[Treatment of syringomyelia in patients with Chiari malformation and craniosynostosis. A case report and review of the literature]. / Tratamiento de la siringomielia en pacientes con malformacion de Chiari y craneosinostosis. Caso clinico y revision de la bibliografia.

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-dorso-lumbar 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.

TITLE: Tratamiento de la siringomielia en pacientes con malformacion de Chiari y craneosinostosis. Caso clinico y revision de la bibliografia.Introduccion. Los pacientes con craneosinostosis complejas o unisuturales presentan frecuentemente malformacion de Chiari y siringomielia. El tratamiento quirurgico de la siringomielia en estos pacientes es controvertido. Caso clinico. Niña de 3 años con craneosinostosis compleja no corregida quirurgicamente. Permanecio asintomatica a pesar de que en la resonancia magnetica craneal se evidencio una malformacion de Chiari y un año despues desarrollo una siringomielia cervicodorsolumbar. Se le realizo una craniectomia suboccipital descompresiva, pero posteriormente sufrio un empeoramiento de la siringomielia. El registro de presion intracraneal resulto patologico, por lo que se decidio realizar una craneotomia descompresiva frontoparietotemporal bilateral y remodelacion de la boveda craneal, con lo que se consiguio una disminucion significativa de la siringomielia. Conclusiones. Tras la revision de la bibliografia, se observa que actualmente no existe un consenso sobre el tratamiento de la siringomielia en los pacientes con craneosinostosis y malformacion de Chiari. Algunos autores recomiendan la simultanea descompresion quirurgica suboccipital y de la boveda craneal, otros solo la descompresion de la boveda craneal, y otros la ampliacion de la fosa posterior con distractores. En los casos en los que se realizo primero la descompresion suboccipital no se consiguio resolver ni estabilizar la siringomielia. Concluimos que el tratamiento mas eficaz para los pacientes con siringomielia y craneosinostosis es la remodelacion descompresiva de la boveda craneal, ya que el principal factor causante de la siringomielia es la hipertension intracraneal y la falta de distensibilidad del craneo.

Servicio de Neurocirugía del H. Infantil Universitario Niño Jesús / Neurosurgery Department of H. Infantil Universitario Niño Jesús

En el Servicio se desarrollan programas de investigación en epilepsia refractaria, cráneosinóstosis e hidrocefalia entre otros. Se describe la actividad asistencial, especialmente en los tumores del sistema nervioso central y la epilepsia (AU)

Research programs in the Department are developed in refractory epilepsy, craneosynostosis and hydrocephalia, among others. The care activity, especially those for central nervous system tumors and epilepsy, is described AU)

[Tumours in the pineal region in the paediatric age. Reports of 23 cases and a review of the literature]. / Tumores de la region pineal en la edad pediatrica. Presentacion de 23 casos y revision de la bibliografia.

INTRODUCTION: Tumours in the pineal region are located at a meeting point of several neurovascular structures that are difficult to reach surgically and for which the possibility of resection is limited; as a result the management of these lesions usually requires associated adjunctive treatment with radiotherapy and/or chemotherapy. PATIENTS AND METHODS: This study is a retrospective analysis of the epidemiological, clinical, neuroimaging and pathological characteristics of 23 patients with tumours in the pineal region who were treated between the years 1997 and 2010 in the Hospital Infantil Niño Jesús. The factors involved in the prognosis of this cohort following surgical or adjunctive treatment are also discussed. RESULTS: Subjects included in the study were 6 girls and 17 boys with ages ranging from 4 months to 18 years. It was found that the initial symptoms in 95% of the patients were signs of acute or subacute hydrocephalus, which required the placement of a ventriculoperitoneal shunt (82%). A histological sample of the tumour tissue was collected in all cases. Biopsy samples were taken in the case of five patients and 18 underwent surgery involving a craniotomy. Germinoma (eight cases) and mature teratoma (one case) were the tumours with the longest survival times; non-germinomatous tumours (three cases), those of the pineal parenchyma (four cases) and gliomas (five cases) presented the highest rates of recurrence and a poorer prognosis. CONCLUSIONS: The study of tumour markers can be used to guide the diagnosis of certain tumours of the pineal region. At present, the recommended procedure involves taking a histological sample of the tumour in order to establish an accurate diagnosis and a specific oncological treatment.

