Características de la trombosis venosa cerebral en pacientes de dos hospitales universitarios de Colombia en el período 2018-2020 / Characteristics of cerebral venous thrombosis in patients from two university hospitals in Colombia between 2018-2020

Introducción. La trombosis venosa cerebral es una causa infrecuente de enfermedad cerebrovascular que viene en aumento a nivel mundial. A pesar de ello, actualmente, en Colombia no se cuenta con estudios suficientes que nos permitan caracterizar epidemiológicamente la enfermedad en nuestra población para identificar los factores de riesgo y las complicaciones más frecuentes en nuestro medio. Objetivo. Describir las características clínicas, demográficas y radiológicas, y los factores de riesgo de una serie de pacientes con trombosis venosa cerebral de dos hospitales de Colombia. Materiales y métodos. Es un estudio descriptivo retrospectivo de pacientes hospitalizados, atendidos en el servicio de neurología de dos hospitales de Bogotá desde diciembre de 2018 hasta diciembre del 2020. Resultados. Se incluyeron 33 pacientes. Las frecuencias más altas correspondieron a mujeres en edad fértil, en puerperio (n=7; 33,3 %) y pacientes con patologías autoinmunes (n=10; 30,3 %). El síntoma inicial más común fue la cefalea (n=31; 93,9 %), seguido de focalización neurológica (n=9; 27,2%) y crisis epiléptica (n=8; 24,2 %). El 51 % (n=17) de los pacientes tuvo un examen físico normal. El infarto venoso cerebral se presentó en el 21,1 % (n=7), la hemorragia subaracnoidea en el 12,1 % (n=4) y el hematoma intraparenquimatoso en el 9 % (n=3) del total de pacientes. El 60,6 % (n=20) quedó con nivel independiente en la escala funcional de Barthel. Ningún paciente falleció. Conclusiones. Se encontraron características sociodemográficas, clínicas y radiológicas similares a lo reportado en la literatura mundial. Con respecto a las diferencias, se encontró en nuestro estudio compromiso de la circulación venosa cerebral profunda en un porcentaje ligeramente mayor a lo descrito, pero sin aumento de complicaciones, ni mortalidad.

Introduction. Cerebral venous thrombosis is an uncommon cause of cerebrovascular disease, which has been increasing worldwide. In Colombia, there are not enough recent studies that allow us to determine epidemiological characteristics of the disease in our population to identify more frequent risk factors and complications according to our living conditions. Objective. To describe clinical, demographic, and radiographic characteristics, and risk factors in a cohort of patients with cerebral venous thrombosis attended at two hospitals in Colombia. Materials and methods. Retrospective descriptive study with patients treated in the hospitalization neurology service of two hospitals in Bogotá, Colombia from December 2018 to December 2020. Results. Thirty-three patients were included. There was a higher incidence of cerebral venous thrombosis in women of childbearing age in the puerperium (n=7; 33.3%) and associated with autoimmune diseases (n=10; 30.3%). The most common initial symptom was headache (n=31; 93.9 %), followed by neurological focal signs (n=9; 27.2%) and seizures (n=8; 24.2%). Fifty-one percent (n=17) of the patients had a normal physical examination. Cerebral venous infarction occurred in 21.1 % (n=7), subarachnoid hemorrhage in 12.1 % (n=4), and intraparenchymal hematoma in 9 % (n=3) of all the patients. Sixty-point six percent (n=20) of the patients had a total independent Barthel functional scale. None of those died. Conclusions. We found similar sociodemographic, clinical, and radiography characteristics to those reported in the world literature. Regarding the differences, deep cerebral venous circulation was higher than that described in previous studies but without complications increase or mortality.

Trombosis de senos venosos cerebrales una manifestación extrapulmonar de COVID-19: Reporte de caso / Cerebral venous sinus thrombosis, an extrapulmonary manifestation of COVID-19: A case report

Resumen La trombosis de senos venosos cerebrales es un evento infrecuente en la población pediátrica y sus manifestaciones clínicas pueden variar dependiendo de la localización y extensión de la lesión, etiología o grupo etario (1); así mismo, la asociación de esta patología con virus es poco común, sin embargo, se han repor tado casos de trombosis de senos venosos en pacientes adultos con SARS-CoV-2 en relación con los mecanismos de lesión endotelial y respuesta inflamatoria que desencadena mecanismos procoagulantes. A continuación se presenta el primer caso reportado en Colombia de un caso de trombosis venosa cerebral en un lactante previamente sano, que debuta con un cuadro infeccioso gastrointestinal que resuelve y una semana después se presenta con cefalea y paralisis del VI par craneal derecho. Se documentó por angioto mografía trombosis del seno venoso sagital con extensión a senos transversos; los laboratorios fueron negativos para otras causas sistémicas y con prueba de anticuerpos para coronavirus positiva.

