Tratamiento de la siringomielia en pacientes con malformación de Chiari y craneosinostosis. Caso clínico y revisión de la bibliografía / Treatment of syringomyelia in patients with Chiari malformation and craniosynostosis. A case report and review of the literature

Introducción. Los pacientes con craneosinostosis complejas o unisuturales presentan frecuentemente malformación de Chiari y siringomielia. El tratamiento quirúrgico de la siringomielia en estos pacientes es controvertido. Caso clínico. Niña de 3 años con craneosinostosis compleja no corregida quirúrgicamente. Permaneció asintomática a pesar de que en la resonancia magnética craneal se evidenció una malformación de Chiari y un año después desarrolló una siringomielia cervicodorsolumbar. Se le realizó una craniectomía suboccipital descompresiva, pero posteriormente sufrió un empeoramiento de la siringomielia. El registro de presión intracraneal resultó patológico, por lo que se decidió realizar una craneotomía descompresiva frontoparietotemporal bilateral y remodelación de la bóveda craneal, con lo que se consiguió una disminución significativa de la siringomielia. Conclusiones. Tras la revisión de la bibliografía, se observa que actualmente no existe un consenso sobre el tratamiento de la siringomielia en los pacientes con craneosinostosis y malformación de Chiari. Algunos autores recomiendan la simultánea descompresión quirúrgica suboccipital y de la bóveda craneal, otros sólo la descompresión de la bóveda craneal, y otros la ampliación de la fosa posterior con distractores. En los casos en los que se realizó primero la descompresión suboccipital no se consiguió resolver ni estabilizar la siringomielia. Concluimos que el tratamiento más eficaz para los pacientes con siringomielia y craneosinostosis es la remodelación descompresiva de la bóveda craneal, ya que el principal factor causante de la siringomielia es la hipertensión intracraneal y la falta de distensibilidad del cráneo (AU)

Introduction. Patients with multisutural or single craniosynostosis, often suffer from Chiari malformation and syringomyelia. The surgical management of syringomyelia in these patients is controversial. Case report. A 3-year-old girl was referred with complex craniosynostosis that had not been corrected surgically. She was asymptomatic despite the cranial MRI showed a Chiari malformation and one year later she developed a cervico-dorsolumbar syringomyelia. She underwent a decompressive suboccipital craniectomy but subsequently suffered a worsening of syringomyelia. The intracranial pressure monitoring was pathological so it was decided to perform a decompressive bilateral fronto-parieto-temporal craniotomy and remodeling of the cranial vault, achieving a significant reduction of syringomyelia. Conclusions. After reviewing the literature, it is noted that there is currently no consensus on the treatment of syringomyelia in patients with craniosynostosis and Chiari malformation. Some authors recommend the simultaneous surgical suboccipital and cranial vault decompression, others only decompression of the cranial vault and other enlargement of the posterior fossa with distractors. In cases where the suboccipital decompression was performed first, the syringomyelia was not improved or stabilized. We conclude that the most effective treatment for patients with syringomyelia and craniosynostosis is decompressive remodeling of the cranial vault, as the main cause of syringomyelia is the raised intracranial pressure and lack of skull compliance (AU)

Cirugía de la epilepsia en niños con displasias corticales focales / Epilepsy surgery in children with focal cortical dysplasias

Introducción. Las displasias corticales focales (DCF) son la primera etiología de cirugía de la epilepsia pediátrica. La evaluación prequirúrgica en DCF a menudo es compleja, ya que son lesiones que pueden ser altamente epileptógenas y, a la vez, conservar función neurológica, y no visualizarse en la resonancia magnética. El éxito de la cirugía depende, en gran medida, de la adecuada identificación de la lesión y de la posibilidad de realizar una resección completa del tejido displá- sico. En este trabajo se revisa la bibliografía relacionada con el tema, en relación con la experiencia de los autores. Desarrollo. Se revisan algunos avances relacionados con la evaluación prequirúrgica y el abordaje neuroquirúrgico de la epilepsia en niños con DCF; se comentan los resultados de las series quirúrgicas en relación con las clasificaciones de DCF y los factores de pronóstico posquirúrgico; se describen algunos fenotipos anatomoelectroclínicos distintivos en niños con DCF y su abordaje quirúrgico; y se comentan brevemente los actuales retos y el futuro del tratamiento quirúrgico de la epilepsia en DCF. Conclusiones. El avance los métodos de diagnóstico prequirúrgico y de procedimientos quirúrgicos está permitiendo ofrecer un tratamiento exitoso en edades tempranas a pacientes con DCF previamente considerados ‘no lesionales’ y a pacientes con lesiones localizadas en la ‘corteza elocuente’. La identificación de fenotipos anatomoelectroclínicos de DCF permite establecer abordajes quirúrgicos y expectativas de pronóstico posquirúrgico adecuadas a cada situación, mejor en las DCF IIb transmantle y en las displasias de fondo de surco que en las DCF multilobares, en su mayoría DCF I (AU)

