Trombosis de Senos Venosos, experiencia en un hospital pediátrico en Buenos Aires, Argentina / Venous sinus thrombosis: Experience at a pediatric hospital in Buenos Aires, Argentina

Introducción: La trombosis de senos venosos cerebrales (TSVC) es infrecuente en pediatría. Está asociada a condiciones como infecciones, deshidratación, fallo renal, traumatismo de cráneo, neoplasias, trastornos hematológicos, etc. Cefalea, vómitos, alteración del sensorio y hemiparesia son los síntomas más frecuentes. El diagnóstico es confirmado por TC con angio y/o RM con angio. La anticoagulación es el tratamiento de elección. Los pacientes suelen evolucionar favorablemente. Materiales y Métodos: Estudio descriptivo observacional de pacientes con TSVC atendidos en el Hospital Garrahan desde 2010 a 2017. Las variables registradas fueron: edad, sexo; manifestaciones clínicas, factores de riesgo; estudios diagnósticos, tratamiento y evolución. Resultados: Se describen 34 pacientes con TSVC. Los adolescentes fueron el grupo mayor. La cefalea fue el síntoma más frecuente. Angio TC, RM y/o angio RM confirmaron el diagnóstico; los senos transverso, sagital superior y sigmoideo fueron los más comprometidos. 21 pacientes tenían patología oncológica y 14 procesos infecciosos. El tratamiento de elección fue la anticoagulación. Tuvieron buena evolución el 82%. Conclusiones: Debemos sospechar esta entidad en dos grupos: el primero formado por lactantes y pre-escolares con patología infecciosa; y un segundo integrado por escolares y adolescentes con patología oncológica, especialmente aquellos que reciben L-ASA.Es importante resaltar el valor de la TC y angio TC para hacer diagnóstico oportuno, resultando accesible las 24 horas en el hospital

Introduction: Cerebral venous sinus thrombosis (CVST) is uncommon in children. CVST is associated with conditions, such as infections, dehydration, renal failure, head trauma, cancer, and hematological disorders. Headache, vomiting, sensory alterations, and hemiparesis are the most common symptoms. Diagnosis is confirmed by angio CT and/or MRA. Anticoagulation is the treatment of choice. Outcome is generally good. Material and Methods: An observational, descriptive study of patients with CVST seen at Garrahan Hospital between 2010 and 2017. The following variables were recorded: age, sex; clinical manifestations, risk factors; diagnostic studies, treatment, and outcome. Results: 34 patients with CVST were studied. Most patients were adolescents. Headache was the most common symptom. Angio CT, MRI, and/or MRA confirmed the diagnosis; the transverse, superior sagittal, and sigmoid sinuses were most frequently affected. Of the patients, 21 had oncological disease and 14 infections. Anticoagulation was the treatment of choice. Outcome was good in 82%. Conclusions: CVST should be suspected in the following two groups: A first group consisting of infants and preschool children with infections and a second group of school-age children and adolescents with cancer, especially those receiving L-ASA. It is important to highlight the role of CT and angio CT for early diagnosis as the study is available day and night at the hospital.

Trombosis venosa cerebral asociada a consumo de sildenafil / Cerebral venous thrombosis associated with sildenafil intake

Se presentó un paciente masculino de 59 años de edad, con antecedentes de hipertensión arterial y consumo regular de sildenafil, que ingresó en el servicio de ictus por presentar convulsión aguda y cefalea. Al examen físico se detectó hemiplejia izquierda. Mediante resonancia magnética nuclear de cráneo, con gadolinio se observó lesión expansiva que captó contraste de forma heterogénea. Se planteó el diagnóstico de posible glioma cerebral de alto grado. Se realizó craneotomía frontoparietal derecha y no se visualizó lesión tumoral. Después de descartar la presencia de un glioma de alto grado se valoró el diagnóstico de infarto venoso hemorrágico. Se efectuó angioTAC y estudio de trombofilia para descartar estados protrombóticos que justificaran dicha entidad nosológica. Los estudios de hematología especial se encontraron dentro de valores normales. Se concluyó el caso como una trombosis de senos venosos asociada al consumo de sildenafil(AU)

