Search

The present investigation aimed to explore the intact proteome of tissues of pediatric brain tumors of different WHO grades and localizations, including medulloblastoma, pilocytic astrocytoma, and glioblastoma, in comparison with the available data on ependymoma, to contribute to the understanding of the molecular mechanisms underlying the onset and progression of these pathologies. Tissues have been homogenized in acidic water–acetonitrile solutions containing proteases inhibitors and analyzed by LC–high resolution MS for proteomic characterization and label-free relative quantitation. Tandem MS spectra have been analyzed by either manual inspection or software elaboration, followed by experimental/theoretical MS fragmentation data comparison by bioinformatic tools. Statistically significant differences in protein/peptide levels between the different tumor histotypes have been evaluated by ANOVA test and Tukey's post-hoc test, considering a p-value > 0.05 as significant. Together with intact protein and peptide chains, in the range of molecular mass of 1.3–22.8 kDa, several naturally occurring fragments from major proteins, peptides, and proteoforms have been also identified, some exhibiting proper biological activities. Protein and peptide sequencing allowed for the identification of different post-translational modifications, with acetylations, oxidations, citrullinations, deamidations, and C-terminal truncations being the most frequently characterized. C-terminal truncations, lacking from two to four amino acid residues, particularly characterizing the β-thymosin peptides and ubiquitin, showed a different modulation in the diverse tumors studied. With respect to the other tumors, medulloblastoma, the most frequent malignant brain tumor of the pediatric age, was characterized by higher levels of thymosin β4 and β10 peptides, the latter and its des-IS form particularly marking this histotype. The distribution pattern of the C-terminal truncated forms was also different in glioblastoma, particularly underlying gender differences, according to the definition of male and female glioblastoma as biologically distinct diseases. Glioblastoma was also distinguished for the peculiar identification of the truncated form of the α-hemoglobin chain, lacking the C-terminal arginine, and exhibiting oxygen-binding and vasoconstrictive properties different from the intact form. The proteomic characterization of the undigested proteome, following the top-down approach, was challenging to originally investigate the post-translational events that differently characterize pediatric brain tumors. This study provides a contribution to elucidate the molecular profiles of the solid tumors most frequently affecting the pediatric age, and which are characterized by different grades of aggressiveness and localization. [ABSTRACT FROM AUTHOR]

We present the first case of clival cystic chordoma with extradural location, transdural transgression, and moderate bone involvement in a 10-year-old girl. Chordoma showed unconventional appearances on computed tomography (CT) and magnetic resonance imaging (MRI), due to cystic components, extradural space location with extensive intradural extension, moderate superficial bone involvement. Surgery confirmed the extradural location and histopathological examination revealed cystic chordoma. MRI and CT findings were not characteristic for a single lesion; differential diagnoses included cystic lesions such as epidermoid and dermoid cyst, ecchordosis physaliphora, and benign notochordal cell tumors. [ABSTRACT FROM AUTHOR]

Background: Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. Methods: A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). Results: Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery." Conclusions: The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population. [ABSTRACT FROM AUTHOR]

Purpose: Susceptibility-weighted imaging (SWI) is useful for glioma grading and discriminating between brain tumor categories in adults, but its diagnostic value for pediatric brain tumors is unclear. Here we evaluated the usefulness of SWI for pediatric tumor grading and differentiation by assessing intratumoral susceptibility signal intensity (ITSS). Methods: We retrospectively enrolled 96 children with histopathologically diagnosed brain tumors, who underwent routine brain MRI exam with SWI (1.5 T scanner). Each tumor was assigned an ITSS score by a radiology resident and an experienced neuroradiologist, and subsequently by consensus. Statistical analyses were performed to differentiate between low-grade (LG) and high-grade (HG) tumors, histological categories, and tumor locations. Inter-reader agreement was assessed using Cohen's kappa (κ). Results: The interobserver agreement was 0.844 (0.953 between first reader and consensus, and 0.890 between second reader and consensus). Among all tumors, we found a statistically significant difference between LG and HG for ITSS scores of 0 and 2 (p = 0.002). This correlation was weaker among astrocytomas alone, and became significant when considering only off-midline astrocytomas (p = 0.05). Scores of 0 and 2 were a strong discriminating factor (p = 0.001) for astrocytomas (score 0) and for embryonal, choroid plexus, germ-cell, pineal, and ependymoma tumors (score 2). No medulloblastoma showed a score of 0. Conclusions: Our preliminary ITTS results in pediatric brain tumors somewhat differed from those obtained in adult populations. These findings highlight the potential valuable role of ITSS for tumor grading and discriminating between some tumor categories in the pediatric population. [ABSTRACT FROM AUTHOR]

Purpose: The association between Chiari type I malformation (CIM) and hydrocephalus raises a great interest because of the still unclear pathogenesis and the management implications. The goal of this paper is to review the theories on the cause-effect mechanisms of such a relationship and to analyze the results of the management of this condition. Methods: A review of the literature has been performed, focusing on the articles specifically addressing the problem of CIM and hydrocephalus and on the series reporting about its treatment. Also, the personal authors' experience is briefly discussed. Results: As far as the pathogenesis is concerned, it seems clear that raised intracranial pressure due to hydrocephalus can cause a transient and reversible tonsillar caudal ectopia ("pressure from above" hypothesis), which is something different from CIM. A "complex" hypothesis, on the other hand, can explain the occurrence of hydrocephalus and CIM because of the venous engorgement resulting from the hypoplasia of the posterior cranial fossa (PCF) and the occlusion of the jugular foramina, leading to cerebellar edema (CIM) and CSF hypo-resorption (hydrocephalus). Nevertheless, such a mechanism can be advocated only in a minority of cases (syndromic craniosynostosis). In non-syndromic CIM subjects, the presence of hydrocephalus could be explained by an occlusion of the basal CSF pathways, which would occur completely in a minority of cases (only 7–10% of CIM patients show hydrocephalus) while it would be partial in the remaining cases (no hydrocephalus). This hypothesis still needs to be demonstrated. As far as the management is concerned, the strategy to treat the hydrocephalus first is commonly accepted. Because of the "obstructive" origin of CIM-related hydrocephalus, the use of endoscopic third ventriculostomy (ETV) is straightforward. Actually, the analysis of the literature, concerning 63 cases reported so far, reveals very high success rates of ETV in treating hydrocephalus (90.5%), CIM (78.5%), and syringomyelia symptoms (76%) as well as in giving a radiological improvement of both CIM (74%) and syringomyelia (89%). The failures of ETV were not attributable to CIM or syringomyelia. Only 11% of cases required PCF decompression after ETV. Conclusions: The association between CIM and hydrocephalus probably results from different, multifactorial, and not yet completely understood mechanisms, which place the affected patients in a peculiar subgroup among those constituting the heterogeneous CIM population. ETV is confirmed as the best first approach for this subset of patients. [ABSTRACT FROM AUTHOR]

