Brainstem and Autonomic Nervous System Dysfunction: A Neurosurgical Point of View.

Central autonomic control nuclei and pathways are mainly integrated within the brainstem, especially in the medulla oblongata. Lesions within these structures can lead to central dysautonomia.Central autonomic control structures can be damaged by tumors, during surgery, or by other neurosurgical pathologies. These may elicit clinical or subclinical autonomic complications that can constitute a serious clinical problem.The authors present a broad review of the central autonomic nervous system, its possible dysfunctions, and the relation between neurosurgery and this "not-well-known system". Preliminary results of an autonomic study of brainstem lesions that is currently being carried out by the authors are also shown.

Autonomic dysfunction elicited by a medulla oblongata injury after fourth ventricle tumor surgery in a pediatric patient.

We report the case of a 9-year-old male patient with a recurrent fourth ventricle anaplastic ependymoma who developed severe arterial hypertension and blood pressure lability during and after surgery. A punctual bilateral lesion located within mid dorsal medulla oblongata caused by both infiltration and surgical resection was observed in postoperative MRI. Three years later, the patient remained neurologically stable but the family referred the presence of a chronic tachycardia as well as palpitations and sweating with flushing episodes related to environmental stress. On autonomic evaluation, an increase in sympathetic outflow with tachycardia together with orthostatic hypotension caused by baroreceptor reflex dysfunction was observed. We postulate that a bilateral injury to both nuclei of the solitary tract may have caused central dysautonomia.

[Neuroendoscopic biopsy. Experience in 31 patients and literature review]. / Biopsia neuroendoscópica. Revisión de la literatura y experiencia en 31 pacientes.

OBJECTIVE. To describe our experience with the endoscopic management of intraventricular tumors, analyzing biopsy effectiveness, and to compare our results with those obtained from an extensive literature review. MATERIALS AND METHODS. Between 2003 and 2010, 31 patients aged between 7 months and 77 years, diagnosed of solid and/or cystic intra and/or periventricular tumors, underwent neuroendoscopic biopsy. We analyze operative technique, pathological result, management of associated hydrocephalus, rate of complications and postoperative technique. RESULTS. 32 endoscopic procedures were done and biopsy was successfully performed in 28 cases, with positive histological result in 25 of them (78% success rate per procedure and 89% success rate per biopsy). Most frequent pathological diagnosis was grade II astrocytoma. 30 patients had associated hydrocephalus that required endoscopic third ventriculostomy (19 cases, with 73.7% success rate) and/or septostomy (12 patients, 3 associated with ventriculostomy and 9 with ventriculo-peritoneal shunt). Frameless neuronavigation was used in three selected cases. During the surgery and the postoperative period the following complications appeared: intraventricular hemorrhage in four cases (two of them died), seizures in two patients, new neurological findings in three cases (Parinaud's sign, transient palsy of third cranial nerve and hemiparesis associated with palsy of third cranial nerve), and cerebrospinal fluid leak and infection in one case. 19 patients received subsequent treatment (microsurgical resection in 1, radiosurgery in 2, radiotherapy in 8, chemotherapy in 5 and chemo-radiotherapy in 3). CONCLUSIONS. Endoscopic management of intraventricular and/or periventricular brain tumors is effective, and allow diagnostic biopsy and simultaneous treatment of the associated hydrocephalus in many cases. So, it could be the treatment of choice in those tumors that are not suitable for microsurgical resection. Although this technique is not exempt of serious complications, morbimortality could be lower than conventional microsurgical approach.

[Ganglioneuroma coinciding with a lumbar disk herniation]. / Ganglioneuroma coincidente con una extrusión discal lumbar.

INTRODUCTION. Ganglioneuromas or gangliomas are tumours of the sympathetic ganglia that contain cells of the neural crest, so they can appear in all body localizations. They are generally benign, more frequent between 10 and 40 years, may secrete hormones and, sometimes, Neurofibromatosis type I and other genetic disorders can be associated. OBJECTIVE. To review the scientific literature related to the topic and to present a case treated in our service. DISCUSSION. The symptoms depend on location and vasoactive secreted hormones. In spite of that, they are generally benign tumours, although sometimes they can spread out. Since laboratory and image test are of limited usefulness, the conclusive diagnosis is anatomopathologic. In symptomatic patients the best procedure is surgical removing. CONCLUSION. Ganglioneuroma and disk herniation association constitute an exceptional disorder. Its treatment implies surgery resection.