A 59 year-old male patient with a history of hypertension and regular consumption of sildenafil entered the service because of acute stroke and headache seizure. Physical examination detected left hemiplegia. By skull nuclear gadolinium magnetic resonance, an expansive lesion is observed which caught heterogeneously contrast. Possible diagnosis of high-grade brain glioma was raised. Right frontoparietal craniotomy was performed and a tumor lesion was not visualized. After ruling out the presence of high-grade glioma, the diagnosis of hemorrhagic venous infarction was assessed. CT angiography and clotting was made to rule out prothrombotic states to justify this disease entity. Special hematology studies were within normal values. The case was concluded as a venous sinus thrombosis associated with the use of sildenafil(AU)

Factors affecting visual loss and visual recovery in patients with pseudotumor cerebri syndrome / Fatores que influenciam na perda e na recuperação visual de pacientes com a síndrome do pseudotumor cerebral

ABSTRACT Purpose: To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome. Methods: We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis. Results: The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042). Conclusions: VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset. .

RESUMO Objetivo: Investigar a frequência de perda visual (PV) e os possíveis fatores preditivos para perda e para melhora visual em pacientes com a síndrome do pseudotumor cerebral (SPC). Métodos: Foram revisados 50 pacientes com SPC submetidos a exame neuroftalmológico no momento do diagnóstico e após o tratamento. Dados demográficos, índice de massa corpórea (IMC), tempo decorrido entre o início dos sintomas e o diagnóstico (TD), pressão intracraniana máxima (PIM), ocorrência de trombose venosa cerebral (TVC), e as modalidades de tratamento foram revisadas. PV foi graduada em discreta, moderada e grave, baseada na acuidade e no campo visual. Fatores preditivos para perda e melhora visual foram avaliados por análise de regressão linear. Resultados: Quarenta e três pacientes eram do sexo feminino. A média de idade, o IMC e a PIM (± desvio padrão) foram: 35,2 ± 12,7 anos, 32,0 ± 7,5 kg/cm2 e 41,9 ± 14,5 cmH2O, respectivamente. Sintomas visuais estavam presentes em 46 e TVC em 8 pacientes. TD (em meses) foi <1 em 21, 1-6 em 15 e >6 em 14 pacientes. Pacientes receberam tratamento clinico apenas (n=30) ou associado a tratamento cirúrgico (n=20). Na apresentação a PV era discreta em 16, moderada em 12 e grave em 22 pacientes. Vinte e oito pacientes melhoraram e 5 pioraram. PIM, TD e hipertensão arterial correlacionaram significativamente com PV grave. O melhor fator preditivo para PV grave foi o TD>6 meses (p=0,04; razão de chances 5,18). TD entre 1 e 6 meses foi o único fator significativamente associado com melhora visual após tratamento (p=0,042). Conclusões: Perda visual é comum na SPC e quando grave se mostra relacionado a atraso no diagnóstico. É usualmente permanente mas pode haver melhora visual especialmente quando a doença é diagnosticada nos primeiros 6 após o início dos sintomas. .

Trombosis venosa cerebral / Cerebral venous thrombosis

La trombosis venosa cerebral (TvC) es una rara enfermedad que se incluye dentro del grupo de trombosis en sitios infrecuentes. El correcto estudio y manejo terapéutico de la misma es de capital importancia por la alta morbimortalidad que conlleva de no diagnosticarse y tratarse correctamente. El desarrollo en el conocimiento de los factores de riesgo como las trombofilias entre otros, ha sido de gran importancia diagnóstica. Aún faltan estudios randomizados y controlados de gran número de pacientes para definir la duración óptima del tratamiento según cada caso particular. Por el momento las recomendaciones terapéuticas en este tipo de trombosis se basan en estudios observacionales y opinión de expertos, los cuales recomiendan anticoagulación a largo plazo para pacientes con trombosis inexplicadas, trombosis recurrentes, o asociadas a trombofilias de alto riesgo o combinadas. El objetivo de esta publicación es presentar una serie de casos clínicos, revisar el tema de TvC dado la importancia por las posibles complicaciones evolutivas; conocer los factores de riesgo y discutir las posibilidades terapéuticas.