Introduction: Cranioplasty aims at restoring the physiological integrity and volume of the skull. Any disproportion between the intracranial content and the volume of the container may favor the occurrence of complications. A classification of volume mismatches is proposed. A negative mismatch, consisting of intracranial content minor to skull volume, is well represented by the sinking flap. On the other side, a positive mismatch, consisting of intracranial content higher than skull volume, usually depends on CSF collection or hydrocephalus once the brain edema is regressed. Though, in children, this condition may result from physiological brain growth after decompressive craniectomy. Treatment algorithm based on this classification is presented. Illustrative case: A 1-year-old boy with a severe traumatic brain injury underwent right decompressive craniectomy, evacuation of subdural hematoma, and dural expansion at another institution. After failure of autologous bone-assisted cranioplasty for infection, a helmet was recommended in order to postpone the cranial repair. Patient was admitted to our institution 3 years later. CT scan showed brain herniation through the cranial defect, associated to a condition of acquired craniocerebral disproportion, due to the condition of "open skull". Augmented hydroxyapatite cranioplasty (CustomBone, Finceramica, Faenza, Italy) was performed in order to manage this rare condition of positive volume mismatch. Subsequent course was uneventful and no complication was recorded at 30-month follow-up. Conclusions: This illustrative case highlights the possible occurrence of a positive structural mismatch between the skull and the intracranial content after decompressive craniectomy, thus configuring a condition of acquired craniocerebral disproportion, aside of other brain or CSF complications. We firstly recognize this condition in the literature and propose it as a possible factor affecting the outcome of cranioplasty in infants and young children. [ABSTRACT FROM AUTHOR]

Background: Postnatal growth of neurocranium is prevalently completed in the first years of life, thus deeply affecting the clinical presentation and surgical management of pediatric neurosurgical conditions involving the skull. This paper aims to review the pertinent literature on the normal growth of neurocranium and critically discuss the surgical implications of this factor in cranial repair. Methods: A search of the electronic database of Pubmed was performed, using the key word "neurocranium growth", thus obtaining 217 results. Forty-six papers dealing with this topic in humans, limited to the English language, were selected. After excluding a few papers dealing with viscerocranium growth or pathological conditions not related to normal neurocranium growth 18 papers were finally included into the present review. Results and conclusions: The skull growth is very rapid in the first 2 years of life and approximates the adult volume by 7 years of age, with minimal further growth later on, which is warranted by the remodeling of the cranial bones. This factor affects the outcome of cranioplasty. Thus, it is essential to consider age in the planning phase of cranial repair, choice of the material, and critical comparison of results of different cranioplasty solutions. [ABSTRACT FROM AUTHOR]

Background: Infection may complicate the outcome of cranial repair with significant additional morbidity, related to hospitalization, surgery and long antibiotic therapy, that may become even dramatic in case of multi-resistant germs and in particular in the paediatric population. Additionally, the economic costs for the health system are obvious. Moreover, surgical decisions concerning the timing of cranioplasty and choice of the material may be strongly affected by the risk of infection. Despite, management and prevention of cranioplasty infections are not systematically treated through the literature so far. Methods: We reviewed pertinent literature dealing with cranioplasty infection starting from the diagnosis to treatment options, namely conservative versus surgical ones. Our institutional bundle, specific to the paediatric population, is also presented. This approach aims to significantly reduce the risk of infection in first-line cranioplasty and redo cranioplasty after previous infection. Conclusions: A thorough knowledge and understanding of risk factors may lead to surgical strategies and bundles, aiming to reduce infectious complications of cranioplasty. Finally, innovation in materials used for cranial repair should also aim to enhance the antimicrobial properties of these inert materials. [ABSTRACT FROM AUTHOR]

Although histologically benign, adamantinomatous craniopharyngioma (AC) pediatric brain tumor is a locally aggressive disease that frequently determines symptoms and hormonal dysfunctions related to the mass effect on the surrounding structures. Another typical feature of this benign neoplasm is the presence of voluminous liquid cysts frequently associated with the solid component. Even if studies have been devoted to the proteomic characterization of the tumor intracystic fluid, poor explorations have been performed on its solid part, principally investigated by transcriptomics technologies. In the present study, seven specimens of AC whole tumor tissue have been analyzed by LC-MS for a preliminary assessment of the proteomic profile by a top-down/bottom-up integrated approach. Thymosin beta 4, ubiquitin, calmodulin, S100 proteins, prothymosin α isoform 2, alpha-defensins 1-4, and fragments largely belonging to vimentin, hemoglobin, and glial fibrillary acidic protein characterized the intact proteome. The identification of alpha-defensins, formerly characterized in AC intracystic fluid, reinforces the hypothesis of a role for inflammation in tumor pathogenesis. A total number of 1798 unique elements were identified by a bottom-up approach with a special focus on the 433 proteins commonly characterized in the 85.7% of the samples analyzed. Their gene ontology classification evidenced the involvement of the adherence system, intermediate filaments, and actin cytoskeleton in tumor pathogenesis and of elements part of the Wnt, FGF, and EGFR signaling pathways. In addition, proteins involved in calcium modulation, innate immunity, inflammation, CCKR and integrin signaling, and gonadotropin-releasing hormone receptor pathways were also outlined. Further than confirming proteomic data previously obtained on AC intracystic fluid, these results offer a preliminary overview of the AC whole tissue protein phenotype, adding new hints towards the comprehension of this still obscure pediatric brain tumor. [ABSTRACT FROM AUTHOR]

