Venous sinus thrombosis in pediatrics. Case series of a tertiary hospital. / Trombosis de senos venosos en pediatría. Serie de casos de un hospital terciario.

INTRODUCTION: Venous sinus thrombosis (VST) is a rare entity in pediatrics, probably under-diagnosed and poten tially serious, described as a cause of stroke in childhood. OBJECTIVE: To describe the clinical presenta tion, risk factors, treatment, and evolution of pediatric patients with VST. PATIENTS AND METHOD: Re trospective study of patients admitted to a referral hospital, diagnosed with VST, aged between one month and seventeen years, from January 2011 to December 2019. The following data were re viewed: age at diagnosis, sex, signs and symptoms of presentation, predisposing mechanisms, study of thrombophilias, treatment and duration of treatment, follow-up protocol, long-term sequelae, and mortality. Due to their differences in clinical presentation, the sample was divided into two age groups: young children between 1 month and 5 years and older children and adolescents between 6 and 17 years. RESULTS: 17 patients were diagnosed with VST, 45% were women, with a median age of 4.5 years. The most frequent symptoms in older children (6-17 years old) were headache (80%) and diplopia (60%). In children under 5 years old, the most frequent clinical presentation was cerebellar ataxia (42%), asymptomatic (34%), and headache (25%). In 23.5% of the total, VST was a casual fin ding in neuroimaging. 13 patients presented relevant histories such as complicated otitis media with mastoiditis (53%), severe traumatic head injury (6%), and resection of a space-occupying lesion of the brain (6%). 23% of the cases were idiopathic and in 23% there were prothrombotic factors. The treatment of choice in all patients was low-molecular-weight heparin. During the short-term follow- up, 11.8% presented self-limited neurological symptoms. One patient presented long-term paresis of the sixth paired cranial nerve. There were no deaths or recurrences of the episode in our series. CONCLUSIONS: VST is a rare entity and it usually appears with signs and symptoms of intracranial hy pertension. It is a potentially serious condition and early diagnosis and treatment can help minimize long-term sequelae.

[Cerebral sinovenous thrombosis in a newborn with mutation of MTHFR C677T treated with enoxaparin]. / Trombosis senovenosa cerebral en un recién nacido con mutación MTHFR C677T tratado con enoxaparina.

INTRODUCTION: Neonatal cerebral sinovenous thrombosis (CSNT) is a rare and generally serious con dition about which there is little knowledge of the responsible pathophysiological mechanisms and, although controversial, it has been suggested that genetic thrombophilia may play a role in its patho genesis. Out of concern for intracranial bleeding, the anticoagulant treatment with low-molecular- weight heparin is controversial. CLINICAL CASE: Full-term newborn who presented at eight days of life breastfeeding rejection, clonic seizures, and locomotor hypoactivity. The MRI neuroimaging showed a CSNT involving multiple venous sinuses, a right thalamic hemorrhagic infarction, and venous con gestion in frontal white matter. Thrombophilia study highlighted a homozygous MTHFR C677T mutation. Treatment with low-molecular-weight heparin was associated with repermeabilization of the superior sagittal sinus after 23 days of starting therapy. CONCLUSIONS: The clinical presentation of CSNT in the neonate is nonspecific, probably related to the extent and severity of the injury and the development of associated complications, such as venous hemorrhagic infarctions and intraparenchymal or intraventricular hemorrhage. These complications are detected through ultrasound or MRI, and they should make us suspect a CSNT. In this experience, the anticoagulant treatment proved to be safe and prevents thrombus propagation.

