Search

Background Laminotomy has been introduced in surgical practice to reduce complications of laminectomy after surgery of tumors in the spinal canal. However, the posterior ligament complex, which is routinely interrupted to remove the laminoplasty segment and gain access to the spinal canal, has a tendency not to heal and can lead to progressive kyphosis and collapse. Case presentation A 5-month-old boy affected by a thoracolumbar extradural tumor extending along seven spinal levels was operated on. The tumor was exposed and completely resected by a one-piece laminotomy with preservation of the integrity of the posterior tension band at both extremities. After 1-year radiological examination ruled out spinal deformity. Conclusion The technique herein presented, which we named in situ laminotomy, allows to fully preserve the posterior tension band without reducing the exposure of the spinal canal in multilevel tumors. Additionally, the technique makes also the reconstruction of the spine elements very easy and rapid. However, longer follow-up is necessary to prove the effectiveness of this procedure in preventing long-term deformity and instability.

To investigate the current practice of neurosurgeons and their perception of complications related to the securement of external drainage (ED) to the skin.We created a 24-points English language questionnaire on Google Forms covering the five main domains of care. The survey was distributed among members of the European Society for Pediatric Neurosurgery (ESPN) in April 2020.The results were entirely self-reported, without any independent validation. Fifty-one neurosurgeons practising in different centres worldwide participated in this survey. Despite well-known complications and drawbacks, sutures are still the most commonly used method to secure cerebrospinal fluid (CSF) ventricular ED (49 out of 51 respondents) and spinal ED (37 out of 51) to the skin. Perception of the risk of pullout is estimated as1% by 25.5% of the respondents, 1-5% by 39.2%, 5-10% by 17.6% and 10% by 11.8%. Twenty out of fifty-one respondents acknowledge that their method of securement has drawbacks, and 49% believe that it may also affect the risk of infection. Factors eventually affecting the risk of pullout are young age (62.7%), aetiology (25.5%), neurological status (90.2%), occipital exit site (37.3%), inadequate length of the subcutaneous tunnel (58%), the duration of ED (70.6%), and hospital stay in service (84.3%). 39.2% of respondents agree that the paediatric population deserves a different device or technique to secure ED to the skin. 21.6% of respondents underestimate the risk of accidental pullout. 86.3% of respondents have never read about the 'sutureless subcutaneous anchoring device'.Complications associated with the securement method, such as the risk of pullout and infection, are most likely underestimated. More research is needed to implement effective guidelines in this field.

Background The enlargement of the posterior cranial fossa volume is considered one of the main steps of the surgical management of children with multiple sutures craniosynostosis. Different management options have been proposed including fixed expansive craniotomy, free bone flap craniotomy, and distraction osteogenesis. Objectives To review indications to “free bone flap” craniotomy for the posterior fossa expansion, detailing advantages, disadvantages, and complications related to the technique. Results and conclusions A review of the literature shows that “free bone flap” posterior expansion cranioplasty still has a role, particularly in infants with thin and “honeycomb” structure of the bone, allowing to gain adequate intracranial volume increases and to postpone to a more adequate time surgery aimed at anterior cranial fossa expansion.

Background The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic. Methods The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia. Results and conclusions In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.

Purpose In the paediatric age group, the overall degree of evidence regarding decompressive craniectomy (DC) and cranioplasty is low, whereas in adults, randomised controlled trials and prospective multicentre registries are available. To improve the evidence-based treatment of children, a consensus was reached to establish a prospective registry under the auspices of the European Society for Pediatric Neurosurgery (ESPN). Methods This international multicentre prospective registry is aimed at collecting information on the indication, timing, technique and outcome of DC and cranioplasty in children. The registry will enrol patients ≤ 16 years of age at the time of surgery, irrespective of the underlying medical condition. The study design comprises four obligatory entry points as a core dataset, with an unlimited number of further follow-up entry points to allow documentation until adolescence or adulthood. Study centres should commit to complete data entry and long-term follow-up. Results Data collection will be performed via a web-based portal (homepage: www.pedccr.com) in a central anonymised database after local ethics board approval. An ESPN steering committee will monitor the project’s progress, coordinate analyses of data and presentation of results at conferences and in publications on behalf of the study group. Conclusion The registry aims to define predictors for optimal medical care and patient-centred treatment outcomes. The ultimate goal of the registry is to generate results that are so relevant to be directly transferred into clinical practice to enhance treatment protocols.