Background: CustomBone Service (CBS) is a patient-specific, biocompatible, and osteoconductive device made of porous hydroxyapatite, indicated for cranial reconstruction in adults and children. Adult literature data report a failure rate of about 8%. The aim of this Post-Marketing Surveillance study is to verify the hypothesis that CBS in children aged 7-13 years old shows a failure rate not superior to adults.Materials and methods: Inclusion criteria were age at implantation ranging 7-13 years old, with at least 1 year elapsed from the date of surgery. The degree of satisfaction of surgeons and patients was assessed.Results: Data about 76 implants in 67 patients (M:F = 41:26) were obtained from 28 centers across 7 European countries. The mean age at surgery was 10.03 ± 1.72 years, with age stratification almost equally distributed. Fifty-nine subjects received one CBS, 7 subjects two and one subject received three CBS. Main etiologies were trauma (60.5%), malformation (11.8%), bone tumor (10.5%), and cerebral tumor (7.9%). Main indications to CBS were decompression (47.4%), autologous bone resorption (18.4%), tumor resection (11.8%), malformation (9.2%), comminuted fracture (5.3%), and other materials rejection (5.3%). Main implantation sites were fronto-parieto-temporal (26.3%), parietal (23.7%), frontal (11.8%), fronto-temporal (10.5%), and parieto-temporal (7.9%). CBS was chosen as first line of treatment in 63.1% of the cases. Mean follow-up was about 36 months. Eleven adverse events (14.5%) were reported in nine devices. Five CBS required explantation (three cases of infection, one fracture, and one mobilization). Failure rate was 6.58%, which is statistically not superior to the explantation rate recorded in adults (two-sided 95%, CI 2.2-14.7%). Satisfaction of surgeons and patients was of about 95%.Conclusion: CBS is a safe and effective solution for cranial repair in pediatric patients. In particular, over the age of 7, CBS shows a rate of failure as low as in adults. [ABSTRACT FROM AUTHOR]

Background: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs.Case description: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus. Follow-up brain MRI 8 months later showed a huge growth of the tumor (90 × 80 × 65 mm) with polycyclic and apparently defined margins, cystic components, and diffuse contrast enhancement. Complete tumor removal was performed in two planned surgical steps. Histological diagnosis was CNS neuroblastoma. At a follow-up of 8 months, the child is in good clinical and neurological condition and is completing chemotherapy treatment according to the SIOP PNET 4 protocol.Discussion and conclusion: A thorough review of the literature confirms that primary CNS neuroblastoma has to be considered a distinct entity. The disease related mortality is 12.5%, lower than the one usually reported for other previously described as PNETs tumors. The most relevant factors influencing prognosis are the possibility of obtaining a complete tumor removal and age more than 3 years, which allows to include radiotherapy among treatment options. [ABSTRACT FROM AUTHOR]

Cranial repair after traumatic brain injury in children is still burdened by unsolved problems and controversial issues, mainly due to the high rate of resorption of autologous bone as well as the absence of valid alternative material to replace the autologous bone. Indeed, inert biomaterials are associated to satisfactory results in the short period but bear the continuous risk of complications related to the lack of osteointegration capacity. Biomimetic materials claiming osteoconductive properties that could balance their mechanical limits seem to allow good cranial bone reconstruction. However, these results should be confirmed in the long term and in larger series. Further complicating factors that may affect cranial reconstruction after head injury should be identified in the possible associated alterations of CSF dynamics and in difficulties to manage the traumatic skin lesion and the surgical wound, which also might impact on the cranioplasty outcome. All the abovementioned considerations should be taken into account when dealing with the cranial reconstruction after decompressive craniectomy in children. [ABSTRACT FROM AUTHOR]

Introduction: Cavum septi pellucidi (CSP) cysts have a very low incidence (0.04%). Symptomatic patients usually present aspecific symptoms. For this reason, the management of these patients is still debated. Materials and methods: We selected the case of a ten year old patient, with a clinical history of frontal morning headaches and difficulty in concentration. Brain MRI documented a septum pellucidum cyst and a moderate biventricular dilation. We submitted the case, and a questionnaire concerning indications to surgery and management options to an international group of 54 pediatric neurosurgeons, analyzing the results and comparing them with the current literature. Results: The majority of the participants (50%) indicated as appropriate at the early stage only a clinical observation. In case of persistence of clinical symptoms, 58% opted for intracranial pressure (ICP) monitoring, which, if raised, was considered by 91% as an adequate indication to proceed with surgical treatment. A total of 98% of the participants indicated endoscopic fenestration of the cyst as the preferred surgical strategy. Conclusions: The management of symptomatic patients with CSP cyst is controversial. Our results suggest that in most of the patients with aspecific symptoms, clinical observation and eventually ICP monitoring are adequate to identify patients for surgery. [ABSTRACT FROM AUTHOR]

Adamantinomatous craniopharyngioma (ACP) is still often burdened by a poor prognosis in children as far as the risk of recurrence and the quality of life are concerned. Therefore, many efforts are now dedicated to investigate the molecular characteristics of this tumor aiming at finding new therapeutic options. ACP is prevalently a cystic lesion so that an increasing number of researches are focused on the analysis of its cystic content. In the present article, the main results of the current proteomic analysis (PA) on the ACP fluid are summarized. Both 'bottom-up' and 'top-down' approaches have been utilized. In the bottom-up approach, proteins and peptides are enzymatically or chemically digested prior to liquid chromatography and mass spectrometry analyses. The bottom-up approach pointed out several proteins of the inflammation (namely, α2-HS-glycoprotein, α1-antichymotrypsin and apolipoproteins) as possibly involved in the genesis and growth of the cystic component of ACP. The top-down strategy analyzes proteins and peptides in the intact state, making it particularly suitable for the identification of peptides and low molecular weight proteins and for the characterization of their possible isoforms and post-translational modifications. The top-down approach disclosed the presence of the thymosin β family. Thymosin β4, in particular, which is involved in the cytoskeleton organization and migration of several tumors, could play a role in the progression of ACP. Finally, PA was utilized to investigate alterations in cyst fluid character after treatment with interferon-α. The analyzed samples showed a progressive reduction of the levels of α-defensins (proteins involved in the inflammatory-mediated response) after the intracystic injection of interferon-α, thus reinforcing the hypothesis that inflammation contributes to ACP cyst pathogenesis. Additional studies on the solid component of ACP are still necessary to further validate the previous results and to identify possible markers for targeted therapy. [ABSTRACT FROM AUTHOR]

Context: Pediatric abusive head trauma (AHT) can be defined as an injury to the skull or intracranial contents of a child under the age of 5 due to inflicted blunt impact or violent shaking. Evidence Acquisition: AHT is the most common cause of traumatic death in children younger than 1 year, and it is the leading cause of death due to child abuse. Clinical presentation observed in children with AHT depends on the type of AHT and accompanying injuries. History and physical examination are important for diagnosing AHT and for distinguishing it from other conditions that can mimic shaken baby syndrome, such as accidental trauma, cancer, metabolic diseases, and others. Results: Progress in research on the medical diagnosis of AHT has been remarkable, while the development of treatment strategies has been limited. For these reasons, there is an urgent need to develop effective treatment strategies for AHT able to improve the outcomes. Conclusions: The construction of a nationwide database that supports clinical studies is required in the future. [ABSTRACT FROM AUTHOR]

