Congenital Aqueductal Stenosis Research Articles

Third Ventricular Diameter is Inversely Related to Thalamic Massa Intermedia Thickness in Hydrocephalus Caused by Congenital Aqueductal Stenosis.

In fetuses with lateral ventriculomegaly and normal posterior fossa cerebrospinal spaces, third ventricular distention is a compelling clue that supports a diagnosis of aqueductal stenosis. However, this association assumes normal ventricular anatomy. Structural constraints can impair pressure-induced compliance. We aimed to determine how thalamic massa intermedia (TMI) size alterations may impact the size of the 3rd ventricle in the setting of congenital aqueductal stenosis (CAS). This retrospective study was performed at a single academic pediatric hospital after IRB approval. We searched our brain MRI reports for all exams describing "aqueductal stenosis" and included all the patients who had both fetal and postnatal exams. Patients with interhypothalamic adhesions and hydrocephalus unrelated to CAS were excluded from this study. We evaluated all the MRIs for the presence of TMI and documented third ventricle diameters (supraoptic recess, central, and suprapineal recess) and the TMI circumference. Spearman correlation was used to identify the potential relationship between the TMI circumference and 3rd ventricle size in fetal and postnatal MRIs. Patients were also stratified into two groups based on the presence or absence of TMI. Mann-Whitney U tests were used to compare third ventricle diameters between these groups. The study included both fetal and postnatal studies from 59 patients. The overall third ventricular diameter was inversely proportional to the circumference of the TMI in both groups (fetal: p=0.001, rho=-0.422, CI=[-0.628-0.181]; postnatal: p<0.001, rho=-0.653, CI= [-0.782-0.479]). Nonetheless, dilation of anterior and posterior recesses still occurred when the mid third ventricle was non-dilated or less severely dilated in patients with an enlarged TMI. Third ventricular dilation was most severe in patients lacking a TMI compared to patients with a TMI (p<0.001). In patients with suspected congenital aqueductal stenosis, lack of significant third ventriculomegaly as conventionally measured can sometimes be explained by thickening of the TMI. In this circumstance, it is important to evaluate the extreme recesses of the 3rd ventricle for evidence of dilatation on fetal MRI.ABBREVIATIONS: TMI = Thalamic massa intermedia; CAS = Congenital aqueductal stenosis.

Scrotal Migration of The Distal End of The Ventriculoperitoneal Shunt: Is There a Role for Early Closure of Patent Processus Vaginalis in The Pediatric Population?

Background: Ventriculoperitoneal shunt (VP) insertion is one of the surgical treatments for patients with hydrocephalus. Infection, malfunction, catheter migration, hernia development, equipment failure, CSF subcutaneous collection and peritoneal pseudocyst formation are considered to be the main complications of this procedure. Case Description: A 6-months-old boy known to have congenital aqueductal stenosis with VP shunt insertion at the age of 1 month, presented with scrotal swelling and generalized hypotonia. He was found to have catheter migration into the scrotum. Conclusion: Scrotal migration of the distal end of the ventriculoperitoneal shunt is a rare but threatening complication that can present with either signs of hydrocephalus or scrotal swelling or hydrocele. Accordingly, we recommend performing a scrotal exam with an ultrasound early upon evaluation of any infant presenting with scrotal swelling or hydrocele after VP shunt insertion. There haven’t been clear recommendations in the literature concerning closure of patent processus vaginalis upon placing a VP shunt in patients below the age of 3 months. Thus, we question whether early closure could help reduce the complications of catheter migration in infants.

329 Prenatally identified congenital aqueductal stenosis: short- and long-term neurodevelopmental outcomes

329 Prenatally identified congenital aqueductal stenosis: short- and long-term neurodevelopmental outcomes

Scrotal migration of the distal end of the ventriculoperitoneal shunt: Is there a role for early closure of patent processus vaginalis in the pediatric population?