[Coccygeal dermal sinus: Clinical relevance and management]. / Sinus dérmico sacrocoxígeo: importancia clínica y manejo.

INTRODUCTION: The presence of a dermal sinus on a child's back is a common cause for paediatric neurosurgical consultation. The relative frequency of these lesions and fears on their possible clinical consequences enhances the importance of early diagnosis and treatment. With this work we aimed to search for the most relevant clinical features that might indicate an intradural penetration of the lesions and that may advise performing neuroimaging studies and a paediatric neurosurgical consultation. PATIENTS AND METHODS: The records pertaining to children diagnosed with sacro-coccygeal pits seen at our institution during the years 2005-2009 were reviewed. A Pub Med search of the most relevant articles on the subject was also performed. RESULTS: Thirty-two children diagnosed with sacro-coccygeal pits were seen at our institution in the study period. Most cases had been sent for neurosurgical consultation by their paediatricians, paediatric surgeons or paediatricians of the emergency services in our region. Seventeen patients were submitted to some neuroimaging study and 15 were evaluated only by physical examination. In no patient was an intradural penetration of the lesion observed. The lack of reports on coccygeal sinuses probably reflects the unimportance given to these lesions in the absence of neurological involvement. CONCLUSIONS: A detailed physical examination can reduce the number of neuroimaging studies, an intergluteal localization of the sinus being the most important diagnostic clue. Doubtful cases of an intradural penetration of the lesion can be further evaluated by ultrasound as a preliminary screening tool and by magnetic resonance and neurosurgical consultation if deemed necessary.

[Presurgery neuropsychological evaluation in pediatric focal epilepsies]. / Evaluación neuropsicológica prequirúrgica en epilepsias focales pediátricas.

INTRODUCTION: Neuropsychological assessment is included in the protocols for evaluation of epilepsy surgery candidates, providing information about the patient's cognitive dysfunctions, allowing for prediction of possible cognitive deficits derived from surgery and yielding objective measures of any post-surgical changes. Neuropsychological disturbances constitute an important co-morbidity of medically intractable epilepsy. An early epilepsy onset in infancy may lead to cognitive dysfunctions that are atypical in terms of brain localization, due to the inherent plasticity and reorganization processes of the immature brain. The analysis of the neuropsychological profiles of paediatric focal epilepsies is much more complex than in the adult population. DEVELOPMENT AND CONCLUSIONS: In this paper, we review the neuropsychological disturbances associated to focal epilepsies (posterior cortex, temporal and frontal epilepsies), stressing the point that there is a considerable lack of rigorous studies on the topic in the literature, in spite of this being an essential part of the presurgical work-up in epilepsy patients.

[Surgical treatment of symptomatic arachnoid cysts in children]. / Tratamiento quirúrgico de los quistes aracnoideos sintomáticos en niños.

INTRODUCTION: When symptomatic, arachnoid cysts (AC) must be treated surgically. The best surgical technique, however, is at the present time still subject to controversy -implantation of a cyst-peritoneal shunt (CPS) or fenestration of the cyst, either by means of a craniotomy or by using endoscopic techniques. PATIENTS AND METHODS: This paper reports the findings from a series of 18 patients under 10 years of age who were treated for symptomatic ACs. An increase in the cranial perimeter was observed in 12 patients, 4 had headaches and 2 children suffered convulsive crises. In 11 cases the location was supratentorial and in 7 it was found to be infratentorial. RESULTS: Treatment involved a cyst-peritoneal or ventriculoperitoneal shunt in 12 cases. Endoscopic treatment of the cyst was carried out in 5 of the patients and in 1 case craniotomy debridement was performed. Seven of the 18 children required a second intervention to resolve the clinical condition, either due to poor valve functioning or because the endoscopic treatment was insufficient. Complications included 2 subdural haematomas, which required surgical treatment. No mortality or morbidity occurred. CONCLUSIONS: The progress being accomplished in endoscopic techniques can make them the ideal form of treatment rather than craniotomy debridement techniques, although the high percentage of no-resolution in children below the age of 15 months must be taken into account. CPS solves the problem of these cysts with a lower degree of surgical risk, but it has a high rate of reintervention, as well as the dependence on the shunt. In the review of the literature we carried out it was seen that reports are still published concerning series treated by both cyst fenestration and by means of shunts.

