid structure and aneurysm with relative sparing of the glomeruli. The glomeruloid structure displayed a proliferation of mainly capillary-sized channels lined by factor viii-related, antigen-positive plump endothelial cells embedded in the edematous connective tissue. These glomeruloid vessels communicated with the aneurysmal segment at the end portion of the arteriolar branch. We believe that the glomeruloid structures in TTP represent not merely organization or recanalization of thrombus but rather active angiogenesis through aneurysmal dilation in the arteriolized vessel, probably initiated by platelet agglutination. ( info) |
13/609. Anti-Kaposi's sarcoma and antiangiogenic activities of sulfated dextrins. Delivery of the sulfated polysaccharide dextrin 2-sulfate by the intraperitoneal route to the lymphatic circulation resulted in a clinically significant improvement in Kaposi's sarcoma in three patients. Our in vitro studies show that although sulfated dextrins do not interfere with the growth of isolated human umbilical vein endothelial cells, they do inhibit the morphological differentiation of endothelial cells into tubes as well as reduce new vessel formation in a placental angiogenesis assay. The antiangiogenic effect of dextrin 6-sulfate is greater than that of dextrin 2-sulfate and is independent of their anti-human immunodeficiency virus type 1 activities. ( info) |
14/609. Regression of large atrial thrombi and coronary neovascularizations with conventional anticoagulation in mitral stenosis--a case report. The authors report a case of angiographically documented multiple coronary neovascularizations originating from the left circumflex artery (LCX) and coursing toward multiple thrombi located in the left atrium in a patient with severe mitral stenosis. The thrombi as well as the neovascularizations underwent near-complete resolution with 4 weeks' anticoagulation therapy with warfarin maintaining an international normalization ratio of 3.5. Percutaneous mitral balloon valvuloplasty was performed successfully without complications. ( info) |
15/609. Multifocal choroiditis with panuveitis and punctate inner choroidopathy: a mini review. Multifocal choroiditis and punctate inner choroidopathy cause scattered acute chorioretinal lesions in the fundus. Secondary choroidal neovascularization and, more rarely, diffuse subretinal fibrosis without obvious neovascularization are associated with both syndromes and cause severe visual loss. Both disorders are of unknown etiology and have many similarities. It is our purpose to present four such cases with emphasis on their fluorescein and indocyanine green angiographic appearance. We review shortly the literature on the subject. ( info) |
16/609. Slender versus threatening angiodysplasias: observe, operate or obturate? A delusive decision. Beside the immediate success of surgery and of embolization of angiodysplasias, certain number of patients return to medical consultation, because recanalization of the arteriovenous fistulae after their resection or obturation. From the presentation of two clinical cases of thoracic angiodysplasia: one of them slender, and threatening the other, the authors point out the complications and recurrence after surgical resection or arterial embolization. The recurrence takes place even when only one artery remains permeable after resection or embolization of the fistula. There are cases where the affected limb has to be amputated. However, angiodysplasia shows itself in the stump of the amputee limb. Uncontrolled angio-genesis is the natural history or postoperatory evolution of angiodysplasias, probably originated by an erroneous genetic program, which persist in spite of resection of the vascular malformation, causing a therapeutic failure. ( info) |
17/609. Giant primary cardiac liposarcoma vascularized via the circumflex coronary artery. A 51-year-old patient presented with a history of chest pain and progressive dyspnea. CT scan demonstrated a lipomatous mass encircling the heart and compressing the left lung. After median sternotomy, an intrapericardial tumor of 3200 g with a vascular pedicle arising from the circumflex coronary artery was resected. A histologic diagnosis of a well-differentiated liposarcoma was made. The patient is alive and well 2 years postoperatively. This tumor with its unusual vascularization is the largest of the few primary cardiac liposarcomas that have been reported. ( info) |
18/609. Cilioretinal artery occlusion with central retinal vein occlusion. BACKGROUND: Combined cilioretinal artery and retinal vein occlusions are infrequently documented retinal vascular disorders of speculative origin. Occlusion of the cilioretinal artery is believed to result from either mechanical compression of the artery as a result of an increase in venous pressure or from a reduction in perfusion pressure in both the cilioretinal and retinal arteries. The ophthalmoscopic and angiographic features of this condition are reviewed. case reports: Two cases of cilioretinal artery occlusion after central retinal vein occlusion are presented, one of which evolved to the development of iris neovascularization. DISCUSSION: The incidence of cilioretinal artery occlusions due to central retinal vein occlusions is infrequently reported in the literature. Excluding those with chronic cystoid macular edema, most patients have a favorable visual outcome. It is possible that the incidence of combined cilioretinal artery and central retinal vein occlusions is grossly underestimated. ( info) |
19/609. Macular fibrosis associated with talc retinopathy. PURPOSE: To investigate a patient with talc retinopathy who developed macular fibrosis with resultant visual loss. methods: A 64-year-old intravenous drug abuser was evaluated for bilateral peripheral retinal neovascularization. He admitted to abusing oral methylphenidate intravenously. Funduscopy showed numerous intravascular talc particles in the macular area of both eyes. Over a period of next 4 years, visually significant macular fibrosis gradually developed in both eyes, the left eye more than the right eye. RESULTS: fluorescein angiography confirmed the presence of bilateral peripheral retinal neovascularization with adjacent areas of ischemic retina. The patient was treated with peripheral laser treatment to the ischemic retina with resultant regression of peripheral retinal neovascularization. Bilateral macular fibrosis accounted for the reduced vision in our patient. CONCLUSION: talc retinopathy can be associated with macular fibrosis with resultant visual loss. ( info) |
20/609. Interferon alpha-2b at low doses as long-term antiangiogenic treatment of a metastatic intracranial hemangioendothelioma: a case report. We describe a case of intracranial haemangioendothelioma in a 20-year old female patient who presented severe neurological symptoms and relapsed after two surgical interventions. The patient was treated with low doses of recombinant interferon alpha-2b (1 MUI three times a week) after surgical resection which led to recovery of daily function and work activity. To our knowledge, this is the ninth patient reported with intracranial hemangioendothelioma, but the only one having diffuse and painful bone metastases resolved by treatment with interferon. After 30 months the patient is free from symptoms and recurrence. The effectiveness shown by recombinant interferon alpha-2b against vascular neoplasms prompted us to look for the possible biological basis of such a property. ( info) |