Background: Ventriculoperitoneal shunt (VP) insertion is one of the surgical treatments for patients with hydrocephalus. Infection, malfunction, catheter migration, hernia development, equipment failure, CSF subcutaneous collection and peritoneal pseudo cyst formation are considered to be the main complications of this procedure. Case description: A 6-months-old boy known to have congenital aqueductal stenosis with VP shunt insertion at the age of 1 month, presented with scrotal swelling and generalized hypotonia. He was found to have catheter migration into the scrotum. Conclusion: Scrotal migration of the distal end of the ventriculoperitoneal shunt is a rare but threatening complication that can present with either signs of hydrocephalus or scrotal swelling or hydrocele. Accordingly, we recommend performing a scrotal exam with an ultrasound early upon evaluation of any infant presenting with scrotal swelling or hydrocele after VP shunt insertion. There hasn’t been clear recommendations in the literature concerning closure of patent processus vaginalis upon placing a VP shunt in patients below the age of 3 months. Thus, we question whether early closure could help reduce the complications of catheter migration in infants.

A Study of Ventriculoperitoneal Shunt in Management of Pediatric Hydrocephalus

Background: Hydrocephalus is one of the most common clinical conditions affecting the central nervous system and Ventriculoperitoneal (VP) shunt is the most common procedure used. We report an outcome analysis of various risk factors and etiological factors for shunt failure in pediatric patients from a single institution. Methods: A single institutional cross-sectional observational study was conducted in 45 operated cases of hydrocephalus below 16 years of age from 2020 to 2022. Etiology, various risk factors, surgery time, cerebrospinal fluid (CSF) analysis and shunt complications were the parameters analysed. The association of parameters was analysed using the Chi-square test and binary logistic regression. Results: Forty-five patients who fulfilled the inclusion criteria were enrolled in the study. About 57.8% of patients were male, and the mean age of patients at the time of VP shunt was six months. The majority of the patients belonged to 0-1 years of age with 68.9%. The commonest etiology of hydrocephalus was congenital aqueduct stenosis (42.22%). Overall shunt complication was found in 20% of patients. Chi-Square analysis revealed emergency surgery (p = 0.01) compared to elective surgery, long duration of surgery (≥ 1 hour; p = 0.01) compared to short duration of surgery (&lt; 1 hour), and increased protein content in CSF analysis (p = 0.001) were the significantly associated with shunt failure. But only increased CSF analysis (p = 0.04) was found to be significant predictor of shunt complication when adjusted with other significant variables in binary logistic regression. Conclusion: Emergency surgery, long duration of surgery, and increase protein content in CSF analysis were found to be associated with shunt complications and protein CSF was found to be significant predictor of shunt failure after adjusting with other variables.

The Role of Early Postoperative Intracranial Pressure Monitoring in Predicting the Outcome of Endoscopic Third Ventriculostomy Performed in Infants With Congenital Hydrocephalus: A Prospective Analysis.

IntroductionHydrocephalus is an excessive accumulation of cerebrospinal fluid (CSF) in the cavity and spaces of the brain. To date, there is no single method to accurately assess the compliance of subarachnoid spaces after endoscopic third ventriculostomy (ETV).ObjectiveTo analyze the intracranial pressure (ICP) trends in the early postoperative period in infants undergoing ETV for congenital hydrocephalus and correlate them with the final outcome.Material and methodsThis is a single-center prospective study conducted at the Department of Neurosurgery of our institute from January 2019 to February 2020. Infants presented with congenital hydrocephalus mandating ETV were included in the study. ICP was continuously monitored for the first three days after the procedure. ICP values were recorded hourly, and 24 ICP values obtained daily were averaged to obtain a daily average value (DAV).ResultsForty patients were recruited in the study. The mean age of the study population was 4.7 ± 2.8 months; 80% of the infants were <6 months of age. The male/female ratio was 5.7:1. The most common etiology was congenital aqueductal stenosis, which was observed in 18 (45%) of the patients, followed by Dandy-Walker malformation (DWM) in 11 (27.5%) of the patients. On considering a difference of >1 mmHg between the first and third postoperative day, the ETV success rate was dropped from 50% in stable trend to 11% in progressive increase trend, which was statistically significant (p = 0.044). At DAV variation of >2 mmHg in progressive increase trend, the sensitivity of stable ICP trend increased to 100% in predicting ETV success. Also, the negative predictive value (the ability of a stable trend to rule out ETV failure) reached 100%. The overall success rates of ETV in our study at one, three, and six months were 62.5%, 40%, and 35%, respectively.ConclusionA progressive increase in the ICP trend (with a difference of >2 mmHg between postoperative days 1 and 3) was the best predictor of ETV failure in our study. It was superior to any other clinical or radiological variable in our study, which was affecting the outcome.