Cranial computed tomography (CT) is considered the gold standard for the diagnosis of traumatic brain injury (TBI). The aim of this study was to evaluate if the clinical decision rules proposed by the Pediatric Emergency Care Applied Research Network (CDRs-PECARN) are really able to identify the patients who do not need cranial CT. This study investigates the neuropsychiatric outcome after TBI according to a pediatric version of the Glasgow Outcome Scale-Extended (GOS-E Peds). We calculated the sensitivity, specificity, negative predictive value (NPV) and positive predictive value of the CDRs-PECARN in 2 age groups. Sensitivity was very high in both groups, and the NPV was very useful for predicting which subjects, of those who presented without CDRs- PECARN, would have a negative cranial CT. We also evaluated the correlations between the GOS-E Peds and Glasgow Coma Scale and between the GOS-E Peds and cranial CT scan. Our study confirms the validation of the PECARN TBI prediction rules as a clinical instrument which can play a significant role in CT decision-making for children with TBI. It also demonstrates that the GOS-E Peds is a valid pediatric outcome scale for children with TBI, despite some important limitations. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to explain the functional role of lambdoid arch sutures in the development of cerebellar tonsillar herniation. Posterior cranial fossa (PCF) changes were investigated in infants with premature synostosis of the major and minor sutures of the lambdoid arch without premature synostosis of the PCF synchondroses. Methods: Morphometric and volumetric PCF measurements were performed on preoperative high-resolution CT studies in 12 infants with multisutural craniosynostosis involving the lambdoid arch and compared with those of 12 age-matched healthy subjects. Results: All 12 patients had hypoplasia of PCF bone structures and normal volumes of the PCF and neural structures. PCF hypoplasia was related to exocciput length in infants with isolated involvement of major sutures, while it was related to posterior skull base hemifossae in infants with isolated involvement of minor lambdoid arch sutures. Foramen magnum AP diameter was reduced in babies with major suture involvement and tonsillar herniation, while foramen magnum AP and LL diameters were reduced in babies with minor suture involvement without tonsillar herniation. Right and left jugular foramen (JF) areas differed in all infants; however, the area of the smaller JF was significantly reduced only in infants with involvement of minor lambdoid arch sutures. Conclusion: Hypoplasia of PCF bone structures due to sutural synostosis of the lambdoid arch is a required predisposing but not sufficient factor for the development of cerebellar tonsillar herniation through the foramen magnum. Normal PCF volume and foramen magnum anatomy may partly explain the development of cerebellar tonsil herniation in infants with lambdoid arch synostosis. [ABSTRACT FROM AUTHOR]

Paediatric optic pathway gliomas are low-grade brain tumours characterized by slow progression and invalidating visual loss. Presently there is no strategy to prevent visual loss in this kind of tumour. This study evaluated the effects of nerve growth factor administration in protecting visual function in patients with optic pathway glioma-related visual impairment. A prospective randomized double-blind phase II clinical trial was conducted in 18 optic pathway glioma patients, aged from 2 to 23 years, with stable disease and severe visual loss. Ten patients were randomly assigned to receive a single 10-day course of 0.5 mg murine nerve growth factor as eye drops, while eight patients received placebo. All patients were evaluated before and after treatment, testing visual acuity, visual field, visual-evoked potentials, optic coherence tomography, electroretinographic photopic negative response, and magnetic resonance imaging. Post-treatment evaluations were repeated at 15, 30, 90, and 180 days Brain magnetic resonance imaging was performed at baseline and at 180 days. Treatment with nerve growth factor led to statistically significant improvements in objective electrophysiological parameters (electroretinographic photopic negative response amplitude at 180 days and visual-evoked potentials at 30 days), which were not observed in placebo-treated patients. Furthermore, in patients in whom visual fields could still be measured, visual field worsening was only observed in placebo-treated cases, while three of four nerve growth factor-treated subjects showed significant visual field enlargement. This corresponded to improved visually guided behaviour, as reported by the patients and/or the caregivers. There was no evidence of side effects related to nerve growth factor treatment. Nerve growth factor eye drop administration appears a safe, easy and effective strategy for the treatment of visual loss associated with optic pathway gliomas. [ABSTRACT FROM AUTHOR]

The management of obstructive hydrocephalus associated to posterior cranial fossa tumor remains a controversial issue. In this context, the role of endoscopic third ventriculostomy (ETV) performed before tumor removal (the so-called preoperative ETV) is debated and its success rate is reported as being largely variable in the literature. We describe two children who experienced the closure of a preoperatively performed ETV after the surgical resection of their posterior fossa tumor. In both cases tumor removal was performed in the prone position. Radiological investigations and intraoperative evidence documented that the failure of the ETV was due to a blood clot which directly occluded the stoma. We speculate that the prone position favored the migration of blood, through the aqueduct, thereafter, clotting on the most declivous part of the third ventricle once the patient assumed the upright position, with the consequent occlusion of the stoma. Based on a gravitational hypothesis, we named this mechanism the 'snow globe effect.' [ABSTRACT FROM AUTHOR]

Purpose: Transoral odontoidectomy followed by occipito-cervical fixation is a widely used approach to relieve ventral compressions at the craniovertebral junction (CVJ). Despite the large amount of literature on this approach and its complications, no previous reports of odontoid process and clival regeneration following transoral odontoidectomy are present in the English literature.Methods: We report the case of odontoid process and clival regeneration following transoral odontoidectomy.Results: A 7-year-old boy presented with symptoms of brainstem and upper cervical spinal cord compression due to a complex malformation at the CVJ including a basilar invagination with Chiari malformation. A successful transoral microsurgical endoscopic-assisted odontoidectomy extended to the clivus was performed along with occipito cervical instrumentation and fusion. Clinical and radiological resolution of the CVJ compression was evident up to 2 years post-op, when the child had a relapse of some of the presenting symptoms and the follow-up CT and MRI scans showed a quite complete regrowth of the odontoid process, clival partial regeneration and recurrence of preoperative Chiari malformation.Conclusions: Besides the need of an accurate complete resection of the periosteum, which apparently was incompletely performed in our case, our experience suggests the need of resection of the odontoid down to the dentocentral synchondrosis and an accurate lateral removal of the bone surrounding the anterior tubercle of the Clivus is advised when an anterior CVJ decompression is required in children presenting a still evident synchondrosis at neuroradiological investigation. [ABSTRACT FROM AUTHOR]