Objective: The objective of the present study was to selectively evaluate the long-term impact of posterior fossa pilocytic astrocytomas, which are known to be among the most benign forms of paediatric brain tumours on neurocognitive and behavioural functions. Methods: Children that were operated on for a posterior fossa pilocytic astrocytoma in the Pediatric Neurosurgery Department of the Catholic University Medical School were selected according to the following criteria: (a) age &gt, 5 years (in order to have a complete set of neurocognitive evaluations data), (b) ability to perform a complete set of tests before and after surgery, and (c) children that had a regular follow-up up to 10 years from the surgical treatment. Results: Forty-three percent of the children selected for the present study showed a borderline IQ before surgery, which is a result corresponding to those previously reported in the literature for children affected by posterior fossa pilocytic astrocytomas, praxis and visual perception were the selective functions that were more frequently affected. Language performance tests scores were below average in 40% of the cases but tended to improve in terms of expressive and receptive skills even at the 1-year follow-up, the improvements became significant at the 5-year and 10-year follow-ups. Conclusions: Recognising and measuring the short- and long-term effects of cerebellar tumours in children and their treatment are the first step towards improving their clinical course and quality of life. Early interventions should be offered to all of them, with specific attention bestowed on visual-spatial stimulation, speech and occupational therapies in order to act on praxic and visuo-perceptive skills, as well as on emotion and behaviour tracts of the neurocognitive profile, which more commonly tend to persist in the long term.

Background The optimal management of neonatal post-hemorrhagic hydrocephalus (PHH) is still debated, though several treatment options have been proposed. In the last years, ventriculosubgaleal shunt (VSgS) and neuroendosdcopic lavage (NEL) have been proposed to overcome the drawbacks of more traditional options, such as external ventricular drainage and ventricular access device. Methods We retrospectively reviewed neonates affected by PHH treated at our institution since September 2012 to September 2020. Until 2017 patients received VSgS as initial treatment. After the introduction of NEL, this treatment option was offered to patients with large intraventricular clots. After NEL, VSgS was always placed. Primary VSgS was reserved to patients without significant intraventricular clots and critically ill patients that could not be transferred to the operating room and undergo a longer surgery. Results We collected 63 babies (38 males and 25 females) with mean gestational age of 27.8 ± 3.8SD weeks (range 23–38.5 weeks) and mean birthweight of 1199.7 ± 690.6 SD grams (range 500–3320 g). In 6 patients, hemorrhage occurred in the third trimester of gestation, while in the remaining cases hemorrhage complicated prematurity. This group included 37 inborn and 26 outborn babies. Intraventricular hemorrhage was classified as low grade (I–II according to modified Papile grading scale) in 7 cases, while in the remaining cases the grade of hemorrhage was III to IV. Mean age at first neurosurgical procedure was 32.2 ± 3.6SD weeks (range 25.4–40 weeks). Death due to prematurity occurred in 5 patients. First-line treatment was VSgS in 49 patients and NEL in the remaining 14 cases. Mean longevity of VSgS was 30.3 days (range 10–97 days) in patients finally requiring an additional treatment of hydrocephalus. Thirty-two patients required one to three redo VSgS. Interval from initial treatment to permanent shunt ranged from 14 to 312 days (mean 70.9 days). CSF infection was observed in 5 patients (7.9%). Shunt dependency was observed in 51 out of 58 surviving patients, while 7 cases remained shunt-free at the last follow-up. Multiloculated hydrocephalus was observed in 14 cases. Among these, only one patient initially received NEL and was complicated by isolated trapped temporal horn. Conclusions VSgS and NEL are two effective treatment options in the management of PHH. Both procedures should be part of the neurosurgical armamentarium to deal with PHH, since they offer specific advantages in selected patients. A treatment algorithm combining these two options may reduce the infectious risk and the risk of multiloculated hydrocephalus.

Background Due to the lack of high-quality evidence which has hindered the development of evidence-based guidelines, there is a need to provide general guidance on cranioplasty (CP) following traumatic brain injury (TBI), as well as identify areas of ongoing uncertainty via a consensus-based approach. Methods The international consensus meeting on post-traumatic CP was held during the International Conference on Recent Advances in Neurotraumatology (ICRAN), in Naples, Italy, in June 2018. This meeting was endorsed by the Neurotrauma Committee of the World Federation of Neurosurgical Societies (WFNS), the NIHR Global Health Research Group on Neurotrauma, and several other neurotrauma organizations. Discussions and voting were organized around 5 pre-specified themes: (1) indications and technique, (2) materials, (3) timing, (4) hydrocephalus, and (5) paediatric CP. Results The participants discussed published evidence on each topic and proposed consensus statements, which were subject to ratification using anonymous real-time voting. Statements required an agreement threshold of more than 70% for inclusion in the final recommendations. Conclusions This document is the first set of practical consensus-based clinical recommendations on post-traumatic CP, focusing on timing, materials, complications, and surgical procedures. Future research directions are also presented.

