A few challenges in mucopolysaccharidosis type I.

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care.

Why fetal neurosurgery?

Introduction to the Special Focus Session on Fetal Neurosurgery.

Neurovascular stents in pediatric population.

Purpose: The purpose of the study is to analyze the results obtained using stents for the treatment of neurovascular diseases in pediatric patients.

Methods: A retrospective study of 6-year period was undertaken evaluating clinical charts and imaging studies of patients treated with stents because of neurovascular diseases.

Results: Nine patients were managed with 10 stents.

Comparison of energy expenditure, body composition, metabolic disorders, and energy intake between obese children with a history of craniopharyngioma and children with multifactorial obesity.

Unlabelled: Craniopharyngioma is a histologically benign brain malformation with a fundamental role in satiety modulation, causing obesity in up to 52% of patients.

Aim: To evaluate cardiovascular risk factors, body composition, resting energy expenditure (REE), and energy intake in craniopharyngioma patients and to compare the data with those from children with multifactorial obesity.

Population: All obese children and adolescents who underwent craniopharyngioma resection and a control group of children with multifactorial obesity in follow-up between May 2012 and April 2013.

Intracranial pial fistulas in pediatric population. Clinical features and treatment modalities.

Purpose: The purpose of the study is to describe the clinical manifestations and treatment modalities of patients having intracranial pial arteriovenous fistulas (PAVFs).

Methods: We retrospectively analyzed the cases of PAVFs from January 2004 to December 2013. Medical charts, diagnostic images, surgical, and endovascular reports were reviewed retrospectively during each of the procedures and follow-up.

Intracranial gliofibroma: a case report and review of the literature.

Gliofibroma is a rare tumor with biphasic morphology, commonly occurring in the first two decades of life. Currently, the tumor is not listed as a distinct entity in the current World Health Organization (WHO) classification of central nervous system tumors. As its biological behavior, histogenesis, and prognostic factors are still debated, the aim of this paper was to describe a case of a gliofibroma and to update the data about these lesions.

Endovascular treatment of post-pharyngitis internal carotid artery pseudoaneurysm with a covered stent in a child: a case report.

Case Report: We report a case of 4-year-old boy patient, who developed after a streptococcal pharyngitis a painful, pulsatile, and growing right-sided mass in the neck. Imaging studies revealed an extracranial right internal carotid artery pseudoaneurysm. The patient was successfully treated with stent-graft deployment.

Simultaneous fronto-orbital advancement and dynamic posterior cranial vault expansion in Apert syndrome.

Craniosynostosis in Apert syndrome is routinely treated by wide frontal and bilateral supraorbital reshaping and posterior cranial decompression. Dynamic cranial vault expansion has proved to be useful in craniofacial surgery, and its use has extended to syndromic patients. Although a controversy remains between conventional osteotomy and application of the spring-mediated technique in surgical treatment of craniosynostosis, there have been several positive clinical reports on expansion techniques for nonsyndromic and syndromic craniosynostosis.

Multiloculated hydrocephalus.

Objective: The study aims to assess the treatment of progressive multiloculated hydrocephalus. In a retrospective study, the authors reviewed their experience with different treatment modalities.

Methods: We have retrospectively evaluated 93 patients with progressive multiloculated hydrocephalus operated between 1988 and 2010.

Neurosurgical vascular malformations in children under 1 year of age.

Purpose: This study aims to analyze the clinical and radiological findings, timing and type of treatment, and outcome in children under 1 year of age that presented with neurosurgical vascular malformations.

Methods: A retrospective review of 23 children under 1 year of age with neurosurgical vascular malformations was performed at a single institution between 1999 and 2009.

Results: The lesions found in this age group were: 10 vein of Galen aneurysmal malformations (VGAMs), 5 arteriovenous malformations (AVM), 2 pial arteriovenous fistulas (AVF; 1 in the brain and 1 in the spinal cord), 2 cavernous malformations, 2 dural sinus malformations (DSMs) in the posterior fossa with negative angiography which after surgery turned out to be embryonal malformations of dural sinuses, 1 sacular aneurysm, and 1 dural arteriovenous fistula (DAVF) that drained toward the vein of Galen.

Cerebral aneurysms in children: are we talking about a single pathological entity?

Purpose: The objective of this article is to highlight the fact that cerebral aneurysms in children are heterogeneous unlike in the adult population.

Material And Methods: This is a retrospective review of 17 children with intracranial aneurysms who were managed at a single institution from 2004 to 2009.

Results: The median age was 12 years (range 10 months-17 years).

Cerebellopontine angle lesions in children.

Background: Cerebellopontine angle (CPA) lesions are more commonly found in adults in which they account for 5-10% of all intracranial tumors. However, they are uncommon in children, with an incidence of only 1%.

Materials And Methods: This is a review of the management of CPA lesions in children admitted to the Hospital Nacional de Pediatría "Profesor Doctor Juan P.

Pinealoblastomas in children.

Introduction: Series of pinealoblastomas (PB) usually comprise small number of cases as this tumor type is extremely rare and occurs mainly in childhood (especially under 9 years of age). Frequently, PB are reported together with others pineal parenchymal tumors (PPT) or pineal tumors, making characterization far from adequate.

Materials And Methods: Our series of CNS pediatric tumors comprises 1,350 cases of whom 16 are PPT, 12 PB, two pineocytomas (PC), and two mixed or transitional tumors (PC/PB).

Radical resection of craniopharyngioma.

Introduction: The best management of craniopharyngioma in children remains a controversial topic among neurosurgeons. The two treatments for craniopharyngioma most commonly discussed in the literature are primary total resection and limited resection followed by radiotherapy. Without ignoring the challenging behavior of these tumors, we strongly believe that the first approach in a child with a craniopharyngioma is to attempt total removal.

Subependymal giant cell astrocytoma in children with tuberous sclerosis.

Methods: Out of 105 patients with tuberous sclerosis (TS) admitted to the Hospital Nacional de Pediatría "Juan P. Garrahan" (Buenos Aires, Argentina), we surgically treated 17 children between January 1988 and December 2000. Two patients were operated on because of epilepsy and 15 patients because of an intraventricular tumor (subependymal giant cell astrocytoma [SGCA]).

Symptomatic supratentorial arachnoid cysts in children.

This study was undertaken to evaluate the clinical and radiologic long-term outcome of symptomatic primary arachnoid cysts in pediatric patients. Thirty-three children, ranging from 2 months to 17 years of age (mean age, 6 years) were treated. Craniotomy and fenestration of the cyst were used for temporal fossa and midline cysts in 24 patients (73%); later, two patients required shunt placement.