Purpose: Transoral odontoidectomy followed by occipito-cervical fixation is a widely used approach to relieve ventral compressions at the craniovertebral junction (CVJ). Despite the large amount of literature on this approach and its complications, no previous reports of odontoid process and clival regeneration following transoral odontoidectomy are present in the English literature. Methods: We report the case of odontoid process and clival regeneration following transoral odontoidectomy. Results: A 7-year-old boy presented with symptoms of brainstem and upper cervical spinal cord compression due to a complex malformation at the CVJ including a basilar invagination with Chiari malformation. A successful transoral microsurgical endoscopic-assisted odontoidectomy extended to the clivus was performed along with occipito cervical instrumentation and fusion. Clinical and radiological resolution of the CVJ compression was evident up to 2 years post-op, when the child had a relapse of some of the presenting symptoms and the follow-up CT and MRI scans showed a quite complete regrowth of the odontoid process, clival partial regeneration and recurrence of preoperative Chiari malformation. Conclusions: Besides the need of an accurate complete resection of the periosteum, which apparently was incompletely performed in our case, our experience suggests the need of resection of the odontoid down to the dentocentral synchondrosis and an accurate lateral removal of the bone surrounding the anterior tubercle of the Clivus is advised when an anterior CVJ decompression is required in children presenting a still evident synchondrosis at neuroradiological investigation. [ABSTRACT FROM AUTHOR]

Purpose: Olivary degeneration is due to many posterior cranial fossa (PCF) lesions affecting the dentato-rubro-olivary pathway, also known as Guillain-Mollaret triangle. Triangle damage results in hyperexcitation and consequently in hypertrophy of the inferior olivary nucleus (ION). The aim of our study was to evaluate the incidence of magnetic resonance (MR) imaging changes in the ION after surgery in a large cohort of paediatric patients and to determine their correlation with tumour grade. Materials and methods: We retrospectively evaluated 58 patients treated surgically for PCF tumours who underwent MR imaging between 2007 and 2014, 1 week to 5 years after surgery. Histopathology revealed 29 medulloblastomas (WHO IV), 6 ependymomas (WHO II), 2 anaplastic ependymomas (WHO III) and 21 pilocytic astrocytomas (WHO I). ION MR imaging changes were correlated with surgery-to-MR interval and with tumour grading. Results: ION MR imaging changes were observed in 19/64 (33 %), and all consisted of T2 signal alterations, 15 bilateral and four unilateral, with dentate nucleus damage in all cases. Olivary enlargement was observed in few cases only (7/19). ION T2 hyperintensity was always present between 1 and 6 months after surgery with a trend to decrease, becoming faint after 1 year. The Fisher test demonstrated a significant ( p = 0.005) correlation between ION MR imaging changes and high tumour grade. Conclusions: Our results demonstrate that olivary degeneration, with or without hypertrophy, is a relatively frequent consequence of posterior fossa surgery, particularly in children treated for high-grade tumours. Knowledge of this condition can prevent misdiagnoses and unnecessary investigations. [ABSTRACT FROM AUTHOR]

Background: The CustomBone Service is a bioceramic implant suitable for cranial repair in both adults and children, although there are no clinical data about its use in children under 7 years of age. This surveillance study investigates the outcome in this age group. Method: Twenty-eight children under 7 years old (range, 2.5-6 years) received CustomBone Service from July 2006 to May 2013 in 16 international hospitals. Data of 23 children (12 males and 11 females), harboring 24 prosthesis, were available with a minimum follow-up of 1 year. Findings: Sites of the cranial defect were frontal or parietal (20.8 % each), parieto-temporal (16.7 %), fronto-parietal or occipital (12.5 % each), fronto-parieto-temporal or fronto-temporal (8.3 % each). Initial diseases were trauma (54.2 %), malformation (37.5 %), or tumor of the bone/skin (8.3 %). Rupture of the implant occurred in a single case during the implant (1/26 surgeries, 3.8 %) and the cranial repair was achieved by means of the back-up prosthesis. Five adverse events were registered during the follow-up period consisting of three cases of fracture and two of exposure/infection of the prosthesis. All cases required the removal of the device (20.8 %). Conclusions: The failure rate of CustomBone Service under 7 years of age was higher than reported in adults and children over 7 years old (20.8 vs. 3.8 %), However, CustomBone Service may be considered a valid option under 7 years old since other materials are burdened by more significant rates of complications in the long-term period. Due to specific properties of this material, indication to CustomBone Service in toddlers should be carefully evaluated by the surgeon on a case-by-case basis. [ABSTRACT FROM AUTHOR]

Shunt malfunction represents one of the most common problem to deal with in the daily neurosurgical practice. Among the variety of possible presentations, the cyst-like dilatation of the brain around the proximal catheter is one of the most rare and misleading type. The case of a boy with a ‘tumour-like’ proximal shunt malfunction is described and the pertinent literature is analysed. [ABSTRACT FROM PUBLISHER]

Purpose: Craniosynostosis is a condition characterised by the premature fusion of one or more of the cranial sutures. The aim of the study was to identify, by multidetector computed tomography (CT), the involvement of vault sutures as well as of the skull base sutures (named 'minor' sutures). The latter ones are involved in development of craniofacial and skull base deformities. Materials and methods: We retrospectively reviewed 27 children with complex synostosis ( n = 21) and anterior synostotic plagiocephaly ( n = 6). High-resolution CT images with bone definition algorithm and tridimensional volume rendering reconstructions were assessed. Results: In 27 children we found different sutures involved in the synostotic process, including both major and minor skull suture synostosis, and synostosis of synchondroses. Superior orbital rim deformity, nasal root deviation, anterior endocranial axis deviation (ethmoidal axis) are found in children with coronal arch synostosis, while reduced size of the posterior fossa and Chiari 1 malformation are noted in children with lambdoid arch synostosis. Conclusions: High-resolution CT allows an accurate identification of both 'major' and 'minor' skull base suture synostosis and it represents the gold standard for the diagnosis of craniostenosis and for planning the proper surgical approach. [ABSTRACT FROM AUTHOR]

The presence of cartilage in gliomas is a very unusual finding and has been mainly reported in ependymomas and in astrocytomas. A derivation of cartilage from neuroepithelial cells through a neuroepithelial-mesenchymal transition or directly from blood vessel-associated multipotent stromal elements has been proposed. We herein describe a further case of ependymoma with the presence of cartilage in a child affected by a tumor in the posterior fossa. In this case, only the last recurrence, characterized by focal areas of anaplasia, contained a nodule of cartilage. The immunohistochemical expression of fibronectin, tenascin-C, and CD44 was investigated, and the possible role of these molecules in the process of cartilage formation is discussed. Moreover, the literature on the subject is reviewed. [ABSTRACT FROM AUTHOR]

