Implementation of an adapted perioperative ventriculoperitoneal shunting protocol in a tertiary center located in a low-to-middle-income country.

OBJECTIVE: Preventative protocols have efficaciously reduced shunt infection in developed countries. However, the generalizability of these protocols in low-to-middle-income countries (LMICs) remains unclear. Previously, shunt insertion in the authors' center was routinely performed under institutional preventative precautions, which was updated via merging with the Hydrocephalus Clinical Research Network (HCRN) protocol. This study aimed to investigate the ventriculoperitoneal shunt (VPS) infection rates in pediatric patients following the implementation of the adapted protocol. METHODS: The adapted protocol was implemented in all first-time VPS implantations between 2011 and 2021. The primary outcome was a 6-month shunt infection. The Pearson chi-square test was used for categorical variables and the Mann-Whitney U-test for numeric variables to evaluate the correlation with shunt infection. RESULTS: A total of 352 first-time VPS procedures were performed adhering to the adapted protocol. The median age was 5 months, and 189 (53.7%) were male. Overall, 37 patients (10.5%) experienced shunt infection, with 30 (8.5%) occurring within the first 6 months, which was lower than 13.3% infection rate of the previous series of the same center. The infection rate was slightly higher than the 5.7% and 6.0% rates reported by HCRN studies. Patients with shunt infection were significantly younger (7.5 versus 17.5 months, P < 0.001). CONCLUSIONS: This study validates the efficacy of an adapted perioperative protocol in mitigating shunt infection in a high-volume center in a LMIC. Adhering to a step-by-step protocol, modified to suit the healthcare resources and financial constraints of LMICs, could maintain low shunt infection rates that are roughly comparable to those of centers in high-income countries.

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.

Perinatal Presentation of a Congenital Intramedullary Capillary Hemangioma in a Neonate Born with Hydrocephalus and Paraplegia.

Spinal intramedullary capillary hemangioma is a rare condition. Although most spinal/spinal cord hemangiomas are inborn in origin, perinatal presentation is uncommon. We present a neonate with an intramedullary hemangioma, born with communicating hydrocephalus and complete paraplegia. Spinal imagining showed an intradural mass with hemorrhagic foci, a low-lying conus medullaris, and scalloping of dorsal elements. Ventriculoperitoneal shunting was performed in an emergent setting, with cerebrospinal fluid showing a brownish appearance with high viscosity which implied a possible old hemorrhage. Tissue sample was taken from the spinal lesion in an elective setting. Histopathological examination revealed capillary lobules and extralobular large vessels resembling abnormal veins. The mentioned clues suggested that this congenital hemangioma had become symptomatic from the prenatal period. Though it is a are event, it should be kept in mind while evaluating a neonate with communicating hydrocephalus and weakness of extremities with or without cutaneous hemangiomatous stigmata.

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.

Odd presentation of shunt malfunction: a case series and review of literature.

OBJECTIVE: Children with previous ventriculoperitoneal shunt (VPS) insertion due to hydrocephalus may refer to the hospital with various clinical complaints. Shunt malfunction is frequently diagnosed in these children necessitating shunt revision. Although increased head circumference, setting sun eye in younger children, and headache, nausea/vomiting, loss of consciousness, visual disturbance, and other signs of intracranial hypertension are common clinical manifestations of shunt malfunction, some patients may present with odd or unusual symptoms. Here, we present a series of patients with shunted hydrocephalus who presented with odd and unexpected clinical manifestations of shunt malfunction. METHODS: Eight children with shunt malfunction were enrolled in this series. The age, sex, age of shunting, etiology of hydrocephalus and management, post-shunt insertion symptoms/sign, revision surgery, outcome, and follow-up were evaluated. RESULTS: Patients were aged from 1 to 13 years (mean, 6.38 years). There were 5 males and 3 females. The odd presentation associated with shunt malfunction included facial palsy in three children, ptosis in 3 children, and torticollis and dystonia each in one child. All patients underwent shunt revision except for one patient in whom a new shunt was inserted. Follow-up showed improvement of the symptoms in all patients. CONCLUSION: In this series, we reported eight patients with unusual signs and symptoms following shunt malfunction that were successfully diagnosed and managed.

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.

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.

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.

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.

External-internal cranial expansion to treat patients with craniocerebral disproportion due to post-shunt craniosynostosis: a case series.

INTRODUCTION: Secondary craniosynostosis subsequent to shunting is one of the late complications of ventricular shunt placement in the early childhood. Several interventions have been used to treat high intracranial pressure associated with this condition. This study aimed to evaluate the patients' clinical symptoms and head circumference before and after a method of decompressive craniotomy, coined as external-internal cranial expansion (EICE). METHODS: A retrospective study was conducted, and the patients who had undergone EICE for the treatment of post-shunt craniosynostosis between 2010 and 2020 were enrolled. This approach was a combination of a hinge multiple-strut decompressive craniectomy and internal cranial flap thinning by drill. Data, extracted from medical records, were used to evaluate the patients' symptoms and head circumferences before and 12 months after surgery. RESULTS: A total of 16 patients were enrolled in the study, of which eight were females. Before the surgery, 9 patients (56.2%) suffered from visual impairment, and all had intractable headache. Papilledema was recorded in all, with 3 cases having optic disc paleness. After cranial expansion, only two patients had headaches, diagnosed as migraine-type and psychosomatic headaches, respectively. In two patients, progressive visual impairments got worsening after surgery, which would be due to severe preoperative optic nerve atrophy. Patients' head circumferences significantly increased after the surgery (mean of 48.97 ± 4.28 cm vs. 45.78 ± 4.31 cm; P value < 0.0001). CONCLUSION: In lower resource countries, where newer technologies like distraction osteogenesis is not easily available, external-internal cranial expansion can be considered an effective alternative for patients with post-shunt craniosynostosis.

