Cervical saccular limited dorsal myeloschisis, so-called "cervical myelomeningocele": long-term follow-up of a single-center series and systematic review.

OBJECTIVE: Cervical saccular limited dorsal myeloschisis (LDM), previously so-called "cervical myelomeningocele," is a rare spinal dysraphism. Although the pathogenesis of true myelomeningocele is primary neurulation failure, LDM results from a delayed abnormality during the final stages of neurulation. The aim of the study was to evaluate the outcome of these patients and to assess the correlation of outcomes with the level and type of lesion. Also, pooled data from the literature on similar lesions were systematically reviewed. METHODS: A retrospective study was conducted at Children's Medical Center (CMC), Tehran, Iran. Information of patients who underwent surgery between 2004 and 2020 (i.e., the recent series) was extracted and combined with data from a previously published series from the same center that were obtained between 2000 and 2003 (CMC series). The literature was reviewed for all published cases, to be combined with the CMC series for further analyses. RESULTS: Twenty-two patients were included in the recent series. Combined with 16 previously published cases, 38 patients with a mean ± SD age at surgery of 11.75 ± 28.64 months were included in the CMC series. The rates of neurological deficit, hydrocephalus, and Chiari malformation type II in the CMC series were 26.32%, 39.47%, and 28.95%, respectively. The lesions were at the upper levels in 17 (44.7%) and lower cervical levels in 21 (55.3%) patients, with 31 cases (81.58%) diagnosed with stalk-type lesions and 7 cases (18.42%) with myelocystocele-type lesions. At final follow-up, 31 patients (81.57%) achieved sphincter continence, and all 36 accessible patients were ambulated, consisting of 28 (73.68%) independent and 8 (21.05%) dependent ambulation patients. The rates of Chiari malformation type II and hydrocephalus were insignificantly higher in patients with upper-level lesions, but those of neurological deficit, ambulation, and sphincter continence were not associated with level. The rates of hydrocephalus (p < 0.01), Chiari type II malformation (p < 0.01), and neurological deficit (p = 0.04) were significantly higher in the myelocystocele group. In the systematic review, 24.77% of patients had neurological deficit. Binary logistic regression showed that older age at surgery (p = 0.03) and associated spinal anomalies (p = 0.04) were significant predictors of deficits. Chiari type II malformation was significantly (p < 0.001) and hydrocephalus was marginally (p = 0.06) more common in patients with myelocystocele-type lesions. The rate of Chiari malformation type II was higher in patients with upper-level lesions (p = 0.02). CONCLUSIONS: Patients with cervical saccular LDM had better outcome compared with those patients with true myelomeningocele in more distal areas. According to the current series, most patients obtained ambulation and voiding continence, regardless of the level or type of lesion. Hydrocephalus, Chiari type II malformation, and neurological deficit were more common in patients with myelocystocele-type lesions.

The Predictors of Seizures in Patients with Encephalocele: An 11-Year Experience from a Tertiary Hospital.

INTRODUCTION: The aim of the study was to investigate and identify the predictors associated with the incidence of seizures in patients with encephalocele (EC). METHODS: A retrospective analysis was undertaken of patients treated for EC at a tertiary medical center in Tehran between 2010 and 2021. Data including age at presentation, gender, location, size, and content of EC, ventriculomegaly, hydrocephalus, associated anomalies, and neurodevelopmental delay (NDD) were evaluated for their prognostic value. In addition, univariate and multivariate analyses were performed to identify the correlation between independent predictors and seizure incidence. RESULTS: One hundred and two cases of EC were identified. Seventy-one ECs (69.6%) were posterior ECs, while 31 (30.4%) were anterior. Neural tissue was found in 43 (42.2%) of the ECs. Thirty-three patients (32.4%) had ventriculomegaly, of which 90.9% underwent shunt placement for progressive or symptomatic hydrocephalus. Seizure was found in 26 (25.5%) patients. On univariate analysis, presence of other anomalies, postoperative infections, and NDD were associated with seizures (p < 0.05). When the anomalies were categorized into intracranial and extracranial groups in univariate analysis, none was associated with statistically significant increase in seizure (p values of 0.09 and 0.61, respectively). Although according to multivariate analysis, only the association between other associated anomalies and seizure was near significant (OR: 2.0, 95% CI: 0.95-4.2, p = 0.049). Children with NDD and postoperative infection were, respectively, 3.04 and 1.3 times more at risk to experience seizures compared to other patients. CONCLUSION: We found a rate of 25.5% risk of seizure in patients with EC. This study could not find any significant predictors of seizure in children with EC. However, pediatric patients with postoperative infections including sepsis, wound infection, and NDD require more consideration to reduce the risk of seizure.

