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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe two children with anomalous intracranial venous return associated with bilateral facial nevi, macrocrania, and cephalic venous hypertension. Both children had functional absence of the jugular bulbs, forcing the intracranial venous effluent to exit through persistent emissary pathways. Both children had sustained intracranial hypertension, with one child developing symptomatic communicating hydrocephalus that responded satisfactorily to shunting. The relationship between these patients and those with Sturge-Weber syndrome is discussed. The embryologic abnormality producing the anomalous venous return is characterized. The link between venous hypertension and the development of hydrocephalus is discussed. The increased cranial compliance seen in this age group may predispose certain pediatric patients to develop hydrocephalus when stressed by venous hypertension.
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PMID:Facial nevi associated with anomalous venous return and hydrocephalus. 93 97

Based on a retrospective evaluation of 107 patients with congenital venous angiodysplasia of the Type Klippel-Trenaunay (n = 76) and Type Servelle-Martorell (n = 31) the frequency and pathogenesis of aneurysma formation in the venous system has been analysed. The vascular patterns include both cylindric ectasia and fusiform aneurysms with an incidence of approximately 40%. Preferred locations are subcutaneous drainage veins, the popliteal, external iliac vein and atypic communicating veins between the superficial and the deep venous system. Complications of the aneurysm such as local thrombosis, recurrent pulmonary embolism or bleeding from ruptur were not observed. The aneurysma formation in venous angiodysplasias results probably from the causative factors: congenital weakness of the venous wall and an abnormal hemodynamical stress situation. The latter is caused by concomitant malformations of the deep venous system. The persistent intermittent venous hypertension associated with a more or less pronounced increase of the venous volume in the affected venous system of the limb results in a deep venous insufficiency respectively venous reflux disease. The therapy of choice is predominantly conservative, i.e., external compression bandages or stockings to reduce the deleterious effects of a chronic deep venous insufficiency respectively venous reflux disease. Surgery is indicated under two conditions: a) in the presence of aneurysm complications or b) for the elimination of a pathological short circuit flow in some drainage veins. Antireflux surgery, i.e., venous valve transfer from the brachial vein, is up to recently still in a stage of experimental-clinical investigation.
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PMID:Aneurysma transformation in congenital venous angiodysplasias in lower extremities. 217 53

Based on a retrospective evaluation of 107 patients with congenital venous angiodysplasia of the Type Klippel-Trenaunay (n = 76) and Type Servelle-Martorell (n = 31) the frequency and pathogenesis of aneurysm formation in the venous system has been analysed. The vascular patterns include both cylindric ectasias and fusiform aneurysms with an incidence of approximately 40%. Preferred locations are subcutaneous drainage veins, the popliteal, external iliac vein and atypic communicating veins between the superficial and the deep venous system. Complications of the aneurysm such as local thrombosis, recurrent pulmonary embolism or bleeding from rupture were not observed. From a pathogenetic point of view the aneurysm formation in venous angiodysplasias results probably from two causative factors, i.e., a congenital weakness of the venous wall (inborn error?) and an abnormal hemodynamical stress situation. The latter is caused by concomitant malformations of the deep venous system (avalvulia, hypo- and/or aplasia). The persistent intermittent venous hypertension associated with a more or less pronounced increase of the venous volume in the affected venous system of the limb results in a deep venous insufficiency respectively venous reflux disease. Surgery is indicated under two conditions: a) in the presence of aneurysm complications or b) for the elimination of a pathological short circuit flow in some drainage veins. Antireflux surgery, e.g., venous valve transfer form the brachial vein, is up to recently still in a stage of experimental-clinical investigation. The therapy of choice is predominantly conservative, i.e., external compression bandages or stockings to reduce the deleterious effects of a chronic deep venous insufficiency respectively venous reflux disease.
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PMID:[Aneurysmic transformation of the venous system in venous angiodysplasias of the limbs]. 254 55

We describe a 23-year-old girl with an extremely uncommon form of cerebral venous drainage and cerebellar leptomeningeal angiomatosis as a possible variant of the Sturge-Weber syndrome. Extensive congenital port-wine stains all over the body, hypoplastic left renal and subclavian and iliac veins, cardiomegaly and ptosis and hypoplasia of the left kidney had been recognised in early childhood. She rapidly developed signs of intracranial hypertension. CT and MRI showed a right medial temporal lesion. Angiography revealed cerebellar pial angiomatosis with enlarged medullary veins and no functioning sigmoid sinuses or jugular veins. Cerebral venous drainage was via enlarged ophthalmic veins. Although the intracranial venous abnormalities were characteristic of the Sturge-Weber syndrome anomalies beyond the encephalofacial territory suggested a more complex developmental abnormality.
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PMID:Cerebral venous drainage via the ophthalmic veins in the Sturge-Weber syndrome. 806 81