[Spinal epidural abscess caused by Acinetobacter baumannii mimicking a herniated lumbar disc]. / Absceso epidural vertebral por Acinetobacter baumanii que simula hernia discal lumbar.

INTRODUCTION: A vertebral epidural abscess usually offers a very varied clinical picture of systemic involvement with signs of infection, general malaise and neurological focus. It is diagnosed by means of magnetic resonance imaging, which reveals large lesions with frequent involvement of soft tissues and peripheral contrast enhancement. CASE REPORT: A 35-year-old male with lumbar-radicular pain in the right S1 with Lasègue's sign at 20 degrees on the right side and abolition of the Achilles' reflex. Magnetic resonance imaging showed an extradural lesion in L5-S1, dependent on the disc space, which suggested a herniated disc. The rest of the anamnesis, explorations and analyses were normal except for a slightly high erythrocyte sedimentation rate. The patient was submitted to surgery and an epidural abscess was observed from which an Acinetobacter baumanii was recovered. Treatment was established with antibiotics and a rigid lumbosacral orthosis. At three months clear signs of discitis were observed in magnetic resonance images; these were completely resolved at eight months, when the patient was asymptomatic. CONCLUSIONS: Epidural abscess must be included in the differential diagnosis of a herniated disc because in the early phases it can give rise to symptoms of lumbar-radicular pain that are identical to those caused by a herniated lumbar disc. This is the first case of an epidural abscess produced by A. baumanii.

[Psychosis in a case of temporal lobe epilepsy associated with a dysembryoplastic neuroepithelial tumour]. / Psicosis en un caso de epilepsia temporal asociado a un tumor neuroepitelial disembrioplasico.

INTRODUCTION: Psychosis is an acknowledged, although infrequent, complication that occurs in 0.5 9% of all cases of epilepsy. In this work, we present a case of complex partial epilepsy due to a dysembryoplastic neuroectodermal tumour in the right hippocampus, which began as acute psychosis and was cured following its surgical removal. Both its aetiology and its pathogenesis are discussed. CASE REPORT: We describe the case of a 17 year old male with a family history of schizophrenia. While studying his last years of secondary education, and apparently well adapted, he was taken into custody and hospitalised urgently because of personality disorders. The patient suffered from somatic and auditory hallucinations, simple visual pseudohallucinations, formal alterations to his thinking, cosmovisions, delusions of influence, persecution, grandeur and of a mystic nature, with no other neurological focus. At the same time, the patient also had complex partial seizures. Magnetic resonance imaging revealed a cystic tumour in the right temporal lobe. Pathology laboratory findings showed a dysembryoplastic neuroepithelial tumour. For a period of two years following surgical removal, the schizophrenic symptoms and the complex partial seizures disappeared and the patient no longer required antipsychotic medication. CONCLUSIONS: Epileptic psychosis may be related to pathological conditions of the hippocampus. In this study we present what we believe to be the first case of a dysembryoplastic neuroepithelial tumour giving rise to such psychosis. Surgical removal of the tumour cured both entities.

[Lumbar disc disease in adolescents]. / Hernia discal lumbar en adolescentes.