Introduction. Focal cortical dysplasias (FCD) are the first cause of surgery in paediatric epilepsy surgery. The pre-surgical assessment in FCD is often complex, since they are lesions that can be highly epileptogenic and at the same time can preserve neurological functioning and may not be displayed in magnetic resonance imaging. The success of the operation largely depends on the proper identification of the lesion and the possibility of performing a complete resection of the dysplastic tissue. In this work we review the literature related with this topic, in relation to the authors’ experience. Development. The study reviews some of the advances made as regards the pre-surgical assessment and the neurosurgical management of epilepsy in children with FCD; results from the surgical series regarding the classifications of FCD and the post-surgical prognostic factors are commented on; some anatomo-clinical phenotypes that are distinctive in children with FCD and their surgical management are described; and current challenges and the future of the surgical treatment of epilepsy in FCD are also briefly discussed. Conclusions. The advances being made in the methods of pre-surgical diagnosis and surgical procedures are making it possible to offer successful treatment at earlier ages in patients with FCD who were previously considered ‘non-lesional’ and in patients with localised lesions in the ‘eloquent cortex’. The identification of anatomo-electro-clinical phenotypes of FCD makes it possible to establish surgical approaches and post-surgical prognostic expectations that are well suited to each situation, which are better in the transmantle-type FCD IIb and in bottom-of-sulcus dysplasias than in multilobe FCD, which are mostly FCD I (AU)

[Epilepsy surgery in children with focal cortical dysplasias]. / Cirugía de la epilepsia en niños con displasias corticalesfocales.

INTRODUCTION: Focal cortical dysplasias (FCD) are the first cause of surgery in paediatric epilepsy surgery. The pre-surgical assessment in FCD is often complex, since they are lesions that can be highly epileptogenic and at the same time can preserve neurological functioning and may not be displayed in magnetic resonance imaging. The success of the operation largely depends on the proper identification of the lesion and the possibility of performing a complete resection of the dysplastic tissue. In this work we review the literature related with this topic, in relation to the authors' experience. DEVELOPMENT: The study reviews some of the advances made as regards the pre-surgical assessment and the neurosurgical management of epilepsy in children with FCD; results from the surgical series regarding the classifications of FCD and the post-surgical prognostic factors are commented on; some anatomo-clinical phenotypes that are distinctive in children with FCD and their surgical management are described; and current challenges and the future of the surgical treatment of epilepsy in FCD are also briefly discussed. CONCLUSIONS: The advances being made in the methods of pre-surgical diagnosis and surgical procedures are making it possible to offer successful treatment at earlier ages in patients with FCD who were previously considered 'non-lesional' and in patients with localised lesions in the 'eloquent cortex'. The identification of anatomo-electro-clinical phenotypes of FCD makes it possible to establish surgical approaches and post-surgical prognostic expectations that are well suited to each situation, which are better in the transmantle-type FCD IIb and in bottom-of-sulcus dysplasias than in multilobe FCD, which are mostly FCD I.

TITLE: Cirugia de la epilepsia en niños con displasias corticales focales.Introduccion. Las displasias corticales focales (DCF) son la primera etiologia de cirugia de la epilepsia pediatrica. La evaluacion prequirurgica en DCF a menudo es compleja, ya que son lesiones que pueden ser altamente epileptogenas y, a la vez, conservar funcion neurologica, y no visualizarse en la resonancia magnetica. El exito de la cirugia depende, en gran medida, de la adecuada identificacion de la lesion y de la posibilidad de realizar una reseccion completa del tejido displasico. En este trabajo se revisa la bibliografia relacionada con el tema, en relacion con la experiencia de los autores. Desarrollo. Se revisan algunos avances relacionados con la evaluacion prequirurgica y el abordaje neuroquirurgico de la epilepsia en niños con DCF; se comentan los resultados de las series quirurgicas en relacion con las clasificaciones de DCF y los factores de pronostico posquirurgico; se describen algunos fenotipos anatomoelectroclinicos distintivos en niños con DCF y su abordaje quirurgico; y se comentan brevemente los actuales retos y el futuro del tratamiento quirurgico de la epilepsia en DCF. Conclusiones. El avance los metodos de diagnostico prequirurgico y de procedimientos quirurgicos esta permitiendo ofrecer un tratamiento exitoso en edades tempranas a pacientes con DCF previamente considerados 'no lesionales' y a pacientes con lesiones localizadas en la 'corteza elocuente'. La identificacion de fenotipos anatomoelectroclinicos de DCF permite establecer abordajes quirurgicos y expectativas de pronostico posquirurgico adecuadas a cada situacion, mejor en las DCF IIb transmantle y en las displasias de fondo de surco que en las DCF multilobares, en su mayoria DCF I.

Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy.

PURPOSE: The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains. METHODS: Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions. RESULTS: At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel's class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal-social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG. CONCLUSIONS: Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization.

Epilepsy surgery in children with developmental tumours.

We report our experience regarding evaluation, surgical treatment and outcomes in a population of 21 children with histopathologically confirmed developmental tumours [nine dysembryoplastic neuroepithelial tumours (DNET), ten gangliogliomas (GG) and two gangliocytomas (GC)] and related epilepsy, analyzing video-EEG, MRI and neuropsychological data, before and after surgery. Most children had focal epilepsy correlating well with lesion location. One patient had epileptic spasms and generalized discharges. Tumours were located in the temporal lobe in 13 patients. Mean age at surgery was 11.16 years. Postsurgical MRI showed residual tumour growth in one DNET. One child had a recurrent ganglioglioma with anaplastic transformation. At latest follow-up (mean 4.68 years) 95.2% of patients were seizure-free and no significant neuropsychological declines were observed. Evidence from our study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.