Clinical and Radiological Outcomes of Dura-Splitting versus Duraplasty Techniques in Pediatric Chiari I Malformation: A Systematic Review and Meta-Analysis.

Type I Chiari malformation is a developmental anomaly with various proposed surgical techniques for its management. The dura-splitting technique is a less invasive approach and involves the resection of the outer layer of the dura while sparing the internal layer. While this less-known approach may minimize the complication rates, there are concerns about its efficacy and outcome. Therefore, we have performed a systematic review and meta-analysis of available data on clinical and radiological outcomes of this technique in the pediatric population and compared them to the foramen magnum decompression and duraplasty technique. We have followed the Meta-analysis Of Observational Studies in Epidemiology guidelines in this review. Based on our predefined search strategy, we performed a systematic database search. Subsequently, the article screening process was done based on defined inclusion/exclusion criteria. Following the quality assessment of included studies, two authors performed data extraction. Finally, the extracted data were summarized and presented in form of tables. Forest plots were used to demonstrate the results of the meta-analysis. A review of 8 included studies consisting of 615 patients revealed the significant advantage of the dura-splitting technique in terms of shorter operation duration and hospital stay. The recurrence rate and clinical and radiological outcomes were almost similar between the two surgical techniques. Complication rates were significantly lower in the dura-splitting technique. Dura-splitting can be an effective and safe approach for the management of pediatric Chiari I malformation. However, these results are mostly extracted from observational studies and future randomized controlled trials are recommended.

A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.

This is a letter to share the clinical experience we had with neonates born with a specific feature of spina bifida. The lesion is like a meningocele containing skim amount of fluid, with a tiny dimple around the center of the cutaneous lesion which causes inward retraction of the dysplastic coverage. This feature is in favor of accompanied split cord malformation (SCM) type I, and the central nidus is in continuous with the dural sleeve around the bony septum of SCM. By recognition of this clinical clue, surgical repair can be planned to perform with appropriate provision, particularly foreseeing the amount of intraoperative bleeding and duration of anesthesia in the newborn patients.

Current Status of Research on Targeted Therapy Against Central Nervous System Tumors in Low- and Lower-Middle-Income Countries.

OBJECTIVE: In recent decades, a significant body of research has focused on targeted therapies for the treatment of central nervous system (CNS) tumors to enhance the effectiveness of management strategies. However, most of these efforts have been centered in high-income countries, which renders the generalizability of their results to low- and middle-income countries questionable. Therefore, in this review, we systematically investigated the status of research conducted on targeted therapy for CNS tumors in low- and lower-middle-income countries to elucidate the contribution of these countries in advancing neuro-oncology. METHODS: A systematic search of 3 databases was performed using a predefined search strategy. After screening the articles based on our inclusion/exclusion criteria, the data were extracted to a predesigned Excel worksheet. RESULTS: A review of 44 included studies showed that India, Iran, and Lebanon were the only countries with a contribution to this field. All included studies were laboratory or animal experiments, and there were no clinical studies in this field. The most investigated CNS tumor was malignant glioma, and gene-targeted therapy was the most investigated category of targeted therapies in these countries. CONCLUSIONS: Low- and lower-middle-income countries comprise more than half of the world population, but they are deprived of targeted therapies against CNS tumors. Although there are basic experiments performed on this subject, they originate in a limited number of these countries. Therefore, targeted therapy is in its preliminary stage in these countries.

A prospective study of prenatal ventriculomegaly: natural course, survival, and neurodevelopmental status.

OBJECTIVE: Prenatal ventriculomegaly (VM) is classified as mild, moderate, or severe on the basis of the diameter of the atrium. Neurodevelopmental status in prenatal VM is associated with various factors such as the course of VM, VM type, progression, and associated anomalies. In this study, the authors aimed to evaluate neurodevelopmental outcome in patients with prenatal VM and to detect possible associated risk factors. METHODS: In this study, 73 pregnancies with VM who were referred to Children's Medical Center, Tehran, Iran, between 2019 and 2021 were prospectively followed. They were followed up every 2-4 weeks with ultrasonography (US) before delivery and were then observed for an average time of 14.6 months. The authors collected demographic and ultrasound information, associated abnormalities, pregnancy outcomes, and developmental status according to Centers for Disease Control criteria. RESULTS: The mean gestational age at the time of diagnosis was 28.1 weeks, and 46.6% of fetuses were female. According to the first US, 46.6% had mild, 21.9% had moderate, and 31.5% had severe VM. Serial US scans showed that VM had regressed in 20.5% of patients, remained stable in 35.6%, and progressed in 43.8%. Other cranial abnormalities were detected in 38.4% of fetuses. During follow-up, 62.5% of cases had normal developmental status, 26.6% had mild delay, and 10.9% had severe neurodevelopmental delay. Pregnancy was terminated in 9 (12.3%) cases. Normal neurodevelopment was reported in 75.8% of patients with mild VM versus 50% of those with severe VM (p = 0.19). Neurodevelopmental status was normal in 72.5% of cases without other cranial abnormalities (p = 0.018) and in 86.7% of cases with regression of VM (p = 0.028). CONCLUSIONS: Despite analysis of different factors in prenatal VM, only progression of VM and associated cranial abnormalities had significant relationships with neurodevelopmental prognosis.