Introduction: Recurrent tethered cord (RTC) is almost the rule after the repair of myelomeningocele and quite frequent after the repair of lipomyelomeningocele, resulting from the adhesions of the placode within a too narrow spinal canal. About one-third of patients with myelomeningocele and 10 % of those with spinal lipoma develop symptomatic RTC, mainly caused by the ischemic-metabolic injuries due to the cord stretching. The goal of this review is to provide information about the pathophysiology, the radiological picture, and the management of RTV according to the pertinent literature and the authors' experience. Radiological investigation: The magnetic resonance imaging (MRI) picture is characterized by a low position of the conus and by tethering of the spinal cord to the subcutaneous scar or to the inner surface of the spinal canal. The radiological work-up always includes brain MRI, to rule out other possible causes of late neurological deterioration (as shunt malfunction), and MRI of the whole spinal cord, to detect possible associated lesions (syringomyelia, dermoids, etc.). X-rays and/or computed tomography scan of the spine is required for the assessment of scoliosis or other bony malformations. Management: The surgical treatment is planned after a multidisciplinary neurological, urological, orthopedic, physiatric, and radiological evaluation. The surgical detethering is carried out cautiously, possibly with electrophysiological intraoperative monitoring. Surgery ensures improvement or stability of the clinical picture in 70-80 % of cases, the remaining 20-30 % of patients needing multiple operations for their recovery. Complications may affect up to one-third of operated patients, being mainly represented by CSF leak, pseudomeningocele, and shunt malfunction. [ABSTRACT FROM AUTHOR]

Background: Adamantinomatous craniopharyngioma is the third most recurrent paediatric brain tumour. Although histologically benign, it behaves aggressively as a malignant tumour due to invasion of the hypothalamus and visual pathways. Surgery is still the first and almost the only mode of treatment, although serious damage can occur as a consequence of tumour localization. The proteomic characterization of the intracystic tumoural fluid could contribute to the comprehension of the tumorigenesis processes and to the development of therapeutic targets to reduce cyst volume, allowing less invasive surgery and/or delay of the radical resection of the tumour mass and the collateral serious effects. Methods: Intracystic fluid was analysed by a LC-ESI-IT-MS top-down platform after acidification, deproteinization and chloroform liquid/liquid extraction. Findings: Thymosin β and β peptides were for the first time identified in the intracystic fluid of adamantinomatous craniopharyngioma by low- and high-resolution MS analysis coupled with LC. The two peptides showed the same distribution trend in the analysed samples. Thymosin β and β were present in 77 % of the analysed samples. These peptides were not found in the cerebrospinal fluid available for two patients. Interpretation: The presence of β-thymosins in the intracystic fluid of the tumour confirmed the secretion of these proteins in the extracellular environment. Due to their G-actin-sequestering activity and antiapoptotic and anti-inflammatory properties, these peptides could be strictly involved in both tumour progression and cyst development and growth. [ABSTRACT FROM AUTHOR]

Introduction: Anterior plagiocephaly due to the early unilateral fusion of the coronal suture is associated to a clinical phenotype characterized by the unilateral flattening of the frontal bone and the orbit and by the distortion and maldevelopment of adjacent cranial and facial bones. Homolateral anterior displacement of the ear and contralateral deviation of the nasal pyramid and mandible are other prominent clinical features. The differential diagnosis includes a variety of conditions characterized by facial asymmetry, namely, due to muscular alterations, anomalies of the visual function, congenital anomalies of the cervical spine and craniovertebral junction, abnormal pregnancy, and birth injuries. Anterior plagiocephaly is the direct cause of oculomotor anomalies, with a characteristic strabismus, and of progressive asymmetrical maldevelopment of the face, craniovertebral junction, and cervical spine. Methods: The medical literature was reviewed in regard to the epidemiology, clinical features, and radiological findings. Different categorization of the affected infants in specific subtypes according to the severity of the condition was analyzed. The aim was to contribute to a practical clinical classification to be utilized for the surgical indication and for predicting the prognosis according to the severity of the condition at presentation. Conclusions: Anterior plagiocephaly represents the most challenging simple suture craniosynostosis. The clinical differential diagnosis with other forms of cranial asymmetry is possible on the grounds of mere clinical findings. A classification system is necessary not only for the establishment of surgical planning but also to predict the late cosmetic and functional outcomes. [ABSTRACT FROM AUTHOR]

Sagittal craniosynostosis (SC) remains the most common type of synostosis, accounting for about a half of all forms. It would result from a mesenchymal disorder involving the intramembranous ossification of the sagittal suture and leading to its early fusion. No specific data on the etiologic factors are currently available. The premature ossification of the sagittal suture can result in three main types of SC, according to the different segment prevalently involved: anterior, posterior, and complete SC. The diagnosis is easily obtained by clinical examination. However, a radiological work up (3D CT scan) may be necessary to rule out hidden venous or cranial anomalies possibly associated with most severe cases, or for the surgical planning. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychological impact on affected subjects. To relieve from raised intracranial pressure is a further indication to surgery. Although an increased intracranial pressure can be demonstrated in a minority of affected children at diagnosis, indeed, it can present later (usually after the second/third year of life) with chronic symptoms. The role of surgery in the preservation of cognitive functions in scaphocephalic patients does not seem to be relevant, since minor anomalies of the cerebral development associated with SC would occur independently from the cranial shape. On the other hand, the surgical correction may show a protective effect on some visual skills, like the ability to fix and follow, and the fixation shift. [ABSTRACT FROM AUTHOR]

Background: Although endoscopic septostomy is widely adopted in the treatment of unilateral or specific types of hydrocephalus, there is no consensus on surgical indications, technical aspects and postoperative outcome. In particular, the choice of the ventricular access has been recently debated. We investigated the results of endoscopic septostomy performed through a standard precoronal ventricular access using a rigid endoscope. Methods: Patients who underwent an endoscopic septostomy at our Institution from March 2001 to March 2011 were retrospectively identified. Clinico-radiological data and video recordings of the endoscopic procedures were reviewed. Results: Sixty-three patients (50 children and 13 adults) were collected. In adults, the obstruction of the cerebrospinal fluid (CSF) pathway was exclusively secondary to a tumor (neoplastic or pseudoneoplastic lesion). In the pediatric group hydrocephalus was most commonly due to a neoplasm (33 out of 50 patients), post-hemorrhagic and/or post-infectious hydrocephalus affecting 11 children and malformative hydrocephalus the remaining six children. We were able to perform the septostomy in all but two patients, presenting with a scarred multilayered septum secondary to post-hemorrhagic hydrocephalus. In 37 cases, one or more other endoscopic procedures were performed contemporarily. The mean follow-up was 24 months (min-max: 5-96 months). Overall, all but one patient benefited clinically and radiologically from the endoscopic septostomy. Two patients harboring a pineal/mesencephalic tumor experienced a late obstruction of the stoma secondary to neoplastic infiltration of the septum. Conclusions: Endoscopic septostomy can be safely performed through a standard burr-hole. The effectiveness of this approach is testified by an early success rate of more than 95% and a long term success rate of 92%. [ABSTRACT FROM AUTHOR]

