RESUMEN
INTRODUCTION: Cerebral venous sinus thrombosis (CVST) is a well-known, although underestimated, cause of stroke in childhood. Its diagnosis requires a high index of suspicion, a correct interpretation of neuroimaging studies and an interrelation between clinicians and radiologists. The clinical features, risk factors and neuroimaging of children under 15 years of age with CVST were analyzed. METHODS: multicenter, retrospective, descriptive, study of a consecutive series of cases of children under 15 years of age, who were admitted due to CVST between January 1st, 2010, and March 1st, 2022. RESULTS: The study included 51 patients: 39% with acute symptoms and 59% with subacute symptoms. Newborns predominantly presented encephalopathic symptoms and seizures, while children exhibited signs of intracranial hypertension (ICH). Risk factors were identified in 90% of the cases. Magnetic resonance with angiography in venous time confirmed the diagnosis in 80% of the patients, with the straight sinus being the most affected in newborns and the lateral sinus in children. Hemorrhagic complications were found in 30.5%, being more frequent in newborns. Anticoagulation was initiated in 82% without complications. Sequelae were present in 44.4% of newborns and 37.9% of children, being more frequent and severe in newborns. CONCLUSIONS: To make an early diagnosis, it is essential to consider CVST in newborns with encephalopathic symptoms and/or seizures, and in children with signs of ICH in the presence of predisposing or triggering conditions.
Introducción: La trombosis de venas y senos venosos cerebrales (TVSC) constituye una causa conocida, aunque subestimada de ictus en la infancia. Su diagnóstico requiere un alto índice de sospecha, una correcta interpretación de la neuroimagen e interrelación entre el clínico y el radiólogo. OBJETIVO: Analizar las manifestaciones clínicas, factores de riesgo y neuroimagen de recién nacidos (RN) y niños menores de 15 años con TVSC. Métodos: Estudio descriptivo, retrospectivo, multicéntrico, de una serie consecutiva de casos de menores de 15 años que ingresaron por TVSC entre el 1 de enero del 2010 y el 1 de marzo de 2022. RESULTADOS: El estudio incluyó 51 pacientes: 39% con síntomas agudos y 59% subagudos. En los RN predominaron síntomas encefalopáticos y convulsiones, mientras en los niños elementos de hipertensión endocraneana (HTEC). Se identificaron factores de riesgo en el 90% de los casos. La resonancia magnética con angiografía en tiempo venoso confirmó el diagnóstico en el 80%, siendo el seno recto el más afectado en RN y el seno lateral en niños. Se encontraron complicaciones hemorrágicas en 30.5%, siendo más frecuentes en los RN. Se inició anticoagulación en el 82% sin complicaciones. Las secuelas estuvieron presentes en 44.4% de RN y 37.9% de niños, siendo más frecuentes y graves en los RN. CONCLUSIONES: Para realizar un diagnóstico precoz es fundamental pensar en TVSC en RN con síntomas encefalopáticos y/o convulsiones y en mayores con clínica de HTEC en presencia de enfermedades predisponentes o desencadenantes.