Abstract Cerebral venous sinus thrombosis is infrequent in the pediatric population and its clinical manifestations may vary depending on the anatomical location and the extent of the lesion, etiology or age group(1). The association of this pathology with viruses is uncommon, however, cases in adults with SARS-Cov2 have been reported triggered by procoagulant mechanisms due to endothelial injury and inflammatory response. The following article is the first reported case in Colombia of cerebral venous thrombosis in a previously healthy child, who debuted with gastrointestinal infectious disease and a week later with headache and sixth right cranial nerve palsy . The diagnosis of sagittal venous sinus thrombosis with extension to transverse sinuses was documented in a computed tomography angiography; laboratories for systemic diseases were negative and antibodies for coronavirus were positive.

Complicaciones de la otitis media con parálisis del sexto par craneal contralateral en pediatría / Cranial complications of otitis media with paralysis of the contralateral sixth cranial pair in pediatrics

Resumen. La otitis media es una infección frecuente en la infancia, la cual puede producir complicaciones, incluidas las neurológicas graves, en cuatro de cada 100 niños en países en desarrollo. Se presenta el caso de una niña de nueve años sin antecedentes de enfermedad que consultó por otitis media derecha, otorrea, síndrome de hipertensión intracraneal y parálisis del VI nervio craneal contralateral a la lesión. La tomografía computarizada de cráneo y la resonancia magnética cerebral revelaron otomastoiditis crónica, apicitis petrosa, y trombosis de los senos transverso y sigmoide, el bulbo yugular y la vena yugular interna derecha. Recibió tratamiento antibiótico y quirúrgico. Este caso refleja el espectro de complicaciones intracraneales y extracraneales asociadas con la otitis media aguda en la era antibiótica. El examen físico permite la detección precoz de la hipertensión intracraneal, con signos como el papiledema y la parálisis del VI par contralateral como hallazgo inusual.

Abstract. Otitis media is a frequent infection during childhood. Complications may be present in up to 4 of 100 children including serious neurological complications, particularly in developing countries. We report the case of a 9-year-old girl with no disease history who presented with otitis media, otorrhea, intracranial hypertension syndrome, and paralysis of the VI cranial nerve contralateral to the lesion. A computed tomography scan of the skull and a brain magnetic resonance imaging revealed chronic otomastoiditis, petrous apicitis, and thrombosis of the transverse and sigmoid sinus, the jugular bulb, and the right internal jugular vein. She received antibiotics and surgical treatment. This case shows the spectrum of intra and extracranial complications associated with acute otitis media in the antibiotic era. The physical examination allows early identification of intracranial hypertension with signs such as papilledema and sixth contralateral nerve palsy as an unusual finding.

Venous sinus thrombosis during COVID-19 infection in pregnancy: a case report

ABSTRACT BACKGROUND: Although it is known that the new coronavirus disease (COVID-19), which was first seen in Wuhan, China, in December 2019 and has affected the whole world, mainly targets the respiratory tract, cases of this disease with a wide clinical spectrum are emerging as information is shared. CASE REPORT: We present the case of a pregnant woman who was diagnosed with venous sinus thrombosis after she developed headache and hemiparesis. Polymerase chain reaction (PCR) positivity lasted for two weeks after COVID-19 had been diagnosed. CONCLUSIONS: In patients with suspected COVID-19, especially in the presence of causes of hypercoagu- lability and presence of atypical features, venous sinus thrombosis needs to be kept in mind in making the differential diagnosis.

