RESUMEN
La aparición de tumores craneales inducidos por radiación en pacientes pediátricos tratados de enfermedades hematológicas como la leucemia/linfoma linfoblástico T (T-ALL/LBL) es un fenómeno conocido que puede incluir lesiones de diferentes grados de malignidad y precisar tratamiento neuroquirúrgico. Presentamos el caso de un varón de 38 años remitido a nuestro servicio por la aparición brusca de diplopía con hemiparesia izquierda progresiva de 6 meses de evolución y caídas frecuentes. Tras realización de pruebas de imagen, se objetivan distintas lesiones intraaxiales y extraaxiales con diferente comportamiento radiológico decidiéndose un abordaje quirúrgico único para su exéresis. La anatomía patológica confirma cuatro variantes histológicas: meningioma (grado 1 y 2 [atípico]), subependimoma y cavernoma. Discutimos el papel teratogénico y efectos biológicos de las radiaciones ionizantes (AU)
The cranial radiation-induced tumors appearance in pediatric patients treated for hematological diseases such as leukemia/T-lymphoblastic lymphoma (T-ALL/LBL) is a known phenomenon that may include lesions of different malignant degrees and require neurosurgical treatment. We present the case of a 38-year-old man referred to our department for a sudden diplopia with 6-month progressive left hemiparesis and frequent falls. After imaging tests, different intra and extraxial lesions with different radiological behavior were observed, performing a single surgical approach for their resection. The pathological anatomy confirms four histological variants: meningioma (grade 1 and 2 [atypical]), subependymoma, and cavernoma. We discuss the teratogenic role of ionizing radiation (AU)
Asunto(s)
Humanos , Masculino , Adulto , Glioma Subependimario/etiología , Hemangioma Cavernoso/etiología , Meningioma/etiología , Neoplasias Inducidas por Radiación/diagnóstico por imagen , Leucemia Linfoide/radioterapia , Neoplasias Encefálicas/etiología , Tomografía Computarizada por Rayos X , Glioma Subependimario/diagnóstico por imagen , Hemangioma Cavernoso/diagnóstico por imagen , Neoplasias Encefálicas/diagnóstico por imagen , Meningioma/diagnóstico por imagenRESUMEN
BACKGROUND: Transient donor hydrops (TDH) is defined as donor hydrops developed within days after laser therapy for twin-twin transfusion syndrome (TTTS) followed by resolution later. The purpose of this study was to evaluate the incidence, neonatal outcomes and predisposing factors of post laser therapy TDH in severe TTTS. METHODS: A total of 142 patients with severe TTTS who received laser therapy were included into this study. The pre-operative characteristics and neonatal outcomes were compared between TTTS with and without post laser therapy TDH. All live neonates received cranial ultrasound examination after delivery, mild cerebral injury was defined as exhibiting at least one of the following: intraventricular hemorrhage (IVH) grade I and II, lenticulostriate vasculopathy and subependymal pseudocysts; severe cerebral injury comprised at least one among the following: IVH grade III or grade IV, cystic periventriculoleukomalacia (PVL) grade II or more, porencephalic cysts, and ventricular dilatation. Fetal survival was defined as living more than 30 days after delivery.
Asunto(s)
Transfusión Feto-Fetal/cirugía , Fetoscopía/efectos adversos , Hidropesía Fetal/epidemiología , Enfermedades del Recién Nacido/epidemiología , Coagulación con Láser/efectos adversos , Adulto , Enfermedad Cerebrovascular de los Ganglios Basales/epidemiología , Enfermedad Cerebrovascular de los Ganglios Basales/etiología , Lesiones Encefálicas/epidemiología , Lesiones Encefálicas/etiología , Estudios de Casos y Controles , Hemorragia Cerebral Intraventricular/epidemiología , Hemorragia Cerebral Intraventricular/etiología , Femenino , Transfusión Feto-Fetal/complicaciones , Transfusión Feto-Fetal/embriología , Fetoscopía/métodos , Glioma Subependimario/epidemiología , Glioma Subependimario/etiología , Humanos , Hidropesía Fetal/etiología , Incidencia , Recién Nacido , Enfermedades del Recién Nacido/etiología , Coagulación con Láser/métodos , Embarazo , Resultado del Embarazo , Embarazo GemelarRESUMEN
Hydrocephalus formation is a frequent complication of neuropathological insults associated with neuroinflammation. However, the mechanistic role of neuroinflammation in hydrocephalus development is unclear. We have investigated the function of the proinflammatory acting inhibitor of κB kinase (IKK)/nuclear factor κB (NF-κB) signaling system in neuroinflammatory processes and generated a novel mouse model that allows conditional activation of the IKK/NF-κB system in astrocytes. Remarkably, NF-κB activation in astrocytes during early postnatal life results in hydrocephalus formation and additional defects in brain development. NF-κB activation causes global neuroinflammation characterized by a strong, astrocyte-specific expression of proinflammatory NF-κB target genes as well as a massive infiltration and activation of macrophages. In this animal model, hydrocephalus formation is specifically induced during a critical time period of early postnatal development, in which IKK/NF-κB-induced neuroinflammation interferes with ependymal ciliogenesis. Our findings demonstrate for the first time that IKK/NF-κB activation is sufficient to induce hydrocephalus formation and provides a potential mechanistic explanation for the frequent association of neuroinflammation and hydrocephalus formation during brain development, namely impairment of ependymal cilia formation. Therefore, our study might open up new perspectives for the treatment of certain types of neonatal and childhood hydrocephalus associated with hemorrhages and infections.