Trombosis senovenosa cerebral en un recién nacido con mutación MTHFR C677T tratado con enoxaparina / Cerebral sinovenous thrombosis in a newborn with mutation of MTHFR C677T treated with enoxaparin

Resumen: Introducción: La trombosis senovenosa cerebral neonatal (TSVC), es una patología rara y generalmente grave, de la cual se conoce poco sobre los mecanismos fisiopatológicos responsables y, aunque controvertido, se ha sugerido que la trombofilia genética, puede desempeñar un rol en la patogénesis. Debido a los temores de un sangrado intracraneal el tratamiento anticoagulante con heparina de bajo peso mole cular es controvertido. Objetivo: presentar un recién nacido con una trombosis senovenosa cerebral neonatal, discutir los factores de riesgo trombofílico, y el manejo con heparina de bajo peso molecu lar de la trombosis venosa cerebral. Caso Clínico: Recién nacido de término que debutó a los 8 días de vida con convulsiones clónicas, rechazo al pecho más hipoactividad motora. La neuroimagen con RM mostró una TSVC involucrando múltiples senos venosos, un infarto hemorrágico talámico dere cho y congestión venosa de la sustancia blanca frontal. El estudio de trombofilia puso de relieve una mutación homocigota del gen MTHFR C677T. El tratamiento con heparina de bajo peso molecular se asoció a repermeabilización del seno sagital superior a los 23 días de iniciada la terapia. Conclusio nes: La presentación clínica de la TSVC en el neonato es inespecífica, probablemente en relación con la extensión y gravedad de la lesión y el desarrollo de complicaciones asociadas, como infartos he morrágicos venosos intraparenquimatosos o hemorragia intraventricular. Estas complicaciones son detectables mediante Ecografia o Resonancia Magnética, y deben hacer sospechar una TSVC. En esta experiencia el tratamiento anticoagulante mostró ser seguro y prevenir la extensión de la trombosis.

Abstract: Introduction: Neonatal cerebral sinovenous thrombosis (CSNT) is a rare and generally serious con dition about which there is little knowledge of the responsible pathophysiological mechanisms and, although controversial, it has been suggested that genetic thrombophilia may play a role in its patho genesis. Out of concern for intracranial bleeding, the anticoagulant treatment with low-molecular- weight heparin is controversial. Objective: To present a case of a newborn with neonatal CSNT, to analyze the thrombophilic risk factors, and the management of cerebral venous thrombosis with low-molecular-weight heparin. Clinical Case: Full-term newborn who presented at eight days of life breastfeeding rejection, clonic seizures, and locomotor hypoactivity. The MRI neuroimaging showed a CSNT involving multiple venous sinuses, a right thalamic hemorrhagic infarction, and venous congestion in frontal white matter. Thrombophilia study highlighted a homozygous MTHFR C677T mutation. Treatment with low-molecular-weight heparin was associated with repermeabilization of the superior sagittal sinus after 23 days of starting therapy. Conclusions: The clinical presentation of CSNT in the neonate is nonspecific, probably related to the extent and severity of the injury and the development of associated complications, such as venous hemorrhagic infarctions and intraparenchymal or intraventricular hemorrhage. These complications are detected through ultrasound or MRI, and they should make us suspect a CSNT. In this experience, the anticoagulant treatment proved to be safe and prevents thrombus propagation.

[Acute mastoiditis with intracranial complication in a pediatric patient]. / Mastoiditis aguda con complicación intracraneal. Reporte de un caso pediátrico.

Acute mastoiditis is an infection that affects the mastoid air-cell system, usually due to the progression of an acute otitis media. The bacteria most frequently isolated in acute mastoiditis are Streptococcus pneumoniae, Streptococcus pyogenes and Staphylococcus aureus. The mastoid infection can extend affecting contiguous structures and producing intra or extracranial complications. The most frequent ones are intracranial complications, including meningitis, temporal lobe or cerebellar abscess, epidural or subdural abscess and venous sinus thrombosis. We present the case of a 4-year-old girl who developed two intracranial complications (intracranial epidural abscess and transverse and sigmoid sinus thrombosis) initiated in an acute mastoiditis produced by Streptococcus pyogenes.

La mastoiditis aguda es una infección de las celdillas mastoideas, generalmente, secundaria a la progresión de una otitis media aguda. Las bacterias aisladas con más frecuencia en las mastoiditis son Streptococcus pneumoniae, Streptococcus pyogenes y Staphylococcus aureus. La infección mastoidea puede extenderse por contigüidad, afectar a estructuras vecinas y dar lugar a complicaciones intra- o extracraneales. Las más frecuentes son las intracraneales, entre las que se incluyen la meningitis, el absceso cerebeloso o del lóbulo temporal, el absceso epi- o subdural y la trombosis de senos venosos. Se presenta el caso de una niña de 4 años que desarrolló dos complicaciones intracraneales (absceso epidural y trombosis de senos venosos transverso y sigmoideo) a partir de una mastoiditis aguda producida por Streptococus pyogenes.

