Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt.

Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder characterized by multiple benign and malignant tumors involving different organs (renal, adrenal, pancreas, liver, urogenital system, central nervous system, and head and neck region) due to mutations in the VHL tumor suppressor gene. Here, we describe a patient with unknown VHL disease who has complained of hypoesthesia of the right lower limb for about six years. A lumbar MRI was performed and revealed an expansive foraminal lesion at the right L3-L4 level and multiple serpiginous intradural and extramedullary flow voids involving the dorsal aspect of the spinal cord. The patient underwent digital subtraction angiography to exclude a spinal dural arteriovenous fistula, which revealed imaging features suggestive of spinal hemangioblastoma. In the presence of a spinal hemangioblastoma, a brain MRI was performed for further evaluation to rule out the possible diagnosis of VHL disease, and a solitary hemangioblastoma on the right cerebellar hemisphere was found. The patient underwent lumbar spine surgery, confirming the suspicious diagnosis of hemangioblastomas related to VHL disease.

The Rare Neurocutaneous Disorders: Update on Clinical, Molecular, and Neuroimaging Features.

Phakomatoses, also known as neurocutaneous disorders, comprise a vast number of entities that predominantly affect structures originated from the ectoderm such as the central nervous system and the skin, but also the mesoderm, particularly the vascular system. Extensive literature exists about the most common phakomatoses, namely neurofibromatosis, tuberous sclerosis, von Hippel-Lindau and Sturge-Weber syndrome. However, recent developments in the understanding of the molecular underpinnings of less common phakomatoses have sparked interest in these disorders. In this article, we review the clinical features, current pathogenesis, and modern neuroimaging findings of melanophakomatoses, vascular phakomatoses, and other rare neurocutaneous syndromes that may also include tissue overgrowth or neoplastic predisposition.

Intrarenal pseudoaneurysm after percutaneous nephrolithotomy. Angiotomographic assessment and endovascular management.

We report a case of intrarenal pseudoaneurysm of the right kidney after percutaneous nephrolithotomy (PCNL) in supine position. Diagnosis was established by angiotomography with a 3-D reconstruction. Treatment was successfully achieved by endovascular occlusion using N-butyl-2-cyanoacrylate.

Intrarenal pseudoaneurysm after percutaneous nephrolithotomy: angiotomographic assessment and endovascular management

We report a case of intrarenal pseudoaneurysm of the right kidney after percutaneous nephrolithotomy (PCNL) in supine position. Diagnosis was established by angiotomography with a 3-D reconstruction. Treatment was successfully achieved by endovascular occlusion using N-butyl-2-cyanoacrylate.

[The cerebral protection technique in angioplasty plus stenting of carotid: an effective procedure against cerebral embolism]. / Técnica de proteção cerebral na angioplastia e "stent" de carótida: um procedimento eficaz contra embolia.

OBJECTIVE: To describe the cerebral protection technique used in the angioplasty and stenting to treat stenotic lesions of carotid artery bifurcation, showing its efficiency in avoiding cerebral emboli during the procedure. METHOD: Forty two patients (47 arteries) were treated with the cerebral protection technique, which consists in the temporary occlusion of the internal carotid artery, above the stenotic lesion, performing the aspiration and flushing of glucose solution into the internal carotid artery after the delivery of the stent and the angioplasty, to remove any atherosclerotic plaque's fragments. RESULTS: The angioplasty was efficient in all cases. There were no cerebral emboli in the patients in whom the complete cerebral protection technique was used, showing its efficiency. Distal embolism occurred in one patient, during pre angioplasty and before the use of cerebral protection. CONCLUSION: The cerebral protection technique was efficient to avoid cerebral emboli in the procedures performed. This data is in agreement with the literature and the use of this technique increases the indications of carotid angioplasty.

Técnica de proteção cerebral na angioplastia e "stent" de carótida: um procedimento eficaz contra embolia / The cerebral protection technique in angioplasty plus stenting of carotid: an effective procedure against cerebral embolism

OBJETIVO: Descrever a técnica de proteção cerebral usada na angioplastia com colocação de "stent" para tratamento de lesões estenóticas da bifurcação carotídea, mostrando sua eficiência em evitar a embolia durante o procedimento. MÉTODOS: Quarenta e dois pacientes (47 artérias) foram tratados com a técnica de proteção cerebral que consistiu na oclusão temporéria da carótida interna acima da estenose, fazendo-se aspiração e injeção de solução de glicose a 5 por cento na carótida interna após a colocação de "stent" e angioplastia, para remover eventuais fragmentos da placa. RESULTADOS: A angioplastia foi eficiente em todos os casos. Não ocorreu embolia nos pacientes deste estudo nos quais se utilizou a técnica de proteção cerebral na sua versão completa. Embolia ocorreu em um paciente, durante pré-dilatação da estenose, antes de se usar a técnica de proteção cerebral. CONCLUSÃO: A técnica de proteção cerebral foi eficiente em evitar a embolia nos procedimentos realizados. Esses dados concordam com os da literatura e o uso desta técnica amplia as indicações da angioplastia carotídea.