Craniosynostosis (CS) is the second most prevalent craniofacial congenital malformation due to the premature fusion of skull sutures. CS care requires surgical treatment of variable complexity, aimed at resolving functional and cosmetic defects resulting from the skull growth constrain. Despite significant innovation in the management of CS, morbidity and mortality still exist. Residual cranial defects represent a potential complication and needdedicated management to drive a targeted bone regeneration while modulating suture ossification. To this aim, existing techniques are rapidly evolving and include the implementation of novel biomaterials, 3D printing and additive manufacturing techniques, and advanced therapies based on tissue engineering. This review aims at providing an exhaustive and up-to-date overview of the strategies in use to correct these congenital defects, focusing on the technological advances in the fields of biomaterials and tissue engineering implemented in pediatric surgical skull reconstruction, i.e., biodegradable bone fixation systems, biomimetic scaffolds, drug delivery systems, and cell-based approaches.

Aim To analyze the current literature on neurocutaneous melanosis (NCM), as well as, our cases, in order to better define the prognosis and the presence of risk factors affecting it, thus, offering better information to the parents. Material and methods Two cases observed at the Pediatric Neurosurgery Unit of the Catholic University Medical School in Rome are described. Both of them had cutaneous stigmata and cerebral MR evidence of intracranial melanin deposits. These two children showed a very different clinical course. Results The present study enlighten the differences among the two cases and review the literature on the subject, with the attempt to understand which are clinical and disease related factors that might influence the prognosis. Conclusion Beside malignant features of cutaneous melanotic lesions, the presence of hydrocephalus at diagnosis and the early appearance of clinical symptoms, when appearing contemporarily, are predicting the rapid progression of the disease and a worse prognosis.

All skeletal bones house osteogenic stem cell niches, in which mesenchymal stromal cells (MSC) provide progenitors for tissue growth and regeneration. They have been widely studied in long bones formed through endochondral ossification. Limited information is available on the composition of the osteogenic niche in flat bones (i.e., skull vault bones) that develop through direct membranous ossification. Craniosynostosis (CS) is a congenital craniofacial defect due to the excessive and premature ossification of skull vault sutures. This study aimed at analysing the expression of GLI1, AXIN2 and THY1 in the context of the human skull vault, using nonsyndromic forms of CS (NCS) as a model to test their functional implication in the aberrant osteogenic process. The expression of selected markers was studied in NCS patients&rsquo, calvarial bone specimens, to assess the in vivo location of cells, and in MSC isolated thereof. The marker expression profile was analysed during in vitro osteogenic differentiation to validate the functional implication. Our results show that GLI1 and AXIN2 are expressed in periosteal and endosteal locations within the osteogenic niche of human calvarial bones. Their expression is higher in MSC isolated from calvarial bones than in those isolated from long bones and tends to decrease upon osteogenic commitment and differentiation. In particular, AXIN2 expression was lower in cells isolated from prematurely fused sutures than in those derived from patent sutures of NCS patients. This suggests that AXIN2 could reasonably represent a marker for the stem cell population that undergoes depletion during the premature ossification process occurring in CS.

Background Lumbosacral lipomas (LLs) may remain asymptomatic or lead to progressive neurological deterioration. However, sudden neurological deterioration is a rare and severe event. Herein, we report rare occurrences of sudden clinical deterioration in two previously asymptomatic children harbouring intradural LLs without dermal sinus tracts or signs of occult dysraphism. A review of the pertinent literature is also included. Case presentation One child exhibited acute deterioration because of an epidural abscess associated with a filar lipoma without a sinus tract (probably caused by haematogenous spreading from a respiratory tract multiple infection), and the other child exhibited acute deterioration because of a very large, holocord syringomyelia-like cyst associated with a small conus lipoma. Both patients were 4 years old. In case #2, a previously undetected, severe tethered cord (conus at the S3-S4 level) was also present. A complete recovery was attained after an urgent surgical operation in both cases (in addition to targeted antibiotic therapy in case #1). All cases of deterioration in the literature were caused by abscess formation in dermal sinus tracts. Conclusions Prophylactic surgery may be indicated even in asymptomatic children that have tethered cord and surgically favourable LLs (small dorsal and filar LLs), especially if the conditions are associated with progressive syringomyelia. Similarly, intradural dermal sinus tracts should be regarded as surgery-indicated, even if the conus is in its normal position and the patient is asymptomatic because there is a consistent risk of severe, infection-related complications. Finally, asymptomatic patients with filar LLs and a normally located conus can be candidates for surgery or an accurate clinical and radiological follow-up.