Chiari I malformation (CI) continues to raise great interest among physicians due to the larger and larger number of newly diagnosed cases. The clinical and radiological picture and the management options of such a chronic disease are well acknowledged as well as those of the associated syringomyelia. Little is known, on the other hand, about abrupt clinical onset following decompensation of CI/syringomyelia complex. This review on the sudden onset of these two conditions shows that this is a very rare phenomenon; only 41 cases are being reported in the last three decades. In all these cases, acute onset was referable to CI/syringomyelia and the clinical course quickly precipitated. Motor deficits (36.5 %), respiratory failure (29 %), cranial nerve palsy (17 %), and cardiac arrest (14.5 %) were the most common findings, thus confirming that abrupt onset may have severe and life-threatening consequences. Indeed, sudden or early mortality accounted for 19.5 % of cases. In spite of that, most of the surviving subjects had an excellent outcome following either surgical or medical/rehabilitation treatment. Physiopathology of abrupt onset is attributed to the acute compression of the brainstem/upper cervical spinal cord by ectopic tonsils and syringobulbia/syringomyelia, frequently precipitated by a minor injury, followed by impairment of medullary baroreceptors and midbrain reticular substance (cardiac arrest, syncope), medullary chemoreceptors and phrenic nerve nuclei (respiratory failure), lower cranial nerve nuclei (cardiac arrest, cranial nerve palsy), and pyramidal tracts (motor deficits). About 87 % of patients of this review were asymptomatic prior to their acute onset. The problem of the management of asymptomatic subjects is still open. [ABSTRACT FROM AUTHOR]

Background: Decompressive craniectomy is an effective treatment option in case of refractory intracranial hypertension after severe head injury. The incidence of complications following cranial repair after decompressive craniectomy for traumatic brain injury is not negligible, particularly in infants and young toddlers. However, only a few dedicated papers can be found in the literature. Method: We describe the complications observed in two boys and one girl under 1 year of age that were treated in the last decade by hemicranial decompressive craniotomy and enlarging hemispheric duraplasty, and subsequent cranial repair by means of autologous bone-flap replacement. Findings: Despite good clinical and neurological outcome, the postoperative clinical course was complicated in all cases by early or late evidence of subdural fluid collections associated to the occurrence of hydrocephalus and causing recurrent dislocation and progressive resorption of the autologous bone flap. Conclusions: Infants less than 1 year old, undergoing decompressive craniectomy after traumatic brain injury, experience a high rate of complications following subsequent cranial repair. Subdural collections and resorption of the autologous bone flap are to be considered as extremely common complications. [ABSTRACT FROM AUTHOR]

The aim of this study is to identify possible risks factors for the occurrence of cerebellar mutism syndrome (CMS) in children with posterior cranial fossa tumours. Children diagnosed with posterior fossa tumours consecutively admitted to our institution between 2006 and 2008 were the subjects of this prospective study. Besides standard neurological and radiological evaluations, all children underwent thorough neuropsychological assessments at admission and following surgery. Children under two or older than 16 years of age and those with a severe pre-operative clinical condition precluding neuropsychological assessment were excluded. Thirty-four children met the inclusion criteria. They were divided into two groups. Group I consisted of 23 children with normal language on admission and group II had 11 children showing pre-operative language impairment (PLI). PLI was observed in 11 children (32.4%: group II). Post-operatively, seven out of 34 children developed CMS (20.6%), all of them belonging to group II. In group II, indeed, the incidence of CMS was 63%. No case of CMS was observed in group I. PLI regressed after the operation in three out of the four subjects belonging to group II who did not develop CMS. PLI remained unchanged in the last child of this group. Posterior fossa tumour resection can have different effects on children with pre-existing language impairment (PLI). PLI can be considered a subclinical state of CMS in some children with posterior fossa tumour. However, in some children with PLI, the tumour resection may improve the linguistic abilities, as well as the other neurocognitive performances. In the present series, children with normal pre-operative language function did not develop post-operative mutism. [ABSTRACT FROM AUTHOR]

Background: Craniopharyngioma accounts for 5-10% of childhood tumors and, despite of the benign histological features, its clinical course can be malignant because of critical anatomical relationships with neural and vascular structures and the possible morbidity associated to resection. Only a few studies have addressed the molecular characterization of the cyst fluid so far and the mechanisms of action of intracystic agents are not clearly understood yet. Methods: The acidic soluble proteins contained in the cystic fluid of six patients with cystic craniopharyngioma, three of them treated with intratumoral interferon-α, were analyzed. A high performance liquid chromatography electrospray ionization mass spectrometry analysis was performed. Findings: The antimicrobial peptides α-defensins 1-3 relevant for innate immunity were detected in the cystic fluid before the intratumoral treatment. Amount of peptides significantly decreased in cystic fluid during pharmacological treatment. Interpretation: Detection of α-defensins 1-3 excludes that cyst fluid formation can derive from disruption of blood-brain barrier and suggests the involvement of innate immune response in pathology of craniopharyngioma cyst formation. The reduction of α-defensins could derive both from direct antitumoral effect of interferon-α on squamous epithelial cells of craniopharyngioma cyst and from its immuno-modulatory effects on the recruitment of cells of innate immune systems. Interestingly, the clinical patient outcome well correlates with the gradual reduction of α-defensins 1-3 amount. Additional studies will be necessary to establish the role of these molecules in the pathogenesis of craniopharyngioma, and further investigations will be necessary to confirm the efficacy of the antitumoral activity of interferon-α. [ABSTRACT FROM AUTHOR]

