The Possible Role of the Superior Sagittal Sinus in Regulating Cerebrospinal Fluid Dynamics among Preterm Infants: A Case Report and a Review of the Literature.

We report the case of a preterm of 27 weeks of gestation who developed posthemorrhagic ventricular dilatation associated to a complete thrombosis of the superior sagittal sinus, for its peculiar interest in clarifying the physiology of the cerebrospinal fluid (CSF) dynamics. The exact CSF volume that must be removed to improve cerebral hemodynamics and outcomes in infants with posthemorrhagic ventricular dilatation is unknown. According to Volpe's studies, a volume of 10 to 15 mL/kg/die of body weight is commonly chosen. The subject we report needed an excessive CSF drainage (up to 32 mL/kg/d), in presence of a functioning external ventricular drain. We review the literature on the topic, and we postulate that the superior sagittal sinus may play an active role in the CSF dynamics of the immature brain (as it happens for the adult brain).

Ultrasonography as first line imaging for the diagnosis of positional plagiocephaly.

BACKGROUND: In the last years, numerous studies evaluated different tools for the diagnosis of positional plagiocephaly (PP). The purpose of this study was to evaluate ultrasonography (US) as a first line screening test of lambdoid sutural patency in child with PP and to compare our results with the literature. METHODS: All consecutive patients who referred to our Institute from January 2016 to October 2017 with the suspicion of PP, were included in the study and performed US examination of the lambdoid sutures. A 3-6-month clinical follow-up was performed by a pediatric neurosurgeon or a pediatrician to confirm the diagnosis of PP. RESULTS: Thirty-five children performed US examination and in all cases the diagnosis of PP was confirmed. No cases of anticipated suture fusion were examined during this period. The concordance between US findings and clinical exam follow-up was 100%. CONCLUSIONS: Ultrasonography of the lambdoid sutures represents an ideal first-line screening test and reliable alternative to other diagnostic techniques for lambdoid sutural patency in child with PP, being radiation free, fast and cheap.

Non-robotized frameless stereotactic magnetic resonance guided laser interstitial thermal therapy for hypothalamic hamartoma.

Various strategies have been proposed for the treatment of gelastic seizures due to hypothalamic hamartomas (HH), advancing from surgical removal techniques toward functional disconnection strategies. One of the most recent procedure is the Magnetic Resonance guided Laser Interstitial Thermal Therapy (MRg-LITT), which has progressively proved to be a safe and effective technique for hamartomas ablation. In this paper, the authors' preliminary experience with the first two patients treated with this technique in Italy is presented, in order to underline the feasibility of a pure non robotized frameless technique (FS MRg-LITT) while confirming the procedure effectiveness on seizure control. Patients undergoing FS MRg-LITT for the treatment of HH related gelastic seizures since January 2020 were included. A two steps procedure was performed by using the neuronavigation system to define the entry point, the trajectories and to assess the accuracy. Visualase Laser Ablation System was then used for the MR guided ablation of the HH. A multidisciplinary (neurosurgeons, epileptologist, neuroradiologist) institutional board evaluated the patients both in the perioperative period and during follow-up. A total number of 2 pediatric patients were described. The mean operative time resulted to be 6 hours while the mean accuracy was 0.4 mm. No perioperative complications were reported. The mean length of stay was 4 days. Lastly, at 1-year follow-up both patients resulted to be seizure free and endocrinological functions were preserved. FS MRg-LITT for the treatment of HH-related epilepsy could represent an effective technique, being able to guarantee adequate level of accuracy and potentially extending the accessibility of MRg-LITT by lowering its costs and simplifying the overall procedure.

Comparison of Qualitative and Quantitative Analyses of MR-Arterial Spin Labeling Perfusion Data for the Assessment of Pediatric Patients with Focal Epilepsies.