Biopsia neuroendoscópica. Revisión de la literatura y experiencia en 31 pacientes / Neuroendoscopic biopsy. Experience in 31 patients and literature review

Objetivos. Presentar nuestra experiencia en el manejo endoscópico de tumores intraventriculares, analizando la efectividad diagnóstica de la biopsia, y comparar los resultados obtenidos con los datos publicados en la literatura. Material y métodos. Presentamos una serie de 31 pacientes con edades comprendidas entre los 7 meses y los 77 años, diagnosticados de proceso expansivo intra y/o periventricular, sólido y/o quístico, e intervenidos quirúrgicamente en nuestro servicio entre 2003 y 2010 para la realización de una biopsia neuroendoscópica. Analizamos la técnica utilizada, el resultado anatomopatológico obtenido, el manejo de la hidrocefalia asociada, la frecuencia de complicaciones del procedimiento y la actitud terapéutica posterior.Resultados. Se realizaron 32 procedimientos neuroendoscópicos, obteniéndose muestra para biopsia en 28 de ellos, con resultado positivo en 25 (78% de éxito por procedimiento y 89% de éxito por biopsia). El diagnóstico histológico más frecuente fue de astrocitoma grado II. 30 pacientes presentaban hidrocefalia asociada practicándose una ventriculostomía premamilar (VPM) en 19 casos (éxito en 14 casos, un 73.7%); además, se realizó una septostomía en 12 pacientes (en 3 casos asociada a VPM y en 9 casos seguida de derivación ventrículo-peritoneal). En 3 casos el procedimiento (..) (AU)

Objective. To describe our experience with the endoscopic management of intraventricular tumors, analyzing biopsy effectiveness, and to compare our results with those obtained from an extensive literature review.Materials and methods. Between 2003 and 2010, 31 patients aged between 7 months and 77 years, diagnosed of solid and/or cystic intra and/or periventricular tumors, underwent neuroendoscopic biopsy. We analyze operative technique, pathological result, management of associated hydrocephalus, rate of complications and postoperative technique.Results. 32 endoscopic procedures were done and biopsy was successfully performed in 28 cases, with (..)(AU)

Ganglioneuroma coincidente con una extrusión discal lumbar / Ganglioneuroma coinciding with a lumbar disk herniation

Introducción. Los ganglioneuromas o gangliomas son tumores de los ganglios simpáticos que contienen células de la cresta neural, pudiendo aparecer en cualquier parte del organismo. Son generalmente benignos, más frecuente entre los 10 y los 40 años, pueden secretar hormonas y, en ocasiones, asociarse a trastornos genéticos, como la neurofibromatosis tipo I.Objetivo. Revisar la literatura científica relacionada con el tema y presentar un caso tratado en nuestro servicio.Discusión. La sintomatología de estas lesiones depende de su ubicación y de las sustancias vasoactivas que puedan secretar. A pesar de que son tumores benignos en su mayoría, en ocasiones pueden metastatizar. Las pruebas de imagen y de laboratorio son inespecíficas, por lo que el diagnóstico definitivo es anatomopatológico. En los casos sintomáticos el tratamiento de elección es la resección quirúrgica.Conclusión. La asociación de un ganglioneuroma y de una hernia discal lumbar es algo excepcional. El tratamiento requiere su resección quirúrgica (AU)

Introduction. Ganglioneuromas or gangliomas are tumours of the sympathetic ganglia that contain cells of the neural crest, so they can appear in all body localizations. They are generally benign, more frequent between 10 and 40 years, may secrete hormones and, sometimes, Neurofibromatosis type I and other genetic disorders can be associated. Objective. To review the scientific literature related to the topic and to present a case treated in our service.Discussion. The symptoms depend on location and vasoactive secreted hormones. In spite of that, they are generally benign tumours, although sometimes they can spread out. Since laboratory and image test are of limited usefulness, the conclusive diagnosis is anatomopathologic. In symptomatic patients the best procedure is surgical revoming.Conclusión. Ganglioneuroma and disk herniation association constitute an excepcional disorder. Its treatment implies surgery resection (AU)

[Pilomyxoid astrocytoma. Three cases and review]. / Astrocitomas pilomixoides. Presentación de tres casos y revisión de la literatura.