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.

Safety and efficacy of aprotinin versus tranexamic acid for reducing absolute blood loss and transfusion in pediatric patients undergoing craniosynostosis surgery: a randomized, double-blind, three-arm controlled trial.

OBJECTIVE: Craniosynostosis surgery is associated with considerable blood loss and need for transfusion. Considering the lower estimated blood volume (EBV) of children compared to adults, excessive blood loss may quickly lead to hypovolemic shock. Therefore, reducing blood loss is important in craniosynostosis surgery. This study was conducted to evaluate the efficacy of aprotinin or tranexamic acid (TXA) in blood loss reduction in these patients. METHODS: In the current randomized controlled trial, 90 eligible pediatric patients with craniosynostosis were randomly divided into three groups to receive either aprotinin, TXA, or no intervention. The absolute blood loss and transfusion amount were assessed for all patients both intraoperatively and 2 and 8 hours postoperatively. RESULTS: Although crude values of estimated blood loss were not significantly different between groups (p = 0.162), when adjusted to the patient's weight or EBV, the values reached the significance level (p = 0.018), particularly when the aprotinin group was compared to the control group (p = 0.0154). The EBV losses 2 hours and 8 hours postoperatively significantly dropped in the TXA and aprotinin groups compared to the control group (p = 0.001 and p < 0.001, respectively). Rates of postoperative blood transfusion were significantly higher in the control group (p = 0.024). Hemoglobin and hematocrit 8 hours postoperatively were lower in the control group than in the TXA or aprotinin treatment groups (p < 0.002 and p < 0.001, respectively). There were no serious adverse events associated with the interventions in this study. CONCLUSIONS: Aprotinin and TXA can reduce blood loss and blood transfusion without serious complications and adverse events in pediatric patients undergoing craniosynostosis surgery.

Single-dose antibiotic prophylaxis compared with multiple-dose protocol in clean pediatric neurosurgical interventions: a nonrandomized, historically controlled equivalence trial.

OBJECTIVE: Guidelines recommend antimicrobial prophylaxis (AMP) preoperatively for "clean" spinal and cranial surgeries, while dose and timing remain controversial. The use of multiple-dose AMP for such surgeries is under debate in the pediatric context. In this clinical study, the authors aimed to compare single-dose with multiple-dose prophylactic antibiotic usage in cranial and spinal neurosurgical interventions of pediatric patients. METHODS: All neurosurgical patients aged 28 days to 18 years who underwent surgery at a single tertiary center were assessed. Three cohorts (noninstrumented clean spinal, noninstrumented cranial, and instrumented cranial interventions), each of which comprised two 50-patient arms (i.e., single-dose AMP and multiple-dose AMP), were included after propensity score-matched retrospective sampling and power analysis. Records were examined for surgical site infections. Using a previously published meta-analysis as the prior and 80% acceptance of equivalence (margin of OR 0.88-1.13), logistic regression was carried out for the total cohort and each subcohort and adjusted for etiology by consideration of multiple-dose AMP as reference. RESULTS: The overall sample included 300 age- and sex-matched patients who were evenly distributed in 3 bi-arm cohorts. There was no statistical intercohort difference based on etiology or type of operation (p < 0.05). Equivalence analysis revealed nondiscriminating results for the total cohort (adjusted OR 0.65, 95% CI 0.27-1.57) and each of the subcohorts (noninstrumented clean spinal, adjusted OR 0.65, 95% CI 0.12-3.44; noninstrumented cranial, adjusted OR 0.52, 95% CI 0.14-2.73; and instrumented cranial, adjusted OR 0.68, 95% CI 0.13-3.31). CONCLUSIONS: No significant benefit for multiple-dose compared with single-dose AMPs in any of the pediatric neurosurgery settings could be detected. Since unnecessary antibiotic use should be avoided as much as possible, it seems that usage of single-dose AMP is indicated.