The role of MR Arterial-Spin-Labeling Cerebral Blood Flow maps (ASL-CBF) in the assessment of pediatric focal epilepsy is still debated. We aim to compare the Seizure Onset Zone (SOZ) detection rate of three methods of evaluation of ASL-CBF: 1) qualitative visual (qCBF), 2) z-score voxel-based quantitative analysis of index of asymmetry (AI-CBF), and 3) z-score voxel-based cluster analysis of the quantitative difference of patient's CBF from the normative data of an age-matched healthy population (cCBF). Interictal ASL-CBF were acquired in 65 pediatric patients with focal epilepsy: 26 with focal brain lesions and 39 with a normal MRI. All hypoperfusion areas visible in at least 3 contiguous images of qCBF analysis were identified. In the quantitative evaluations, clusters with a significant z-score AI-CBF ≤ −1.64 and areas with a z-score cCBF ≤ −1.64 were considered potentially related to the SOZ. These areas were compared with the SOZ defined by the anatomo-electro-clinical data. In patients with a positive MRI, SOZ was correctly identified in 27% of patients using qCBF, 73% using AI-CBF, and 77% using cCBF. In negative MRI patients, SOZ was identified in 18% of patients using qCBF, in 46% using AI-CBF, and in 64% using cCBF (p < 0.001). Quantitative analyses of ASL-CBF maps increase the detection rate of SOZ compared to the qualitative method, principally in negative MRI patients.

Technical description of a novel device for external ventricular drainage in neonatal and pediatric patients: Results from a single referral center experience.

BACKGROUND: Since external ventricular drainage (EVD) related infections are usually due to skin flora, an extradural intra/extra-cranial accessory device, developed for pediatric patients under three years of age undergoing EVD positioning, is described. The aim of this paper is to provide technical description of this device, underlining the possibility to reduce infective risk and to prevent EVD dislocation. METHODS: Patients undergoing A-D device EVD placement between 1990 and 2017 at authors' institution were retrospectively considered. The device was made of a fully MRI-compatible inert material (Ketron-Peek-1000), composed of two pieces securely fixable to the skull, bridging the catheter directly from the epidural space to the extracranial space without letting it come in contact with the skin. RESULTS: A total number of 350 patients were considered. The mean age was 1.4 years, being the youngest patient a newborn of 25 weeks of gestational age. Mean time of EVD maintenance was 45 days, ranging from 21 to 81 days. 2 cases (0.6%) of EVD related infections were reported, while, pull-out of the ventricular catheter occurred in 3 cases (0.9%). No cases of bone fractures related to the clamp effect provided by A-D device were reported in the series CONCLUSIONS: This device could represent a safe and feasible option to reduce EVD related infections and catheter pull-out in pediatric patients. The encouraging results could strength the aim of the device to allow safer and longer length of CSF drainage. Moreover, the fully MRI-compatible nature and its non-magnetic properties allow to use it with neuronavigation systems.

Magnetic Resonance-Guided Laser Interstitial Thermal Therapy (MR-gLiTT) in Pediatric Epilepsy Surgery: State of the Art and Presentation of Giannina Gaslini Children's Hospital (Genoa, Italy) Series.

Magnetic resonance-guided laser interstitial thermal therapy (MR-gLiTT) is a novel minimally invasive treatment approach for drug-resistant focal epilepsy and brain tumors. Using thermal ablation induced by a laser diode implanted intracranially in a stereotactic manner, the technique is highly effective and safe, reducing the risk associated with more traditional open surgical approaches that could lead to increased neurological morbidity. Indications for MR-gLiTT in pediatric epilepsy surgery include hypothalamic hamartoma, tuberous sclerosis complex, cavernoma-related epilepsy, SEEG-guided seizure onset zone ablation, corpus callosotomy, periventricular nodular heterotopia, mesial temporal lobe epilepsy, and insular epilepsy. We review the available literature on the topic and present our series of patients with drug-resistant epilepsy treated by MR-gLiTT. Our experience, represented by six cases of hypothalamic hamartomas, one case of tuberous sclerosis, and one case of dysembryoplastic neuroepithelial tumor, helps to confirm that MR-gLiTT is a highly safe and effective procedure for several epilepsy conditions in children.

The Role of Muscle Biopsy in Diagnostic Process of Infant Hypotonia: From Clinical Classification to the Genetic Outcome.