BACKGROUND AND PURPOSE: Dynamic susceptibility contrast MR perfusion imaging has limited results in children due to difficulties in reproducing technical standards derived from adults. This prospective, multicenter study aimed to determine DSC feasibility and quality in children using custom administration of a standard dose of gadolinium. MATERIALS AND METHODS: Eighty-three consecutive children with brain tumors underwent DSC perfusion with a standard dose of gadobutrol administered by an automated power injector. The location and size of intravenous catheters and gadobutrol volume and flow rates were reported, and local and/or systemic adverse effects were recorded. DSC was qualitatively evaluated by CBV maps and signal intensity–time curves and quantitatively by the percentage of signal drop and full width at half-maximum, and the data were compared with the standards reported for adults. Quantitative data were grouped by flow rate, and differences among groups were assessed by analysis of covariance and tested for statistical significance with a t test. RESULTS: No local or systemic adverse events were recorded independent of catheter location (63 arm, 14 hand, 6 foot), size (24–18 ga), and flow rates (1–5 mL/s). High-quality CBV maps and signal intensity–time curves were achieved in all patients, and quantitative evaluations were equal or superior to those reported for adults. No significant differences ( P ≥ .05) were identified among the higher-flow-rate groups in the quantitative data. CONCLUSIONS: A custom administration of a standard dose of gadobutrol allows safe and high-quality DSC MR perfusion imaging in children.

Nonsyndromic craniosynostosis (NCS) is the premature ossification of skull sutures, without associated clinical features. Mutations in several genes account for a small number of NCS patients; thus, the molecular etiopathogenesis of NCS remains largely unclear. Our study aimed at characterizing the molecular signaling implicated in the aberrant ossification of sutures in NCS patients. Comparative gene expression profiling of NCS patient sutures identified a fused suture-specific signature, including 17 genes involved in primary cilium signaling and assembly. Cells from fused sutures displayed a reduced potential to form primary cilia compared to cells from control patent sutures of the same patient. We identified specific upregulated splice variants of the Bardet Biedl syndrome-associated gene 9 (BBS9), which encodes a structural component of the ciliary BBSome complex. BBS9 expression increased during in vitro osteogenic differentiation of suture-derived mesenchymal cells of NCS patients. Also, Bbs9 expression increased during in vivo ossification of rat sutures. BBS9 functional knockdown affected the expression of primary cilia on patient suture cells and their osteogenic potential. Computational modeling of the upregulated protein isoforms (observed in patients) predicted that their binding affinity within the BBSome may be affected, providing a possible explanation for the aberrant suture ossification in NCS.

Cerebrospinal fluid (CSF) overproduction results from either CSF infection or choroid plexus hypertrophy or tumor, with only a single idiopathic case described so far. We report a unique case of a male infant with Crouzon syndrome who presented with intracranial hypertension, caused by up to 4-fold increase in CSF daily production. Conditions related to CSF overproduction, namely central nervous system infections and choroid plexus hypertrophy or tumor, were ruled out by repeated magnetic resonance imaging and CSF samples. Medical therapy failed to reduce CSF production and the patient underwent several shunting procedures, cranial expansion, and endoscopic coagulation of the choroid plexus. This article thoroughly reviews pertinent literature on CSF production mechanisms and possible therapeutic implications.

Unfortunately in the original publication, the affiliation provided for the author G. Tamburrini was incorrect. The correct affiliation for G. Tamburrini should read as follows.

Cephalohematoma, one of the most common neonatal head injuries, generally undergoes spontaneous resorption. When calcified, it may cause cranial vault distortion and depression of the inner skull layer, although it remains asymptomatic. Surgery, indeed, is usually performed for cosmetic purposes. For these reasons, the long-term effects of calcified cephalohematoma (CC) are widely unknown. The authors report the case of an 11-year-old girl with a persistent calcified CC causing skull deformity and delayed electroencephalography (EEG) anomalies. These anomalies were detected during routine control EEG and were not clinically evident. The young girl underwent surgical removal of the CC for cosmetic purpose. The EEG abnormalities disappeared after surgery, thus reinforcing the hypothesis of a correlation with the brain “compression” resulting from the CC. To the best of the authors' knowledge this is the first time that CC-associated EEG anomalies have been described: even though these anomalies cannot be considered an indication for surgery, they merit late follow-up in case of skull deformity.

