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.

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 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.

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.

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.

Trombosis de seno venoso secundaria a traumatismo encéfalo craneano: caso clínico / Cerebral vein thrombosis secondary to closed head injury: Report of one case

Intracranial sinus thrombosis (1ST) after closed head injury is an uncommon but potentially serious complication. It has no correlation with the severity of the injury. The symptoms and clinical course are highly variable. The most frequent but least specific symptom is severe headache. Cerebral lesions and neurologic signs develop in half of patients with IST. We report a 29 year-old male who had an IST after a severe closed head injury. The patient initially developed headache and had later 2 secondarily generalized seizures. The magnetic resonance imaging showed a superior sagittal sinus thrombosis. Anticoagulation with unfractionated heparin and intravenous phenytoin was started. At the moment of this report he is asymptomatic and continues with oral anticoagulants and phenytoin.

Trombose de seios venosos cerebrais: estudo de 15 casos e revisão de literatura / Cerebral venous thrombosis: study of fifteen cases and review of literature

OBJETIVO: Analisar uma série de 15 pacientes com trombose venosa cerebral (TVC) e comparar os resultados com dados da literatura. MÉTODOS: Foram avaliados, por meio de estudo retrospectivo, transversal e descritivo, as características epidemiológicas, o quadro clínico, os fatores de risco e o prognóstico de 15 pacientes com TVC admitidos no serviço de Neurologia da Santa Casa de Belo Horizonte no período de abril de 2007 a Dezembro de 2008. RESULTADOS: O diagnóstico de TVC foi confirmado por exame de ressonância nuclear magnética de encéfalo em 14 casos e por angiografia cerebral em um caso. Os principais fatores de risco identificados foram o uso do anticoncepcional oral (40 por cento) e uma história prévia ou familiar de trombose venosa profunda. Trombofilia foi encontrada em dois pacientes (13 por cento). O seio mais acometido foi o transverso (73 por cento), seguido pelo sagital superior, em 53 por cento. Quatro pacientes apresentaram acidente vascular cerebral e outros 5 apresentaram-se apenas com cefaleia isolada. Doze pacientes foram tratados com heparina e anticoagulação oral sequencial. CONCLUSÃO: A terapêutica com heparina na fase aguda seguida do anticoagulante oral demonstrou-se segura e eficaz na prevenção da progressão da doença, de sua recidiva e na rápida recuperação do quadro neurológico de todos os pacientes tratados. A TVC deve ser considerada no diagnóstico de cefaleia secundária mesmo em pacientes com ausência de outros sinais ou sintomas.

OBJECTIVE: To analyze a series of 15 patients with cerebral venous thrombosis (CVT) who had follow-ups at the neurology service of Santa Casa de Belo Horizonte Hospital from April, 2007 to December, 2008. These results were compared with data in literature. METHODS: Cases were evaluated by retrospective study of the epidemiologic characteristics, signs and symptoms, risk factors and prognosis of 15 patients with cerebral venous thrombosis. RESULTS: Diagnoses were reached through magnetic resonance imaging of the brain in 14 cases and through an angiography in one. The main risk factors identified were use of birth control pills (40 percent) and history of family member with deep venous thrombosis. Thrombophilia was found in two patients (13 percent). The veins more affected were the transverse sinus (73 percent) followed by the upper sagital sinus (53 percent). Four patients had strokes and five had only headaches as isolated symptoms. Twelve patients were treated with heparin and oral anticoagulant. CONCLUSION: Treatment with heparin in the acute phase followed by an oral anticoagulant was shown as safe and efficient to prevent worsening of the disease, recurrence and for quick improvement of neurological symptoms of all treated patients. CVT is one of the possible diagnoses of headaches as an isolated symptom.

Trombose de seio dural: relato de caso / Dural sinus thrombosis: case report

Relata-se o caso de uma gestante de 24 anos, encaminhada ao serviço de neurologia por apresentar agitação, alucinações, confusão mental, cefaléia, perda de visão, afasia e convulsões. Exame neurorradiológico compatível com trombose de seios durais e veias corticais. Foi realizado tratamento com abciximab e efetuada a lise mecânica do trombo obtendo restauração do fluxo venoso cerebral. Após o procedimento, apresentou hematoma frontal o qual foi retirado cirurgicamente. A paciente evoluiu com melhora neurológica progressiva. Discute-se esta infreqüente patologia segundo quadro clínico, patogênese, exames de imagem e terapêutica.

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.

Ulcerative colitis with sagittal sinus thrombosis with normal coagulation profile.

Thromboembolic events are serious complications in patients with inflammatory bowel diseases. We describe a 30-year-old man with ulcerative colitis complicated by sagittal sinus thrombosis with normal coagulation profile; he achieved clinical remission with subcutaneous heparin.

Trombose séptica do seio cavernoso: relato de seis caso / Septic thrombosis of cavernous sinus: report of 6 cases

O seio cavernoso é freqüentemente afetado por trombose séptica. Os sítios primários de infecção são comumente a face, as órbitas, as tonsilas palatinas, o palato mole e os seios etmoidal e esfenoidal. A apresentação clínica típica inclui febre e edema periorbital seguido de cefaléia, ptose e paralisia de músculos oculares. O diagnóstico é eminentemente clínico e o prognóstico diretamente influenciado pela instalação de um tratamento precoce e adequado. Relatamos seis casos de trombose séptica do seio cavernoso decorrentes de sítios infecciosos diferentes e revisamos os principais aspectos clínicos, diagnósticos e terapêuticos.

Trombosis venosa cerebral profunda: comunicación de un caso / Deep cerebral venous thrombosis: report of 1 case

We report a 39 years old female presenting with headache, proggressive clouding of sensorium and long pathway deficit signs. Brain CAT scan showed a process involving basal ganglia, thalami and Herophilus press. A thrombosis of this vessel was suspected. The first magnetic resonance imaging was informed as a diffuse inflammatory process compatible with an encephalitis. The patient aggravated and a second magnetic resonance imaging demonstrated a partial thrombosis of sinus rectus, Galenus vein, Herophilus press and both internal cerebral veins. Intravenous heparin was started with a progressive recovery of the patient. Two months later she had an almost complete recovery with a persistent mild right brachial paresis and a memory deficit for recent events

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)

Trombosis venosa cerebral durante el embarazo y puerperio / Cerebral Venous Thrombosis During Pregnancy and Puerperium.

Se presentan seis casos de trombosis venosa cerebral durante el embarazo y el puerperio. Cinco durante el embarazo y uno en el puerperio. Se discute la etiopatogenia, los aspectos clínicos diagnóstico diferencial, diagnóstico por imagenes y los alcances terapéuticos. Tratamiento activo con anticoagulantes fue llevado a cabo en dos pacientes y los otros fueron manejados sintomáticamente. Tres pacientes fallecieron y tres sobrevivieron todos con secuelas neurológicas.