The role of muscle biopsy in the diagnostic workup of floppy infants is controversial. Muscle sampling is invasive, and often, results are not specific. The rapid expansion of genetic approach has made the muscle histopathology analysis less crucial. This study aims to assess the role and efficacy of muscle histopathology in the diagnostic algorithm of hypotonia in early infancy through a retrospective analysis of 197 infants who underwent muscle biopsy in their first 18 months of life. Data analysis revealed that 92/197 (46.7%) of muscle biopsies were non-specific (80) or normal (12), not allowing a specific diagnosis. In 41/197 (20.8%) cases, biopsy suggested a metabolic or mitochondrial myopathy, while in 23/197 cases (11.7%), we found evidence of muscular dystrophy. In 19/197 cases (9.7%), histopathology characteristics of a congenital myopathy were reported. In 22/197 cases (11.7%), the histopathological study indicated presence of a neurogenic damage. Overall, 46 diagnoses were then achieved by oriented genetic tests. Muscle biopsy results were consistent with genetic results in 90% of cases. Diagnostic algorithms for the diagnosis of a floppy infant are largely missing. Muscle biopsy alone can lead to a diagnosis, help the clinician in the choice of a genetic test, or even modify a diagnosis made previously.

Hemispheric surgery for severe epilepsy in early childhood: a case series.

Hemispheric surgery is an effective and cost-effective option for hemispheric epilepsy. Data specifically focusing on very early infancy are scant. In our study, we report the results of hemispheric surgery in children under three years of age, along with clinical, neuroradiological and EEG features, from two Italian epilepsy surgery centres. After reviewing our epilepsy surgery databases (2008-2018), we identified 14 patients (seven males) submitted to hemispheric surgery, under three years (range: 2-29 months), with a follow-up of at least 12 months. No deaths occurred, and surgical complications were observed in 3/17 procedures. At final follow-up visit (mean: 30.8 months; range: 12-90), 10/14 patients (71.4%) achieved Engel Class I (eight Class 1A, one Class 1B, and one Class 1C). Antiepileptic drugs were completely discontinued in three and reduced in eight, thus a significant decrease in drug regimen after surgery was achieved in 11/14 patients (78.6%). Before surgery, severe developmental delay was present in 10 patients, moderate in two and mild in two. At the last follow-up visit, the degree of developmental delay changed from severe to moderate in five patients, remained unchanged in six cases (four severe and two moderate), and changed from mild to moderate in two following surgery. In many cases, hemispheric surgery in children under three years is effective in achieving seizure freedom or reducing seizure frequency, with the possibility of simplifying complex drug regimens. Moreover, it appears to be a safe and well tolerated procedure, leading to improvement in cognition and posture.

Multimodal approach in the pre-surgical evaluation of focal epilepsy surgery candidates: how far are we from a non-invasive ESI-based "sourcectomy"?

The management of drug-resistant patients with focal epilepsy is often challenging. Surgery is recognised as a useful and effective treatment option. The identification of the epileptogenic zone relies on the integration of clinical, neurophysiological, and neuroimaging findings. The role of non-invasive functional neuroimaging techniques has been reported to add diagnostic accuracy to first-line evaluations, avoiding invasive presurgical examinations in selected cases. In this view, we report the case of a 16-year-old male suffering from drug-resistant focal epilepsy with episodes rarely evolving to a bilateral tonic-clonic seizure. Conventional 1.5T and 3T MRI were considered uninformative. Based on electro-clinical data, focal cortical dysplasia was suspected. The epileptogenic zone was identified with the integration of further non-invasive functional neuroimaging techniques ([18F]-fluorodeoxyglucose positron emission tomography and arterial spin labelling), where electrical source imaging played the main role. All techniques pointed towards a cortical region, where a 7T brain MRI identified a signal alteration consistent with focal cortical dysplasia. A tailored resection of the lesion located in the inferior frontal sulcus was performed, guided by intraoperative electrocorticography (strip and depth electrodes). Postoperative seizure freedom was achieved. The histopathology confirmed the suspicion of focal cortical dysplasia type IIa. With this case report, we highlight the importance of a multimodal approach in the presurgical evaluation of candidates for epilepsy surgery, which, in selected cases, may allow invasive procedures, such as stereo-EEG, to be avoided in the investigation of the epileptogenic zone. Moreover, we underline the pivotal role of EEG source imaging, especially when focal cortical dysplasia is suspected.

Temporal lobe epilepsy surgery in children and adults: A multicenter study.