Hydrocephalus in patients with encephalocele: introduction of a scoring system for estimating the likelihood of hydrocephalus based on an 11-year experience from a tertiary center.

OBJECTIVE: The goal of this study was to investigate and identify the predictors associated with the incidence of hydrocephalus requiring shunt insertion in patients with encephalocele (EC), and to develop a scoring system to estimate the probability of hydrocephalus occurrence over time in these patients. METHODS: A retrospective analysis was undertaken on data from patients treated for EC at a tertiary medical center between 2010 and 2021. Data including patient age at presentation, sex, sac location, sac size, contents, presence of ventriculomegaly/hydrocephalus, CSF leakage, and other associated intracranial/extracranial anomalies were among the variables evaluated for their predictive value. In addition, logistic regression analyses were performed to identify the independent predictors. A predictive scoring system was developed based on regression coefficients. RESULTS: A total of 102 cases of EC were identified. The patient group consisted of 52 boys and 50 girls. Seventy-one patients (69.6%) had posterior ECs. Forty-three (42.2%) of the ECs contained neural tissue. Thirty-three patients presented with ventriculomegaly (32.4%), 30 of whom (90.9%) underwent ventriculoperitoneal shunt placement for hydrocephalus. Multivariate analysis revealed that the presence of other associated anomalies (OR 2.8, 95% CI 1.1-7.4, p = 0.027), larger EC sac size (OR 1.3, 95% CI 1.01-1.6, p = 0.042), and infections (OR 6.8, 95% CI 1.3-34.8, p = 0.034) were associated with ventriculomegaly. The logistic regression model consisted of 5 variables including the patients' history of meningitis, their sex, sac location, sac size, and presence of other other associated anomalies; analysis resulted in the maximum accuracy of 86% for the prediction of hydrocephalus occurrence. CONCLUSIONS: According to the findings, the presence of other associated anomalies, a larger sac, and infections are significant independent predictors of hydrocephalus. By considering these 3 predictors as well as sac location and the patient's sex, it will be possible to predict hydrocephalus occurrence in patients with EC with significant accuracy.

Spinal myelolipoma - an extremely rare pathology within the lumbar spine: a case report and literature review.

Myelolipoma is a benign tumor containing mature adipose cells and a combination of myeloid and erythroid elements. This tumor is typically found in the adrenal glands; however, it has been detected outside the adrenal glands in rare cases. We report an extremely rare case of myelolipoma in the lumbar spine causing significant neural compression due to the involvement of the posterior spinal elements. Given the significant neurological deficit, the patient was surgically managed as soon as possible. Extra-adrenal myelolipomas are rare lesions, and only one case has been reported in the spine so far. However, this diagnosis should be considered in cases with its characteristic imaging features.

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology.

BACKGROUND AND IMPORTANCE: Papillary glioneuronal tumor is a recently known entity in central nervous system tumors. These benign WHO grade I tumors are mostly seen in young adults. Pediatric PGNT is rare and there is no report of these tumors in toddlers. Headache, nausea/vomiting and seizure are most common clinical symptoms. Acute presentation with focal neurological deficits or loss of consciousness are not amongst the expected presentations. These tumors are typically cystic with enhancing mural nodule. Although case with chronic intermittent microhemorrhages are reported in the literature but overt intra-tumoral hemorrhage is an odd radiological presentation with just one reported case in the literature. CLINICAL PRESENTATION: We present an extremely rare case of PGNT presenting with sudden onset hemiparesis and impaired consciousness due to acute intra-tumoral hemorrhage in a toddler which was surgically treated with favorable outcome. CONCLUSION: PGNTs can also be seen in very young children even in toddlers. Also, it should be kept in mind that these tumors have potential for overt intra-tumoral hemorrhage and acute presentation with focal neurological deficits mimicking more common pathologies which should be considered to plan optimal patient management.