Papiledema como primera manifestación de trombosis de senos venosos en un paciente pediátrico. Reporte de caso y revisión de literatura / Papilledema as an initial finding in venous sinus thrombosis in a child. Case report and literature review

Introducción: la trombosis de senos venosos (TSV) en niños, sin factores de riesgo es una patología rara pero potencialmente fatal. La cefalea es el síntoma principal y muy pocos pacientes se presentan sin este. El papiledema como hallazgo inicial se presenta sólo en el 9% de los pacientes. Presentamos el caso de una menor de 13 años, sin factores de riesgo para TSV, cuya manifestación inicial fue el papiledema en ausencia de cefalea. Objetivo: describir el caso clínico de una paciente menor de edad sin comorbilidades, factores de riesgo ni cefalea, con papiledema como única manifestación de TSV. Diseño de estudio: reporte de caso. Resumen del caso: menor de 13 años, sin antecedentes, con cuadro clínico de 12 horas de evolución de dolor en ojo derecho de instauración progresiva, tipo punzada, asociado a visión doble y un episodio emético. Al examen físico con agudeza visual (AV) 20/20 en ambos ojos, con papiledema; sin hallazgos positivos al examen neurológico ni en otros sistemas. Conclusión: La TSV en pacientes pediátricos sin factores de riesgo ni cefalea es rara. El papiledema es uno de los hallazgos principales de esta patología y en pocas ocasiones es la manifestación inicial. Es importante conocer los síntomas y signos oft almológicos de la TSV, ya que pueden ser el único hallazgo en esta patología.

Background: venous sinus thrombosis (VST) in children, without risk factors, is a rare but potentially fatal pathology. Headache is the main symptom and only few patients present without it. Papilledema as an initial finding occurs only in 9% of patients. We present the case of a 13 years old girl, without risk factors for VST, with papilledema as initial manifestation in the absence of headache. Objective: to describe a clinical case of with venous sinus thrombosis without comorbidities or risk factors, who had papilledema as first symptom. Study design: case report and literature review. Case summary: 13 years old girl, without medical history, with a clinical picture of 12 hours of right eye progressive pain, prick type, associated with double vision and an emetic episode. Physical examination with visual acuity (VA) 20/20 in both eyes and papilledema; without positive findings in the neurological examination or in other systems. Conclusion: venous sinus thrombosis in pediatric patients without risk factors or headache is rare. Papilledema is one of the main fi ndings of this pathology and in few cases it is the initial manifestation. It is important to know the symptoms and ophthalmological signs of VST because it could be the only finding in this pathology.

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.

Dural sinus thrombosis following epidural analgesia for delivery: a clinical case / Trombose de seios durais após analgesia peridural para parto: caso clínico

Abstract Background and objectives: Neurological complications of spinal anesthesia are rare conditions. Headache caused by low pressure of the cerebrospinal fluid is one of the most frequent, which occurs after post-dural puncture. A comprehensive history and physical exam must be carried out before making the diagnosis of Post-Dural Puncture Headache (PDPH) and additional tests are necessary to exclude the possibility of developing serious neurological complications such as Dural Sinus Thrombosis (DST). According to the Case Report a differential diagnosis between Dural Sinus Thrombosis with PDPH is discussed. Case report: A 22 year-old lady, ASA Physical Status Class I was admitted at 39 weeks of gestation for delivery. For labor pain relief she requested epidural for analgesia, but unfortunately accidental dural puncture occurred. She developed an occipital headache and neck pain in the second day postpartum which was relieved by both lying down and supporting treatment such as rehydration, analgesics and caffeine. On day third postpartum she was discharged without complaints. On day fifth postpartum the pain returned and became more intense and less responsive to oral analgesics. She was admitted to the hospital to do a complete neurological and image investigation that showed a lesion consistent with the diagnosis of cortical vein thrombosis and Dural Sinus Thrombosis (DST). She was treated with oral anticoagulants. After two days, a repeated magnetic resonance image (MRI) showed partial canalization of the central sinus thrombus. The patient was discharged from hospital five days after her admission without any of the initial symptoms. Conclusion: The report describes a patient who developed severe headache following continuous epidural analgesia for delivery. Initially it was diagnosed as PDPH, however with the aid of MRI the diagnosis of DST was later established and treated. DST is a rare condition and is often underdiagnosed. Because of its potentially lethal complications, it should always be considered in acute headache differential diagnosis.