OBJECTIVE: To assess seizure and cognitive outcomes and their predictors in children (<16 years at surgery) and adults undergoing temporal lobe epilepsy (TLE) surgery in eight Italian centers. METHODS: This is a retrospective multicenter study. We performed a descriptive analysis and subsequently carried out multivariable mixed-effect models corrected for multiple comparisons. RESULTS: We analyzed data from 511 patients (114 children) and observed significant differences in several clinical features between adults and children. The possibility of achieving Engel class IA outcome and discontinuing antiepileptic drugs (AEDs) at last follow-up (FU) was significantly higher in children (P = .006 and < .0001). However, percentages of children and adults in Engel class I at last FU (mean ± SD, 45.9 ± 17 months in children; 45.9 ± 20.6 months in adults) did not differ significantly. We identified different predictors of seizure outcome in children vs adults and at short- vs long-term FU. The only variables consistently associated with class I outcome over time were postoperative electroencephalography (EEG) in adults (abnormal, improved,odds ratio [OR] = 0.414, P = .023, Q = 0.046 vs normal, at 2-year FU and abnormal, improved, OR = 0.301, P = .001, Q = 0.002 vs normal, at last FU) and the completeness of resection of temporal magnetic resonance (MR) abnormalities other than hippocampal sclerosis in children (OR = 7.93, P = .001, Q = 0.003, at 2-year FU and OR = 45.03, P < .0001, Q < 0.0001, at last FU). Cognitive outcome was best predicted by preoperative performances in either age group. SIGNIFICANCE: Clinical differences between adult and pediatric patients undergoing TLE surgery are reflected in differences in long-term outcomes and predictors of failures. Children are more likely to achieve sustained seizure freedom and withdraw AEDs after TLE surgery. Earlier referral should be encouraged as it can improve surgical outcome.

Listeria meningitis complicated by hydrocephalus in an immunocompetent child: case report and review of the literature.

BACKGROUND: Listeria monocytogenes is a Gram-positive bacteria transmitted to human by animal stools, contaminated water and food. In children, Listeria monocytogenes typically affects newborns and immunocompromised patients often leading to invasive syndromes including sepsis, brain abscesses, meningitis, meningoencephalitis and rhombencephalitis. In healthy and immunocompetent children, Listeria meningitis is rare, but can progress rapidly and may be associated with severe complications (hydrocephalus, ventriculitis, cranial nerves palsy and cerebrospinal abscesses) and high mortality rate. CASE PRESENTATION: We describe a very uncommon case of meningoencephalitis due to Listeria monocytogenes in a 11-month-old immunocompetent girl. Cerebrospinal fluid (CSF) culture was positive on the second day. Antibiotic therapy was promptly started but the disease was complicated by neurological deterioration and decompensated hydrocephalus. The child required a very demanding pediatric and neurosurgical management and was discharged after 40 days without major sequelae. CONCLUSION: Listeria is difficult to isolate and it is not susceptible to first-line treatment for bacterial meningitis with extended-spectrum cephalosporins. Early recognition is therefore crucial for a positive outcome. Pediatricians have to perform close clinical monitoring of these children and be aware of possible complications. A review of all cases of Listeria meningitis complicated by hydrocephalus in healthy children has been performed, to provide an overview on clinical features, treatment options and outcome.

Spontaneous rupture of middle fossa arachnoid cysts: surgical series from a single center pediatric hospital and literature review.

PURPOSE: Arachnoid cysts may present with symptoms deriving from cyst rupture, usually causing intracystic hemorrhage and subdural hematoma or hygroma. Rupture is usually caused by minor trauma, spontaneous rupture is an exceptional event, and 57 cases have been described in literature. We here present and discuss the largest series of spontaneously ruptured middle fossa arachnoid cysts in order to investigate clinical presentation and best treatment available. METHODS: We report a retrospective series of 17 pediatric patients surgically treated for middle fossa arachnoid cyst with signs of cyst rupture without a history of trauma in the previous 90 days. We describe clinical presentation, treatment, and outcome at follow-up discussing our results with a literature review including all reported cases of spontaneous rupture of middle fossa arachnoid cysts. RESULTS: In our experience patients most frequently presented with subdural hygroma, in literature, a chronic hematoma was most frequently reported. Headache is the most reported symptom at presentation. Neurological deficits and consciousness alterations are rare. Surgical treatment may resolve brain compression only or reduce rupture recurrence risk. Conservative treatment has also been proposed. Different treatments are reported and discussed focusing on indications, contraindications, risks, and expected benefits. CONCLUSION: We propose, when safely possible, microsurgical cyst fenestration in skull base cisterns as the treatment of choice for these patients as long as it addresses both immediate decompression and risk of rupture recurrence. We report good outcomes and low incidence of complications from our series with a mean postoperative follow-up of 30 months.