Lactococcus lactis is one of the most important microorganisms in the dairy industry and has “generally recognized as safe” (GRAS) status. L. lactis belongs to the group of lactic acid bacteria (LAB) and is encountered in a wide range of environments. Recently, the use of the intramammary infusion of a live culture of LAB has been investigated as a new antibiotic alternative for treating mastitis in dairy ruminants. Controversial results are described in literature regarding its efficacy and safety. In this study we conducted in-depth investigation of the mammary gland immune response induced by intramammary inoculum of a live culture of L. lactis LMG 7930 using the mouse mastitis model. Overnight cultures either of L. lactis (≈ 107 CFU) or of the mastitis pathogens Staphylococcus chromogenes (≈ 105 CFU) or S. aureus (≈ 102 CFU/ml) were injected into the mouse inguinal glands. A double injection, consisting of S. chromogenes first and then L. lactis, was also investigated. Bacterial recovery from the gland and inflammatory cell infiltration were assessed. L. lactis-treated and control glands were analysed for proinflammatory cytokine production. Microbiological results showed that L. lactis was able to survive in the mammary gland 24 h post infection, as were the mastitis pathogens S. chromogenes and S. aureus. L. lactis reduced S. chromogenes survival in the glands and increased its own survival ability by coexisting with the pathogen. Histology showed that L. lactis-treated glands presented variable histological features, ranging from undamaged tissue with no inflammatory cell infiltrate to severe PMN infiltrate with focal areas of tissue damage. S. aureus-treated glands showed the most severe histological grade of inflammation despite the fact that the inoculum size was the smallest. In contrast, most S. chromogenes-treated glands showed normal structures with no infiltration or lesions. Significant increases in IL-1β and TNF-α levels were also found in L. lactis-inoculated glands. The above findings seem to suggest that food-grade L. lactis at a high-inoculum dose such as an overnight culture may elicit a suppurative inflammatory response in the mammary gland, thus becoming a potential mastitis-causing pathogen. Because of the unpredictable potential of L. lactis in acting as a potential mastitis pathogen, this organism cannot be considered a safe treatment for bovine mastitis.

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. 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. 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. 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.

Descending transtentorial herniation (DTH) is a complication of raised pressure in the supratentorial compartment, usually resulting from mass lesion of several etiologies. The authors report an exceptional case of DTH complicating the implant of a CSF shunting device in the trapped fourth ventricle of a 17-year-old boy in whom a second CSF shunting device had been implanted for neonatal posthemorrhagic and postinfectious hydrocephalus. The insidious clinical and radiological presentation of DTH, mimicking a malfunction of the supratentorial shunt, is documented. Ultimately, the treatment consisted of removal of the infratentorial shunt and endoscopic acqueductoplasty with stenting. The absence of supratentorial mass lesion and other described etiologies of DTH prompted the authors to speculate on the hydrodynamic pathogenesis of DTH in the present case.

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.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.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.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%.

Sexual hormones have been related to the growth of meningiomas, also due to the almost constant expression of hormonal receptors by tumoral cells. A case of a woman with previous history of multiple treatment for infertility, harboring a huge meningioma is here described. The tumor was surgically resected and the immunohistochemical examination revealed a high expression of progesterone receptors on tumoral cells surface. A putative role of past progesterone administration in the growth of meningioma has been hypothesized. Particular caution should be paid whenever adopting sexual hormonal therapy, especially for fertility. A radiological examination (ideally MRI) could be advised before starting therapy, in order to rule out any intracranial meningioma.