International Women in Neurosurgery.

The history of women in neurosurgery worldwide has been characterized by adversity and hardships in a male-dominated field, where resilient, tenacious, and ingenious women have nevertheless left their mark. The first women in neurosurgery appeared in Europe at the end of the 1920s, and since then have emerged in all continents in the world. Women neurosurgeons all over the globe have advanced the field in numerous directions, introducing neurosurgical subspecialties to their countries, making scientific and technical advances, and dedicating themselves to humanitarian causes, to name a few. The past 30 years, in particular, have been a period of increasing involvement and responsibility for women in neurosurgery. We must now focus on continual system improvements that will promote a diverse and talented workforce, building a welcoming environment for all aspiring neurosurgeons, in order to advance the specialty in the service of neurosurgical patients.

Gorham-Stout disease of skull base leading to cranial settling and rhinorrhea: a case-based review.

PURPOSE: Gorham-Stout disease (GSD) is a rare progressive osteolytic disorder, theoretically caused by lymphovascular endothelial proliferation. Spinal involvement carries a dismal prognosis because of neurological consequences. Lesions of the skull base are extremely rare and entail even more devastating prognosis due to cervical instability and cerebrospinal fluid (CSF) leakage. Due to scarcity of this condition, the aim of this study was to give an overview of skull base GSD and review the cases with such condition reported in the literature. METHODS: In this case-based review, different aspects of skull base GSD are discussed, and a sample clinical case of GSD leading to cranial settling and rhinorrhea is presented. The characteristics, symptoms, and managements of all English-language PubMed-reported cases were reviewed, and different features of presentation and methods of treatments were analyzed. RESULTS: Based on the literature review, most of the cases encountered serious problems in the course of the disease. Meningitis/CSF leakage was detected in 12 of 26 collected cases, followed by hearing loss/tinnitus/otitis media in 10 cases, headache in 8, and neck pain/stiffness in 8 patients. Despite a variety of treatments, improvement was only observed in 8 of 26 collected cases. The reminders showed either stable condition or worsening and death. CONCLUSION: All cases of GSD of the skull base should be evaluated for rhinorrhea/otorrhea and cranial settling, both of them being among the most life-threatening conditions. Since definite treatment, in order to stop disease progression, is sometimes impossible, symptomatic and supportive treatment should be started as possible.

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.

Spontaneous regression of congenital brain tumors: a report of two cases.

BACKGROUND: Vanishing brain tumor is defined as spontaneously disappearing or decreasing of the initial brain mass volume to ≤ 70% before establishing the definitive diagnosis. The condition is rare and can be attributed to different factors. The exact mechanism is under debate, but the increasing rate and accuracy of neuroimaging studies and occurrence of similar scenario in other pathologies rather than brain tumors can be of particular importance in finding vanishing brain lesions. CASE REPORT: We present two unusual cases of congenital brain masses which underwent spontaneous shrinkage within the first months of life. CONCLUSION: The condition is scarcely observed in congenital brain masses. As congenital brain lesions are distinct entities with peculiar characteristics, this rare phenomenon may reflect different aspects in this age group.

Prenatal ventriculomegaly: natural course, survival, and neurodevelopmental status.

OBJECTIVE: Prenatal ventriculomegaly is classified as mild, moderate, or severe based on the atrium diameter. The natural course and intrauterine progression of mild and moderate ventriculomegaly associated with the neurodevelopmental status of these children has been widely reported. METHODS: One hundred twenty-two pregnancies with mild and moderate ventriculomegaly referred to the pediatric neurosurgery clinic of Children's Medical Center between 2010 and 2018 were retrospectively studied. The authors collected demographic and first and sequential ultrasonographic information, associated abnormalities, information about pregnancy outcomes, and the latest developmental status of these children according to Centers for Disease Control and Prevention criteria by calling parents at least 1 year after birth. RESULTS: The mean gestational age at the time of diagnosis was 29.1 weeks, and 53% of fetuses were female. The width of the atrium was registered precisely in 106 cases, in which 61% had mild and 39% had moderate ventriculomegaly. Information on serial ultrasound scans was collected in 84 cases in which ventriculomegaly regressed in 5, remained stable in 67, and progressed in 12 patients. Fourteen cases (29.7%) in the mild ventriculomegaly group and 6 cases (16.2%) in the moderate group had associated abnormalities, with corpus callosum agenesis as the most frequent abnormality. The survival rate was 80% in mild and 89.4% in moderate ventriculomegaly. Considering survival to live birth and progression of the ventriculomegaly, the survival rate was 100% in regressed, 97% in stable, and 41.6% in progressed ventricular width groups (p < 0.001). Neurodevelopmental status was evaluated in 73 cases and found to be normal in 69.8% of the cases; 16.4% of children had mild delay, and 5.4% and 8.2% of cases were diagnosed with moderate and severe delays, respectively. CONCLUSIONS: In spite of a high rate of missed data in our retrospective study, most patients with mild or moderate ventriculomegaly had a stable or regressed course. Most cases had near-normal developmental status. Prospective studies with a larger sample size and detailed developmental evaluation tests are needed to answer the questions related to the natural course, survival, and prognosis of prenatal ventriculomegaly.

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).