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder that is typically associated with progressive neurological deterioration. We describe a 12-year-old girl with SWS who suffered a permanent cerebral insult as the result of a period of protracted status epilepticus. The case illustrates the unique susceptibility of patients with SWS to uncontrolled venous hypertension and emphasises the need for optimal seizure control and preservation of venous outflow. We discuss the relevance of our observations to haemodynamic concepts of neurological decline in SWS.
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PMID:Status epilepticus and venous infarction in Sturge-Weber syndrome. 988 20

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. Sturge-Weber Syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. Cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.
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PMID:Intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow. 1209 50

The paper deals with hemorrhagic stroke (HS) pathogenesis and diagnosis in young people. Among cerebrovascular diseases in the young acute hemorrhagic strokes take noticeable place. Arterial hypertension, diabetes mellitus, smoking, alcoholism are among risk factors of subarachnoidal hemorrhage (SAH). Massive hemorrhages occur in the rupture of arterial aneurysms and arteriovenous malformations. HS in the young may be caused by blood diseases, i.e. leukemias, hemophilias, idiopathic thrombocytopenic purpura, coagulopathies; vasculitis in diffuse diseases of the connective tissue; non-inflammatory arteriopathies; drug addiction. Genetic predisposition to HS development is discussed with focus to such diseases as a family form of moya-moya disease, glucocorticoid-depressed hyperaldosteronism, elastic pseudoxanthoma, Marfan's syndrome, renal olycystosis, Sturge-Veber syndrome. It is recommended to use wider updated methods of neurovisualization (CT, MRT, angiography) in diagnosis of HS. The conclusion is made that HS diagnosis, especially in the young, needs a multidisciplinary approach with active participation of neurologist, neurosurgeon, therapist, endocrinologist, hematologist.
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PMID:[Specific features of pathogenesis and diagnosis of hemorrhagic stroke in young patients]. 1511 67

The aim of this report is to explain the historical differences between Klippel-Trenaunay (KT), Klippel-Trenaunay-Weber (KTW), and Klippel-Trenaunay-Servelle (KTS) syndromes. Furthermore, an attempt will be made to describe the different causes, symptoms, and consequences of the pathology, largely as a consequence of venous hypertension. The significance of these syndromes within the pediatric population is highlighted.
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PMID:Klippel-Trenaunay-Servelle syndrome in pediatrics. 1562 26

According to a new, unifying view of the pathogenesis of Sturge-Weber syndrome and related syndromes, signs and symptoms all arise from localized primary venous dysplasia, with effects of venous hypertension transmitted to nearby areas via persisting communicating venous passageways and compensatory collateral venous channels. Port-wine stains result from a vascular disorder rather than a neural disorder. Symptoms depend upon the extent and location of the venous dysplasia. This hypothesis is supported by published data and by original observations and Doppler ultrasonographic studies of orbital venous flow in patients with the Sturge-Weber syndrome. This new understanding of underlying pathophysiology also elucidates the mechanism for tissue hypertrophy. Therapies aimed at obliterating port-wine stains to minimize the cosmetic blemish will reduce collateral venous blood-flow passageways. In some instances, this reduction may worsen blood stasis within the brain and potentially exacerbate neurologic symptoms.
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PMID:Sturge-weber syndrome: a unified pathophysiologic mechanism. 1832 99

Neurofibromatosis type 1 (NF-1) is an autosomal dominant, hereditary, neurocutaneous syndrome that may, primarily or secondarily, affect different organs or systems of the body including the cardiovascular system. The most common vascular abnormality in patients with NF-1 is renal artery stenosis. Here we report the case of a middle-aged gentleman who presented at Sultan Qaboos University Hospital, Oman, with end stage renal disease and severe hypertension and was diagnosed to have NF-1 with bilateral renal artery stenosis. He was started on renal replacement therapy.
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PMID:Neurofibromatosis with bilateral renal artery stenosis and end stage renal disease: case report. 2150 95


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