ABSTRACT
INTRODUCTION: Since its first description at the beginning of the 19th century, greater awareness of this disorder has been achieved, becoming nowadays a recognized but sometimes underdiagnosed cause of ischemic stroke in childhood. AIM. To describe a population of children with cerebral venous thrombosis, analyzing clinical presentation, risk factors, treatment and evolution. PATIENTS AND METHODS: A descriptive, retrospective and longitudinal review of children from 0 to 17 years of age diagnosed with sinovenous thrombosis was conducted, during the period of time between 2004 and 2009. RESULTS: Thirty one cases with cerebral sinus thrombosis were identified, 18 of them were boys. Clinical presentation included 18 patients with seizures and 13 with high intracranial pressure. We found infections as a risk factor in 23 patients, 3 newborns with obstetric trauma, 5 patients with prothrombotic states, 4 with chronic systemic diseases, 5 with more than one risk factor and 1 with none. Diagnosis was done by magnetic resonance imaging in 18 patients, computed tomography in 11 and magnetic resonance angiography in the other 2. Regarding location, 16 patients showed compromise of the transverse sinus, 4 of the superior longitudinal sinus, another 4 an affectation of the sigmoid sinus, 2 of the cavernous sinus and 5 with a combination of them. Anticoagulation therapy was done in 21 patients and treatment abstention in 10. None of the patients suffered complications or death due to anticoagulation therapy or sinovenous thrombosis. No recurrent symptomatic thrombosis in long term follow up was found. CONCLUSIONS: Sinovenous cerebral thromboses are an important but underestimated cause of stroke in childhood. Clinical suspicion should be high, in patients with risk factors and intracranial hypertension or seizures.
Subject(s)
Intracranial Thrombosis/diagnosis , Intracranial Thrombosis/pathology , Intracranial Thrombosis/therapy , Venous Thrombosis/diagnosis , Venous Thrombosis/pathology , Venous Thrombosis/therapy , Adolescent , Anticoagulants/therapeutic use , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Intracranial Thrombosis/complications , Magnetic Resonance Imaging , Male , Retrospective Studies , Risk Factors , Stroke/etiology , Venous Thrombosis/complicationsABSTRACT
La enfermedad o síndrome de moyamoya es una rara afección que provoca una oclusión de las carótidas intracraneales y determina una red vascular colateral anormal en la base del cráneo y que, por su aspecto arteriográfico, hace ya más de 50 años un grupo de neurocirujanos japoneses denominaron moyamoya, y de la cual persisten muchas interrogantes sin resolver. Se realizó un estudio descriptivo y longitudinal de 19 niños con síndrome o enfermedad de moyamoya. La edad promedio de inicio fue de seis años. Del total de niños incluidos, doce debutaron en forma aguda con infarto cerebral; dos pacientes (con antecedentes de epilepsia y retraso) lo hicieron, uno con infarto y otro con hemorragia cerebral, otros dos con epilepsia y retraso, uno con trastorno del lenguaje, siendo en los dos restantes un hallazgo casual. Entretanto doce pacientes se consideraron idiopáticos (portadores de enfermedad de moyamoya) y siete asociados a enfermedades sistémicas (síndrome de moyamoya). El diagnóstico inicial se confirmó por angiorresonancia en diez casos y por arteriografía convencional en nueve. El seguimiento promedio fue de cinco años, once evidenciaron progresión clínica y ocho estabilidad, con un seguimiento promedio de siete y dos años respectivamente. Todos mostraron progresión angiográfica. En siete niños se realizaron diez cirugías de revascularización sin complicaciones y con buenos resultados clínicos y angiográficos. Dos niños fallecieron (uno por hemorragia cerebral y otro por un infarto expansivo) y catorce presentaron secuelas leves a moderadas. Resulta importante identificar los niños que se pueden beneficiar con la cirugía de revascularización, una de las pocas opciones terapéuticas para evitar la progresión y complicaciones de esta grave enfermedad.
Moyamoya's disease or syndrome is a rare illness wich causes symptoms of cerebral ischemia due to intracranial arteries' stenosis, with secondary abnormal vasculature networks at the base of the brain. Since the first description of moyamoya disease done by a group of Japanese neurosurgeons more than 50 years ago, this rare illness is still considered an intriguing disease. The clinical features, treatment, imaging findings and outcomes of a series of not Asian children with Moyamoya disease are described in this study. A total of 19 six year old patients were analyzed. The initial presentation in 12 patients was infarctions, 2 with epilepsy and mental retardation, another 2 who had personal history of epilepsy and mental retardation developed symptoms of ischemic stroke (one of them), and hemorrhagic stroke the other one; 1experienced language problems, while in the last two the diagnosis was casual. On the other hand, 12 patientes were considered idiopathic cases (moyamoya's disease) and 7 were related to systemic illnes (moyamoya's syndrome). Initial diagnosis was achieved by magnetic resonance angiography in 10 cases, and conventional angiography in 9. All patients were followed up for a mean period of 5 years. While 11 patients experienced clinical worsening of symptoms, 6 asymptomatic. However, all of them showed angiographic progression. Seven patients underwent bypass surgery with no further complications, 2 passed away and 14 remained with moderate handicaps. A number of surgical procedures have been developed for revascularization, improving the outcome of some of these patients by preventing ischemic and hemorrhagic stroke. Due to the fact that moyamoya disease is not a silent disorder and its progress cause complications, surgical revascularization should always be considered in the management of these patients.
