Child's Nervous System最新文献

Introduction: The management of open neural tube defects (ONTD) has significantly improved with fetal surgery, but many children remain ineligible for fetal therapy. This study assesses the impact of intraoperative neurophysiological monitoring (IONM) during postnatal myelomeningocele (MMC) repair and its potential to optimize functional outcomes.

Patients and methods: Seven newborns with thoracolumbar or lumbar MMC underwent postnatal surgical repair using IONM. Neuromonitoring included motor-evoked potentials (MEP), sensory-evoked potentials (SEP), electromyography (EMG), and bulbocavernosus reflex (BCR). Preoperative neurological assessments were compared with postnatal outcomes at 2 years, along with anatomical levels on MRI and IONM results.

Results: At birth, 6 of 7 newborns showed better functional levels than expected by the anatomical level of the ONTD in fetal MRI. Intraoperative EMG responses were normal in all but one patient, but only 30% of patients had normal MEP and SEP responses. IONM was useful to detect undue mechanical stress during ONTD repair surgery and intraoperative identification of functional nerve roots. Neurological function deteriorated in the early postoperative period but stabilized at a level above the anatomical MRI level in 85% of the patients by 2 years of age.

Conclusion: IONM in postnatal ONTD repair is a safe and valuable tool that has the potential to increase surgical safety by detecting undue mechanical stress to the neural structure. Standardized postnatal management strategies, including the use of IONM, should be refined. Further studies are necessary to evaluate the prognostic value of EMG during postnatal surgery and its potential to result in better long-term outcome by preventing additional intraoperative damage.

Purpose: This retrospective monocentric study aims to describe the characteristics of neurosurgical site infections (n-SSI) in neurooncological pediatric patients. The primary goal was to assess infection rates and identify common pathogens in this population.

Methods: We considered pediatric patients (0-18 years) who underwent neurosurgery for brain tumors at Meyer Children's Hospital in Florence between January 1, 2017, and December 31, 2021. Children with suspected/confirmed n-SSI were included. Data were retrospectively collected from patients' medical records, and covered tumor and surgery type, presence of foreign bodies, microbiological findings, and treatment. Infections were classified into categories according to literature. Statistical analysis was performed using GraphPad Prism. A p value of < 0.05 was considered significant.

Results: Of 352 children undergoing neurosurgery, 43 (12.22%) had suspected/confirmed n-SSI, with a confirmed infection rate of 4%. The most frequent n-SSI was postoperative meningitis (37.2%), followed by wound infections (25.6%). A foreign body was present in 74.4% of cases. The most prevalent pathogens were Staphylococcus aureus (40%) and coagulase-negative staphylococci (33%). Lumbar puncture (LP) performed before antibiotics significantly increased pathogen isolation (p = 0.01). Most patients (95.3%) had fever, and 53.5% had CSF leakage. Antibiotic therapy was administered in all cases, and 65.1% required therapy adjustment. No significant correlation was found between foreign body and clinical symptoms or microbiological positivity.

Conclusion: The study reveals a high rate of n-SSI, emphasizing the importance of early diagnostic measures like LP to improve microbiological diagnosis and optimize antimicrobial treatment. The most frequent pathogen was S. aureus; however, the absence of methicillin-resistant strains is notable. These findings highlight the role of a multidisciplinary approach in managing n-SSI and the potential for n-SSI to delay adjuvant cancer treatments.

Purpose: Intrathecal baclofen (ITB) therapy is a key intervention for managing severe spasticity in pediatric cerebral palsy (CP) patients. However, standard surgical techniques for ITB pump placement pose challenges in low body mass index (BMI) patients, who have limited soft tissue coverage, increasing the risk of complications such as infections and skin erosions. This study compares three techniques-subcutaneous (SC), subfascial (SF), and a novel subfascial with umbilicus detachment (SFUD) approach-specifically aimed at reducing these risks and improving surgical outcomes in this vulnerable population.

Methods: Retrospective cohort study on 54 pediatric CP patients (BMI ≤ 18.5 kg/m²) who underwent initial ITB implantation from January 2004 to July 2018. Patients were divided into SC, SF, and SFUD groups, and outcomes such as surgical time, blood loss, length of hospital stay (LOS), complications, and pump explants were analyzed.

Results: The SFUD group had the highest successful implant rate (93.7%), compared to 79.2% for SF and 57.1% for SC (p < 0.05). SFUD also showed the lowest complication rates, with minimal infections and no skin erosions or pump removals. The SC group experienced the highest complications, including infections and skin erosions. Mean LOS was significantly shorter in the SFUD group.