Asunto(s)
Encefalopatías , Trombosis de los Senos Intracraneales , Recién Nacido , Humanos , Niño , Estudios Retrospectivos , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/etiología , Convulsiones/etiología , AngiografíaRESUMEN
OBJECTIVE: To determine the prevalence of and risk factors for cerebral sinus venous thrombosis (CSVT) in neonates undergoing congenital heart disease (CHD) repair. STUDY DESIGN: Neonates who had CHD repair with cardiopulmonary bypass and postoperative brain magnetic resonance imaging (MRI) between 2013 and 2019 at a single tertiary care center were identified from institutional databases. Demographic, clinical, and surgical data were abstracted from these databases and from the medical record; 278 neonates with CHD had cardiopulmonary bypass, 184 of whom had a postoperative brain MRI. RESULTS: Eight patients (4.3%) had a CSVT. Transposition of the great arteries with an intact ventricular septum (P < .01) and interrupted aortic arch (P = .02) were associated with an increased risk for CSVT. Other risk factors for CSVT included cross-clamp time (98 [IQR, 77.5-120] minutes vs 67 [IQR, 44-102] minutes; P = .03), units of platelets (3.63 [IQR, 3-4] vs 2.17 [IQR, 1-4]; P < .01) and packed red blood cells (0.81 [IQR, 0.25-1] vs 1.21 [IQR, 1-1]; P = .03) transfused intraoperatively, and time between surgery and MRI (10 [IQR, 7-12.5] days vs 20 [IQR, 12-35] days; P < .01). Five patients (62.5%) were treated with anticoagulation. All patients had complete or partial resolution of their CSVT, regardless of treatment. CONCLUSIONS: Brain MRI after cardiopulmonary bypass in neonates revealed a low prevalence of CSVT (4.3%). Further studies are needed to establish best practices for surveillance, prevention, and treatment of CSVT in this population.
Asunto(s)
Cardiopatías Congénitas , Trombosis de los Senos Intracraneales , Transposición de los Grandes Vasos , Trombosis de la Vena , Anticoagulantes/uso terapéutico , Cardiopatías Congénitas/complicaciones , Humanos , Lactante , Recién Nacido , Trombosis de los Senos Intracraneales/epidemiología , Trombosis de los Senos Intracraneales/etiología , Transposición de los Grandes Vasos/complicaciones , Trombosis de la Vena/complicacionesRESUMEN
BACKGROUND AND AIM: Shunt dysfunction is a common event, especially in children who have this intervention performed early in life. The consequences of chronic shunt overdrainage can be multiple since the cerebral hydrodynamics is altered. A thrombotic event with consequent symptoms of intracranial hypertension is discussed in this article. MATERIAL AND METHODS: We performed a detailed review of cerebral hydrodynamics and intracranial pressure compensation mechanisms and how this can alter cerebral venous circulation. Next, we report the case of a 4-year-old child with such a clinical presentation that was conducted by our team. RESULTS: A child with a history of hydrocephalus treated with a ventriculo-peritoneal (VP) shunt in his early childhood presented with symptoms of intracranial hypertension, initial computed tomography (CT) demonstrating reduced-sized ventricles. Complementary investigation showed bilateral papilledema, cranial suture closure, changes compatible with Chiari type I, and venous sinus thrombosis (transverse and sigmoid, bilaterally). The case was managed conservatively with full anticoagulation with enoxaparin. Four months after the onset of symptoms, there was an improvement in the clinical and imaging status. CONCLUSION: A condition of severe headache in a patient with an apparently functioning shunt and small ventricles on initial CT should open up a range of diagnostic possibilities, with pseudotumor cerebri syndrome and cerebral venous sinus thrombosis being suggested. The therapeutic approach in these cases must be individualized.
Asunto(s)
Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Trombosis de los Senos Intracraneales , Preescolar , Senos Craneales/diagnóstico por imagen , Senos Craneales/patología , Humanos , Hipertensión Intracraneal/complicaciones , Papiledema/etiología , Seudotumor Cerebral/cirugía , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/etiologíaRESUMEN
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.