Densidad escanográfica de los senos venosos en pacientes sanos a 2600 metros sobre el nivel del mar / Computed Tomography Density of the Venous Sinuses in Healthy Patients at 2600 Meters Above Sea Level

Introducción: Existe relación entre la densidad escanográfica de las estructuras vasculares medida en unidades Hounsfield (UH) y el hematocrito, el cual se incrementa en las personas que viven a mayor altitud sobre el nivel del mar. Se ha descrito que una densidad de 70 UH es el valor límite superior de normalidad de densidad de los senos venosos en personas sin trombosis venosa que habitan a una altitud de 1.000 m s. n. m.; sin embargo, no se ha establecido esta medida en personas que habitan en altitudes geográficas mayores. En este estudio se determinó dicho valor para personas sin trombosis de senos venosos que viven a 2.600 m s. n. m. Objetivo: Este estudio tiene como propósito caracterizar la densidad normal de los senos venosos en pacientes que habitan en altitudes geográficas de 2.600 m s. n. m. Metodología: Se analizaron las densidades escanográficas de los senos venosos longitudinal superior y de los sitios de unión entre los senos transversos y senos sigmoides de 240 sujetos que habitan a 2.600 m s. n. m., quienes asistieron a la institución entre enero de 2008 y junio de 2016. Estas personas consultaron por cefalea, convulsiones, alteración del estado de conciencia o focalización neurológica. A estos pacientes se les realizó inicialmente un estudio escanográfico de cráneo simple, hemoglobina y hematocrito con diferencia no mayor a 3 días entre el estudio escanográfico y el hemograma, así como estudios confirmatorios de positividad o ausencia de trombosis de senos intracraneales mediante resonancia magnética simple o angiorresonancia. Resultados: La media de UH fue de 59,8 con un rango entre 43,2 y 74,9, para pacientes normales. Se encontró una correlación positiva de 0,49 entre el hematocrito y las UH como es ampliamente conocido en la literatura. Conclusión: El rango en UH de los pacientes sin trombosis de senos venosos es amplio y supera por 4 puntos el informado en la literatura. Esto puede ser atribuible al aumento del hematocrito en individuos que viven a 2.600 m s. n. m. Para establecer normalidad en los pacientes con valores superiores a 70 UH se sugiere tomar en cuenta la simetría de la densidad escanográfica con respecto al seno venoso contralateral, así como evaluar la morfología del borde anterior del seno venoso y la adecuada localización del ROI (Region of Interest) para medir la densidad venosa.

Introduction: There is a relationship between the intravascular density measured in Hounsfield units (HU) in computed tomography (CT) and the hematocrit levels, which increases in people who live above sea level. The expected density in venous sinuses is lower than 70 HU in healthy people living at 1000 meters above sea level. However, this measure has not been established in people living at higher geographic altitudes. In this study, this value was determined for normal people living at 2,600 meters above sea level. Objective: The purpose of this study is to characterize the normal density of the venous sinuses in patients who live at geographical altitudes of 2,600 m above sea level. Methodology: We measured the density on CT at the superior longitudinal venous sinus and at the junction between the transverse sinus and sigmoid sinus of 240 subjects living at 2600 meters above sea level from January 2008 to June 2016. These patients consulted for headache, seizures, altered state of consciousness or neurological focus. Initially, all participants had a non-enhanced CT brain, hemoglobin and hematocrit levels with a difference of no more than 3 days between the CT study and the hemogram, as well as confirmatory studies of positivity or absence of intracranial sinus thrombosis by non-enhanced MR or MRA. Results: The mean HU was 59.8 with a range between 43.2 and 74.9, for normal patients. A positive correlation of 0.49 was found between hematocrit and HU, as is widely known in the literature. Conclusion: We obtained a wide range in the HU of healthy patients compared to the values reported in other papers, and exceeds the highest value by 4 points. This may be attributable to the increased hematocrit in healthy patients living at 2,600 meters above sea level. In order to establish whether patients with venous sinus densities greater than 70 HU have venous thrombosis, our suggestion is to evaluate the symmetry of the density compared to the contralateral venous sinus, as well as to evaluate the morphology and the location of the ROI used to measure venous density.

Massive thrombosis and phlegmasia cerulea dolens while taking rivaroxaban: case report and review / Trombose maciça e flegmasia cerúlea dolens durante o uso de rivaroxabana: relato de caso e revisão

Abstract Our study describes a fatal case of phlegmasia cerulea dolens and massive venous thrombosis in a patient taking rivaroxaban regularly to treat cerebral venous sinus thrombosis. Blood tests samples were positive for lupus anticoagulant. The unique evolution of the case, as well as the positivity for lupus anticoagulant, raises the possibility of an acquired hypercoagulation syndrome. We highlight the fact that the test recommended as the first line for lupus anticoagulant diagnosis (dilute Russell viper venom time) is the most affected by rivaroxaban, leading to a high prevalence of false-positive results. We also discuss potential diagnoses for the current case and review the current state-of-the-art of use of the novel oral anticoagulation agents in this unusual situation. So far, there are no recommendations to use such agents as first options in cerebral venous sinus thrombosis or in hypercoagulation syndromes.

