Intracranial complications of sinogenic and otogenic infections in children: an ESPN survey on their occurrence in the pre-COVID and post-COVID era.

BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.

Ruptured Sylvian arachnoid cysts: an update on a real problem.

PURPOSE: Sylvian arachnoid cysts (SACs) are the most common type of arachnoid cysts and the most prone to undergo a rupture. This event is considered rare but potentially severe. No definite information is available on its occurrence or management. The goal of the present article is to provide an update on the epidemiological, etiological, and clinical aspects and the management of this peculiar clinical condition. METHODS: A comprehensive review of the English literature of the last 40 years on this topic has been realized. Moreover, a personal series of children investigated and treated in the last 20 years is presented. These patients were managed as follows: (1) treatment of the subdural collection; (2) identification of candidates for surgical treatment of the residual cyst (brain MRI, perfusion brain MRI, prolonged invasive ICP monitoring (selected cases), EEG, neuropsychological tests); (3) surgical treatment of the cyst in the patients with pathological perfusion MRI and/or ICP measurement and/or clear neurophysiological and neuropsychological correlations. RESULTS: A total of 446 patients (430 from the literature and 16 from the personal series), mainly children, adolescents, and young adults, have been analyzed leading to the following results: (1) SAC rupture is rare but not negligible (yearly risk of rupture: 0.04%; overall risk up to 10% in children affected by SCAs). Prophylactic surgery in asymptomatic cases is not advisable. (2) The mechanism of rupture is not known but an impact of SAC against the sphenoid wing and/or a direct injury on SAC through a thinned temporal bone, with possible laceration of the cyst wall vessels and/or tear of the bridging veins, can be hypothesized. A head injury is often not reported (may be misdiagnosed). (3) Subdural collection (hygroma > chronic hematoma) is the most common finding followed by intracystic bleeding, extradural hematoma, and other types of bleeding. Signs or symptoms of raised intracranial pressure are the most frequent ones. (4) The complication of the rupture is usually treated in emergency or in the acute period by burr hole or craniotomic evacuation of the subdural collection, although a conservative management is possible in some cases. Following the rupture, the majority of SACs are treated (70%), often at the same time of the complication, but no specific investigations are routinely performed to select candidates. According to our protocol, only 43.7% of SACs needed to be treated. CONCLUSIONS: The "spontaneous" or posttraumatic rupture of SACs is a rare but potentially significant complication followed by a generally good outcome. The course of the cyst is independent from the outcome of the complication, consequently requiring specific investigations for individuating those lesions interfering with CSF dynamics and/or cerebral blood flow.

Consensus statement from the international consensus meeting on post-traumatic cranioplasty.

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.

Post-traumatic hematoma of the "transdural" virtual space. A possible cause of incorrect surgical treatment of epidural hematomas.

The space existing between the external and internal dural layer has been anatomically described as a virtual space; no clear clinical significance has been given to it to date. We hereby describe a case of a child with what was expected to be a purely epidural hematoma, at surgery, was found to be composed of two equally coexistent components, one in the epidural space and the second one between the two dural layers. The recognition of a possible involvement of the transdural space in the case of post-traumatic epidural hematomas is relevant for a correct conclusion of the surgical treatment of epidural hematomas, extensively considered basic practice among neurosurgical procedures.

Single-suture craniosynostosis: is there a correlation between preoperative ophthalmological, neuroradiological, and neurocognitive findings?

BACKGROUND: In spite of literature data stating that children with single-suture craniosynostosis have an increased risk for neuropsychological deficits, no data are present clarifying the potential risk factors. METHODS: All children with non-syndromic single-suture craniosynostosis operated on from January 2014 to January 2017 were enrolled. A comprehensive neurocognitive and neuro-ophthalmological evaluation was performed before surgery and 6 months after surgery. A further neurocognitive evaluation was performed 12 months after surgery. All children had a preoperative CT/MR study. RESULTS: One hundred forty-two patients were enrolled; 87 are affected by sagittal craniosynostosis, 38 by trigonocephaly, and 17 by plagiocephaly. A global neurocognitive impairment was documented in 22/87 children with scaphocephaly, 5/38 children with trigonocephaly, and 6/17 children with anterior plagiocephaly. There was a significant relationship between results of the ophthalmological evaluation, global IQ, and CT findings at diagnosis (r = 0.296, p < 0.001; r =0.187, p 0.05). Though a significant recovery was documented after surgery, a persistence of eye coordination deficits was present at 6 months in 1 out of 3 children with abnormal preoperative exams. A significant correlation was found between pathological CT findings and persistence of below average neuro-ophthalmological and neurocognitive findings 6 months after surgery, as well as between CT findings and neurocognitive scores at the 1 year follow-up (r = 0.411; p < 0.01). CONCLUSION: The presence of neuroradiological abnormalities appears to be related to both ophthalmological and neurocognitive deficits at diagnosis. This relationship is maintained in spite of the surgical treatment in children who show the persistence of ophthalmological and neurocognitive deficits during the follow-up.