Neuroophthalmological manifestations of congenital aqueductal stenosis.

Congenital aqueductal stenosis (CAS) is a common etiology of hydrocephalus that occurs in a subset of infants and may be linked to an increased incidence of ophthalmological abnormalities and delayed developmental milestones. Although hydrocephalus is common and widely studied, sparse literature exists on patients with isolated (no identifiable genetic link) CAS along with analysis of ophthalmological manifestations. In this study, the authors sought to describe the ophthalmological abnormalities and delayed developmental milestones of patients with isolated CAS. Data of patients with CAS were prospectively entered and monitored in a surgical database maintained by the Department of Neurological Surgery at Children's Hospital of Pittsburgh from January 2005 to October 2016. Patients with a family history of congenital hydrocephalus, positive testing for genetic forms of aqueductal stenosis, other congenital abnormalities suggesting an underlying genetic syndrome, and stenosis/obstruction due to secondary causes were excluded from this study. Prenatal and perinatal history, CSF diversion history, and a variety of outcomes, including ophthalmological deficits and developmental milestones, were collected and analyzed. A total of 41 patients with isolated CAS were identified, with a mean follow-up duration of 6 years. Among that cohort, 26 patients (63.4%) developed neuroophthalmological complications, which were further stratified. Fourteen patients (34.1%) developed strabismus and 11 (26.8%) developed astigmatism, and 1 patient (2.4%) with papilledema was recorded. Among patients with ophthalmological abnormalities, 76.9% had delayed developmental milestones (p = 0.045). Patients with CAS were found to have increased risk of ophthalmological abnormalities requiring correction, along with an increased risk of delayed developmental milestones. Importantly, there was a significant correlation between the development of ophthalmological abnormalities and delayed developmental milestones that was independent of CSF diversion history. Larger patient cohort studies are required to explore whether earlier development of hydrocephalus, as is the case in CAS, causes elevated rates of neurological and ophthalmological complications, and if earlier CSF diversion correlates with improved outcomes.

Age Related Role of Endoscopic Third Ventriculostomy in Treatment of Hydrocephalic Children

Goal: To understand endoscopic third ventriculostomy (ETV) surgical result in the hydrocephalus. Setting: Benha University Hospitals Department of Neurosurgery. Between January 2016 to February 2020. Materials and processes: A total of 58 hydrocephalus patients were included in this research, regardless of gender prejudice. CSF loculation or tumour hydrocephalus was excluded. Hydrocephalus CT scan brain and MRI were diagnosed. Information on patient specifics, causes of hydrocephalus and operation problems were recorded in Performa patient. The data were examined in version 26 of SPSS. Frequency and percentage for categorical variables were computed. Mean ± SD for age has been computed. Results were presented as tables. Results: The research comprised a total of 58 individuals with hydrocephalus. Of the 58 patients, 32 were male (55.2 per cent) and 26 were female (44.8 percent ). The average age was 22.86 months. The most frequent cause of hydrocephalus was congenital aqueductal stenosis. CSF leaking was observed in 20 individuals after surgery (34.5 percent ). 6 individuals have postoperative infection (10.3 percent ). Conclusions: third ventriculostomy endoscopic is safe and effective. Patients who fulfil the requirements are offered the option of freeing themselves from shunt dependence via endoscopic third ventriculostomy.

An adjustable gravitational valve for initial VP-shunt treatment in hydrocephalic preterm neonates and infants below 1 year of age