Resumo Nosso estudo descreve um caso fatal de flegmasia cerúlea dolens e trombose venosa maciça em um paciente usando regularmente rivaroxabana para o tratamento de trombose de seio venoso cerebral. A investigação laboratorial foi positiva para o anticoagulante lúpico. A evolução única do caso aumenta a possibilidade de uma síndrome de hipercoagulabilidade adquirida, bem como a positividade para o anticoagulante lúpico. Destacamos o fato de que o teste recomendado como primeira linha para o diagnóstico de anticoagulante lúpico (veneno de víbora de Russel diluído) é o mais afetado pela rivaroxabana, levando a uma alta prevalência de resultados falso-positivos. Também discutimos os potenciais diagnósticos para o presente caso e revisamos o estado da arte atual dos novos agentes de anticoagulação oral usados nessa situação incomum. Até o presente momento, não há recomendações para o uso de tais agentes como primeira opção na trombose de seios venosos cerebrais ou nas síndromes de hipercoagulação.

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.

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

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

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.

Deficiencia de cobalamina C de inicio temprano: presentación de caso clínico / Early onset cobalamin C deficiency: clinical case presentation

Cobalamin C (CblC) deficiency is an autosomal recessive disorder caused by mutations of the MMACHC gene that results in impaired synthesis of the methylcobalamin and adenosylcobalamin co-factors. This brings an impaired conversion of dietary cobalamin and therefore dysfunction of two key enzymes generating hyperhomocysteinemia, hypometionimemia and methylmalonic aciduria. It is the most common intracellular metabolism disorder of cobalamin. The early clinical form is the most frequent disorder and appears as a multisystemic disease with developmental delay, failure to thrive, and ocular, renal and hematological involvement during the first year of life. The thromboembolic events are associated with small vessel involvement, generating thrombotic microangiopathy responsible for renal involvement and pulmonary thromboembolism. The late-onset form is characterized by leukoencephalopathy, psychiatric disorders, subacute degeneration of the spinal cord, and thromboembolic events of medium to large vessels. The treatment currently available increases the survival of the patient and improves growth, neurological manifestations, biochemical, hematological profile and hydrocephalus. We present the neonatal debut of a case of CblC deficiency that appeared as a multisystem disease with initial neurological, ocular and hematological manifestations. The onset of symptoms was acute, a characteristic that is not frequent in CblC. The patient started treatment early, but in an unsatisfactory fashion, which led to increased neurological deterioration. Due to MRI images performed during the evolution of his condition, a superior and transverse sagittal sinus thrombosis, a rare manifestation of the disease, was observed.

La deficiencia de cobalamina C (CblC) es un defecto autosómico recesivo causado por la mutación del gen MMACHC, que resulta en la síntesis alterada de los cofactores metilcobalamina y adenosilcobalamina. Esto trae aparejado una disfunción de dos enzimas claves, lo cual genera hiperhomocisteinemia, hipometionimemia y aciduria metilmalónica. La presentación clínica de la deficiencia de CblC es heterogénea, y varía desde las formas de inicio temprano graves y potencialmente mortales, hasta los fenotipos más leves de inicio tardío. La forma clínica temprana es la más frecuente y se manifiesta como una enfermedad multisistémica, con restricción del desarrollo, restricción del crecimiento y alteraciones oculares, renales y hematológicas durante el primer año de vida. Las manifestaciones tromboembólicas están asociadas con el compromiso de pequeños vasos, lo que causa microangiopatía trombótica, responsable de compromiso renal y de tromboembolismo pulmonar. La forma tardía se caracteriza por leucoencefalopatía, trastornos psiquiátricos, degeneración subaguda de la médula espinal y eventos tromboembólicos de medianos o grandes vasos. El tratamiento disponible actualmente aumenta la supervivencia de la enfermedad y mejora el crecimiento, las manifestaciones neurológicas, el perfil bioquímico y hematológico y la hidrocefalia. Presentamos el debut neonatal de un caso de deficiencia de CblC que se manifestó con compromiso inicial neurológico, ocular y hematológico. El comienzo de los síntomas fue agudo, característica que no es frecuente en la deficiencia de CblC. El tratamiento se inició tempranamente, pero en forma insatisfactoria, con evolución de deterioro neurológico. En la evolución de su enfermedad en las imágenes de resonancia magnética, se puso de manifiesto trombosis de los senos sagital superior y transversos, una rara manifestación de la deficiencia de CblC.