Resumo Justificativa e objetivos: As complicações neurológicas da raquianestesia são condições raras. A cefaleia causada pela baixa pressão do fluido cerebrospinal é uma das mais frequentes e ocorre após a punção dural. Anamnese completa e exame físico geral devem ser feitos antes de fazer o diagnóstico de cefaleia pós-punção dural (CPPD) e testes adicionais são necessários para excluir a possibilidade de complicações neurológicas graves, como trombose de seios durais (TSD). De acordo com o relato do caso, discutiremos o diagnóstico diferencial entre TSD e CPPD. Relato de caso: Paciente de 22 anos, estado físico ASA I, foi admitida com 39 semanas de gestação para o parto. Para alívio da dor do trabalho de parto, a paciente solicitou analgesia peridural, mas infelizmente ocorreu uma punção dural acidental. A paciente desenvolveu cefaleia occipital e dor cervical no segundo dia pós-parto - ambas aliviadas com repouso e terapia de suporte, como reidratação, analgésicos e cafeína. No terceiro dia pós-parto, a paciente recebeu alta sem queixas. No quinto dia pós-parto, a dor retornou e ficou mais intensa e com pouca resposta aos analgésicos orais. Ela foi admitida no hospital para uma completa investigação neurológica e de imagem que mostrou uma lesão compatível com o diagnóstico de trombose venosa cortical e TSD. A paciente foi tratada com anticoagulantes orais. Após dois dias, a repetição de ressonância nuclear magnética (RM) mostrou canalização parcial de trombo do seio central. A paciente recebeu alta hospitalar cinco dias após a admissão, sem quaisquer dos sintomas iniciais. Conclusão: O caso descreve uma paciente que desenvolveu cefaleia grave após epidural contínua para o parto. Inicialmente ela foi diagnosticada como CPPD, contudo com o auxílio da RNM foi estabelecido o diagnóstico tardio de TSD. TSD é uma condição rara e frequentemente subdiagnosticada. Ela deve sempre ser considerada como diagnóstico diferencial de cefaleia aguda em decorrência de suas complicações potencialmente letais.

Frosted branch angiitis and cerebral venous sinus thrombosis as an initial onset of neuro-Behçet's disease: a case report and review of the literature.

BACKGROUND: Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Diagnosis of Behçet's disease was based on the current 2014 International Criteria for Behçet's Disease and the International consensus recommendation criteria for neuro-Behçet's disease. In addition, a literature review using search parameters of "frosted branch angiitis", "Behçet" and "neuro-Behçet" in the PubMed database is presented. CASE PRESENTATION: A 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset. A fundus examination showed bilateral widespread retinal vasculitis with venous and arterial white sheathing, optic disc swelling, macular edema, and retinal hemorrhages, leading to the diagnosis of frosted branch angiitis. An extensive systemic workup for retinal vasculitis was uneventful, except for brain magnetic resonance imaging demonstrating cerebral venous sinus thrombosis and lymphocytic aseptic meningitis. A diagnosis of neuro-Behçet's disease was made, and treatment was started with methylprednisolone therapy 1 g/day for 5 consecutive days, followed by oral mycophenolate mofetil and infliximab 5 mg/kg infusion. The patient's response was rapid, with improvement of visual acuity to hand movement and counting fingers by day 7 and final visual acuity of counting fingers and 20/130. CONCLUSIONS: Frosted branch angiitis may be associated with infectious, noninfectious, or idiopathic causes. An extensive workup should be done to exclude systemic vasculitis such as Behçet's disease. Treatment with systemic steroids must be promptly initiated in association with specific treatment aimed at inflammation control and blindness risk reduction.