Conclusion: The SFUD technique provides a safe and effective alternative for ITB pump implantation in CP patients with low BMI, reducing complications and improving implant stability. Further studies are warranted to confirm these findings and support broader clinical adoption.

Purpose: This is a description of the main middle cerebral artery (MCA) anomalies from the neuroradiological and embryological point of view, discussing the practical implications and the association with other vascular diseases.

Methods: Among the patients who underwent digital subtraction angiography (DSA) at our institution in a short time period (from 1 January 2021 to 31 December 2023), we selected three cases with high-quality images, representative for the main MCA anomalies, i.e., accessory, duplicated, and twig-like MCA.

Results: We describe a series of three cases, illustrating the three main embryological anomalies of MCA, including accessory MCA, duplicated MCA, and twig-like MCA. All patients underwent neuroradiological investigations at our institution because of the acute occurrence of neurological symptoms and the findings are presented using computed tomography angiography (CTA), magnetic resonance angiography (MRA), and DSA with a detailed description. These three variants have a very low prevalence and they were reported and described more systematically in the Asian population than in the European population. Both noninvasive and invasive neuroimaging techniques were used for investigating these patients, and catheter angiography still provides the more informative content for precisely imaging the angioarchitecture of these variants and the association with aneurysms on other cerebral arteries. The knowledge of the embryology of these variants is of paramount importance in order to make easier their identification and reporting and to enhance their understanding.

Conclusions: MCA anomalies should be known and carefully checked in order to avoid misdiagnosis with disease and may affect the treatment of acute stroke.

Purpose: Managing hydrocephalus in infants can be very challenging. The most used permanent hydrocephalus treatment in young patients is a ventriculoperitoneal shunt (VPS) placement. Obstructive hydrocephalus in selected young patients can be treated with endoscopic third ventriculostomy (ETV). However, in infants less than 6 months of age, the outcome of both procedures remains to be bothered with complications, revision surgeries and long-term shunt dependency. This retrospective study analyzes the management of hydrocephalus in 54 very young infants with different etiological causes.

Methods: Data was collected retrospectively from a single center university hospital over a 5-year period (2018-2022). All patients under 3 months of age with progressive ventriculomegaly confirmed by cranial ultrasound (cUS), who required neurosurgical intervention, were eligible for this study. Hydrocephalus was treated with serial tapping from a ventricular access device (VAD), placement of ventriculoperitoneal shunts and/or performing a thulium laser-assisted ETV.

Results: Twelve patients benefited sufficiently from a VAD to normalize ventricular volume lastingly. Forty-two patients required permanent treatment (28 underwent a VPS; 14 an ETV if there was obstructive hydrocephalus) at an average age of 2.5 months. The VPS failure rate was 32.1% and the ETV failure rate was 50%. Although not significantly different, patients with failed ETV tended to be younger than patients with successful ETV (p = 0.38). One week before permanent ETV treatment, relatively large ventricular volumes were measured in failed ETV patients, as compared to successful ETVs.

Conclusions: Managing hydrocephalus in very young infants remains challenging regarding surgical strategy, reducing shunt dependency and decreasing current complication rates. In addition to a VPS, an ETV has shown to be a successful treatment option for hydrocephalus in well-selected very young infants. The opportunity to decrease ventricular volume with a VAD could have contributed to the success of an ETV in this young patient group.

The role of connectivity in the function and development of the human brain has been intensely studied over the last two decades. These findings have begun to be translated to the clinical setting, particularly in the context of epilepsy. Determining connectivity in the epileptic brain can be challenging and is even more difficult in the pediatric patient. In pediatric epilepsy, resting-state functional magnetic resonance imaging (rs-fMRI) has emerged as a powerful method for determining connectivity. Resting-state fMRI is a non-invasive method of determining correlated activity (functional connectivity) between brain regions in a task-free manner. This modality is especially useful in the pediatric population as it can be done under sedation and requires minimal cooperation from the patient. Over the last decade, rs-fMRI has been increasingly used and studied in pediatric epilepsy. In this article, we review this recent work and discuss the current state of rs-fMRI in the diagnosis and management of the different pediatric epilepsy syndromes. We first provide an overview of rs-fMRI in practice, including the different methods of analysis. We then describe the connectivity findings in pediatric epilepsy that have been revealed by rs-fMRI and the current state of rs-fMRI use in practice. Finally, we discuss what rs-fMRI has revealed about postoperative changes in connectivity and provide several recommendations for future research.