In general, cranioplasty is viewed as a straightforward surgical procedure, and for many years the complications associated with the procedure have been underestimated. We reviewed our 5-year experience consisting of 218 cranioplasties. Study outcomes focused specifically on the occurrence of complications after cranioplasty. Autologous bone-assisted and prosthetic cranioplasties were considered. Variables described by other authors to be associated with complications were studied, including history of previous cranioplasty, wider craniectomy size, bifrontal craniectomy, and delayed cranioplasty. We also analyzed the influence of material used for craniectomy on the occurrence of complications. The overall complication rate was 19.7%. Nineteen cases of infection (8.7%), 5 cases of postoperative wound dehiscence (2.3%), 6 cases of epidural hemorrhage (2.8%), and 13 cases of cranioplasty dislocation (5.9%) were observed. Bifrontal cranioplasties were more frequently associated with complications (p=0.01; Fisher's exact test) and infection (p0.0001; Fisher's exact test). Postoperative wound dehiscence was more frequently observed with hand-made or custom-made cranioplasties compared with autologous cranioplasties (p=0.02). Early cranioplasty (3 months from craniectomy) was significantly associated with cranioplasty dislocation (p=0.03). Logistical regression analysis showed that the only factor independently associated with complication was the site of cranioplasty (p=0.01). In particular, patients with a bifrontal cranioplasty had a 2-fold increased risk of complication (CI 95 1.1-3.6, p=0.017) and a 2.5-fold increased risk of developing infection (CI 95 1.3-4.9, p=0.009) compared with hemispheric/bihemispheric cranioplasty. Our analysis confirms that cranioplasty is burdened by a significant complication rate. In this context, bifrontal cranioplasty is related to a higher risk of complication and, in particular, infection.

We report on an unusual case of a patient, not affected by neurofibromatosis, harbouring two radiologically spatially contiguous tumours within the same cerebello-pontine angle. Pathological findings were consistent with the diagnosis of two spatially distinct primary tumours, namely a meningioma and a schwannoma. We proposed a classification of tumours occurring at the same location consistent with the different spatial arrangement and histological nature of these conditions. The correct classification of these nosological entities will allow further more accurate evaluations of these cases in order to clarify the pathogenesis, prognosis and best treatment of each one.

A 7-year-old boy underwent resection of a posterior fossa medulloblastoma. Two days later, he developed facial diplegia, left abducens nerve palsy, and mild hypalgesia below the neck. MRI documented 2 dot-like ischemic foci in the pons (figure). We diagnosed the unusual bilateral occurrence of Gasperini syndrome, a lesion of the sixth and seventh cranial nerve nuclei and the lateral spinothalamic tract that produces ipsilateral cranial nerve palsies with contralateral hemisensory deficits.1 We hypothesized a mechanism of postoperative spasm of long circumferential branches of the basilar artery. This mechanism should be considered in cases of postoperative neurologic deficits with delayed onset.2

Posterior quadrant dysplasia (PQD) is a rare variant of cortical dysplasia involving the posterior regions of a single hemisphere. It is always associated with early onset, refractory epilepsy often characterized by a "catastrophic" evolution. The experience on its surgical management during the first year of life is limited to sporadic, isolated cases. Between 2002 and 2005, four children less than one-year-old and affected by drug-resistant epilepsy associated with PQD were admitted to our Institution and underwent surgical treatment. One patient remained seizure-free during all the follow-up (Engel I). The remaining three children showed a recurrence of the seizures, requiring subsequent surgical procedures in two cases. In one case (Engel II), the seizure control has been obtained thanks to pharmacological treatment. The other two patients respectively had only a partial (Engel III) and a less relevant reduction of the number of seizures (Engel IV). Both the epileptic and the neuropsychological outcome of our series were significantly influenced by persistent contralateral interictal anomalies rather than by the timing of the surgical procedure. Unpredictable results should be expected in this kind of patients if there is the detection of contralateral independent epileptiform activities on the EEG at diagnosis. Parents and relatives should be aware of the results' variability, even though a reduction of seizures may be expected, enabling an easier handling of the child's condition.

Craniopharyngioma has long been considered a benign tumor because of its pathological aspect. This primordial view of craniopharyngioma fit with the primitive treatment attempts based on blind resection of the tumor each time it recurred. The limits of this management strategy were proven early by the high morbidity related to the resection and recurrence risk despite radical lesion removal. Nowadays, craniopharyngioma must be considered a complex molecular disease, and a detailed explanation of the mechanisms underlying its aggressive biological and clinical behavior, despite some benign pathological features, would be the first step toward defining the best management of craniopharyngioma. Indeed, advances in the knowledge of the molecular mechanisms at the base of craniopharyngioma oncogenesis will lead to comprehension of the critical checkpoints involved in neoplastic transformation. The final research target will be the definition of new biological agents able to reverse the neoplastic process by acting on these critical checkpoints. This biological approach will lead to a refined therapy combining higher efficacy and safety with lower morbidity. In this paper the authors reveal state-of-the-art comprehension of the molecular biology of craniopharyngioma and the consequent therapeutic implications.