Child's Nervous System最新文献

Introduction: Choroid plexus cysts (CPCs) are rare, benign neuroepithelial cysts that can cause obstructive hydrocephalus, although most are asymptomatic. Symptomatic CPCs are uncommon, and hydrocephalus from these cysts, especially intermittent in nature, is rarely reported. Diagnosing CPC-induced intermittent hydrocephalus can be challenging due to the cysts' CSF-like content and thin walls, often requiring advanced MRI sequences for detection.

Case report: We present the case of a previously healthy 6-month-old male who developed intermittent triventricular hydrocephalus, presenting with projectile vomiting, lethargy, a bulging fontanel, and limited upward gaze. CT imaging revealed ventricular enlargement, and MRI revealed a thin membrane and a suspected choroid plexus cyst located within the choroid plexus of the anterior roof of the third ventricle, which is consistent with the dynamic obstruction of cerebrospinal fluid flow. After initial symptom resolution, the patient experienced recurrence within 24 h. Bilateral neuroendoscopic surgery was performed, involving cyst fenestration, shrinkage, and preventive endoscopic third ventriculostomy (ETV). Pathology confirmed a choroid plexus cyst. Postoperative imaging showed resolution of hydrocephalus and disappearance of the cyst. At follow-ups over 18 months, the patient remained symptom-free with no recurrence.

Discussion: This case highlights the challenges of diagnosing and managing intermittent hydrocephalus caused by a septated choroid plexus cyst. Neuroendoscopy offers a minimally invasive treatment for obstructive hydrocephalus, particularly with complex cystic lesions. Our case required a bilateral approach due to the cyst's septation, which posed unique surgical challenges. A literature review revealed similar favorable outcomes with endoscopic techniques, but our case stands out due to the rare presentation of a septated CPC.

Conclusion: This report demonstrates the effectiveness of bilateral neuroendoscopic surgery in managing intermittent hydrocephalus caused by a septated choroid plexus cyst. The case underscores the importance of individualized surgical strategies and advanced imaging techniques in managing complex cases of hydrocephalus.

Purpose: There are currently no specialized pediatric neurosurgeons in Pakistan. The extent to which this impacts the outcomes of children undergoing surgery for neurological conditions in the country is unclear. We aimed to investigate whether outcomes of brain tumor resections in children at our high-volume center in Pakistan were comparable to those performed by pediatric neurosurgeons in a large, validated US surgical database.

Methods: A multi-center collaborative collected clinical data on supratentorial and infratentorial craniotomy procedures (SC and IC, respectively) for malignant brain tumors in children < 18 years at a single center in Pakistan from 2015 to 2022. Similarly, the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) 2021 dataset was queried to extract a comparable cohort of patients. Rates of adverse outcomes and quality metrics were compared between the groups using appropriate statistical tests.

Results: We collected data on 105 Pakistan patients and 570 NSQIP-P patients. Patient demographics were similar for both groups. A total of 350 and 325 children underwent SC and IC, respectively. Postoperatively, children in the Pakistan cohort had worse neurological outcomes, including higher rates of postoperative seizures (SC, p < 0.001; IC, p = 0.003) and focal neurological deficits (SC, p = 0.003; IC, p < 0.001). Furthermore, Pakistani children undergoing SC had higher rates of postoperative mortality (p = 0.002), surgical site infections (p = 0.015), and deep wound infections (p = 0.027), while those undergoing IC had higher rates of unplanned intubations (p < 0.001) and prolonged postoperative mechanical ventilation > 48 h (p = 0.004) compared to their US counterparts captured in NSQIP-P data.

Conclusion: Despite the availability of neuroimaging, cancer therapeutics, and intensive care at our center, children undergoing brain tumor resections had worse outcomes than their US counterparts. There is likely a need for specialized pediatric neurosurgical health services to improve outcomes of children undergoing complex neurosurgical procedures in Pakistan.

Background and purpose: Achondroplasia, a common form of skeletal dysplasia, can be associated with cervical spine compression and cerebrospinal fluid (CSF) flow compromise, potentially leading to neurological complications. Accurate assessment of the cervical spine is essential for identifying children at increased risk of neurological injury. However, concerns regarding the safety of dynamic MRI under anesthesia in young children have limited its use. This study evaluates the safety of dynamic MRI under anesthesia in pediatric patients with achondroplasia, utilizing the largest dataset reported to date.