INTRODUCTION: Intervertebral disc herniation is a very common neurosurgical pathology. Most commonly disc disease occurs in adults and series published in children are only few and each reports only few cases. A genetic factor has been considered but in none of our patients there was a family history of disc disease. PATIENTS AND METHODS: We report ten patients with disc herniations in adolescents occurring among 1920 patients operated of herniated discs between 1988 and 1998. These herniations in adolescents were all related to some sport activities, and they occurred in the age range of 12 to 16 years. Symptoms were back pain and lower extremities pains of roots L5 or S1. All were studied with magnetic resonance imaging and in all patients the herniated disc was either in L4 L5 or L5 S1 intervertebral spaces. All disc were subligamental and 81.5% were posterolateral in position. Pathological studies of the herniated discs showed elastic consistency with high water content without degenerative changes. RESULTS: After microscopic removal of the herniated discs pain disappeared and remained so in the follow up. It was not necessary to reoperate in any of these patients and the postoperative follow up was between 2 and 5 years. CONCLUSIONS: Herniated disc disease is very infrequent in adolescents and all recovered completely after surgery, and the localization is posterolateral and subligamental.

Hernia discal lumbar en adolescentes / Lumbar disc disease in adolescents

Introducción. Las hernias discales son una patología habitual dentro de la Neurocirugía, pero la presentación suele producirse en la edad adulta y las series publicadas en niños son pocas y con un número de casos pequeño. Se ha invocado un factor genético, pero en ninguno de nuestros casos existía antecedente familiar de hernia de disco. Pacientes y métodos. Se presentan 10 hernias discales lumbares (HDL) en adolescentes en una serie de 1.920 HDL, que abarca desde 1988 hasta 1998. Todos los casos de la serie se presentaron asociados a la realización de algún tipo de deporte. Las edades estaban comprendidas entre los 12 y los 16 años. La clínica fue un cuadro de dolor lumbar con irradiación por el territorio radicular asociado (L5 o S1). Todos los casos se estudiaron con resonancia magnética y su localización fue, en el 100 por ciento, entre L4-L5 y L5-S1; todas eran hernias subligamentosas y el 81,5 por ciento aparecieron en posición posterolateral. La anatomía patológica demostró una consistencia elástica y alto contenido en agua, sin fenómenos degenerativos. Resultados. En todos los casos desaparecía el cuadro doloroso y se hacían asintomáticas tras la realización de extirpación por el procedimiento de microdisectomía. No se precisó reintervención en ningún caso, y el control postoperatorio fue de entre 2 y 5 años. Conclusiones. La HDL del adolescente es una patología poco frecuente y en todos los casos los resultados fueron buenos después de la intervención quirúrgica. La localización generalmente es subligamentosa posterolateral (AU)

Introduction. Intervertebral disc herniation is a very common neurosurgical pathology. Most commonly disc disease occurs in adults and series published in children are only few and each reports only few cases. A genetic factor has been considered but in none of our patients there was a family history of disc disease. Patients and methods. We report ten patients with disc herniations in adolescents occurring among 1920 patients operated of herniated discs between 1988 and 1998. These herniations in adolescents were all related to some sport activities, and they occurred in the age range of 12 to 16 years. Symptoms were back pain and lower extremities pains of roots L5 or S1. All were studied with magnetic resonance imaging and in all patients the herniated disc was either in L4-L5 or L5-S1 intervertebral spaces. All disc were subligamental and 81.5% were posterolateral in position. Pathological studies of the herniated discs showed elastic consistency with high water content without degenerative changes. Results. After microscopic removal of the herniated discs pain disappeared and remained so in the follow up. It was not necessary to reoperate in any of these patients and the postoperative follow up was between 2 and 5 years. Conclusions. Herniated disc disease is very infrequent in adolescents and all recovered completely after surgery, and the localization is posterolateral and subligamental (AU)

Use of epsilon aminocaproic acid (EACA) in the preoperative management of ruptured intracranial aneurysms.

The authors report their experience with the use of epsilon aminocaproic acid (EACA) in the preoperative management of a series of patients with ruptured intracranial aneurysms. A similar series of patients was taken as control. They found that EACA is of definite value in preventing recurrent hemorrhage in the preoperative period. The significance of antifibrinolytic therapy in ruptured intracranial aneurysms is discussed.