The neurodevelopmental outcomes of children with encephalocele: a series of 102 patients.

OBJECTIVE: The overall prognosis of encephalocele (EC) is not well described. However, the presence of some risk factors may result in neurodevelopmental delay (NDD) and negatively affect the prognosis of affected patients. The goal of this study was to evaluate neurodevelopmental outcome, as well as the impact of a number of factors on the outcome in patients with ECs. METHODS: This was an observational, retrospective study including 102 children with EC who were followed at the pediatric neurosurgery department of a tertiary medical center between the years 2010 and 2021. The authors evaluated NDD status according to the Centers for Disease Control and Prevention classification via clinical evaluation and parent interviews in the outpatient setting. RESULTS: There were 52 boys and 50 girls. The median age at the time of surgery was 4 months (range 1 day-7.5 years). Seventy-one patients (69.6%) had posterior ECs, whereas 31 (30.4%) had anterior ECs. Forty-three (42.2%) of the ECs contained neural tissue. Of the 102 patients, 33 (32.4%) had ventriculomegaly. In terms of NDD, 14 (14.9%) had mild/moderate delay, whereas 17 patients (18.1%) had severe NDD. On univariate analysis, posterior location, size of sac, presence of neural tissue, ventriculomegaly, symptomatic hydrocephalus, and postoperative infection were correlated with NDD. On a multivariate logistic regression model, only neural tissue presence had a statistically significant association with NDD (OR 7.04, 95% CI 1.33-37.2, p = 0.022). Although not statistically significant, children with ventriculomegaly were 2.6 times as likely to have NDD (95% CI 0.59-11.19, p = 0.362). CONCLUSIONS: This is a single-center study with a large sample size in which the neurodevelopmental status of patients with EC was assessed, and the authors tried to find the risk factors of NDD in these patients. The results showed that the presence of neural tissue within the EC sac was the only risk factor that had independent statistically significant association with NDD.

Infantile Spasms, Clinical Manifestations of a Rare Brain Tumor: A Case Report and Literature Review.

Desmoplastic infantile ganglioglioma (DIG) has a favorable prognosis and is classified as a benign infantile brain tumor. The DIG is more common in children under 2 years of age than in other age groups. This report introduces a 5.5 month-old infant who was referred with infantile spasms and diagnosed with a brain tumor. Brain magnetic resonance imaging showed a large heterogeneous mass in the right hemisphere with shifting to the other side. The patient underwent surgery. The extra-axial mass was completely resected, and the diagnosis of DIG grade I was confirmed by pathology. After one year, patient development was normal, and the seizures did not recur. In addition, the general condition was good. With a brief review and search in the literature, 13 case reports were identified 9 of which were male cases. The mean age of initial manifestation to final tumor diagnosis was 4 months. Out of 13 patients, 8 cases were reported with the mass origin in the right hemisphere. The most commonly observed tumors were glioma (n=4) and hypothalamic hamartoma (n=3). Except for three patients who died, the remaining had a complete recovery after tumor removal with a seizure-free interval at follow-up.

A Giant Sacrococcygeal Chordoma: A Case Report.

Sacrococcygeal chordoma is a rare malignant bone tumor. Although there are tough membranes such as the periosteum and presacral fascia (which resist transgression by the tumors), chordoma usually invades the rectal wall. The serious problem with these tumors is the late diagnosis and its high likelihood to become enlarged. The main treatment options for this tumor is surgical resection, radiotherapy, and chemotherapy. Due to the tumor vicinity to important organs such as bladder and its neurovascular structures, it makes surgical excision extremely challenging. The aim of this study is to describe a 50-year-old man with a giant sacrococcygeal mass. The novelty of this case report is the huge and unique size of the tumor which has not reported previously as well the special surgical approaches performed to remove the tumor.

Characteristics and In-Hospital Outcomes of Pediatric Traumatic Spinal Injuries in A Referral Trauma Center.