Neurobrucellosis and venous sinus thrombosis: an uncommon association.

Brucellosis is a commonly diagnosed zoonosis and neurological involvement is rare. A 30-year-old woman presented with a pulsatile headache that was exacerbated by the Valsalva maneuver and refractory to analgesic therapy. The patient also had nausea, cough, and coryza that evolved over 7 days. The neurological examination was unremarkable. Thrombosis of the lateral and sigmoid sinus and ipsilateral internal jugular vein were diagnosed and anticoagulation therapy was started. Brucella spp was identified in a sample of cerebrospinal fluid (CSF); five months after treatment with rifampicin and doxycycline, CSF was sterile. Cerebral venous thrombosis is a very uncommon sign of brucellosis.

Neurobrucellosis and venous sinus thrombosis: an uncommon association

Abstract: Brucellosis is a commonly diagnosed zoonosis and neurological involvement is rare. A 30-year-old woman presented with a pulsatile headache that was exacerbated by the Valsalva maneuver and refractory to analgesic therapy. The patient also had nausea, cough, and coryza that evolved over 7 days. The neurological examination was unremarkable. Thrombosis of the lateral and sigmoid sinus and ipsilateral internal jugular vein were diagnosed and anticoagulation therapy was started. Brucella spp was identified in a sample of cerebrospinal fluid (CSF); five months after treatment with rifampicin and doxycycline, CSF was sterile. Cerebral venous thrombosis is a very uncommon sign of brucellosis.

[Cerebral venous sinus thrombosis: Epidemiology, clinical characteristics, imaging and prognosis]. / Trombosis de senos venosos cerebrales: epidemiología, características clínicas, imaginología y pronóstico.

INTRODUCTION: Cerebral venous sinus thrombosis represents 0.5 - 1% of all cerebrovascular diseases. OBJECTIVE: The aim of this study was to determine the epidemiological, clinical, and imaging features of the disease, as well as the outcomes of patients with cerebral venous sinus thrombosis, and to explore the characteristics associated with unfavorable patient outcomes. MATERIALS AND METHODS: In this cross-sectional, retrospective study, the medical records of 37 patients with cerebral venous sinus thrombosis were analyzed. RESULTS: Eighty-six percent of the patients were women, and the mean patient age was 41 years. The most frequently reported symptom was headache (86.5%); headache was the single presenting symptom in 40.5% of the patients. Sixty-eight percent of the patients had at least one risk factor, the most frequent of which was obesity (24.3%). A total of 43.2% of the patients had no focal neurological findings. The most common finding on computerized tomography (CT) was hyperdense venous sinuses; on Magnetic Resonance Imaging (MRI), the most common finding was venous infarction. On average, 2.27±1.3 sinuses were involved; most frequently, the transverse venous sinuses were affected. The average hospital stay was 7.8±3.6 days. At hospital discharge, the outcomes were favorable in 92% of the patients, and the mortality rate was 5.4%. CONCLUSIONS: Cerebral venous sinus thrombosis is a different type of cerebrovascular disorder, with distinct epidemiology, risk factors, clinical presentations and functional outcomes. The diagnosis is based on clinical suspicion because of the unspecific clinical presentation of the disease.

[Intracranial sinus venous thrombosis in ulcerative colitis. Report of one case]. / Trombosis de senos venosos intracraneales: una complicación infrecuente y grave de la colitis ulcerosa. Primera comunicación de un caso en Uruguay.

Intracranial sinus venous thrombosis (ICSVT) is a rare complication of ulcerative colitis that affects from 1.7 to 7.5% of patients. We report a 22 year-old male with ulcerative colitis in treatment with mesalazine and prednisone presenting with headache and speech disturbances. A magnetic resonance imaging of the brain showed a left temporal hemorrhagic infarct with thrombosis of the ispilateral superficial vein and sigmoid venous sinus. No cause of thrombophilia was detected. Anticoagulation with heparin was started which was changed to oral anticoagulation with warfarin. The patient was discharged ten days after admission.

