Seudotumor cerebri en niños: etiología, características clínicas y evolución / Pseudotumour cerebri in children: Aetiology, clinical features, and progression

Introducción: El síndrome de hipertensión intracraneal idiopática o seudotumor cerebri (STC) en niños está en constante revisión, respecto a su definición, etiologías asociadas, diagnóstico y terapéutica más apropiada. Objetivos y métodos: Se revisaron los casos de STC < 15 años de edad en un hospital de referencia en los últimos 12 años. Se estudiaron las características clínico-epidemiológicas y el procedimiento diagnóstico-terapéutico empleado. Se definió STC como presión intracraneal > 25cmH2O por punción lumbar (PL), con estudio de resonancia magnética cerebral sin lesión ocupante de espacio. Resultados: Se registró a 12 niños con STC, media de edad de 10 años, 90% mujeres. Todos presentaban peso normal. El 82% manifestaba síntomas: cefalea (66%), diplopía (8%) o baja visión (8%). Todos asociaban papiledema (17% unilateral). La PL fue diagnóstica en el 100% y la neuroimagen fue normal en el 91%. Se evidenció un posible desencadenante en 5 casos (2 farmacológico y 3 infeccioso por Mycoplasma pneumoniae [M. pneumoniae]). El 91% recibió tratamiento médico: en el 75% consistió en PL repetidas y en el 42% solo acetazolamida y/o prednisona. La evolución fue favorable en todos ellos. Conclusiones: La incidencia de STC fue de aproximadamente 1/100.000 niños/año, similar a estudios previos. En esta población, el sobrepeso no es un factor de riesgo. La infección por M. pneumoniae podría actuar como desencadenante de STC y favorecer recurrencias tardías. La ausencia de síntomas parece independiente del grado de presión intracraneal. El tratamiento con acetazolamida es eficaz en la mayoría de los casos, desterrando el uso de PL repetidas

Introduction: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. Objectives and methods: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure> 25 cmH2O) and absence of space-occupying lesions in brain MR images. Results: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. Conclusions: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP

Pseudotumour cerebri in children: Aetiology, clinical features, and progression. / Seudotumor cerebri en niños: etiología, características clínicas y evolución.

INTRODUCTION: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. OBJECTIVES AND METHODS: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images. RESULTS: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. CONCLUSIONS: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP.

Quistes neuroentéricos, una causa infrecuente de hemorragia intestinal / Neurenteric cysts; an uncommon cause of intestinal bleeding

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Feocromocitoma y paraganglioma en la infancia: a propósito de 2 casos / Pheochromocytoma and paraganglioma in childhood: A report of 2 cases report

Los feocromocitomas y los paragangliomas son tumores neuroendocrinos infrecuentes en pediatría; sin embargo, estos representan el tumor endocrino más frecuente en la infancia. La mayoría son esporádicos, cobrando mayor relevancia los síndromes familiares en edad pediátrica. Los avances en el campo de la genética, en bioquímica y en técnicas de imagen han adaptado el manejo de estos tumores; de modo que el estudio bioquímico debe comenzarse ante todo diagnóstico clínico de sospecha, seguido del estudio por imagen y molecular, más aún ante la existencia de un síndrome familiar conocido. Revisamos aspectos clínicos, diagnósticos y terapéuticos a propósito de 2 casos, presentando el segundo paciente antecedentes de neurofibromatosis tipo 1

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors in children and most of them are sporadic. However, they represent the most common endocrine tumor in childhood, and hereditary tumor syndromes are most relevant in these age. Advances in genetic, biochemistry and imaging techniques have revised the management of these tumors; thus A biochemical study should be always initiated once the clinical diagnosis is suspected, followed by imaging and molecular studies, particularly in the context of known familial disease. The diagnostic and therapeutic features are reviewed after the presentation of two clinicalcases, where the second one is a patient with type 1 Neurofibromatosis

[Pheochromocytoma and paraganglioma in childhood: a report of 2 cases report]. / Feocromocitoma y paraganglioma en la infancia: a propósito de 2 casos.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors in children and most of them are sporadic. However, they represent the most common endocrine tumor in childhood, and hereditary tumor syndromes are most relevant in these age. Advances in genetic, biochemistry and imaging techniques have revised the management of these tumors; thus A biochemical study should be always initiated once the clinical diagnosis is suspected, followed by imaging and molecular studies, particularly in the context of known familial disease. The diagnostic and therapeutic features are reviewed after the presentation of two clinical cases, where the second one is a patient with type 1 Neurofibromatosis.