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Christina Huang, Elias Rizk, Mark Iantosca, Andrea L. Zaenglein, Klaus F. Helm, Arabinda K. Choudhary and Mark S. Dias

Multifocal lymphangioendotheliomatosis with thrombocytopenia is a congenital vascular disorder characterized by the presence of multiple cutaneous macules, papules, and plaques. The number and size of these lesions vary, but hundreds can be seen ranging in size from a few millimeters to several centimeters. Similar lesions can also be found in other organs, including the gastrointestinal tract most commonly, as well as the lung, synovium, muscle, bone, bone marrow, kidney, liver, spleen, and brain. The gastrointestinal lesions can be quite numerous and involve the

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Marian Gaballah, Phillip B. Storm, Deborah Rabinowitz, Rebecca N. Ichord, Robert W. Hurst, Ganesh Krishnamurthy, Marc S. Keller, Adeka McIntosh and Anne Marie Cahill

, Nelson M , Giannotta SL , McComb JG : Intraoperative angiography in the management of pediatric vascular disorders . Pediatr Neurosurg 30 : 16 – 22 , 1999 15 Grossart KW , Turner JW : Operative angiography in cerebral vascular surgery . Br J Radiol 46 : 401 – 402 , 1973 16 Heros RC , Korosue K , Diebold PM : Surgical excision of cerebral arteriovenous malformations: late results . Neurosurgery 26 : 570 – 578 , 1990 17 Hino A , Fujimoto M , Iwamoto Y , Takahashi Y , Katsumori T : An adult case of recurrent

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Päivi Koroknay-Pál, Hanna Lehto, Mika Niemelä, Riku Kivisaari and Juha Hernesniemi

classic connective tissue disorders were not diagnosed in our series led us to suspect these patients had some vascular disorders that would predispose them in the future to cardiac diseases or other cerebrovascular events. However, this was not the case, with the exception of de novo aneurysms. The incidence of hypertension, hypercholesterolemia, stroke, and other conditions was no higher than in an age-matched Finnish population. Also, there was no increase in the incidence of autoimmune disorders. The mean proportion of smokers in Finland has been reported to be 22

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Kenichi Amagasaki, Hiromichi Yamazaki, Kimie Ohmori, Hidehito Koizumi, Kazuhiro Hashizume and Nobuo Sasaguchi

lymphomatosis that demonstrated an unusual association with venous stenosis manifesting as multifocal vascular disease and rapidly progressive dementia. This disease is one of the diagnostic possibilities that must be considered in any patient with a multifocal vascular disorder and rapidly progressive dementia. Case Report This 55-year-old man presented at another neurosurgical institution with paraparesis, sensory disturbance of the lower extremities, and dysuria. Neurological examination revealed paraparesis with hypoactive reflexes of the lower extremities and sensory

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Arya N. Bagherpour, Gustavo J. Rodriguez, Chetan Moorthy, Todd T. Trier and Alberto Maud

. Workup and Treatment Considerations The yield of spinal axis imaging in nonaneurysmal SAH is low, particularly in perimesencephalic SAH. 3 However, imaging of the spine should be considered in certain patients. At our institution, we perform MRI of the spine particularly when cerebral digital subtraction angiography (DSA) is nonrevealing (especially after 2 or more consecutive negative cerebral DSA studies) and in the following cases: posterior fossa–predominant SAH, certain predisposing vascular disorders, and pediatric patients because AVMs are more commonly a

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Andrew S. Jack, Michael M. Chow, Loretta Fiorillo, Thea Chibuk, Jerome Y. Yager and Vivek Mehta

disorder and may represent the most common neurocutaneous vascular disorder. 19 , 21 , 22 Several large registries and cohort studies have been reported outlining the prevalence and incidence of the disease’s different abnormalities. However, little has been reported regarding the natural history of the syndrome. More specifically, few have reported on the natural history of the intracranial arteri-opathy, which affects approximately 90% of PHACE patients. 13 Although rates of intracranial structural and cerebrovascular anomalies vary among PHACE patients, these

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Ayako Ro and Norimasa Kageyama

, report of 3 new cases, and comments on the role of endothelin-1 in its pathogenesis . Int J Surg Pathol 15 : 121 – 134 , 2007 15 Slavin RE , Yaeger MJ : Segmental arterial mediolysis—an iatrogenic vascular disorder induced by ractopamine . Cardiovasc Pathol 21 : 334 – 338 , 2012 16 Takagi T , Takayasu M , Suzuki Y , Yoshida J : Prediction of rebleeding from angiographic features in vertebral artery dissecting aneurysms . Neurosurg Rev 30 : 32 – 39 , 2007 17 Yamada M , Kitahara T , Kurata A , Fujii K , Miyasaka Y

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Shih-Shan Lang, Lauren A. Beslow, Robert L. Bailey, Arastoo Vossough, Joanna Ekstrom, Gregory G. Heuer and Phillip B. Storm

, Pardatscher K , Fiore DL : Cerebral arteriovenous malformations in children (56 cases) . Childs Brain 8 : 356 – 371 , 1981 8 Ghosh S , Levy ML , Stanley P , Nelson M , Giannotta SL , Mc-Comb JG : Intraoperative angiography in the management of pediatric vascular disorders . Pediatr Neurosurg 30 : 16 – 22 , 1999 9 Hladky JP , Lejeune JP , Blond S , Pruvo JP , Dhellemmes P : Cerebral arteriovenous malformations in children: report on 62 cases . Childs Nerv Syst 10 : 328 – 333 , 1994 10 Hoh BL , Carter BS , Ogilvy

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Pankaj K. Agarwalla, Christopher J. Stapleton, Michael T. Phillips, Brian P. Walcott, Andrew S. Venteicher and Christopher S. Ogilvy

natural history. Finally, associated conditions were studied in our patient population, and outside of the risk factors documented in Table 2 , there were no associated conditions such as anemia, radiation exposure, vasculitis, hereditary phakomatoses, associated vascular disorders, trauma, or a positive family history. One patient had Graves' disease, which has been associated with MMS. 13 Interestingly, there were a number of patients who had headache disorders (32%) or psychiatric conditions (24%) associated with moyamoya. Discussion Moyamoya disease and