Asunto(s)
Trombosis de los Senos Intracraneales , Trombosis de la Vena , Adolescente , Anticoagulantes/uso terapéutico , Niño , Preescolar , Femenino , Estudios de Seguimiento , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Factores de Riesgo , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Trombosis de los Senos Intracraneales/etiología , Trombosis de los Senos Intracraneales/mortalidad , Centros de Atención Terciaria , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/tratamiento farmacológico , Trombosis de la Vena/etiología , Trombosis de la Vena/mortalidadRESUMEN
OBJECTIVE: Severe complications of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) include arterial ischemic stroke (AIS) in adults and multisystem inflammatory syndrome in children. Whether stroke is a frequent complication of pediatric SARS-CoV-2 is unknown. This study aimed to determine the proportion of pediatric SARS-CoV-2 cases with ischemic stroke and the proportion of incident pediatric strokes with SARS-CoV-2 in the first 3 months of the pandemic in an international cohort. METHODS: We surveyed 61 international sites with pediatric stroke expertise. Survey questions included: numbers of hospitalized pediatric (≤ 18 years) patients with SARS-CoV-2; numbers of incident neonatal and childhood ischemic strokes; frequency of SARS-CoV-2 testing for pediatric patients with stroke; and numbers of stroke cases positive for SARS-CoV-2 from March 1 to May 31, 2020. RESULTS: Of 42 centers with SARS-CoV-2 hospitalization numbers, 8 of 971 (0.82%) pediatric patients with SARS-CoV-2 had ischemic strokes. Proportions of stroke cases positive for SARS-CoV-2 from March to May 2020 were: 1 of 108 with neonatal AIS (0.9%), 0 of 33 with neonatal cerebral sinovenous thrombosis (CSVT; 0%), 6 of 166 with childhood AIS (3.6%), and 1 of 54 with childhood CSVT (1.9%). However, only 30.5% of neonates and 60% of children with strokes were tested for SARS-CoV-2. Therefore, these proportions represent 2.9, 0, 6.1, and 3.0% of stroke cases tested for SARS-CoV-2. Seven of 8 patients with SARS-CoV-2 had additional established stroke risk factors. INTERPRETATION: As in adults, pediatric stroke is an infrequent complication of SARS-CoV-2, and SARS-CoV-2 was detected in only 4.6% of pediatric patients with ischemic stroke tested for the virus. However, < 50% of strokes were tested. To understand the role of SARS-CoV-2 in pediatric stroke better, SARS-CoV-2 testing should be considered in pediatric patients with stroke as the pandemic continues. ANN NEUROL 2021;89:657-665.
Asunto(s)
COVID-19/epidemiología , Accidente Cerebrovascular Isquémico/epidemiología , Trombosis de los Senos Intracraneales/epidemiología , Síndrome de Respuesta Inflamatoria Sistémica/epidemiología , Adolescente , COVID-19/complicaciones , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Accidente Cerebrovascular Isquémico/etiología , Masculino , SARS-CoV-2 , Trombosis de los Senos Intracraneales/etiología , Encuestas y Cuestionarios , Síndrome de Respuesta Inflamatoria Sistémica/complicacionesRESUMEN
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.
Asunto(s)
Anticoagulantes/uso terapéutico , Enoxaparina/uso terapéutico , Homocistinuria/diagnóstico , Metilenotetrahidrofolato Reductasa (NADPH2)/deficiencia , Espasticidad Muscular/diagnóstico , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/etiología , Femenino , Marcadores Genéticos , Homocistinuria/complicaciones , Homocistinuria/genética , Homocigoto , Humanos , Recién Nacido , Metilenotetrahidrofolato Reductasa (NADPH2)/genética , Espasticidad Muscular/complicaciones , Espasticidad Muscular/genética , Mutación , Trastornos Psicóticos/complicaciones , Trastornos Psicóticos/diagnóstico , Trastornos Psicóticos/genética , Trombosis de los Senos Intracraneales/tratamiento farmacológicoRESUMEN
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.
Asunto(s)
Humanos , Femenino , Recién Nacido , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/etiología , Enoxaparina/uso terapéutico , Metilenotetrahidrofolato Reductasa (NADPH2)/deficiencia , Homocistinuria/diagnóstico , Espasticidad Muscular/diagnóstico , Anticoagulantes/uso terapéutico , Trastornos Psicóticos/complicaciones , Trastornos Psicóticos/diagnóstico , Trastornos Psicóticos/genética , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Marcadores Genéticos , Metilenotetrahidrofolato Reductasa (NADPH2)/genética , Homocistinuria/complicaciones , Homocistinuria/genética , Homocigoto , Espasticidad Muscular/complicaciones , Espasticidad Muscular/genética , MutaciónRESUMEN
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.