Abducens Nerve Palsy Complicated by Inferior Petrosal Sinus Septic Thrombosis Due to Mastoiditis

We present a very rare case of a 29-month-old boy with acute onset right abducens nerve palsy complicated by inferior petrosal sinus septic thrombosis due to mastoiditis without petrous apicitis. Four months after mastoidectomy, the patient fully recovered from an esotropia of 30 prism diopters and an abduction limitation (-4) in his right eye.

A Case of Atypical Skull Base Osteomyelitis with Septic Pulmonary Embolism

Skull base osteomyelitis (SBO) is difficult to diagnose when a patient presents with multiple cranial nerve palsies but no obvious infectious focus. There is no report about SBO with septic pulmonary embolism. A 51-yr-old man presented to our hospital with headache, hoarseness, dysphagia, frequent choking, fever, cough, and sputum production. He was diagnosed of having masked mastoiditis complicated by SBO with multiple cranial nerve palsies, sigmoid sinus thrombosis, and septic pulmonary embolism. We successfully treated him with antibiotics and anticoagulants alone, with no surgical intervention. His neurologic deficits were completely recovered. Decrease of pulmonary nodules and thrombus in the sinus was evident on the follow-up imaging one month later. In selected cases of intracranial complications of SBO and septic pulmonary embolism, secondary to mastoiditis with early response to antibiotic therapy, conservative treatment may be considered and surgical intervention may be withheld.

Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension.

Retrospective descriptive study reporting the rate of occurrence of cerebral venous sinus thrombosis (CVST), highlighting the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in patients with presumed idiopathic intracranial hypertension (IIH). Study was conducted in the department of neuro-ophthalmology at a tertiary eye care center in South India. Data from 331 patients diagnosed with IIH from June 2005 to September 2007 was included. Inclusion criteria were: Elevated opening cerebrospinal fluid (CSF) pressure of more than 200 mm of water on lumbar puncture, normal CSF biochemistry and microbiology, and normal neuroimaging as depicted by computed tomography(CT) scan. Exclusion criteria were: Space-occupying lesions, hydrocephalus, meningitis, intracranial pressure within normal range, abnormal CSF biochemistry and microbiology. The remaining patients were evaluated with MRI and MRV. CVST was present in 11.4% of patients who were presumed to have IIH (35/308). MRI alone identified 24 cases (68%) of CVST, while MRI used in combination with MRV revealed an additional 11 cases (32%). Risk factors associated with CVST were identified in nine out of 35 patients (26%). CVST may be misdiagnosed as IIH if prompt neuroimaging by MRI and MRV is not undertaken. Risk factors of CVST may not be apparent in all the cases and these patients are liable to be missed if CT scan alone is used for neuroimaging, hence MRI, combined with MRV should be undertaken to rule out CVST.

Hemorragia subaracnoidea: presentación inusual de una trombosis de senos venosos: caso clínico y revisión de literatura / Subarachnoid hemorrhage: unusual presentation of a venous sinus thrombosis: case report and review of literature

La hemorragia subaracnoidea (HSA) usualmente sugiere la presencia de una lesión vascular, tal como ruptura de un aneurisma o sangrado de una malformación arteriovenosa. El hallazgo de una HSA se considera una urgencia neuroquirúrgica y se debe tratar con mucha delicadeza. La HSA puede ser secundaria a otras causas, y por tal razón se debe estar pendiente de los examenes a realizar para conocer la causa de esta. Otra causa poco frecuente de HSA e la Trombosis de Senos Venosos (TSV). La TSV es difícil de diagnosticar y se complica aún más su diagnóstico cuando se presenta con HSA. El objetivo de este artículo es la presentación de un caso de trombosis de seno venoso y revisar la literatura al respecto.

Subarachnoid Hemorrhage (SAH) usually suggests the presence of a vascular lesion as a ruptured aneurism or a bleeding from an arterial-venous malformation. The finding of a SAH is considered a neurosurgical emergency and it should be treated as that. SAH can also be caused by other etiologies and for this reason the cause of this finding should be investigated. Cerebral Sinus Thrombosis is one rare cause of SAH. Cerebral Sinus Thrombosis is usually difficult to diagnose and the presence of a SAH increases the difficulty of diagnosing this entity. The objective of this article is to present a case report and to review the literature with respect to this case.

