J. Hinojosa

Intraventricular cavernoma in pediatric age

Abstract Introduction. Cavernomas rarely occur in the ventricular system. Only 10 pediatric cases out of 46 well-documented cases have been published. Case report. We report the case of an 11-year-old girl operated on for a voluminous cavernoma at the ventricular trigone which was diagnosed after absence seizures. Conclusion. Interestingly, the case we report showed a hypointense rim on T2-weighted magnetic resonance images which has not been a common finding in the cases previously reported.

Endoscopic-assisted osteotomies for the treatment of craniosynostosis

IntroductionThe development of multidisciplinar units for Craniofacial Surgery has led to better postoperative results and a considerable decrease in morbidity in the treatment of complex craniofacial patients.Materials and methodsStandard correction of craniosynostosis involves calvarial remodeling, often considerable blood losses that need to be replaced and lengthy hospital stay. The use of minimally invasive techniques for the correction of some of these malformations are widespread and allows the surgeon to minimize the incidence of complications by means of a decreased surgical time, blood salvage, and shortening of postoperative hospitalization in comparison to conventional craniofacial techniques.Discussion and conclusionSimple and milder craniosynostosis are best approached by endoscopy-assisted osteotomies and render the best results. Extended procedures other than simple suturectomies have been described for more severe patients. Different osteotomies resembling standard fronto-orbital have been developed for the correction, and the use of postoperative cranial orthesis may improve the final cosmetic appearance. Thus, endoscopic-assisted procedures differ from the simple strategy of single suture resection that rendered insufficient results in the past, and different approaches can be tailored to solve these cases in patients in the setting of a case-to-case bases.

Operative treatment of the anterior synostotic plagiocephaly: analysis of 45 cases.

Abstract An operative series of 45 patients with anterior synostotic plagiocephaly is analyzed. In the cases of six children it was not possible to visualize synostosis of the cranial sutures on plain X-ray films or three-dimensional CT. Primary procedures were performed at an average age of 14 months with an average postoperative follow-up of 47.5 months. Children were operated on either with lateral canthal advancement (5 cases), unilateral orbital advancement with “tongue in groove” (12 cases), or bilateral orbital advancement (28 cases). Excellent results were obtained in 33/45 patients, while 8/45 children required secondary minor revisions. Of the three different surgical techniques, the results achieved by the bilateral approach were considered better than those attained with the unilateral techniques. Osteotomies of the nasal bones are recommended in the cases of older children with severe nasal deviation associated with dislocation of the nasoethmoidal complex.

Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature

INTRODUCTION Primitive neuroectodermal tumors (PNETs) are malign neoplasms of the central nervous system which mainly locate in cerebellum (medulloblastoma). Primary intraspinal PNETs are rare. Within this group, we have found ten cases of purely intramedullary PNETs (IPNETs). In this report, we describe a new IPNET case and review the literature about these infrequent intramedullary tumors. CASE REPORT A 17 month-old boy showed progressive decrease of motion in his lower extremities. Spine magnetic resonance imaging revealed an intramedullary expansive lesion from T3 to T10. A near-total removal was performed. The pathological diagnosis was PNET. Subsequent chemotherapy was recommended. Six months after operation, holocord progression has occurred. CONCLUSION IPNETs are uncommon tumors affecting children and young adults. They are characterized by recurrence, progression or intracranial dissemination. Outcome is dismal: most patients die within two years in spite of surgical resection followed by radiotherapy and chemotherapy.

Hamartoma of the suprasellar cistern in a 5-year-old girl.

Abstract A 5-year-old girl with precocious puberty secondary to a suprasellar hamartoma is presented. Magnetic resonance imaging (MRI) revealed a lesion without attachment to the tuber cinereum or the mamillary bodies. Total resection of the tumour was performed.

Gliomas del tronco encefálico

Resumen El conocimiento de los gliomas de tronco ha sufrido una importante progresion en las dos ultimas decadas, siendo considerados en la actualidad un grupo heterogeneo de lesiones con especial predileccion por la edad infantil, donde alcanzan un 10–20% de los tumores cerebrales. Ademas del tradicional tumor difuso, se han identificado subgrupos biologicamente diferentes, susceptibles de reseccion quirurgica y con mejores expectativas pronosticas, lo que ha dado lugar a numerosas definiciones y clasificaciones. Por otra parte, recientemente se ha prestado atencion a los rasgos especiales que presentan los gliomas de tronco en la edad adulta. Basados en una revision de la literatura existente, describimos los diferentes tipos conocidos de gliomas de tronco, con particular interes sobre las modalidades terapeuticas y las diferencias entre los tumores pediatricos y los del adulto, empleando iconografia propia.

Syringomyelia secondary to posthemorrhagic hydrocephalus in a preterm infant.