ObjectiveShunt treatment for hydrocephalus in children should aim for sustainable flexibility in regard to optional, perspective pressure level adjustment during advancing physical and mental development. Gravitation-assisted shunt valves are designed to prevent hydrostatic over-drainage frequently observed in the long course of shunt-treated hydrocephalus. We prospectively studied and analyzed the implication, safety, and feasibility for an adjustable gravitational unit combined with a fixed differential-pressure (DP) valve for neonates and infants primary shunted within the first 12 months of life.MethodsClinical course of hydrocephalic neonates and infants who received initial VP-shunt insertion in the early post-natal phase were monitored prospectively on the basis of our digital institutional Hydrocephalus & Shunt Registry. All patients were equipped with a fixed DP valve combined with a programmable gravitational unit activated in upright body position. Patients with a minimum shunt follow-up of 24 months were considered for further statistical analysis regarding hydrocephalus etiology, surgical setting, pre- and post-operative ventricular enlargement, head circumference, valve pressure setting, implication for the adjustment option of the gravitational unit, type and number of shunt complications, and revision-free shunt and valve survival.ResultsSeventy-eight pediatric patients received primary VP-shunt insertion at a mean age of 10 weeks with age gestationally corrected for preterm neonates. Hydrocephalus was related to perinatal IVH (64%), CNS malformation (11%), spina bifida (9%), congenital aqueductal stenosis (9%), and idiopathic (4%) or post-infectious etiology (3%). Fifty-two patients (70%) presented with history of prematurity (gestational age 23–36 weeks). Regular follow-up carried out for a mean period of 63 months demonstrated that ventricular enlargement decreased significantly after applied treatment and excessive head growth could be counteracted effectively. At least one pressure level adjustment was performed in 31% of all patients after 12 months, in 42% after 24 months, and in 64% at the time of last clinical follow-up since initial shunt insertion. Pressure level adjustments were successful in cases of clinical or radiographic signs of under- or over-drainage for individual patients of various ages during entire clinical course. Mean pressure setting for upright position was 24.1 cm H2O at the time of initial shunt insertion and increased to 26.4 cmH2O at the time of last clinical follow-up. Revision-free shunt-survival rates after 12 and 24 months were 79% and 70% and valve-survival rates 91% and 90%, respectively.ConclusionThe combination of a fixed DP valve with an adjustable gravitational unit utilized as first-line shunt regimen was feasible and safe in a highly vulnerable subgroup of hydrocephalic infants. The adjustment option for the gravitational unit showed frequent and increasing implication over time and was beneficial even during the very early developmental stage of limited autonomous mobility. To our knowledge this is the first ever reported long-term investigation of an age-consistent pediatric patient collective primary shunted with an adjustable gravitational valve system.

Letter to the Editor Regarding “Prospective Study of Surgery for Traumatic Brain Injury in Addis Ababa, Ethiopia: Trauma Causes, Injury Types, and Clinical Presentation”

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Hydrocephalus Research.

This paper highlights the hydrocephalus research efforts undertaken at AIIMS, New Delhi, supporting progress in the field. Over a period of three decades, basic research, clinical investigations, and multicentric studies were undertaken. This report will review the work mainly to emphasize the need for future generations to pursue further research. Studies that impacted hydrocephalus care (mainly in India) are described, and some of these findings may be useful in other resource-challenged situations. Investigative studies on the effect of shunting on brainstem auditory evoked responses (BAER), transcranial Doppler (TCD), and CT-SPECT were published offering management options for patients. Participation in the International Infant Hydrocephalus Study (IIHS) study offered opportunities to compare our approaches and develop modifications in patient care. This effort proved shunting was equal or better for young children with congenital aqueductal stenosis. Shunt infection protocols and changes made in a systematic manner helped develop local protocols to reduce postoperative shunt infections. Hydrocephalus research over three decades at AIIMS, New Delhi was productive and educational, confirming that locally performed investigative work can help in decision making. Further studies and active participation in international efforts are necessary to advance the field.

Pediatric Ventriculoperitoneal Shunt: Outcome Analysis in a Tertiary Care Center in Nepal

Introduction Ventriculoperitoneal (VP) shunt is a commonly performed neurosurgical procedure in pediatric surgical practice. It results in a dramatic improvement in patient survival and neurological function but is associated with several complications requiring multiple shunt revisions throughout a patient’s lifetime. The main aim of the study was to evaluate the outcome especially complications of shunt surgery at our center.&#x0D; MethodsA single-institutional, retrospective observational study was conducted in 60 operated cases of congenital hydrocephalus with a minimum follow-up for up to 6 months. Parameters for analysis included demographic data, etiology of hydrocephalus, surgery time, and type of complications.&#x0D; ResultsOut of 86 patients, 60 patients who fulfilled the inclusion criteria were enrolled in the study. The mean age of the patients at the time of VP shunt placement was 3 months, range from 8 days to 15 years with male preponderance. The majority of the patients belonged to the 0-1-year age group (76.8%). The commonest etiology of hydrocephalus was congenital aqueduct stenosis. Fifteen patients (25%) developed complications within six months follow-up period. Eight patients (13.3%) developed clinical shunt infection with four cases having culture positive. Four (6.67%) had mechanical complications in the form of shunt obstruction. Three patients had ascites, subdural collection, and shunt extrusion from the anus. The shunt related mortality was 1.67%.&#x0D; ConclusionThe shunt infection and obstruction still remain the most important complications. Despite inherent problems, shunt surgery is the procedure of choice until a safer effective alternative is available.