Infarto bitalámico por trombosis venosa cerebral profunda: hallazgos clínicos y radiológicos / Bilateral thalamic infarcts due to deep cerebral venous thrombosis: clinical and radiological findings

La enfermedad cerebrovascular es un problema de salud pública mundial, considerada dentro de las primeras causas de mortalidad. La trombosis venosa cerebral (TVC) es una de sus patologías, que a pesar de ser infrecuente puede llevar a severas complicaciones en el paciente. Es por eso que se presentan dos casos de infartos bitalámicos secundarios a trombosis venosa cerebral profunda, con hallazgos clínicos e imagenológicos inusuales que hicieron aún más difícil su diagnóstico. Debido a las diferentes funciones que posee el tálamo además de la infrecuencia de la trombosis venosa cerebral profunda, la presentación clínica sigue siendo atípica y es usual que se consideren otros diagnósticos al inicio del evento, por lo tanto, consideramos importante proyectar estudios con muestras de mayor tamaño para definir con claridad la clínica y los hallazgos radiológicos de esta patología.

Cerebrovascular disease is a global public health problem and is a major cause of mortality. Cerebral venous thrombosis (CVT) is an uncommon but serious type of cerebrovascular disease. Thus, we report two cases of bilateral thalamic infarcts secondary to deep cerebral venous thrombosis presenting with unusual clinical and radiological features which made diagnosis more difficult. The functional complexity of the thalamus and the uncommon presentation of deep cerebral venous thrombosis lead to atypical clinical manifestations hence various conditions are considered in the differential diagnosis at onset of the event. Therefore, we emphasize the importance of conducting future studies with a larger sample size in order to further elucidate the clinical and radiological characteristics of this condition.

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.

Cerebral Venous Sinus Thrombosis in a Child with Idiopathic Nephrotic Syndrome: a case report / Trombose Venosa Cerebral na apresentação de Síndrome Nefrótica Pediátrica: caso clínico

ABSTRACT Complications are rare in pediatric cases of idiopathic nephrotic syndrome (NS). Thromboembolism ranks among the most uncommon and difficult complications to diagnose, particularly in the first episode of NS, since clinical signs might be unspecific. This report describes the case of a 5-year-old girl with NS for the first time presenting with severe hypoalbuminemia (< 2g/dL). The patient responded poorly to therapy with corticosteroids. On day 8 of hospitalization she started having headaches and vomiting; she did not present hemodynamic alterations, fever or exanthems, and her neurological parameters were normal. The patient was suspected for intracranial hypertension, and computed tomography scans revealed she had cerebral venous sinus thrombosis (CVST). She was started on anticoagulants and showed clinical signs of improvement. The patient had no evident prothrombotic risk factors. She had three other episodes since she was diagnosed, one in which her plasma antithrombin level was low. Although antithrombin levels were normal in her first episode, she was tested after the resolution of proteinuria. The low levels of antithrombin seen in the first recurrence might have mirrored the initial drop in plasma antithrombin levels, an idea supported by the severe hypoalbuminemia she had when diagnosed. This severe manifestation of acquired thrombophilia might be in the origin of CVST. This report presents a rare case of thromboembolic complication in a pediatric patient with NS. The patient progressed well since she was started on anticoagulants. Although she did not present any evident risk factors at first, the development of her case indicated that severe acquired thrombophilia might have worked as the pathophysiological mechanism leading to CVST.