Syringomyelia is often associated with hydrocephalus, especially in Chiari malformations, but it has never been described as a complication of posthemorrhagic hydrocephalus after preterm birth. We report on a premature infant who presented this exceptional association. He was born at 29 weeks of gestational age and suffered a grade 3 intraventricular hemorrhage. Progressive ventricular dilatation developed despite repeated lumbar punctures, and a ventricular reservoir had to be inserted for cerebrospinal fluid drainage. Two weeks later he presented a flaccid, areflexic paralysis of his left upper limb. Magnetic resonance imaging disclosed a remarkable tetraventricular hydrocephalus and a cervical hydrosyringomyelia expanding from the C(5) to T(1) segments. After shunt surgery, the cephalic perimeter stabilized, and the infant began to move his arm. On follow-up, a minimal paresis of the left hand persisted. This case highlights an unreported outcome of posthemorrhagic hydrocephalus. In this context, syringomyelia should be included in the differential diagnosis of any infant with who presents segmental signs of acute or progressive onset.

1. Cavernomas intraventriculares. Presentación de un caso y revisión de la literatura

Los cavernomas intraventriculares son lesiones muy infrecuentes, habiendose descrito previamente 45 casos en la literatura. Revisamos la literatura y presentamos el caso de una nina de 5 anos de edad diagnosticada de tumor intraventricular en atrio izquierdo tras presentar 3 episodios de crisis de ausencia con desorientacion posterior. El estudio anatomopatologico informo de cavernoma. No existen datos clinicos ni radiologicos que, prequirurgicamente, nos sirvan para sospechar la naturaleza vascular de esta lesion en esa localizacion.

Hemispheric low-grade gliomas

The common concept of low-grade gliomas as benign tumors that in principle might be cured has found its limit in real life in the frequent involvement of eloquent areas of the brain, the frequent intraoperative evidence of tissue with macroscopic aspects not easily distinguished from the surrounding normal brain, and the difficulties of eventually needed complimentary treatments because of the relative resistance of a not negligible part of lowgrade gliomas to both chemotherapy regimens and radiotherapy. In the last decade, efforts have been therefore directed in three directions. The first is towards improving our preoperative and intraoperative planning through more advanced MR imaging (DTI sequences and functional MR studies), a more extensive use of intraoperative mapping and monitoring in order to reduce the risk of postoperative neurological sequelae, and the attempts to apply the techniques of awake surgery also to the pediatric population. The second significant effort has been made in the field of intraoperative imaging. Neuronavigation based on preoperative imaging has indeed demonstrated its limits for the intradural steps of the surgery for this kind of lesions, especially when located far from the midline. Conventionaland contrast-enhanced ultrasounds have been widely utilized, presenting the advantage to be the tools of contained cost and easy practical application, but the limit of being operator dependent. Intraoperative MR is nowadays increasingly utilized for extending safe removal for low-grade gliomas in the pediatric population as it does not bear the risk of intraoperative radiations, compared with intraoperative CT scan, and its usefulness and reliability. However, the high cost constitutes a significant limit, especially for pediatric neurosurgery centers. The third direction to improve the management of children with low-grade gliomas certainly involves a better understanding of both the genetic and molecular nature of these tumors, in the hope to achieve results similar to that obtained for malignant tumors (e.g., medulloblastomas). Indeed, starting from the knowledge of genetic syndromes associated with lowgrade gliomas, related molecular pathways are increasingly demonstrating their possible involvement as targets for ultraselective individualized treatments. Thanks to the abovementioned efforts and advances, more extensive removals of low-grade gliomas have become nowadays possible not only for tumors developing in eloquent cortical areas but also for deep-sited lesions, e.g., insular and thalamic tumors, to which more conservative approaches were offered for a long time, to preserve the quality of life. Actually, although it is well known that even larger tumor residuals might remain stable for a long time after surgical treatment, the prolonged postoperative observation demonstrates that the extent of tumor resection (EOR), even for low-grade gliomas, is a significantly positive survival factor related to both longer overall survival (OS) and progressionfree survival (PFS). Moreover, it is well stated that the EOR is significantly related to the possibility of epilepsy control in children presenting with seizures. The annual issue of this year is an attempt to give an overview of the achieved knowledge in the abovementioned three directions, keeping also an eye to possible fields of further knowledge on these tumors which can lead to their further better management, such as the possibility to predict the tumor grade based on preoperative selective neurocognitive evaluations up to possible alternatives (e.g., MRI-guided laser tumor ablation ) to open surgery and advanced complimentary treatments. * Gianpiero Tamburrini gianpiero.tamburrini@unicatt.it

Currarino triad with presacral meningocele: A study with intrathecal gadopenthate dimeglumine and surgical management

The Currarino triad is a rare hereditary caudal malformation complex consisting of sacral bone defect, anorectal malformation and presacral mass, which is transmitted in an autosomal dominant pattern with variable phenotypic expression. We report a case of complete Currarino triad with anterior meningocele that was studied with intrathecal gadolinium-diethylene triamine pentaacetic acid magnetic resonance imaging, a novel technique that may be useful for surgical decision and planning in disorders involving the cerebrospinal fluid pathways.