Radiation-Induced Moyamoya Syndrome in Children with Brain Tumors: Case Series and Literature Review.

BACKGROUND: Over the last decades, significant advancements have been achieved in the treatment of pediatric brain tumors as a result of radiation therapy (RT). With the increasing diffusion of this treatment, iatrogenic damage to cerebrovascular tissues contouring the radiation target volume has become the subject of debate, especially radiation-induced moyamoya syndrome (RIMS). METHODS: A systematic literature search was performed on the association between moyamoya vasculopathy and cranial irradiation in children. Large case series of patients with moyamoya were analyzed and clinicoradiologic data were collected reviewing pediatric patients treated with RT for primary brain tumors at our institution. RESULTS: The risk of developing RIMS is higher in younger children, in patients with optic pathway glioma, and in those receiving higher radiation doses. Headache is the most common presenting symptom and cerebral infarction is frequent. The preferred surgical techniques were pial synangiosis and encephaloduroarteriosynangiosis. In our case series, surgical revascularization led to neovascularization, with clinical improvement or stability in all patients. Medical therapy did not significantly affect the clinical course. CONCLUSIONS: Pediatric patients receiving involved field RT for the treatment of brain tumors have an increased risk of developing RIMS. Prompt diagnosis and early surgical revascularization play a pivotal role in decreasing the clinical impact of this complication. The use of new techniques, such as the intensity-modulated RT, and the increasing dose saving for the organs at risk, are essential to prevent iatrogenic vasculopathy. The combination of appropriate medical therapy and surgery will improve patient management and clinical outcome.

Pure Bilateral Lambdoid and Posterior Sagittal Synostosis (Mercedes-Benz Syndrome): Case Report and Literature Review.

BACKGROUND: Bilateral lambdoid and posterior sagittal synostosis is a rarely encountered multisutural craniosynostosis accounting for 0.3%-0.7% of all craniosynostoses. It has been named "Mercedes-Benz Syndrome" (MBS) because of the bone ridges that resemble the Mercedes-Benz logo. The typical head shape is usually described as anterior turricephaly combined with mild brachycephaly. We describe a case of pure MBS without involvement of other sutures. Since we believe that MBS is a different pathology when other sutures are involved, we will discuss our case with a review of the literature, including all reported cases of bilateral lambdoid and posterior sagittal synostosis with no other sutures involved but sagittal and bilateral lambdoid. CASE DESCRIPTION: A 3-month-old female baby came to our attention because of a turrycephalic appearance of the head. Findings of the neurologic examination were unremarkable. Computed tomography scan showed premature fusion of posterior third of sagittal suture and bilateral lambdoid suture. Magnetic resonance imaging did not show relevant intracranial abnormalities. She underwent sagittal decompression extended posteriorly below the lambdoid suture combined with biparietal decompression to obtain expansion of both parieto-frontal bones and posterior fossa. Post-operative course was uneventful. Follow-up at 3 months showed a good aesthetic result, and results of the neurologic examination were unremarkable. CONCLUSIONS: Pure MBS is a rare condition that unlike other multisutural complex craniosynostosis is rarely associated with genetic syndromes, has a low rate of associated intracranial anomalies, and has less need for more skull-remodeling surgery. The choice of the most suitable surgical procedure must be tailored on the patient, considering preoperative head appearance and eventually associated anomalies.

Grisel Syndrome in Pediatric Age: A Single-Center Italian Experience and Review of the Literature.

