Fourth ventricle neurocysticercosis presenting with Bruns’ syndrome: A case report

Background: Neurocysticercosis (NCC) is the most common helminthic infection of the central nervous system. We present a case of the fourth ventricle NCC that presented with Bruns’ syndrome (with headaches worsened by head movements, gait ataxia, and vomiting) and the operative technique used for cyst removal. Case Description: A 39-year-old woman was admitted to the emergency department with moderate-to-severe headaches that got worse in the afternoon and were triggered by head movements, relieved on prone position, and were associated with gait ataxia and vomiting. A brain magnetic resonance imaging (MRI) showed a fourth ventricle cyst, with an eccentric enhancing nodule compatible with a larvae scolex and associated obstructive hydrocephalus. The patient was positioned prone and underwent a microsurgical resection of the cyst without rupture through a telovelar approach to the fourth ventricle. The postoperative MRI confirmed complete cyst removal and resolution of the hydrocephalus. At 12-month follow-up, the patient remains with no signs of disease recurrence. Conclusion: In this report, we depict a case of intraventricular NCC successfully treated with a single surgery, allowing intact cyst removal and achieving effective hydrocephalus treatment with no need to resort to cerebrospinal fluid diversion techniques.

Methods for increasing the efficiency of hydrocephalus treatment in neonatal practice

Posthemorrhagic hydrocephalus in newborns with ventricular occlusion leads to decompensation of CSF dynamics with a high risk of neurological disorders and child disability.Objective. To improve the effectiveness of treatment of hydrocephalus in newborns.Characteristics of children and research methods. The authors analyzed the treatment of hydrocephalus in 327 newborns in 2000– 2018. 184children (Group 1)underwent standard treatment with lumbar and ventricular punctures with 20-22G needles, and with progression of hydrocephalus – ventriculo-subgaleal drainage and ventriculo-peritoneal shunting. 143 children (Group 2) with ventricular occlusion underwentcomplex treatment with coronary-transluminal subarachnoventriculostomy (RF patent No. 2715535) and ventricular drainage with a ventriculo-subarachnoid stenting system (RF patent No. 2721455).Results. The effectiveness of treatment with the inclusion of the proposed options is improvedby eliminatingocclusion, restoring circulation and absorption of cerebrospinal fluid, prolonged sanitation of cerebrospinal fluid with isotonic sodium chloridesolution. A positive result with compensation for hydrocephalus was achievedin 75,4% of cases versus 28,2% with the «standard» protocol (p <0,001).Compensation of hydrocephalus was accompanied by regression of hypertensive symptoms, stabilization of the ventricular size. The authors demonstrate a decrease in the timethe child spent in the hospital after surgery to 3-6 weeks with dynamic observation after discharge in the follow-up room, neurovisual monitoring.>Conclusion. The data obtained allowus to consider the effectiveness of the inclusion of the proposed options in the modern algorithm for the treatment of hydrocephalus in newborns, to expand the possibilities for early rehabilitation and restorative treatment together with pediatricneurologists and doctors of related specialties.

Postnatal Repair of Open Neural Tube Defects: A Single Center with 90-Month Interdisciplinary Follow-Up

After publication of the Management of Myelomeningocele Study (MOMS) there is confusion regarding which treatment of open neural tube defects (NTD) is best. We report our results of postnatally repaired open NTDs born between 2007–2018 (n = 36) in critical reflection of the MOMS study. Neurosurgical, orthopedic, and urologic data were assessed. We also introduce a new entity: “status post prenatal repair”. FU ranged from 29 to 161 months (mean: 89.1 m) in 7 cases of myeloschisis and 24 myelomeningoceles in the final collective n = 31. The shunt rate was 41.9%, and the endoscopic third ventriculostomy rate was 16.1%. Hydrocephalus requiring treatment was not associated with the anatomical level, but with premature birth (p = 0.048). Myeloschisis was associated with shunt placement (p = 0.008). ROC analysis revealed birth <38.5th week predicts the necessity for hydrocephalus treatment (sensitivity: 89%; specificity: 77%; AUC= 0.71; p = 0.055). Eight (25.8%), patients are wheelchair-bound, 2 (6.5%) ambulate with a posterior walker, 10 (32.3%) with orthosis and 11 (35.5%) independently. One (3.2%) patient underwent detethering at 5.5 years. A total of three patients underwent five Chiari decompressions (9.6%). Further, nineteen orthopedic procedures were performed in nine patients (29.0%). A total of 17 (54.8%) patients self-catheterize, which was associated with an anatomical lesion at L3 or below (p = 0.032) and 23 (74.2%) take anticholinergic medication. In conclusion, shunt dependency is associated with myeloschisis, not with the anatomical defect level. Hydrocephalus treatment is associated with premature birth. In this postnatal cohort with significantly longer follow-up data than the MOMs study, the ambulation rate is better, the shunt rate lower and the secondary tethered cord rate better compared to the MOMS study.