INTRODUCTION: Pilomyxoid astrocytoma (PMA) is a central nervous system (CNS) tumour with peculiar clinicopathological features, that turn it into an entity different from pilocytic astrocytoma (PA). It appears in 2007 WHO classification of tumours of the CNS as an PA subtype belonging to the group of astrocytic tumours. Nowadays little is still known about this tumour entity; the histological origin and clinical behavior remain controversial, and there is no consensus about its management. OBJECTIVE: To review the scientific literature related to the topic and to present three cases treated at our service. CONCLUSIONS: PMA is an histological entity related to PA with a greater trend to regrowth and cerebrospinal fluid dissemination, therefore strict follow-up and oncological treatment is recommended.

Astrocitomas pilomixoides. Presentación de tres casos y revisión de la literatura / Pilomyxoid astrocytoma. Three cases and review

Introducción. El astrocitoma pilomixoide (APM) esun tumor del sistema nervioso central (SNC) con característicasclínicas y anatomopatológicas propias que loconvierten en una entidad diferente del astrocitomapilocítico (AP). Este tumor aparece en la clasificaciónde tumores del SNC de la OMS en su cuarta edición(año 2007) como subtipo de AP dentro del grupo delos tumores de origen astrocitario. Actualmente siguesiendo una entidad tumoral poco conocida, existiendocontroversia sobre su origen histológico y su comportamientoclínico, y una falta de consenso en cuanto a sumanejo terapéutico.Objetivo. Revisar la literatura científica relacionadacon el tema y presentar tres casos valorados en nuestroservicio.Conclusiones. El APM es una entidad anatomopatológicarelacionada con el AP aunque con una mayortendencia a la recidiva y diseminación por el líquidocefalorraquídeo, por lo que se recomienda seguimientoestricto y tratamiento oncológico adyuvante (AU)

Introduction. Pilomyxoid astrocytoma (PMA) is acentral nervous system (CNS) tumour with peculiarclinicopathological features, that turn it into an entitydifferent from pilocytic astrocytoma (PA). It appearsin 2007 WHO classification of tumours of the CNSas an PA subtype belonging to the group of astrocytictumours. Nowadays little is still known about thistumour entity; the histological origin and clinical behaviorremain controversial, and there is no consensusabout its management.Objective. To review the scientific literature relatedto the topic and to present three cases treated at ourservice.Conclusions. PMA is an histological entity related toPA with a greater trend to regrowth and cerebrospinalfluid dissemination, therefore strict follow-up and oncologicaltreatment is recommended (AU)

[Ventricular haemorrhage in preterm neonates and posthemorrhagic hydrocephalus. Proposal of a management protocol based on early ventriculo-peritoneal shunt]. / Hemorragia intraventricular del prematuro e hidrocefalia post-hemorrágica.Propuesta de un protocolo de manejo basado en la derivación ventrículo-peritonealprecoz.