Imaging in craniosynostosis: when and what?

PURPOSE: Currently, the interest on craniosynostosis in the clinical practice is raised by their increased frequency and their genetic implications other than by the still existing search of less invasive surgical techniques. These reasons, together with the problem of legal issues, make the need of a definite diagnosis for a crucial problem, even in single-suture craniosynostosis (SSC). Although the diagnosis of craniosynostosis is primarily the result of physical examination, craniometrics measuring, and observation of the skull deformity, the radiological assessment currently plays an important role in the confirmation of the diagnosis, the surgical planning, and even the postoperative follow-up. On the other hand, in infants, the use of radiation or the need of sedation/anesthesia raises the problem to reduce them to minimum to preserve such a delicate category of patient from their adverse effects. METHODS, RESULTS AND CONCLUSIONS: This review aims at summarizing the state of the art of the role of radiology in craniosynostosis, mainly focusing on indications and techniques, to provide an update not only to pediatric neurosurgeons or maxillofacial surgeons but also to all the other specialists involved in their management, like neonatologists, pediatricians, clinical geneticists, and pediatric neurologists.

Bony decompression vs duraplasty for Chiari I malformation: does the eternal dilemma matter?

PURPOSE: The management of Chiari I malformation (CIM) still raises the problem of the optimal surgical treatment, with special regard to the "eternal dilemma" of the posterior fossa bony decompression alone (PFBD) or with duraplasty (PFBDD). The goal of the present review is to update the results (outcome and complications) of both techniques to better understand the correct indication for each of them. METHODS: A review of the literature has been performed, focusing on the articles and the meta-analyses specifically addressing the problem of PFBD vs PFBDD. Also, the personal authors' experience is briefly discussed. RESULTS: PFBD (usually with C1 laminectomy, often with delamination of the external dural layer) is the most commonly used technique in children, especially if syringomyelia is absent. It ensures a high success rate, with > 80% clinical improvement and about 75% reduction of the syringomyelia, and a very low risk of complications, hospital stay, and costs. A certain risk of recurrence is present (2-12%). PFBDD (with autologous tissues or dural substitutes), on the other hand, is mostly used not only in adults but also in children with large syringomyelia. It is burdened by a higher risk of complications (namely, the CSF-related ones), longer hospital stay, and higher costs; however, it warrants a better clinical improvement (> 85%) and a lower risk of reoperation (2-3.5%). Eight meta-analyses of the literature (three on pediatric series and five in adult series) and one prospective study in children, published in the last decade, largely confirm these findings. CONCLUSION: PFBD and PFBDD are different techniques that are indicated for different types of patients. In children, PFBD has been demonstrated to represent the best choice, although some patients may require a more aggressive treatment. Therefore, the success in the management of CIM, with or without syringomyelia, depends on the correct indication to surgery and on a patient-tailored choice rather than on the surgical technique.

Brain DSC MR Perfusion in Children: A Clinical Feasibility Study Using Different Technical Standards of Contrast Administration.

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.

PrPC Governs Susceptibility to Prion Strains in Bank Vole, While Other Host Factors Modulate Strain Features.

Bank vole is a rodent species that shows differential susceptibility to the experimental transmission of different prion strains. In this work, the transmission features of a panel of diverse prions with distinct origins were assayed both in bank vole expressing methionine at codon 109 (Bv109M) and in transgenic mice expressing physiological levels of bank vole PrPC (the BvPrP-Tg407 mouse line). This work is the first systematic comparison of the transmission features of a collection of prion isolates, representing a panel of diverse prion strains, in a transgenic-mouse model and in its natural counterpart. The results showed very similar transmission properties in both the natural species and the transgenic-mouse model, demonstrating the key role of the PrP amino acid sequence in prion transmission susceptibility. However, differences in the PrPSc types propagated by Bv109M and BvPrP-Tg407 suggest that host factors other than PrPC modulate prion strain features. IMPORTANCE: The differential susceptibility of bank voles to prion strains can be modeled in transgenic mice, suggesting that this selective susceptibility is controlled by the vole PrP sequence alone rather than by other species-specific factors. Differences in the phenotypes observed after prion transmissions in bank voles and in the transgenic mice suggest that host factors other than the PrPC sequence may affect the selection of the substrain replicating in the animal model.

Preoperative neurocognitive evaluation as a predictor of brain tumor grading in pediatric patients with supratentorial hemispheric tumors.