Trombosis de senos venosos cerebrales: epidemiología, características clínicas, imaginología y pronóstico / Cerebral venous sinus thrombosis: Epidemiology, clinical characteristics, imaging and prognosis

Introducción. La trombosis de senos venosos cerebrales representa entre 0,5 y 1 % de las enfermedades cerebrovasculares en adultos, y sus factores de riesgo son diferentes a los del resto de dichas enfermedades. Objetivo. Determinar la epidemiología, las características clínicas e imaginológicas, así como los resultados en pacientes con trombosis de senos venosos cerebrales, y explorar los aspectos asociados con los puntajes desfavorables en la escala modificada de Rankin. Materiales y métodos. Se llevó a cabo un estudio retrospectivo de corte transversal en el Instituto Neurológico de Colombia entre marzo de 2006 y junio de 2011, periodo en el que se analizaron las historias clínicas de 37 pacientes con diagnóstico confirmado por neuroimágenes. Resultados. El 86,5 % de los pacientes eran mujeres, con una edad promedio de 41 años. El síntoma más frecuente fue la cefalea (86,5 %), el cual se presentó como único síntoma en el 40,5 % de los casos. El 68 % tenía, por lo menos, un antecedente de riesgo para trombosis de senos venosos cerebrales, siendo el más frecuente la obesidad (24,3 %), seguido del uso de anticonceptivos hormonales (21,9 %). El examen neurológico fue normal en el 43,2 %. El hallazgo más frecuente en la tomografía fue la hiperdensidad de los senos venosos (33 %) y, en la resonancia magnética, el infarto venoso (37,5 %). El promedio de senos comprometidos fue de 2,27+1,3, siendo más frecuente el compromiso de los transversos. La estancia hospitalaria promedio fue de 7,8+3,6 días. El 92 % de los pacientes presentaba resultados funcionales favorables al dárseles de alta. La mortalidad hospitalaria fue de 5,4 % y se relacionó directamente con la trombosis de senos venosos cerebrales. Conclusiones. La trombosis de senos venosos cerebrales difiere de los otros tipos de enfermedad cerebrovascular en cuanto a su perfil epidemiológico, factores de riesgo, presentación clínica y pronóstico. Es una condición que exige un alto grado de sospecha diagnóstica, pues su presentación clínica es inespecífica.

Introduction: Cerebral venous sinus thrombosis represents 0.5 - 1% of all cerebrovascular diseases. Objective: The aim of this study was to determine the epidemiological, clinical, and imaging features of the disease, as well as the outcomes of patients with cerebral venous sinus thrombosis, and to explore the characteristics associated with unfavorable patient outcomes. Materials and methods: In this cross-sectional, retrospective study, the medical records of 37 patients with cerebral venous sinus thrombosis were analyzed. Results: Eighty-six percent of the patients were women, and the mean patient age was 41 years. The most frequently reported symptom was headache (86.5%); headache was the single presenting symptom in 40.5% of the patients. Sixty-eight percent of the patients had at least one risk factor, the most frequent of which was obesity (24.3%). A total of 43.2% of the patients had no focal neurological findings. The most common finding on computerized tomography (CT) was hyperdense venous sinuses; on Magnetic Resonance Imaging (MRI), the most common finding was venous infarction. On average, 2.27±1.3 sinuses were involved; most frequently, the transverse venous sinuses were affected. The average hospital stay was 7.8±3.6 days. At hospital discharge, the outcomes were favorable in 92% of the patients, and the mortality rate was 5.4%. Conclusions: Cerebral venous sinus thrombosis is a different type of cerebrovascular disorder, with distinct epidemiology, risk factors, clinical presentations and functional outcomes. The diagnosis is based on clinical suspicion because of the unspecific clinical presentation of the disease.