INTRODUCTION: About 50% of the preterm neonates with a ventricular haemorrhage will develop posthaemorrhagic hydrocephalus. Medical treatment is not effective neither safe, does not reduce shunt's dependence and therefore can not be recommended; early and repetitive ventricular or lumbar punctures and the use of intraventricular fibrynolitic treatment have showed no effect on reducing patient's disability, shunt's necessity or mortality of these patients and furthermore, they can have several and important side effects. The ventriculo-peritoneal shunt can be in many cases the only option for definitive treatment, despite well-known infective and obstructive complications and there is an ongoing debate about the ideal moment for the intervention. OBJECTIVE: To present a diagnostic and treatment protocol for post-haemorrhagic hydrocephalus of the preterm and describe our initial experience with its application on the Paediatric Neurosurgical Department at the Hospital Materno-Infantil Carlos Haya of Málaga. MATERIALS AND METHODS: A total of 21 patients with diagnosis of preterm post-haemorrhagic hydrocephalus were surgically treated at our hospital with ventriculoperitoneal shunt between January 2003 and September 2006 following the designed protocol. All the cases were Papile's grade III or IV with severe ventricular dilation (Thalamus-Caudate index over 1.5 cm) and subacute or chronic presentation. We used medium pressure valves and antibiotic impregnated catheters. We considered 1500 g as the minimum weight permitted for the intervention. We report the early and late postoperative complications and the patients functional state at the ambulatory follow up classifying them in 4 grades (Excellent or Grade 1; Good or Grade 2; Regular or Grade 3; Poor or Grade 4) according to the presence of neurological focal signs, relation with the surrounding environment, response to stimuli and presence of seizures. RESULTS: The most frequent complications were escaphocephalic cranium in 5 patients, persistent subgaleal collections in 2 patients, symptomatic slit ventricles in 2 patients and surgical wound dehiscence with shunt infection in 1 patient. One patient presented a systemic fungical infection with non-diagnosed meningeal compromise previous to the shunt. 7 patients required shunt replacement (14 procedures); in 2 cases of tabicated hydrocephalus an endoscopical septostomy (associated with an ETV that did not function) was done, and in a third case ETV and shunt removal was performed after shunt malfunction, with delayed failure of ETV. For the functional results 9 patients were classified as Grade 1, 5 patients as Grade 2, 3 patients as Grade 3 and 4 patients as Grade 4. This means a 67% of good or excellent results. CONCLUSIONS: We propose a diagnostic and treatment protocol for preterm neonates with haemorrhagic hydrocephalus that we have been using since 2003 at our department. In our experience it is possible to shunt patients starting at 1500 g with low morbidity. The use of protocols can help in reducing complications and improving functional results in these patients.

Hemorragia intraventricular del prematuro e hidrocefalia post-hemorrágica. Propuesta de un protocolo de manejo basado en la derivación ventrículo-peritonealprecoz / Ventricular haemorrhage in preterm neonates and posthemorrhagic hydrocephalus. Proposal of a management protocol based on early ventriculo-peritoneal shunt

Introducción: Aproximadamente el 50% de los pacientes pretérminos diagnosticados de hemorragia intraventricular desarrollará una hidrocefalia posthemorrágica. La derivación ventrículo-peritoneal de LCR puede constituir en muchos casos la única opción de tratamiento definitivo, aunque se han descrito elevados porcentajes de complicaciones infecciosas o por obstrucción, existiendo además debate sobre cual es el momento más adecuado para la intervención. Objetivo: Presentar un protocolo de diagnóstico y tratamiento de la hidrocefalia post-hemorrágica del prematuro y describir nuestra experiencia inicial con su aplicación en el Hospital Materno-Infantil Carlos Haya de Málaga. Material y métodos: Un total de 21 pacientes con diagnóstico de hidrocefalia post-hemorrágica del prematuro fueron intervenidos mediante derivación ventrículo-peritoneal entre enero de 2003 y diciembre de 2006 de acuerdo al protocolo de diagnóstico y tratamiento elaborado en nuestro centro. En todos los casos se trataba de hidrocefalias grado III o IV de Papile, con dilataciones ventriculares graves (índice tálamo-caudado superior a 1.5cm) y de presentación subaguda o crónica. Se emplearon válvulas de presión media y catéteres con impregnación antibiótica. Se consideró 1500gr como el peso mínimo necesario para la intervención. Se valoró la aparición de complicaciones postquirúrgicas y la situación funcional de los pacientes en el seguimiento ambulatorio clasificándola en cuatro grados (excelente o grado 1, buena o grado 2, regular o grado 3 y mala o grado 4) de acuerdo con la presencia de focalidad, la relación con el entorno y presencia o no de crisis comiciales. Resultados: Las complicaciones más frecuentes fueron: cráneo escafocefálico en 5 pacientes, colección subgaleal persistente en 2 casos, ventrículo en hendidura sintomático en 2 casos, dehiscencia de herida quirúrgica en 1 caso con posterior infección valvular. Un paciente presentó una infección micótica sistémica con compromiso meníngeo no diagnosticado previo a la derivación. Se requirió recambio del sistema de derivación en 7 pacientes (14 reintervenciones); en 2 de estos casos se realizó septostomía endoscópica por hidrocefalia tabicada (junto con ventriculostomía de III ventrículo fallida) y en un tercer caso se realizó una ventriculostomía endoscópica y retirada valvular tras un episodio de disfunción valvular, con fallo diferido de la ventriculostomía. En cuanto a resultados funcionales 9 pacientes se clasificaron como grado 1, 5 pacientes como grado 2, 3 pacientes como grado 3 y 4 pacientes como grado 4, con un 67% de resultados buenos o excelentes. Conclusiones: Proponemos un protocolo para el diagnóstico y tratamiento de la hidrocefalia posthemorrágica del prematuro, de aplicación en nuestro Servicio desde Enero de 2003. En nuestra experiencia es posible derivar pacientes a partir de 1500gr de peso con baja morbilidad. La protocolización puede ayudarnos a reducir complicaciones y a mejorar el pronóstico funcional de estos pacientes (AU)