RESUMO A Síndrome Nefrótica (SN) idiopática em crianças pode, raramente, complicar-se. O tromboembolismo é uma das complicações mais raras, principalmente no primeiro episódio, e de diagnóstico mais difícil, uma vez que a clínica pode ser inespecífica. Descrevemos o caso de uma criança de 5 anos com episódio inaugural de SN, destacando-se hipoalbuminemia inicial grave (< 2g/dL). Apresentou fraca resposta inicial à corticoterapia e, após 8 dias de internamento, iniciou quadro de cefaleias e vômitos, sem alterações hemodinâmicas, sem febre, sem exantema e com exame neurológico normal. Perante a suspeita de hipertensão intracraniana, foi realizada TC-CE, que mostrou trombose venosa cerebral (TVC). Foi então iniciada terapêutica anticoagulante com posterior boa evolução clínica. Trata-se de uma criança sem fatores de risco pró-trombóticos evidentes. Desde o diagnóstico, teve 3 recaídas, uma das quais com níveis baixos de antitrombina, que no episódio inaugural eram normais, apesar de avaliados já numa fase não proteinúrica. Suspeita-se, assim, que esse déficit plasmático em antitrombina na recaída poderá mimetizar a queda plasmática inicial, hipótese também apoiada pela hipoalbuminemia grave ao diagnóstico. Esta trombofília grave adquirida poderá ter sido mecanismo etiológico para a trombose venosa cerebral. O interesse deste caso prende-se com a raridade de complicações tromboembólicas na SN Pediátrica, ainda mais raras no episódio inaugural. Nesse caso, a boa evolução foi possível após a associação da terapêutica anticoagulante. Embora sem fatores de risco iniciais evidentes, a evolução do caso permitiu a suspeita de uma trombofília adquirida grave como mecanismo fisiopatológico do tromboembolismo cerebral.

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.

Spontaneous intracranial hypotension and its complications / Hipotensão intracraniana espontânea e complicações

ABSTRACT Spontaneous intracranial hypotension (SIH) is a syndrome that was unknown until the advent of magnetic resonance imaging (MRI). It is a cause of orthostatic headache, which remains underdiagnosed and, rarely, can result in several complications including dural venous sinus thrombosis, subdural hematoma and subarachnoid hemorrhage. Some of these complications are potentially life-threatening and should be recognized promptly, mainly by imaging studies. We reviewed the MRI of nine patients with SIH and describe the complications observed in three of these patients. Two of them had subdural hematoma and one had a dural venous sinus thrombosis detected by computed tomography and MRI. We concluded that MRI findings are of great importance in the diagnosis of SIH and its complications, which often influence the clinical-surgical treatment of the patient.

RESUMO Hipotensão Intracraniana Espontânea (HIE) é uma síndrome desconhecida até o advento das imagens de Ressonância Magnética (RM). É uma causa de cefaleia ortostática que permanece subdiagnosticada e raramente resulta em complicações, como trombose de seios venosos durais, hematoma subdural e hemorragia subaracnoidea. Algumas dessas complicações são potencialmente ameaçadoras à vida e devem ser prontamente reconhecidas pelos estudos de imagem. Nós revisamos as RM de 9 pacientes com HIE e descrevemos as complicações observadas em 3 casos. Dois deles tiveram hematoma subdural e um teve trombose de seio venoso dural detectados por tomografia computadorizada e RM. Concluímos que achados de RM são de grande importância no diagnóstico de HIE e suas complicações, frequentemente influenciando o tratamento clínico-cirúrgico do paciente.

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.

Déficit de proteína S en un caso pediátrico de trombosis de senos venosos cerebrales / Protein S deficiency in a pediatric case of cerebral venous sinus thrombosis

RESUMEN La trombosis venosa cerebral (TVC) representa el 0,5 % de todos los eventos vasculares cerebrales a nivel mundial; es una entidad compleja y de difícil diagnóstico, dada la variabilidad en el modo de presentación, el pronóstico y la diversidad de condiciones médicas que la originan. Ocurre por obstrucción del drenaje venoso cerebral. Su principal manifestación en lactantes y niños son las convulsiones. Este es el caso de una TVC por déficit de proteína S en un paciente pediátrico, el objetivo de este caso es destacar la importancia de la sospecha e identificación temprana de la enfermedad, así como la búsqueda del factor desencadenante. La TVC es una entidad grave, es potencialmente tratable y su pronóstico dependerá de la oportunidad del diagnóstico e intervención.

SUMMARY Cerebral venous sinus thrombosis represents 0.5 % of all cases worldwide. Is a complex pathology cause the variability in presentation mode, prognosis and different medical conditions that cause it. The cause is the obstruction of cerebral venous drainage, the main manifestation in infants and children are seizures. This is the case of cerebral venous sinus thrombosis in a pediatric patient with protein S deficiency, the goal of this case is to recognize the importance of suspicion and early identification of the disease and the search of the trigger. The cerebral venous sinus thrombosis is a serious entity, but potentially treatable and prognosis depend on the timing of diagnosis and intervention.