Pediatric Ventriculoperitoneal Shunt: Outcome Analysis in a Tertiary Care Center in Nepal

Introduction: Ventriculoperitoneal (VP) shunt is a commonly performed neurosurgical procedure in pediatric surgical practice. It results in a dramatic improvement in patient survival and neurological function but is associated with several complications requiring multiple shunt revisions throughout a patient’s lifetime. The main aim of the study was to evaluate the outcome especially complications of shunt surgery at our center. Methods: A single-institutional, retrospective observational study was conducted in 60 operated cases of congenital hydrocephalus with a minimum follow-up for up to 6 months. Parameters for analysis included demographic data, etiology of hydrocephalus, surgery time, and type of complications. Results: Out of 86 patients, 60 patients who fulfilled the inclusion criteria were enrolled in the study. The mean age of the patients at the time of VP shunt placement was 3 months, range from 8 days to 15 years with male preponderance. The majority of the patients belonged to the 0-1-year age group (76.8%). The commonest etiology of hydrocephalus was congenital aqueduct stenosis. Fifteen patients (25%) developed complications within six months follow-up period. Eight patients (13.3%) developed clinical shunt infection with four cases having culture positive. Four (6.67%) had mechanical complications in the form of shunt obstruction. Three patients had ascites, subdural collection, and shunt extrusion from the anus. The shunt related mortality was 1.67%. Conclusion: The shunt infection and obstruction still remain the most important complications. Despite inherent problems, shunt surgery is the procedure of choice until a safer effective alternative is available.

Ventriculoperitoneal Shunt Complication in Pediatric Hydrocephalus: Risk Factor Analysis from a Single Institution in Nepal.

Objective:Ventriculoperitoneal (VP) shunt surgery is one of the commonly performed neurosurgical procedures. Complications due to shunt failure are associated with high morbidity and mortality. We report an analysis of risk factors for shunt failure in pediatric patients from a single institution in Nepal.Materials and Methods:A retrospective analytical study with prospective data was designed. All children younger than 15 years, with first time VP shunting, at a tertiary government hospital in Kathmandu during 2014-2017 were followed up. Association of independent variables with the primary outcome variable (complication of VP shunt) was analyzed using Chi-square test. Bivariate logistic regression was performed to identify unadjusted odds ratio (OR) with 95% confidence interval (CI). Multivariate logistic regression model was designed to calculate adjusted OR with 95% CI.Results:Of 120 patients, more than half (55.8%) of the patients were male. Mean age was 62.97 months. Maximum duration of follow-up was 30 months. Most common cause of hydrocephalus was congenital aqueductal stenosis (40.8%) followed by tumors (29.2%). Overall shunt complication was found in 26.7% (95% CI 19.0%–35.5%). Shunt infection was seen in 5% while malfunction without infection was found in 21.7%. Bivariate logistic regression showed duration of surgery more than 1 h (OR 2.67, 95% CI 1.11–6.42, P = 0.028) compared to 1 h or less, experienced surgeon (OR 0.37, 95% CI 0.16–0.89, P = 0.026) compared to residents, and emergency surgery (OR 3.97, 95% CI 1.69–9.29, P = 0.001) compared to elective surgery as significant risk factors, while emergency surgery was the only significant variable for shunt failure on multivariate regression analysis (OR 3.3, 95% CI 1.16–9.35, P = 0.025).Conclusion:Longer duration of surgery, less experience of the surgeon, and the priority of the case (emergency) were independent risk factors for shunt complications.