Purpose: Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder characterized by premature aging. Cerebral hypoperfusion and stroke have been described in HGPS however revascularization techniques have not been reported from a neurosurgical perspective in HGPS patients.

Methods: We present a case of a pediatric patient with HGPS who underwent cerebral bypass along with a review of the literature.

Results: Cerebral hypoperfusion and resulting ischemic strokes have been reported in children with HGPS, as early as 4 years old. Medical treatment, including antiplatelet agents, following standard ischemic stroke guidelines has been commonly reported. There is a paucity of literature regarding surgical management of cerebral hypoperfusion in HGPS patients. As an illustrative case we report a 4-year-old boy with HGPS who presented with acute left ischemic stroke from ipsilateral internal carotid artery stenosis and arteriopathy. He underwent encephaloduroarteriosynangiosis for indirect cerebral artery bypass, with neoangiogenesis on follow up imaging and with good clinical status. Wound healing in this patient became a challenging complication, with multidisciplinary input required for successful resolution.

Conclusion: Children with HGPS can present with cerebral hypoperfusion due to cerebral flow deficits and indirect bypass can be cautiously considered as an option to restore cerebral blood flow. Neoangiogenesis and collateralization can occur in patients with HGPS following indirect bypass, though tissue fragility in this population makes them at high risk for wound healing issues. We discuss preoperative and perioperative considerations, as well as medical and surgical maneuvers undertaken to achieve successful healing.

Intratumoral hemorrhage is a known complication of cerebrospinal fluid (CSF) drainage surgeries, such as ventriculoperitoneal shunts or external ventricular drainage, which are usually associated with upward herniation of the posterior fossa contents. In contrast, this is extremely rare after an endoscopic third ventriculostomy (ETV). This technique should drain the CSF in a more physiological way by limiting the pressure gradient between the infratentorial and supratentorial compartments, thus avoiding upward herniation of the tumor and intratumoral hemorrhage. Only three cases of intratumoral hemorrhage after ETV have been reported in the literature. Here, we describe a 9-year-old boy affected by hydrocephalus secondary to a large posterior fossa tumor who presented intratumoral hemorrhage after ETV. He required urgent hematoma evacuation and tumor removal. Histological and molecular diagnosis was WNT-activated medulloblastoma, a tumor associated with an increased risk of hemorrhage.

A 7-year-old boy presented to the hospital with recurrent nausea and vomiting over 2 weeks. A cranial MRI revealed a large heterogeneous lesion in the posterior fossa extending from the fourth ventricle to the foramen magnum with contact to the brainstem. The lesion showed moderate diffusion restriction and multiple small cystic components. The child underwent gross total resection. The primary histological findings suggested proliferative active tumor without further definition. The extended histological examination 3 weeks later confirmed the diagnosis of astroblastoma. Due to complete resection and full recovery of the patient, watchful waiting with radiological follow-up was recommended. Astroblastoma is an extremely rare tumor especially in the posterior fossa. However, it should be considered as a differential diagnosis in every tumor presenting the discussed radiological and histological features, especially in young aged patients.

Purpose: Pediatric brachial plexus injuries (BPI) are uncommon, representing about 10% of BPIs overall and 0.1% of pediatric trauma cases. These injuries often result from high-energy trauma and are unique to neonatal and adult cases. This study aims to provide a contemporary, large-scale analysis of pediatric BPI mechanisms and management patterns.

Methods: The Trauma Quality Improvement Program (TQIP) database was used to identify pediatric BPI cases from 2017 to 2020. Mechanisms of injury (MOI), concomitant injuries, imaging, and treatment intervention were determined. Pediatric age groups were defined as children (0-9 years), young adolescents (10-14), and older adolescents (15-17).

Results: Of the 746,817 recorded pediatric trauma cases in TQIP, 285 BPI cases were identified (0.038%), with the majority (55%) in older adolescents. Firearm injuries accounted for 23% of cases, surpassing sports (22%) and MVAs (21%) as the leading mechanisms. Children under 10 years were most affected by MVAs (20%), young adolescents by sports (32%), and older adolescents by firearms (30%). Associated injuries included orthopedic (44%), head (40%), and vascular (24%) injuries. Imaging was performed in 82% of cases, with CT (57%) and MRI (50%) being the most common. Immediate BPI repair was rare (4.3%), mainly occurring in cases with concurrent vascular injuries (91%).

Discussion: Pediatric BPIs are increasingly linked to firearms and often present alongside complex polytrauma, requiring specialized care. Immediate repair is rare, typically reserved for cases with vascular injury. These findings emphasize the need for further research to refine management, particularly in high-energy and firearm-related BPIs.