Introduction: About 50% of the preterm neonates with a ventricular haemorrhage will develop post haemorrhagic hydrocephalus. Medical treatment is not effective neither safe, does not reduce shunt's dependence and therefore can not be recommended; early and repetitive ventricular or lumbar punctures and the use of intraventricular fibrynolitic treatment have showed no effect on reducing patient's disability, shunt's necessity or mortality of these patients and furthermore, they can have several and important side effects. The ventriculo-peritoneal shunt can be in many cases the only option for definitive treatment, despite well-known infective and obstructive complications and there is an ongoing debate about the ideal moment for the intervention. Objective: To present a diagnostic and treatment protocol for post-haemorrhagic hydrocephalus of the preterm and describe our initial experience with its application on the Paediatric Neurosurgical Department at the Hospital Materno-Infantil Carlos Haya of Málaga. Materials and methods: A total of 21 patients with diagnosis of preterm post-haemorrhagic hydrocephalus were surgically treated at our hospital with ventriculoperitoneal shunt between January 2003 and September 2006 following the designed protocol. All the cases were Papile's grade III or IV with severe ventricular dilation (Thalamus-Caudate index over 1.5cm) and subacute or chronic presentation. We used medium pressure valves and antibiotic impregnated catheters. We considered 1500g as the minimum weight permitted for the intervention. We report the early and late postoperative complications and the patients functional state at the ambulatory follow up classifying them in 4 grades (Excellent or Grade 1; Good or Grade 2; Regular or Grade 3; Poor or Grade 4) according to the presence of neurological focal signs, relation with the surrounding environment, response to stimuli and presence of seizures. Results: The most frequent complications were escaphocephalic cranium in 5 patients, persistent subgaleal collections in 2 patients, symptomatic slit ventricles in 2 patients and surgical wound dehiscence with shunt infection in 1 patient. One patient presented a systemic fungical infection with non-diagnosed meningeal compromise previous to the shunt. 7 patients required shunt replacement (14 procedures); in 2 cases of tabicated hydrocephalus an endoscopical septostomy (associated with an ETV that did not function) was done, and in a third case ETV and shunt removal was performed after shunt malfunction, with delayed failure of ETV. For the functional results 9 patients were classified as Grade 1, 5 patients as Grade 2, 3 patients as Grade 3 and 4 patients as Grade 4. This means a 67% of good or excellent results. Conclusions: We propose a diagnostic and treatment protocol for preterm neonates with haemorrhagic hydrocephalus that we have been using since 2003 at our department. In our experience it is possible to shunt patients starting at 1500g with low morbidity. The use of protocols can help in reducing complications and improving functional results in these patients (AU)