Quantifying the Utility of Low Resolution Brain Imaging:: Treatment of Infant Hydrocephalus

As low-field MRI technology is being disseminated into clinical settings, it is important to assess the image quality required to properly diagnose and treat a given disease. In this post-hoc analysis of an ongoing randomized clinical trial, we assessed the diagnostic utility of reduced-quality and deep learning enhanced images for hydrocephalus treatment planning. Images were degraded in terms of resolution, noise, and contrast between brain and CSF and enhanced using deep learning algorithms. Both degraded and enhanced images were presented to three experienced pediatric neurosurgeons accustomed to working in LMIC for assessment of clinical utility in treatment planning for hydrocephalus. Results indicate that image resolution and contrast-to-noise ratio between brain and CSF predict the likelihood of a useful image for hydrocephalus treatment planning. For images with 128x128 resolution, a contrast-to-noise ratio of 2.5 has a high probability of being useful (91%, 95% CI 73% to 96%; P=2e-16). Deep learning enhancement of a 128x128 image with very low contrast-to-noise (1.5) and low probability of being useful (23%, 95% CI 14% to 36%; P=2e-16) increases CNR improving the apparent likelihood of being useful, but carries substantial risk of structural errors leading to misleading clinical interpretation (CNR after enhancement = 5; risk of misleading results = 21%, 95% CI 3% to 32%; P=7e-11). Lower quality images not customarily considered acceptable by clinicians can be useful in planning hydrocephalus treatment. We find substantial risk of misleading structural errors when using deep learning enhancement of low quality images. These findings advocate for new standards in assessing acceptable image quality for clinical use.

Efficacy and safety of flexible versus rigid endoscopic third ventriculostomy in pediatric and adult populations: a systematic review and meta-analysis

Endoscopic third ventriculostomy (ETV) is a well-established surgical procedure for hydrocephalus treatment, but there is sparse evidence on the optimal choice between flexible and rigid approaches. A meta-analysis was conducted to compare efficacy and safety profiles of both techniques in pediatrics and adults. A comprehensive search was conducted on PubMED, EMBASE, and Cochrane until 11/10/2019. Efficacy was evaluated comparing incidence of ETV failure, while safety was defined by the incidence of perioperative complications, intraoperative bleedings, and deaths. Random-effects models were used to pool the incidence. Out of 1365 studies, 46 case series were meta-analyzed, yielding 821 patients who underwent flexible ETV and 2918 who underwent rigid ETV, with an age range of [5 days–87 years]. Although flexible ETV had a higher incidence of failure in adults (flexible: 54%, 95%CI: 22–82% vs rigid: 20%, 95%CI: 22–82%) possibly due to confounding due to etiology in adults treated with flexible, a smaller difference was seen in pediatrics (flexible: 36%, pediatric: 32%). Safety profiles were acceptable for both techniques, with a certain degree of variability for complications (flexible 2%, rigid 18%) and death (flexible 1%, rigid 3%) in pediatrics as well as complications (rigid 9%, flexible 13%), death (flexible 4%, rigid 6%) and intra-operative bleeding events (rigid 6%, flexible 8%) in adults. No clear superiority in efficacy could be depicted between flexible and rigid ETV for hydrocephalus treatment. Safety profiles varied by age but were acceptable for both techniques. Well-designed comparative studies are needed to assess the optimal endoscopic treatment option for hydrocephalus.

Hydrocephalus treatment in patients with craniosynostosis: an analysis from the Hydrocephalus Clinical Research Network prospective registry

OBJECTIVE Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis. METHODS Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated. RESULTS In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC). CONCLUSIONS This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Multimodal Sensing Capabilities for the Detection of Shunt Failure

Hydrocephalus is a medical condition characterized by the abnormal accumulation of cerebrospinal fluid (CSF) within the cavities of the brain called ventricles. It frequently follows pediatric and adult congenital malformations, stroke, meningitis, aneurysmal rupture, brain tumors, and traumatic brain injury. CSF diversion devices, or shunts, have become the primary therapy for hydrocephalus treatment for nearly 60 years. However, routine treatment complications associated with a shunt device are infection, obstruction, and over drainage. Although some (regrettably, the minority) patients with shunts can go for years without complications, even those lucky few may potentially experience one shunt malfunction; a shunt complication can require emergency intervention. Here, we present a soft, wireless device that monitors distal terminal fluid flow and transmits measurements to a smartphone via a low-power Bluetooth communication when requested. The proposed multimodal sensing device enabled by flow sensors, for measurements of flow rate and electrodes for measurements of resistance in a fluidic chamber, allows precision measurement of CSF flow rate over a long time and under any circumstances caused by unexpected or abnormal events. A universal design compatible with any modern commercial spinal fluid shunt system would enable the widespread use of this technology.

The Role of the Liliequist Membrane in the Third Ventriculostomy

Endoscopic third ventriculostomy (ETV) is a hydrocephalus treatment procedure that involves opening the Liliequist membrane (LM). However, LM anatomy has not been well-studied neuroendoscopically, because approach angles differ between descriptive and microsurgical anatomical explorations. Discrepancies in ETV efficacy, especially among children age 2 and younger, may be due to incomplete LM opening. The objective of this study was to characterize the LM anatomically from a neuroendoscopic perspective to better understand the impact of anatomical features during LM ostomy and the ETV success rate. Additionally, the ETV success score was tested to predict patient outcome after the intraoperatively difficult opening of LM. Fifty-four patients who underwent ETV were prospectively analyzed with a mean follow-up of 53.1 months (1–90 months). The ETV technical parameters of difficulty were validated by seven expert neurosurgeons. The pediatric population (44) of this study represents the majority of patients (81.4%). The overall ETV success rate was 68.5%. Anomalies on the IIIVT floor resulted in an increased rate of ETV failure. The IIIVT was anomalous, and LM was thick in 33.3% of cases. Fenestration of LM was difficult in 39% of cases, and the LM and TC were opened separately in 55.6% of cases. The endoscopic third ventriculostomy success score (ETVSS) accurately predicted the level of difficulty opening the LM (p = 0.012), and the group with easy opening presented greater durability in ETV success. Neurosurgeons should be aware of the difficulty level of the overture of LM during ETV and its impact on long-term ETV effectiveness.