BACKGROUND: Nontraumatic atlantoaxial subluxation, also known as Grisel syndrome, is a rare disease that usually affects children. The typical presentation is torticollis in patients with a history of surgical operations or airway infections. METHODS: We describe 5 patients with Grisel syndrome, referring to medical care for a torticollis, a few weeks after an airway infection, with no trauma associated. Radiologic confirmation of the diagnosis, with computed tomography and magnetic resonance imaging studies, was carried out. RESULTS: The patients were treated with external immobilization for 3 months, followed by surgical fixation in the case of recurrence after collar removal or inveterate subluxation. We performed a review of the literature to define the best management of this disease. CONCLUSIONS: Management of Grisel syndrome depends on the degree of subluxation basing on the Fielding and Hawkins classification. The initial nonsurgical management consists of close reduction and immobilization. Surgical fixation is indicated in cases of conservative treatment failure.

Burr Holes Revascularization in Three Pediatric Cases of Moyamoya Syndrome: Easy Choice or Insidious Trap? Case Series and Review.

INTRODUCTION: Moyamoya disease is a steno-occlusive cerebrovascular disease of unknown etiology involving the terminal portion of the internal carotid artery and the proximal portions of the anterior and middle cerebral arteries with associated collateral vascular network. When the vascular pattern is associated with a particular condition (e.g., Type 1 neurofibromatosis, Down syndrome), it is defined as moyamoya syndrome (MMS) (or quasi-moyamoya). Among different indirect bypass techniques used to prevent ischemic injury by increasing collateral blood flow to hypoperfused areas of the cortex, multiple burr holes technique is an easy and diffuse indirect revascularization approach in the treatment of moyamoya. DISCUSSION: While the effectiveness in patients with moyamoya disease was demonstrated, its role in MMS remains uncertain. In this study, we describe surgical and diagnostic implications in three pediatric cases of moyamoya sydrome unsuccessfully treated with multiple cranial burr hole technique. A critical review of the literature about the use of the surgical indirect revascularization techniques in pediatric patients was also reported.

Dermoid and Epidermoid Cysts of Scalp: Case Series of 234 Consecutive Patients.

BACKGROUND: Dermoid and epidermoid cysts are among the most frequent lesions of the scalp in the pediatric population. Optimal management of a suspected dermoid or epidermoid cyst in children is debated: Some authors advocate conservative management on the basis of the benign histology of these entities, while others prefer surgical excision. OBJECTIVE: Our objective is to demonstrate that excision of dermoid and epidermoid cysts is a safe procedure and that early surgery is effective in preventing potential complications related to cyst growth. METHODS: We retrospectively collected data on patients who underwent surgery for excision of proven dermoid or epidermoid cysts between January 2006 and October 2017. RESULTS: In 234 patients, 237 cysts were excised. Mean age at presentation was 26.99 ± 32.7 months; 48.7% of patients were operated on between 1 and 3 years of age, and 32.9% were younger than 1 year of age. Cysts were more frequently located in the frontal bone than in occipital and supraorbital regions. In 36.28% of cases there was no significant cranial impingement, while 21.94% of the cysts eroded through a partial thickness of the cranium, 12.23% were in the full thickness of the skull, and 0.84% had epidural extension. We identified 22 intradiploic cysts. Statistical analysis demonstrated significant association between frontal and pterional localization and bone erosion. Neither major complications nor cyst recurrence were observed. CONCLUSIONS: Excision of dermoid and epidermoid cysts is a safe procedure for neurosurgeons dealing with this disease, even in young patients. Early resection is recommended due to the potential adverse effects that may occur if these cysts are left untreated.

Iatrogenic Pseudoaneurysm of Superficial Temporal Artery After Surgery for Scaphocephaly: Case Report and Review of Literature.

BACKGROUND: Iatrogenic pseudoaneurysm of the superficial temporal artery after surgery for craniosynostosis is a complication that has never been described in the pertinent literature. Although reported for other types of surgeries, no case has been described in the pediatric population. CASE DESCRIPTION: We report on a case of pseudoaneurysm of the superficial temporal artery that occurred 9 days after corrective surgery for scaphocephaly. We also describe the management of this complication. CONCLUSION: Pseudoaneurysm is an exceptional complication in surgery for craniosynostosis, but it should be considered in case of swelling in the temporal region.