OBJECTIVE: To evaluate the characteristics and in-hospital outcomes of traumatic spinal injuries among children admitted to a local trauma center in Iran. METHODS: Patients aged 0-18 years who had been admitted to Shahid Kamyab trauma center for acute traumatic spinal injury (Mashhad, Iran) between 2011 and 2018 were evaluated retrospectively. Various demographic, clinical, radiological, and outcome variables were recorded and analyzed. RESULTS: A total of 127,300 trauma patients were evaluated and amongst them, 61 children had spinal trauma. The mean age was 11.1 and there was no significant sex preponderance (54% males). Most of the injuries were occurred in summer (34.4%) and the most common trauma mechanism was motor vehicle accidents (55.7%) followed by falling (36.1%). Almost all patients (95.1%) had vertebral fractures, which were in the cervical, thoracic, and lumbosacral area in order to decrease incidence. 67.2% of patients were managed non-surgically. The mean hospital stay was 8.9 days and 82.0% of patients had been discharged with normal motor function. CONCLUSION: Pediatric spinal trauma is less studied entity in the field of traumatology due to the lower prevalence of these injuries in pediatric patients worldwide. But our study shows a higher prevalence of such injuries in the pediatric population. Although controversial, the leading cause of these injuries is motor vehicle accidents. Fortunately, short term in-hospital outcome seems to be good in such injuries.

The effect of combination therapy with erythropoietin and methylprednisolone in patients with traumatic cervical spinal cord injury: a pilot randomized controlled trial.

STUDY DESIGN: Pilot double-blinded randomized controlled trial. OBJECTIVES: To investigate the additive effect of recombinant human erythropoietin (rhEPO) on functional outcome and disability in patients with traumatic cervical spinal cord injury (TCSCI). SETTINGS: University-affiliated hospital in Mashhad, Iran. METHODS: Patients with acute TCSCI admitted within 8 h after injury were randomly assigned to receive only methylprednisolone (M group) or rhEPO 500 IU/mL plus methylprednisolone (M + E group). All the patients underwent surgery within the next several days. Neurological function was assessed on admission, and at 6th and 12th months after the injury according to the sphincter function and American spinal cord injury association (ASIA) scale. RESULTS: Overall, 54 patients (mean age: 39.7 ± 13.3 years) including 46 (85%) males were studied in two groups of 27. The likelihood of developing adverse neurological outcomes (ASIA impairment score of A compared to D or E) was not significantly different between the groups after 6 (OR = 0.39, 95% CI = 0.03-4.80, P = 0.46) and 12 months (OR = 0.83, 95% CI = 0.11-6.11, P = 0.86). The groups also showed no significant difference in 1-year mortality (OR = 0.83, 95% CI = 0.25-2.74, P = 0.76). CONCLUSIONS: It is not clear whether combination therapy with erythropoietin compared to methylprednisolone alone improves neurological functions of patients with TCSCI. Our study provides interim data to guide future larger definitive trials.

Outcomes of dura-splitting technique compared to conventional duraplasty technique in the treatment of adult Chiari I malformation: a systematic review and meta-analysis.

Chiari malformation type I is a developmental abnormality with an array of surgical techniques introduced for the management of it. The most common technique is foramen magnum decompression with duraplasty. Dura-splitting technique as one of the non-dura-opening techniques is a less known procedure that spares the internal layer of the dura and can theoretically result in fewer complications compared to duraplasty. So, we performed a review of literature and meta-analysis on different clinical and radiological aspects of this technique and compared its outcomes to duraplasty. MOOSE guidelines were followed. A systematic search of three databases based on predefined search strategy and inclusion/exclusion criteria was performed. After quality assessment and data extraction by two authors, summarized data were presented in form of tables, and meta-analysis results were illustrated in forest plots. A review of 10 included studies consisting of 370 patients revealed significantly shorter operation duration and less intraoperative blood loss in the dura-splitting technique compared to duraplasty. Interestingly, there was no significant difference between these two techniques in terms of clinical and radiological outcomes. Overall complication rate and incidence of CSF-related complications or infections were significantly in favor of the dura-splitting technique. Dura-splitting technique can be considered as a safe and effective surgical procedure for Chiari I malformation with comparable outcomes and fewer complications compared to duraplasty, although this interpretation is derived from retrospective observational studies and lack of a prospective clinical trial is evident.

Strengths and weaknesses of frontal versus occipital ventriculoperitoneal shunt placement: A systematic review.