Subarachnoid haemorrhage as a presentation of cerebral venous sinus thrombosis.

Cerebral venous sinus thrombosis can present with a variety of clinical symptoms ranging from isolated headache to deep coma. Subarachnoid haemorrhage is a rare presentation. In the report, we describe a case of non-puerperal venous thrombosis of many dural sinuses manifesting on imaging solely as localized subarachnoid haemorrhage without underlying parenchymal involvement.

Pseudotumor cerebral secundário a trombose venosa dural: relato de caso pediátrico / Pseudotumor cerebri secondary to dural sinus thrombosis: pediatric case report

O pseudotumor cerebral é uma síndrome neurológica relativamente comum na adolescência. Na maioria dos casos, a etiologia é idiopática, mas pode haver complicações graves, como cegueira, relacionadas com a hipertensão intracraniana. O objetivo deste artigo é enfatizar o diagnóstico diferencial do pseudotumor cerebral, com atenção especial às etiologias tratáveis. Relatamos o caso de um adolescente de 12 anos que se apresentou com diplopia e cefaléia 9 dias após otite média e mastoidite à direita. A tomografia computadorizada do crânio foi normal, mas a ressonância magnética do encéfalo detectou trombose dos seios transverso e sigmóideo ipsilaterais, a qual respondeu à anticoagulação precoce. A conclusão é que a ressonância magnética do encéfalo é essencial nos pacientes com diagnóstico clínico de pseudotumor cerebral para exclusão de causas tratáveis, como a trombose venosa dural.

Perda visual bilateral grave como sinal de apresentação de trombose de seio venoso cerebral: relato de caso / Severe bilateral visual loss as the presenting sign of cerebral venous sinus thrombosis: case report

O objetivo é relatar um caso de trombose de seio venoso cerebral causando síndrome de pseudotumor, onde a principal manifestação clínica foi uma perda visual bilateral grave. Homem de 24 anos apresentando perda visual bilateral grave, obscurecimentos transitórios da visão, cefaléia, papiledema bilateral e campos visuais constrictos. Tomografia computadorizada, bem como exame neurológico, apresentavam-se normais. Punção lombar mostrou um aumento da pressão liquórica inicial e o hemograma mostrou aumento do número de plaquetas. A ressonância magnética de crânio apresentava sinais característicos de papiledema crônico e sinais sugestivos de trombose de seio sagital superior. A venorressonância magnética de crânio mostrou trombose de seios sagital superior, transversos e sigmóides. Paciente foi tratado com descompressão de bainha de nervo óptico do olho esquerdo, acetazolamida e anticoagulação. O exame oftalmológico após o tratamento mostrou redução do papiledema, mas com persistência de perda visual acentuada. O pseudotumor cerebral com perda visual severa pode ser um sinal de apresentação de trombose venosa cerebral secundária a doença hematológica grave.

Cerebral sinovenous thrombosis in a nephrotic child

Nephrotic syndrome in infancy and childhood is known to be associated with a hypercoagulable state and thromboembolic complications, but cerebral sinovenous thrombosis (CST) is a very rare and serious one, with only a few isolated reports in the literature. A case is presented of a 9-year-old boy with nephrotic syndrome that acutely developed signs and symptoms of intracranial hypertension syndrome. CST was diagnosed on cranial CT and MRI and he gradually recovered after treatment with anticoagulants. The diagnosis of CST should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. The discussion of this case, coupled with a review of the literature, emphasizes that early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. This report also illustrates the difficulties that may be encountered in managing such a patient

Stroke in children.

Stroke is defined as the sudden occlusion or rupture of cerebral arteries or veins resulting in focal cerebral damage and neurological deficits. Forms of stroke resulting from vascular occlusion are arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) and those resulting from vascular rupture are called hemorrhagic stroke. Stroke in children is relatively rare and frequently results in a lack of recognition and delay in diagnosis. The etiologies of stroke in children are legion and multiple risk factors coexist unlike unifactorial etiology in adults. Heart disease whether congenital or acquired, malformations, metabolic and hematological disorders and vasospastic conditions like migraine are seen more often in childhood strokes. The purpose of diagnostic evaluation includes confirmation of the presence of a cerebrovascular lesion, exclusion of other types of neurological dysfunction and identification of etiology of the stroke. The treatment of stroke in children has been primarily directed toward stabilizing systemic factors and management of the underlying causes. Various antithrombotic and non antithrombotic therapies are discussed. The use of anticoagulant therapy appears to be increasing in pediatric AIS. Mortality after stroke in children ranges from 20% to 30% depending on the location and the underlying cause. Residual neurological dysfunction is present in more than 50% of survivors.