Materials and methods: In this retrospective study, we reviewed the medical records of 81 patients with achondroplasia who underwent a total of 124 flexion-extension MRIs under anesthesia. All imaging procedures were performed by MR technologists and anesthesiologists without direct supervision by a neurosurgeon or radiologist. Data reviewed included anesthesia type, neurological examinations by a senior pediatric neurosurgeon before and after imaging, surgical intervention, follow-up MRIs, and the presence of CSF obstruction at the craniocervical junction.

Results: A total of 81 patient charts were reviewed (mean age, 2.03 ± 2 years; age range, 1 month to 6 years). Of the 124 flexion-extension MRIs, 113 were performed under general anesthesia, and 11 under sedation alone. Foramen magnum stenosis with CSF flow compromise was documented in 38 cases (46%). No adverse events, neurological deficits, or anesthesia-related complications were documented. Neurological examinations conducted by the senior author, a pediatric neurosurgeon, before and after imaging remained stable across all cases.

Conclusion: Flexion-extension MRI did not result in adverse outcomes in this cohort of pediatric patients with achondroplasia. While these findings support the dynamic MRI's safety in appropriate settings, further studies are needed to validate these results and explore its broader application.

Background: Intraoperative neuromonitoring (IONM) is utilized in many neurosurgical procedures. It is often employed when operating in close proximity to eloquent regions as it can delineate such areas from the targeted resection. IONM can additionally provide surgeon assurance and confidence in chosen intraoperative maneuvers. However, there is no clear consensus regarding whether it provides significant value in pediatric corpus callosotomy. This study reviews the current literature to evaluate intraoperative utility, prognostic value, and medicolegal considerations of IONM in pediatric corpus callosotomy.

Methods: An extensive literature review was performed through PubMed, OVID Medline, SCOPUS, and Embase. Reference lists were manually screened to identify additional applicable articles. Studies were evaluated qualitatively for inclusion and reported descriptively.

Results: The search strategy yielded 1523 articles. Following duplicate removal, initial screening, and full-text review, 8 were included. Both disruption of bisynchronous epileptiform discharges (EDs) and lateralization of previously generalized EDs have been proposed as having a prognostic value in callosotomy. No significant correlation between intraoperative ED changes and seizure outcomes has been found in the studies evaluated here. Guidance of posterior dissection extent has also been suggested to be of intraoperative utility in callosotomy. Given that the majority of corpus callosotomies in the pediatric population are complete as opposed to an anterior two-thirds technique, the overall utility in dissection guidance is low.

Conclusion: IONM has not been found to provide significant prognostic value nor strongly contribute to improving surgical outcomes during corpus callosotomies for pediatric patients with intractable epilepsy. Medicolegal considerations across the field of neurosurgery have grown in recent years; the majority of litigation perspectives are isolated to spine surgery, with IONM functioning more for documentation purposes in pediatric corpus callosotomy. The use of IONM during pediatric corpus callosotomy remains the surgeon's preference as it has not been defined as the standard of care.

Purpose: Encephalocele is a herniation of intracranial structures associated with a skull anomaly. In Western countries, posterior encephaloceles are more common than anterior encephaloceles and may occur in the parietal (parietal encephalocele, PE) or occipital (occipital encephalocele, OE) region. Although those entities are relatively common in pediatric neurosurgery, large clinical series are scarce, and their clinical outcomes are poorly documented in the literature.

Methods: We retrospectively analyzed the clinical and radiological findings, post-operative long-term outcomes of consecutive patients diagnosed with posterior encephaloceles from 2010 to 2021 in 3 centers: Hôpital Femme Mère Enfant (Lyon, France); Hôpital Roger Salengro (Lille, France); and Charité Universitätsmedizin (Berlin, Germany).

Results: We collected 79 observations, 46 PEs and 33 OEs. Cerebral anomalies were more common in OEs than PEs (15/33, 45% vs 7/46, 15%, p = 0.001). Vascular anomalies were more common in PEs than OEs (41/46, 88% vs 5/33, 15%). All children underwent a surgical correction of the malformation. CSF disorders requiring surgical management were present in 4/33 OEs and 4/46 PEs. During the mean follow-up of 33 months (12-160 months), 3 OEs patients died, and various degrees of psychomotor impairment were found in both entities: 18/33 (54%) OEs and 8/46 (17%) PEs (p = 0.005); however, in most cases of PE, developmental delay was mild.

Conclusions: The clinical evolution in OEs is significantly more unfavorable than in PEs. However, even in case of PEs, psychomotor impairments are not uncommon. The presence of herniated cerebral tissue, hydrocephalus, and syndromic context increase the risk of developmental delay.

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.