The efficacy of endoscopic third ventriculostomy in children 1 year of age or younger: A systematic review and meta-analysis

Hydrocephalus is a major cause of morbidity in the pediatric population, with potentially severe consequences if left untreated. Two viable strategies for management of non-communicating hydrocephalus are endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunting. However, there is uncertainty over the safety and efficacy of ETV in younger infants aged 1 year or below. In this systematic review, we aim to elucidate the success rate and procedural risks of ETV in this age group. A multi-database (PubMed, Embase, Web of Science) literature search between January 1990 and April 2018 was performed in accordance with PRISMA guidelines. Eligible studies were included if they (i) examined non-communicating hydrocephalus; (ii) quantified the success/failure rates of ETV; and (iii) assessed outcomes in children 1 year of age or younger. A total of 19 articles with 399 patients were eligible for inclusion. Mean age at procedure was 4.2 months (range 34 weeks gestation to 12 months), with 116 females and 143 males. Commonest underlying aetiology was congenital aqueductal stenosis (AS) (60.4%). Remaining causes included post-haemorrhagic, post-infection, Chiari malformations, malignancies and others. Overall and AS mean success rates were 51.6% and 56.5% respectively. Overall complication rate was 10.0%, consisting mainly of CSF leak, infection, and haemorrhage. Younger age was significantly associated with poorer ETV success rate when divided into <6 months and 6-12 months of age (44.4 vs 66.7%; p=0.0007). Underlying pathology had no significant association with ETV outcome when divided into AS and other pathologies (p=0.53). Age is significantly associated with ETV success rates. Pathology-dependent effects were not found in this age group. Despite a lower ETV success rate at younger ages (44.4 vs 66.7%), it offers a comparable safety profile that is independent of age. ETV remains a viable treatment option for non-communicating hydrocephalus for infants aged 1 year or younger.

Evaluation Of Findings On Imaging Of Brain In Children With First Recognized Episode Of Fits-Experience At Tertiary Care Hospital Of Quetta

Objective: To evaluate various causes of fits in children on MRI presenting in Tertiary Care Hospital, Quetta. Study Design and Setting: A Cross sectional study was conducted in the department of Radiology, Bolan Medical Complex Hospital Quetta from October 2017 to March 2018. Methodology: A total of 100 children aged 06 months to 13 years were included in the study who presented with seizures to emergency department and neuro clinics. Information obtained from history, clinical examination and MRI brains were recorded. The data was analyzed in SPSS 20. Results: Among the total 100 children, MRI examination was unremarkable in 45% (n=45). Neoplastic lesions were the second most common abnormal MRI finding and constituted 10% (n=10). Perinatal ischemia and Periventricular leucomalacia were recorded in 9% (n=9). Congenital aqueductal stenosis in 9% (n=9), along with Encephalitis/Meningitis also in 8% (n=8). Brain atrophy was noted in 6% (n=6). Three cases each of Vascular and Post traumatic changes/gliosis (n=6, 6%) and one case of each of Hydrocephalus/Aqueductal stenosis, Infarct, Malformation of cortical development, Leucodystrophy, Agenesis of corpus callosum, Arachnoid cyst and Hydatid cyst (n=7, 7%) Conclusion: Brain magnetic resonance imaging was successful in detecting structural abnormalities and it can be trusted to detect seizure foci in pediatric patients.

Fetal brain MRI findings and neonatal outcome of common diagnosis at a tertiary care center.

Identify common brain malformations on fetal MRI and evaluate perinatal course. Fetal consultations from 10/2016 to 12/2017 reviewed. Hundred consultations were requested; 94 were completed. Findings included: posterior fossa malformations (19%), agenesis/dysgenesis of corpus callosum (15%), congenital aqueductal stenosis (CAS) (14%), ventriculomegaly (11%), isolated cortical malformations (8.5%), and holoprosencephaly (6%). Posterior fossa malformations were more likely to be associated with genetic conditions and cardiac malformations. Patients with CAS all required intensive care unit admission. Overall, few patients with congenital brain malformations required feeding or respiratory support at discharge. None had seizures as neonates except two with early epileptic encephalopathy syndromes. Even though long term neurological prognosis is poor for many conditions including high lifetime risk of epilepsy, most are discharged with no feeding or respiratory support. Seizures are rarely seen in the neonatal period.

Comparing the Efficiency of Two Treatment Methods of Hydrocephalus: Shunt Implantation and Endoscopic Third Ventriculostomy.