Pseudotumor syndrome associated with antiphospholipid antibodies and cerebral venous sinus thrombosis.

Antiphospholipid antibodies have been recognized as a marker for an increased risk of thrombosis, including cerebral venous thrombosis. This is a clinical study of three patients who presented with features of raised intracranial tension. Investigations revealed normal CT of brain and CSF examination in two patients. MRI of brain revealed dural venous sinus thrombosis in all the patients and positive antiphospholipid antibodies in the blood. All patients recovered with anticoagulant therapy. Antiphospholipid antibodies should be considered in the differential diagnosis of pseudotumor syndrome related to cerebral venous thrombosis.

Systemic lupus erythematosus complicated with cerebral venous sinus thrombosis: a report of two cases

A rare manifestation of systemic lupus erythematosus (SLE) is cerebral venous sinus thrombosis (CVST), in which early diagnosis and aggressive therapy are of prime importance for favorable outcome. The pathogenesis of CVST is largely unknown, but it is thought to be caused by cerebral vasculitis, antiphospholipid antibodies or other conditions associated with enhanced coagulability. We describe two cases of SLE with CVST which were not associated with antiphospholipid antibodies. Both cases were treated with immunosuppressants (intravenous methylprednisolone and cyclophosphamide pulse therapy) and anticoagulant drugs (heparin and subsequent maintenance therapy with warfarin). There was a marked improvement of neurologic symptoms with the disappearance of thrombus in a follow-up MRI. The possibility of CVST should be considered in any patients with SLE who show neuropsychiatric manifestations.

Meningite neonatal por Streptococcus pyogenes e trombose de seio sagital: relato de caso / Neonatal Streptococcus pyogenes meningitis and sagital sinus thrombosis: case report

Relatamos um caso de meningite por Streptococcus pyogenes em menina de 18 dias de vida, com evoluçao complicada por trombose de seio sagital. Sao discutidos alguns aspectos da patogênese, tratamento e seguimento da doença. Frente ao aumento mundial das infecçoes estreptocócicas graves nos últimos 10 anos, é provável que a meningite neonatal por Streptococcus pyogenes se torne mais frequente no futuro, sendo importante estar alerta para o diagnóstico precoce e as possíveis complicaçoes dessa infecçao potencialmente letal.

Thrombose du sinus caverneux. retard au diagnostic and complications

We report a case of cavernous sinus thrombosis in an 8-year old boy, following a neglected and untreated nasal furuncle. This rare entity is manifested by hyperthermia, with headache, vomiting and involvement of the III-IV AND VI cranial nerves. It is usually a complication of a facial infection. The diagnosis is established by CT scan or MRI. Without appropriate and early treatment the prognosis is poor with a fatal outcome or severe sequelaes

Cerebral sinovenous thrombosis in a patient with paroxysmal nocturnal haemoglobinuria

Paroxysmal nocturnal haemoglobinuria may be complicated by life-threatening thrombosis. A patient who presented with the classical clinical picture of cerebral sinus thrombosis is described. Management of the condition is discussed.

Isquemias de territorio carotídeo en trombosis séptica del seno cavernoso / Brain ischemic lesions within the carotid artery territory en cavernous sinus thrombosis

Se presentan dos pacientes con clínica de tromboflebitis del seno cavernoso, quienes desarrollaron complicaciones isquémicas del territorio carotídeo asociado. Uno de ellos presentó embolia de la arteria central de la retina y trombosis carotídea proximal. El segundo mostró un infarto silviano homolateral al proceso infeccioso. El mecanismo iniciador de este inusual tipo de complicación podría ser la arteritis y estenosis focal del segmento intravenoso de la carótida interna, a partir del cual se desencadenarían fenómenos embólicos (distales al estrechamiento) y/o trombóticos (proximales al mismo)