Asunto(s)
Absceso Epidural/etiología , Mastoiditis/diagnóstico , Trombosis de los Senos Intracraneales/etiología , Infecciones Estreptocócicas/diagnóstico , Streptococcus pyogenes/aislamiento & purificación , Enfermedad Aguda , Preescolar , Absceso Epidural/diagnóstico , Femenino , Humanos , Mastoiditis/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico , Infecciones Estreptocócicas/complicacionesRESUMEN
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.
Asunto(s)
Humanos , Femenino , Preescolar , Trombosis de los Senos Intracraneales/etiología , Síndrome Nefrótico/complicacionesRESUMEN
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.
Asunto(s)
Hipotensión Intracraneal/complicaciones , Hipotensión Intracraneal/diagnóstico por imagen , Adulto , Pérdida de Líquido Cefalorraquídeo/diagnóstico por imagen , Pérdida de Líquido Cefalorraquídeo/etiología , Angiografía por Tomografía Computarizada/métodos , Femenino , Cefalea/etiología , Hematoma Intracraneal Subdural/diagnóstico por imagen , Hematoma Intracraneal Subdural/etiología , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/etiologíaRESUMEN
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.
Asunto(s)
Síndrome Nefrótico/complicaciones , Trombosis de los Senos Intracraneales/etiología , Preescolar , Femenino , HumanosRESUMEN
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.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Hipotensión Intracraneal/complicaciones , Hipotensión Intracraneal/diagnóstico por imagen , Trombosis de los Senos Intracraneales/etiología , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Hematoma Intracraneal Subdural/etiología , Hematoma Intracraneal Subdural/diagnóstico por imagen , Pérdida de Líquido Cefalorraquídeo/etiología , Pérdida de Líquido Cefalorraquídeo/diagnóstico por imagen , Angiografía por Tomografía Computarizada/métodos , Cefalea/etiologíaRESUMEN
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.
Asunto(s)
Colitis Ulcerosa/complicaciones , Trombosis de los Senos Intracraneales/etiología , Antiinflamatorios/uso terapéutico , Anticoagulantes/uso terapéutico , Infarto Cerebral/diagnóstico , Colitis Ulcerosa/tratamiento farmacológico , Enoxaparina/uso terapéutico , Cefalea/tratamiento farmacológico , Cefalea/etiología , Humanos , Masculino , Mesalamina/uso terapéutico , Prednisona/uso terapéutico , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Trastornos del Habla/tratamiento farmacológico , Trastornos del Habla/etiología , Adulto JovenRESUMEN
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.
Asunto(s)
Humanos , Masculino , Adulto Joven , Colitis Ulcerosa/complicaciones , Trombosis de los Senos Intracraneales/etiología , Antiinflamatorios/uso terapéutico , Anticoagulantes/uso terapéutico , Infarto Cerebral/diagnóstico , Colitis Ulcerosa/tratamiento farmacológico , Enoxaparina/uso terapéutico , Cefalea/tratamiento farmacológico , Cefalea/etiología , Mesalamina/uso terapéutico , Prednisona/uso terapéutico , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Trastornos del Habla/tratamiento farmacológico , Trastornos del Habla/etiologíaRESUMEN
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.
Asunto(s)
Preescolar , Humanos , Masculino , Síndrome Nefrótico/complicaciones , Trombosis de los Senos Intracraneales/etiología , Cefalea/etiología , Convulsiones/etiología , Trombosis de los Senos Intracraneales/diagnósticoRESUMEN
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.
Asunto(s)
Síndrome Nefrótico/complicaciones , Trombosis de los Senos Intracraneales/etiología , Preescolar , Cefalea/etiología , Humanos , Masculino , Convulsiones/etiología , Trombosis de los Senos Intracraneales/diagnósticoRESUMEN
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.