OBJECTIVE: The objective of the present study was to retrospectively evaluate the relationship between tumor grading and a selective evaluation of neurocognitive and behavioral functions in children with supratentorial hemispheric brain tumors. METHODS: Children admitted with a diagnosis of supratentorial hemispheric tumors involving the cerebral hemispheres or the thalamus at the Pediatric Neurosurgery Unit of the Catholic University of Rome between January 2008 and January 2014 were considered for the present study. Exclusion criteria were represented by age less than 2 years, severe neurological deficits, seizures, and a metastatic disease. A selective neurocognitive and behavioral workout was used for children aged less and more than 5 years. RESULTS: Global cognitive functions as well as selective neurocognitive and behavioral profiles were found to be significantly worse in children with low-grade tumors, compared with those affected by higher-grades histotypes. Frontal locations for cortical tumors and thalamic lesions were significantly related with worse results, with a clear contribution of dominant vs. nondominant hemisphere involvement and an age higher than 5 years. CONCLUSIONS: Preoperative global and selective neurocognitive evaluation might contribute to the prediction of the tumor aggressiveness. Due to a longer clinical history, more benign tumors more frequently arrive to the diagnosis with a neurocognitive compromise in spite of an apparently mild presence of neurological symptoms and signs.

Cerebellar mutism.

INTRODUCTION: Cerebellar mutism (CM) is defined as a peculiar form of mutism that may complicate the surgical excision of posterior cranial fossa tumor. The incidence is variable in the literature, occurring in up to one third of cases in some series. Commonly occurring peculiar features of CM are delayed onset following surgery, limited duration, and spontaneous recovery usually associated with dysarthria. METHODS: A review has been performed concerning anatomical substrates and circuits actually considered to be involved in the development of cerebellar mutism, as well as risk factors for its development that have been documented in the literature. Attention has also been given to the long-term prognosis and the possibilities of rehabilitation that can be considered in these children, which has been compared with the authors' institutional experience. RESULTS AND CONCLUSIONS: Tumor infiltration of the brainstem seems to represent the most relevant feature related to the development of CM, along with the histological diagnosis of medulloblastoma. On the other hand, hydrocephalus does not represent an independent risk factor. The higher rate of CM in children seems to be related to the higher incidence in children of tumors with malignant histology and brain stem involvement. Surgical technique does not seem to have a definite role; in particular, the use of a telovelar approach as compared to vermian split to reach the fourth ventricle extension of the tumor has not been demonstrated to prevent the development of cerebellar mutism. Concerning long-term prognosis, around one third of the children who develop cerebellar mutism after surgery have a persistent dysarthria, the remaining ones showing a residual phonological impairment. Long-term dysarthric features tend to be more severe and less prone to recovery in children presenting at diagnosis with associated combined procedural memory and defective neurocognitive functions.

Myelomeningocele: the management of the associated hydrocephalus.

BACKGROUND: The pathogenesis of the hydrocephalus associated with myelomeningocele (MMC) has been the subject of an extensive number of studies. The contemporary reduction of the incidence of the Chiari II malformation and of the associated active hydrocephalus after closure of the spinal defect in utero is in line with previous studies suggesting a prominent role of the posterior cranial fossa abnormalities, where even the increased venous pressure might be at least mostly a consequence of the constriction of the posterior cranial fossa structures. Pure absorptive abnormalities however coexist, the main ones documented to be abnormal cisternal spaces and peculiar cerebrospinal fluid chemical features. MATERIALS AND METHODS: We reviewed the pertinent literature concerning the pathogenesis and management of the hydrocephalus associated to MMC. We also reviewed our personal experience in managing the hydrocephalus in such patients through an endoscopic third ventriculostomy. RESULTS AND CONCLUSIONS: The literature review demonstrated an overall reduction in more recent series of children with MMC needing to be treated for the associated hydrocephalus postnatally, questioning the role of the prenatal care of the disease in this context. Less severe conditions and a more conservative neurosurgical attitude have certainly contributed to the reduction of the reported active postnatal hydrocephalus rate. Long-term cognitive evaluation of the children with MMC that we managed with an endoscopic third ventriculocisternostomy (ETV) as primary as well as secondary procedure did not demonstrate significant differences in the outcome compared with non-complicated extrathecally shunted children, favouring ETV as a valuable option in this subset of patients.

Epilepsy surgery of posterior quadrant dysplasia in the first year of life: experience of a single centre with long term follow-up.

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.

[Determination of skeletal sex using discriminant analysis of ulnar measurements]. / Determinazione del sesso scheletrico mediante analisi discriminante di misurazioni ulnari.

Twelve ulnar measurements take on a series of 80 skeletons (40 male, 40 female) of a known Southern Italian population have been used in 9 combinations to produce discriminant functions for skeletal sex determination. The highest percentage of correct sex classification (95%) was obtained by the association of the minimum circumference and the maximal length. Using other four discriminant functions sex is correctly identified in 93.75% of the sample; in addition two of these functions, obtained by the associations of: minimum circumference and distal epiphyseal breadth, height of proximal articular surface, superior breadth and corio-olecranic distance, allowed to have a sex determination even by fragmented ulnae. For each discriminant function proposed, coefficients of discrimination, section points, male and female centroid and the percentage of misclassification are reported. Practical applications of this method will be certainly of aid in sexual identification in case of forensic interest.