The effect of external ventricular drain tunneling length on CSF infection rate in pediatric patients: a randomized, double-blind, 3-arm controlled trial.

OBJECTIVE: The role of tunneling an external ventricular drain (EVD) more than the standard 5 cm for controlling device-related infections remains controversial. METHODS: This is a randomized, double-blind, 3-arm controlled trial done in the Children's Medical Center in Tehran, Iran. Pediatric patients (< 18 years old) with temporary hydrocephalus requiring an EVD and no evidence of CSF infection or prior EVD insertion were enrolled. Patients were randomly assigned (1:1:1) into the following arms: 5-cm (standard; group A); 10-cm (group B); or 15-cm (group C) EVD tunnel lengths. The investigators, parents, and person performing the analysis were masked. The surgeon was informed of the length of the EVD by the monitoring board just before operation. Patients were followed until the EVD's fate was established. Infection rate and other complications related to EVDs were assessed. RESULTS: A total of 105 patients were enrolled in three random groups (group A = 36, group B = 35, and group C = 34). The EVD was removed because there was no further need in most cases (67.6%), followed by conversion to a new EVD or ventriculoperitoneal shunt (15.2%), infection (11.4%), and spontaneous discharge without further CSF diversion requirement (5.7%). No statistical difference was found in infection rate (p = 0.47) or EVD duration (p = 0.81) between the three groups. No group reached the efficacy point sooner than the standard group (group B: hazard ratio 1.21, 95% CI 0.75-1.94, p = 0.429; group C: hazard ratio 1.03, 95% CI 0.64-1.65, p = 0.91). CONCLUSIONS: EVD tunnel lengths of 5 cm and longer did not show a difference in the infection rate in pediatric patients. Indeed, tunneling lengths of 5 cm and greater seem to be equally effective in preventing EVD infection. Clinical trial registration no.: IRCT20160430027680N2 (IRCT.ir).

Suprahepatic space as an alternative site for distal catheter insertion in pseudocyst-associated ventriculoperitoneal shunt malfunction.

OBJECTIVE: Distal catheter malfunction due to pseudocyst formation or intraabdominal adhesion is a significant problem, especially in pediatric patients who have limited sites for distal catheter insertion. In this study, the authors present a series of 12 patients with intractable distal shunt malfunctions due to peritoneal pseudocyst formation who underwent distal catheter replacement in the suprahepatic space to reduce the risk of distal catheter malfunction. METHODS: Twelve consecutive patients with shunt malfunction due to pseudocyst formation who had undergone ventriculosuprahepatic shunting from 2014 to 2019 were identified. According to medical records, after primary evaluations, shunt removal, and antibiotic therapy, they underwent revision surgeries with placement of a distal catheter into the suprahepatic space. RESULTS: Nine boys and 3 girls, ranging in age from 5 months to 14 years, with one or more episodes of pseudocyst formation, underwent shunt revision with placement of a distal catheter into the suprahepatic space. After a median follow-up of 31 months, none of the patients experienced further distal malfunction. CONCLUSIONS: The suprahepatic space appears to be a safe place to secure the distal end of a ventricular catheter following pseudocyst formation, with less risk of re-adhesion. This lower risk might be attributable to the lack of omentum in the suprahepatic space.

Reevaluation of Classic Posterior Ventricular Puncture Sites Using a 3-Dimensional Brain Simulation Model.