Trombose do seio dural em idade pediátrica / Dural sinus thrombosis in pediatric age

A trombose do seio dural é uma situação clínica rara, que resulta normalmente da complicação de processos infecciosos dos seios perinasais. Os sintomas e sinais são extremamente variados e inespecíficos sendo o diagnóstico feito através da ressonância magnética nuclear. Esse trabalho relata a ocorrência de trombose do seio dural em um paciente com idade pediátrica. Paciente com 10 anos de idade, sexo masculino, foi enviado ao serviço de urgência devido à diplopia e endotropia no olho esquerdo. No exame oftalmológico foi detectado papiledema bilateral, diplopia binocular e endotropia do olho esquerdo. Apresentava acuidade visual de 10/10 bilateralmente. Diante da suspeita de lesão ocupando espaço do sistema nervoso central, foi realizada ressonância magnética nuclear que confirmou o diagnóstico de TSD. Para avaliar a pressão intracraniana foi efetuada uma punção lombar com manometria, e esta demonstrou uma pressão intracraniana de 20mmHg (normal: <15mmHg). Perante isto a criança ficou internada para tratamento médico (enoxaparina de baixo peso molecular 1,5 mg/kg/dia subcutâneo (60 mg/dia), prednisolona 35 mg/dia oral e acetazolamida 250 mg/dia oral) durante 10 dias. Após 1 mês de follow-up verificou-se agravamento oftalmológico. A realização de nova punção lombar apresentou uma pressão intracraniana de 40 mmHg que não cedia ao tratamento médico. Após discussão multidisciplinar do caso optou-se pela realização de derivação lombo-peritoneal. A necessidade de uma grande dose de suspeição clínica, tanto para o diagnóstico inicial quanto para a monitorização das complicações, tornam a abordagem da trombose do seio dural um processo singular.

Dural sinus thrombosis is a rare condition, usually results from a late complication of an infection of the paranasal sinuses. The signs and symptoms are extremely varied and nonspecific, being the diagnosis made through magnetic resonance imaging. Ten-year-old male patient that was sent to our emergency department with left endotropia and diplopia. Ophthalmic examination was performed and showed papilledema with margin blurred right and left eye, binocular diplopia and left eye endotropia. Visual acuity was 10/10 bilaterally. Given the suspected space occupying lesion of the central nervous system, the MRI was performed and confirmed the diagnosis of DST. For evaluating the intracranial pressure (IP), a lombar puncture (LP) with manometry was carried out and revealed IP of 20 mmHg (normal values: <15mmHg). Towards this, the child’s was admitted for medical treatment (low molecular weight enoxaparin subcutaneous 1,5 mg/kg/day (60 mg/day), prednisolone 35 mg/per day and acetazolamide 250 mg/per day) over 10 days. After 1 month of follow-up there was deterioration of the ophthalmologic condition. A new LP was made and showed IP of 40 mmHg resilient to medical treatment. After multidisciplinary discussion of the case, it was decided for conducting lumboperitoneal shunt. The need for a great deal of suspicion for both the initial diagnosis and for monitoring complications make DST approach a special process.

Trombosis de senos venosos intracraneales: una complicación infrecuente y grave de la colitis ulcerosa. Primera comunicación de un caso en Uruguay / Intracranial sinus venous thrombosis in ulcerative colitis: Report of one case

Intracranial sinus venous thrombosis (ICSVT) is a rare complication of ulcerative colitis that affects from 1.7 to 7.5% of patients. We report a 22 year-old male with ulcerative colitis in treatment with mesalazine and prednisone presenting with headache and speech disturbances. A magnetic resonance imaging of the brain showed a left temporal hemorrhagic infarct with thrombosis of the ispilateral superficial vein and sigmoid venous sinus. No cause of thrombophilia was detected. Anticoagulation with heparin was started which was changed to oral anticoagulation with warfarin. The patient was discharged ten days after admission.