Introduction:Hydrocephalus is one of the most common diseases in children, and its treatment requires brain operation. However, the pathophysiology of the disease is very complicated and still unknown.Methods:Endoscopic Third Ventriculostomy (ETV) and Ventriculoperitoneal Shunt (VPS) implantation are among the common treatments of hydrocephalus. In this study, Cerebrospinal Fluid (CSF) hydrodynamic parameters and efficiency of the treatment methods were compared with numerical simulation and clinical follow-up of the treated patients.Results:Studies have shown that in patients under 19 years of age suffering from hydrocephalus related to a Posterior Fossa Brain Tumor (PFBT), the cumulative failure rate was 21% and 29% in ETV and VPS operation, respectively. At first, the ETV survival curve shows a sharp decrease and after two months it gets fixed while VPS curve makes a gradual decrease and reaches to a level lower than ETV curve after 5.7 months. Post-operative complications in ETV and VPS methods are 17% and 31%, respectively. In infants younger than 12 months with hydrocephalus due to congenital Aqueduct Stenosis (AS), and also in the elderly patients suffering from Normal Pressure Hydrocephalus (NPH), ETV is a better treatment option. Computer simulations show that the maximum CSF pressure is the most reliable hydrodynamic index for the evaluation of the treatment efficacy in these patients. After treatment by ETV and shunt methods, CSF pressure decreases about 9 and 5.3 times, respectively and 2.5 years after shunt implantation, this number returns to normal range.Conclusion:In infants with hydrocephalus, initial treatment by ETV was more reasonable than implanting the shunt. In adult with hydrocephalus, the initial failure in ETV occurred sooner compared to shunt therapy; however, ETV was more efficient.

Prenatal aqueduct stenosis: Association with rhombencephalosynapsis and neonatal outcome.

To examine prenatal MRI and postnatal imaging in fetuses with congenital aqueductal stenosis (CAS) to determine the frequency of association of rhombencephalosynapsis (RES) and how it may affect neonatal intensive care unit (NICU) course. A single center IRB-approved retrospective study of children with CAS was performed. Prenatal MRI, postnatal images, and clinical data were reviewed. Statistical analysis was performed with SAS statistical software package version 9.3. Aqueduct obstruction was confirmed for all 30 participants. Hydrocephalus required shunting in all but one (97%). Fifteen neonates had CAS with rhomboencephalosynapsis (RES) (50%). Although neonatal course between the two groups was comparable, 53% of CAS with RES neonates required feeding assistance versus 20% in CAS only (P=0.128). Shunting in the CAS with RES group occurred at average of 6days of life versus CAS group at 55days (P=0.196). Biometry measurements showed a statistically significant decrease in pons antero-posterior diameter in both groups (CAS only P=0.0049 and CAS with RES P=0.0003) when compared with norms for gestational age. CAS has a high association with RES. Feeding assistance in the NICU and earlier neurosurgical intervention may be required in patients with CAS who also have RES.

Atlantoaxial Subluxation Secondary to Unstable Os Odontoideum in a Patient With Arrested Hydrocephalus Due to Congenital Aqueductal Stenosis: A Case Report.

In a small percentage of children born with congenital hydrocephalus, enlargement of the head and the presence of ventriculomegaly may halt and ultimately stabilize the condition designated as arrested hydrocephalus. Arrested hydrocephalus in children is typically due to congenital aqueduct stenosis, which can be described appropriately as a stasis existing within the channel between the third and fourth ventricles. Os odontoideum (OO) is an uncommonly occurring pathology at the craniovertebral junction. Although the clinical and radiologic features of its existence and the therapeutic options for its pathology have been widely discussed within the medical literature, its true etiology has been a source of divisive debate, proposing both a traumatic as well as a congenital mechanism. The etiology of OO has been heartily debated in the literature for several years as well. Most authors have come to support a posttraumatic causality. However, strong evidence exists to support a congenital origin to this rarely observed malformation. Within this case study we present a 24-year-old woman with atlantoaxial subluxation that exists secondary to an orthotropic OO. The patient had a history of arrested hydrocephalus due to congenital aqueductal stenosis beginning in early childhood. She presented with normal intelligence and was neurologically without deficits before the occurrence of an atlantoaxial dislocation. Unfortunately, the pathology was initially misdiagnosed as a decompensation state of the arrested hydrocephalus, and after 8 months the patient became wheelchair bound. Following this unfortunate event the correct diagnosis was ultimately uncovered. Subsequently a C2-1 instrumentation procedure resulted in excellent alignment and fusion. To the best of our knowledge, this is the first example of an aqueduct stenosis in the setting of an existing OO, a combination that might be another clue in favor of a congenital etiology.