OBJECTIVE: To revalidate the craniometric dimensions of classic posterior burr holes for ventricular catheter insertion in hydrocephalic patients, based on ideal catheter position on a 3-dimensional simulated computed tomography (CT) reconstruction model of the ventricles. METHODS: Fifteen patients with hydrocephaly underwent multislice, thin-cut CT to geometrically determine the Cartesian coordinates of a new point for optimal posterior ventricular catheterization. The success rate for ventricular puncture and the thickness of brain traversed by the catheter with 3 approaches (Frazier, Keen, and the suggested point) were compared. RESULTS: The suggested burr hole point for posterior ventricular catheterization is 51 and 57 mm posterior and 58 and 60 mm above the external auditory meatus parallel to the orbitomeatal plane on the right and left sides, respectively, significantly different from the classical Frazier and Keen points. The success rate was 100% for approaches using the suggested point and the Frazier point, compared with 83% using the Keen point. This 17% difference was marginally significant (P = 0.052). The parenchymal mantle for the Frazier point was thicker than that of the suggested point on both sides, although the difference was statistically significant only on the right side (P = 0.006). The parenchymal mantle was thinner in the Keen approach compared with the suggested approach, but the difference was not statistically significant. CONCLUSIONS: The use of a suggested burr hole point for posterior ventricular catheterization may decrease the amount of parenchymal mantle of the brain transgressed by the catheter, and may marginally improve the chance of successful posterior ventricular catheterization.

Expanded Endoscopic Transnasal Approach to the Chordoid Glioma of the Third Ventricle : The First Case Ever Reported.

Chordoid glioma of the third ventricle is a rare and challenging tumor to surgery because of its unique anatomical location and its close juxtaposition to the neurovascular structures and hypothalamus. The authors report a case of chordoid glioma of the third ventricle in a 43-year-old woman, who presented with headache and somnolence. The tumor was approached by endoscopic transnasal technique with a favorable result. Histopathologic examination disclosed a neoplastic tissue composed of eosinophilic epithelioid cells, mucinous, periodic acid Schiff-diastase positive, extracellular matrix, and scattered lymphoplasmacytic infiltrates. The best treatment option remains controversial. Customarily, the surgical route to remove chordoid glioma is transcranial; however, the undersurface of the optic chiasm and optic nerves preclude an adequate surgical visualization. In contrast, an expanded endoscopic transnasal approach provides a direct midline corridor to this region without any brain retraction.

Case Report: Combination Therapy with Mesenchymal Stem Cells and Granulocyte-Colony Stimulating Factor in a Case of Spinal Cord Injury.

INTRODUCTION: Various neuroregenerative procedures have been recently employed along with neurorehabilitation programs to promote neurological function after Spinal Cord Injury (SCI), and recently most of them have focused on the acute stage of spinal cord injury. In this report, we present a case of acute SCI treated with neuroprotective treatments in conjunction with conventional rehabilitation program. METHODS: A case of acute penetrative SCI (gunshot wound), 40 years old, was treated with intrathecal bone marrow derived stem cells and parenteral Granulocyte-Colony Stimulating Factor (G-CSF) along with rehabilitation program. The neurological outcomes as well as safety issues have been reported. RESULTS: Assessment with American Spinal Injury Association (ASIA), showed neurological improvement, meanwhile he reported neuropathic pain, which was amenable to oral medication. DISCUSSION: In the acute setting, combination therapy of G-CSF and intrathecal Mesenchymal Stem Cells (MSCs) was safe in our case as an adjunct to conventional rehabilitation programs. Further controlled studies are needed to find possible side effects, and establish net efficacy.

Bilateral occipito-condylar hyperplasia: a very rare anomaly treated with endoscopic endo-nasal approach.

INTRODUCTION: Occipito-condylar hyperplasia is a very rare anomaly of the cranio-vertebral junction that was only reported in two patients before and managed through posterior approach. CASE MATERIAL: A 10-year-old girl with a sudden attack of quadriparesis and respiratory distress was admitted to our center. A detailed work up favored a high cervical myelopathy due to bilateral occipito-condylar hyperplasia and Chiari malformation. RESULTS: An endoscopic endo-nasal approach under navigation guide was used to drill the compressive lesion. CONCLUSION: Our patient is added to the literature as the third one that was approached through a different surgical corridor. Successful decompression with excellent results was gained.