Trombose de seios venosos em criança com síndrome nefrótica: relato de caso e revisão da literatura / Venous sinus thrombosis in a child with nephrotic syndrome: a case report and literature review

A síndrome nefrótica associa-se a um estado de hipercoagulabilidade, apresentando risco aumentado de complicações tromboembólicas. A trombose dos seios venosos cerebrais é uma complicação rara da síndrome nefrótica, com poucos casos descritos na literatura, mas com diagnósticos cada vez mais frequentes. A verdadeira incidência pode estar subestimada, uma vez que muitos eventos são assintomáticos ou não são diagnosticados a tempo. Descrevemos aqui o caso de uma criança do sexo masculino, de 2 anos e 10 meses, com síndrome nefrótica, que apresentou, na evolução, cefaleia, crises epilépticas e rebaixamento sensorial, com o diagnóstico de trombose do seio sagital superior e transverso. Foi realizada revisão da literatura internacional por meio de estratégia de busca definida, nas bases de dados PubMed, SciELO e Lilacs, utilizando os termos “nephrotic syndrome” e “cerebral sinovenous thrombosis”. O diagnóstico de trombose venosa deve ser considerado em qualquer paciente com síndrome nefrótica que manifeste sinais e sintomas neurológicos, destacando que a suspeita clínica precoce tem relação com um desfecho favorável.

Nephrotic syndrome is associated with a hypercoagulable state and an increased risk of thromboembolic complications. Cerebral venous sinus thrombosis is a rare complication of nephrotic syndrome, with few cases described in the literature, although the disease may be under-diagnosis. The true incidence of cerebral venous sinus thrombosis may be underestimated because many events are asymptomatic or are not diagnosed in time. Here, we describe the case of a male child, 2 years and 10 months old, with nephrotic syndrome presenting with headache, epileptic seizures and sensory inhibition who was diagnosed with superior sagittal and transverse sinuses thrombosis. An international literature review was performed with a defined search strategy in the PubMed, SciELO and Lilacs databases using the terms ‘nephrotic syndrome’ and ‘cerebral sinovenous thrombosis’. The diagnosis of venous thrombosis should be considered in any patient with nephrotic syndrome who presents with neurological signs and symptoms, as early clinical diagnosis promotes favorable outcomes.

Trombosis de los senos intracraneales: Reporte de tres casos y revision de la literatura / Intracranial sinuses thrombosis: Report of three cases and literature review

La trombosis venosa intracraneal es un tipo de accidente cerebrovascular poco frecuente cuyo diagnóstico muchas veces ha sido demorado debido a sus diversas causas y presentaciones. Sin embargo, hoy en día su diagnóstico está aumentando debido al mayor empleo de la resonancia magnética nuclear, la angioresonancia y a la mejor comprensión del cuadro clínico. Se presentan los casos de tres mujeres con cefalea y déficit focal de evolución subaguda. Los estudios de neuroimágenes fueron compatibles con trombosis venosa intracraneal. Las pacientes tuvieron diferentes factores de riesgo, fueron tratadas con heparina y presentaron buena respuesta al tratamiento...

Intracranial sinuses thrombosis is an infrequent cerebrovascular accident whose diagnosis is usually delayed due to diverse causes and varying clinical presentations. However, with the advent of magnetic resonance imaging, angioresonance and with better knowledge of the clinical picture its diagnosis has improved nowadays. We present here three women cases with headache and focal deficits of sub-acute onset. Neuroimaging studies were compatible with intracranial sinuses thrombosis. Patients had different risk factors; they were treated with heparin and responded well to treatment...

Venous sinus thrombosis in a child with nephrotic syndrome: a case report and literature review.

Nephrotic syndrome is associated with a hypercoagulable state and an increased risk of thromboembolic complications. Cerebral venous sinus thrombosis is a rare complication of nephrotic syndrome, with few cases described in the literature, although the disease may be under-diagnosis. The true incidence of cerebral venous sinus thrombosis may be underestimated because many events are asymptomatic or are not diagnosed in time. Here, we describe the case of a male child, 2 years and 10 months old, with nephrotic syndrome presenting with headache, epileptic seizures and sensory inhibition who was diagnosed with superior sagittal and transverse sinuses thrombosis. An international literature review was performed with a defined search strategy in the PubMed, SciELO and Lilacs databases using the terms 'nephrotic syndrome' and 'cerebral sinovenous thrombosis'. The diagnosis of venous thrombosis should be considered in any patient with nephrotic syndrome who presents with neurological signs and symptoms, as early clinical diagnosis promotes favorable outcomes.