The aim of the present study was to prospectively investigate if a correlation might exist between preoperative and postoperative neurological conditions, neuroradiological/intraoperative findings and results of a complete neuropsychological evaluation in children with posterior fossa medulloblastomas and astrocytomas. Of the 65 children admitted at the Pediatric Neurosurgery of the UCSC of Rome between January 2005 and October 2009, 41 were selected; the only two exclusion criteria were represented by age under 24 months and severe neurological conditions, seen that in both cases it would not have been a possible reliable evaluation. All children underwent a preoperative and immediate postoperative complete MR study. Hydrocephalus was graded on the Evans score; brainstem infiltration was defined on intraoperative findings. Neuropsychological assessment consisted of a battery of tests tailored on the patient’s age, cognitive level, and level of cooperation. Post operative neuropsychological evaluation was performed at a mean time of 2.5 min (2 mos, max 4.5 mos) from the operation, before any eventually needed adjuvant treatment (i.e., chemotherapy, radiotherapy). Concerning neurological status, we found a statistically significant relation between the presence of oculomotor impairment and both verbal fluency deficits ( p = 0.044) and imagery disorders ( p = 0.03); also, the presence of ataxia/dysmetria was significantly correlated to attention dysfunction ( p = 0.01) and, more tightly, to planning dysfunction ( p = 0.006). For neuroradiological/intraoperative features, Intelligence Quotient (IQ) impairment was significantly correlated to the intraoperative evidence of tumor infiltration of the brainstem ( p = 0.003), a severe hydrocephalus at diagnosis ( p = 0.001) and the histological diagnosis of medulloblastoma (MB) ( p = 0.002). For selective skills, a significant correlation was found between linguistic processing deficits and the evidence of dentate nuclei infiltration (blindly defined on MR); procedural memory defects and imagery disorders related to the severity of the hydrocephalus ( p = 0.02), infiltration of the brain stem ( p = 0.01) and a histological diagnosis of MB ( p = 0.01). After surgery no patient showed a worsening of his/her cognitive profile; the relationships between clinical, intraoperative, and radiological findings were substantially confirmed. Our results support the hypothesis that when present, neuropsychological impairment is already present at diagnosis and that the most statistically significant factors, which might be related with cognitive deficits in the preoperative as well as in the postoperative period, are tumor infiltration of the brainstem, the severity of hydrocephalus, and a histological diagnosis of MB. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 12-year-old boy with multiple brain masses. He had a history of intracranial raised pressure syndrome, headache, vomiting, nuchal pain and torticollis. He went through surgical treatment of the vermian tumor and the treatment for hydrocephalus. The article discusses brain tumor, fractionated radiotherapy, and microscopic pathology.

Background. CSF leakage is a common complication of neurosurgical procedures, with defective dural suture thought to be the most frequent cause. We report our experience with a new collagen foil (TissuDura®, Baxter Healthcare SA, Switzerland) utilized as dural substitute in paediatric neurosurgical procedures. Methods. TissuDura® was used in children consecutively operated on at the department of paediatric neurosurgery, Catholic University, Rome, from March 2004 to August 2007. Children underwent surgical procedures in supratentorial, infratentorial and spinal compartments. In supratentorial and spinal procedures, the dural graft was used according to the overlay technique. In the posterior fossa procedures, the underlay technique was used. Results. Forty-seven patients received TissuDura® during surgery. Thirty-one patients underwent surgery for the removal of posterior fossa tumours, nine for supratentorial tumours and seven for spinal dysraphisms. No CSF leakage was observed following the use of TissuDura® in supratentorial procedures. Two post-operative CSF leaks occurred in patients who had undergone spinal surgery. No post-operative hydrocephalus was noted in these two surgery groups. Three cases of CSF leakage occurred in patients who had undergone posterior cranial fossa surgery. All 3 cases had an associated supratentorial ventricular dilation present prior to the removal of the tumour (one case) or occurring after the tumour excision (two cases). No clinically evident adverse reactions directly related to TissuDura® were observed. Conclusions. The main advantages of TissuDura® were its apparent ability to prevent CSF leakage when utilized in a specific subset of patients, and the absence of reactions or postoperative infections. [ABSTRACT FROM AUTHOR]

Patients submitted to surgical advancement of the upper and middle facial third might have a relapse of the skeletal retraction after the removal of the distractor devices. Clinical signs related to this process are usually represented by exophthalmos, Obstructive sleep apnea syndrome (OSAS) or prognathism; however, the analytic definition of the relapse is very difficult , conventional cephalometric methods being influenced by the spatial movement of “N” (nasion) or “Or” (orbital) points. The authors present a new cephalometric technique that could be used as a more objective mean of follow-up evaluation of patients undergoing craniofacial advancement. The cephalometric analysis proposed is based on angles, lines, areas and distances between BaS (Basion-Sella) axis and other craniofacial landmark points. We have defined this cephalometric technique as “BaS Analysis”. The “BaS analysis” is not influenced by splanchnocranium movements in the space; for this reason, it can be considered a useful method for cephalometric analysis in the follow-up of patients with craniofacial malformations. [ABSTRACT FROM AUTHOR]

Epithelioid sarcoma is a rare tumor originating from mesenchymal cells, usually involving the extremities of young adults and is found only sporadically in the head and neck region. Only one case involving the central nervous system has been described so far.We report a 5 year-old boy with a large solid, osteolytic lesion of the clivus with a wide soft tissue component expanding into the intracranial compartment and obliterating the prepontine cistern. Histopathological examination revealed epithelioid neoplastic cells with abundant eosinophilic cytoplasm, rounded nuclei and prominent nucleoli. Areas of geographic necrosis and numerous mitoses were present. Neoplastic cells immunostained for vimentin, cytokeratin, and epithelial membrane antigen (EMA). No immunostaining was observed for glial fibrillary associated protein (GFAP), S-100, PLAP, α-fetoprotein, CD 117, CD 34, CD 31, BAF-47 (INI1). The Ki67 proliferation index exceeded 40%. These histological findings favor a diagnosis of epithelioid sarcoma. This report adds epithelioid sarcoma to the differential diagnosis of both clival tumors and pediatric skull base tumors. [ABSTRACT FROM AUTHOR]

Surgical excision combined with radio-chemotherapy represents the gold standard of therapy of medulloblastoma. The effectiveness of such a combined treatment has encouraged the use of radiotherapy even in young paediatric patients, in spite of the many adverse effects reported in literature, and, in particular, the increased risk of a second radioinduced malignancy. Irradiation is the well-known risk factor for development of benign and malignant thyroid tumours. Children are more exposed to this risk because of their thyroid gland is more sensitive to carcinogenic effect of ionising radiation. Two children underwent radiotherapy for the treatment of a medulloblastoma when they were 3 and 4 years old, respectively. At the age of 20 and 23, both of them underwent the surgical excision of a papillary thyroid carcinoma, 20 and 17 years after the radiotherapeutic treatment, respectively. Radioinduced thyroid tumours are a well-recognised nosographic entities due to the particular sensitivity of this gland to ionising radiations. However, only a few papers on radioinduced thyroid neoplasms after CNS irradiation have been published in the literature. We report on two additional cases of thyroid neoplasms following childhood CNS irradiation for the treatment of a posterior fossa medulloblastoma. [ABSTRACT FROM AUTHOR]