Excessive accumulation of cerebrospinal fluid within the brain ventricles is called hydrocephalus, which results in increased intracranial pressure preventing brain growth or causing damage to intracranial structures due to raised intracranial pressure. One of the most common treatment options for this pathology includes the placement of a ventriculoperitoneal shunt to drain the excess fluid. The location of catheterization is traditionally considered as an important factor affecting shunt survival. In this study, we aimed to systematically review all available documents to determine the advantage and superiority of frontal or occipital shunt entry points as the two main approaches. A database search was performed in PubMed, Scopus, Embase, Web of Science, Medline, Ovid, and Google Scholar using "ventriculoperitoneal", "shunt placement", and "hydrocephalus" as the main key terms. Resultant articles were screened for relevancy based on predefined inclusion and exclusion criteria by two authors independently. After excluding irrelevant documents, the data of 11 related articles consisting of 3947 patients were extracted and qualitative data synthesis and pooled analysis were performed. The results of the included studies showed that although the outcomes of a higher percentage of the total review population were in favor of frontal shunt placement, there was no significant superiority for neither of these two approaches after pooled analysis of available failure rates. Findings have shown that each approach has benefits and drawbacks, and there may be other factors such as age and valve design besides the position of shunt placement that may affect the survival rate. Also, the accuracy of shunt placement as an independent factor affects the failure rate and can be improved with various image-guidance methods to minimize shunt failure.

Role of available adjuvant therapies following surgical resection of atypical choroid plexus papilloma-a systematic review and pooled analysis.

BACKGROUND: Atypical choroid plexus papilloma is a recently introduced entity with intermediate pathological characteristics. These tumors are relatively rare and the optimal management of these tumors is a matter of debate. Therefore, we performed a systematic review and pooled analysis about the effects of adjuvant therapies on outcome measures of these patients. We also compared these effects on totally and partially resected tumors and pediatric and adult populations. METHODS: A systematic search of 3 databases based on inclusion/exclusion criteria was performed. Data extraction was separately performed by 2 authors, and the summarized data were presented in the form of tables. Pooled estimates of different outcome measures were calculated for each adjuvant therapy and presented separately for studies with pediatric, adult, or mixed populations. RESULTS: A review of 14 included studies consisting of 144 patients revealed the effect of adjuvant treatment on reduction of tumor recurrence, metastasis, and reoperation rates and increasing survival rates in patients with subtotal tumor resection. This advantage was not seen in the case of gross total tumor resection. Almost all outcome measures were more favorable in the pediatric population. CONCLUSIONS: It can be concluded that whenever gross total resection is not feasible, the implementation of adjuvant therapy can improve the outcome and prognosis. In other cases, it should be decided on an individual basis. Also, more aggressive behavior and higher rates of recurrence and mortality in the adult population suggest the consideration of more aggressive adjuvant treatments for adult patients.

Conus Medullaris Teratoma: Case Report and Literature Review.

Teratomas are benign germ cell tumors that usually found out of their anatomical origin. Teratomas usually are found in sacrococcygeal area, gonads, mediastinum, cervicofacial region and intracranial fossa. Spinal teratomas are rare. In this study we describe a case of conus medullaris teratoma which was diagnosed based on imaging studies. The patient underwent surgery. We did bilateral laminectomy. The mass lesion had an obvious and rigid attachment to the conus medullaris. The wall of the lesion was resected as much as possible, but total resection of the lesion's wall could not be done due to changes in neural monitoring. Previous related studies are reviewed.

A rare case of disseminated tuberculosis; multiple intracranial and intramedullary tuberculomas with concurrent tuberculous spondylitis and meningitis.

Concomitant presentation of intracranial and intramedullary tuberculomas is a rare entity. We report a 38-year-old immunocompetent woman with intramedullary and intracranial tuberculomas with concurrent tuberculous spondylitis and meningitis, who presented with paraparesis and back pain. Despite initial antituberculous therapy, the patient's neurologic status deteriorated with the development of paraplegia. Following surgical intervention, the patient gradually recovered muscle strength and bladder function.

Bilateral Fusiform Aneurysms of the Internal Carotid Arteries Presenting with Subarachnoid Hemorrhage.

Fusiform aneurysms of cerebral arteries are less prevalent than saccular aneurysms and are rarely associated with subarachnoid hemorrhage (SAH). In this article, we report SAH due to a rare case of bilateral fusiform aneurysm of the supraclinoid segment of the internal carotid arteries (ICAs) (C4 segment of the ICA).