Isquemia talámica bilateral secundaria a trombosis venosa cerebral: tratamiento endovascular, presentación de un caso / Bilateral thalamic stroke due to cerebral vein thrombosis: endovascular treatment, case report

RESUMEN La trombosis venosa cerebral (TVC) es una entidad poco frecuente y potencialmente devastadora que se presenta con mayor frecuencia en adultos jóvenes, especialmente en mujeres. La TVC representa el 0,5 % a 1 % de todos los ACV, su amplio espectro clínico la convierte en un desafío diagnóstico, especialmente en aquellos casos de mujeres jóvenes sin factores protrombóticos conocidos que debutan con cefalea como único síntoma. La TVC puede ocasionar isquemia o infartos talámicos y gangliobasales bilaterales con delirio y deterioro del estado de conciencia, en especial cuando hay compromiso del sistema venoso profundo. El diagnóstico se confirma con resonancia magnética cerebral (RM) y venografía por resonancia. La anticoagulación sigue siendo la piedra angular del tratamiento; sin embargo, los pacientes que no responden a terapia médica intensiva pueden beneficiarse de la trombectomía endovascular mecánica (TEM). A continuación presentamos el caso clínico de una paciente de 29 años con una isquemia talámica y gangliobasal bilateral secundaria a trombosis venosa cerebral que fue llevada a trombectomía endovascular mecánica.

SUMMARY Thrombosis of the dural sinus and/or cerebral veins (CVT) is a rare but potentially devastating type of stroke that tends to occur in young adults, especially women. CVT represents about 0.5% of all strokes and can be challenging to diagnose because headache, rather than focal neurologic symptoms, is the prominent feature. However, some patients (especially those with deep cerebral venous occlusion) may present with bilateral thalamic or basal infarction and often will have decreased level of consciousness and rapid neurologic deterioration. The diagnosis of CVT is confirmed with MRI and magnetic resonance venogram (MRV). The mainstay of acute management is anticoagulation. However, some patients do not respond to medical therapy and these might benefit from endovascular mechanical thrombectomy. We present the case of a 29 year old female patient with bilateral thalamic and gangliobasal ischemia secondary to CVT, who was treated with anticoagulation and endovascular mechanical thombectomy.

Trombosis seno sagital superior en el puerperio: reporte de un caso clinico / Upper sagittal sinus thrombosis in the puerperium: report of a clinical case

Gestante de 34 semanas de gestación, diagnosticada de preeclampsia con restricción del crecimiento fetal intrauterino. La paciente presentaba alteración en las cifras tensionales con proteinuria positiva en muestra aislada y un índice de proteínas/creatinina en orina de 3.07 mg/mg. Por estos motivos, previa maduración pulmonar, y una vez establecido el tratamiento hipotensor adecuado, se le realizó una cesárea con buena recuperación posterior y normalidad en las cifras tensionales por lo que la paciente fue dada de alta sin precisar tratamiento hipotensor de control. Una semana después del alta, la paciente acude de nuevo al centro hospitalario refiriendo cefalea holocraneal con pérdida de la sensibilidad en el miembro superior derecho. Tras los estudios pertinentes la paciente fue diagnosticada de trombosis del seno sagital superior, iniciándose tratamiento anticoagulante con buena tolerancia al mismo y una recuperación completa del cuadro sin secuelas.

Pregnant 34 weeks gestation diagnosed with preeclampsia with fetal intrauterine growth restriction. The patient had alteration blood presure with positive test of sample isolated proteinuria and an index of protein / creatinine ratio 3.07 mg / mg. For these reasons, previous lung maturation and the antihypertensive treatment was established, a cesárea was realized with good later recovery and normal blood presure. The patient went home without antihypertensive therapy. The patient came to the hospital again a week later, with intense holocraneal headache with loss of sensation in the right arm. After the relevant studies, the patient was diagnosed with thrombosis of the superior sagittal sinus starting anticoagulant therapy with good tolerance to it and a full recovery without sequelae. Pregnancy and specifically puerperium involves stages of risk for development of vascular disease in the brain especially in pathological processes such as preeclampsia. It is important to identify these patients to treat them, also to establish prevention protocols for future pregnancies to help reduce both maternal and fetal morbidity.