Negative-pressure hydrocephalus in the course of a complex postoperative intracranial pressure disturbance: illustrative case.

BACKGROUND: Negative-pressure hydrocephalus (NePH) is a rare clinical entity that presents on the background of ventriculomegaly with atypical symptoms. Its diagnosis is difficult, and some patients experience several shunt revisions until the proper solution is found. OBSERVATIONS: The authors present a patient who developed acute deterioration due to iatrogenic NePH after surgery for a vertebral artery thrombosed giant aneurysm. The deterioration occurred after the insertion of a lumbar drain by which the authors intended to reduce a postoperative subcutaneous cerebrospinal fluid (CSF) collection. The drainage created an unexpected negative-pressure gradient in the CSF spaces, which resulted in NePH. Interventions, such as extraventricular drainage and blood patch, corrected the negative transmantle pressure and stabilized the patient's condition. LESSONS: Because the pathophysiology of NePH is theoretically considered to be caused by negative transmantle pressure, the intervention should be performed in order to deal with the coexistence of obstruction in the CSF pathways and a CSF leak. A blood patch would be an effective option in treating the CSF leak when the site of leakage is certain. This is the first case in which a blood patch was effectively applied in the treatment for NePH with a favorable outcome without any permanent CSF diversion.

Endoscopic third ventriculostomy for VP shunt malfunction during the third trimester of pregnancy: illustrative case.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is a successful procedure for treating noncommunicating hydrocephalus as an alternative to initial ventriculoperitoneal (VP) shunt placement and as a salvage procedure when a VP shunt fails. Physiological changes of pregnancy can lead to VP shunt failure and complicate the management of shunt malfunction, particularly in the third trimester. OBSERVATIONS: The authors present a case in which an ETV was successfully used in the third trimester (31 weeks of gestation) of pregnancy for acute hydrocephalus due to VP shunt malfunction, and the patient went on to deliver a healthy baby at term; the patient remained well in the long-term follow-up. An English-language PubMed literature review revealed four cases of VP shunt failure successfully treated with an ETV in the first or second trimester but no such reports in the third trimester of pregnancy. LESSONS: ETV appears to be a safe and effective alternative to VP shunt replacement in the late prenatal period of pregnancy.

Management challenges associated with a pineal region chordoma: illustrative case.

BACKGROUND: Chordomas, which are rare malignant neoplasms arising from notochordal remnants, often cause gradually progressive clinical symptoms. Intradural cranial chordomas (ICCs) are extremely rare and generally have a favorable prognosis. However, the authors reported the case of a primary ICC originating in the pineal gland presenting with recurrent thalamic hemorrhage and displaying an aggressive postoperative clinical course. OBSERVATIONS: A 41-year-old man arrived at the emergency department with morning headaches and recurrent syncope that had lasted several months. Computed tomography and magnetic resonance imaging (MRI) revealed a pineal gland mass causing obstructive hydrocephalus and a subacute hematoma in the right thalamus. Three weeks after an endoscopic third ventriculostomy was performed, recurrent hemorrhage was observed in the right thalamus. The tumor was promptly removed surgically. The yellowish-white tumor did not exhibit abundant bleeding. No evidence of intratumoral hemorrhage around the hematoma pocket was found. Histopathological examination revealed the characteristics of a chordoma with minimal vascularity. MRI performed 10 weeks postoperatively for worsening headaches revealed abnormal enhancement of multiple cranial nerves, suggesting leptomeningeal seeding (LMS) of the tumor. LESSONS: Despite radiotherapy and intrathecal chemotherapy, the patient's neurological status worsened; he died 2 years postoperatively. A pineal ICC may cause recurrent thalamic hemorrhage and potentially fatal LMS, even in the early postoperative period.

The quality of YouTube videos on endoscopic third ventriculostomy and endoscopic third ventriculostomy with choroid plexus cauterization procedures available to families of patients with pediatric hydrocephalus.

OBJECTIVE: YouTube has become an important information source for pediatric neurosurgical patients and their families. The goal of this study was to determine whether the informative quality of videos of endoscopic third ventriculostomy (ETV) and endoscopic third ventriculostomy with choroid plexus cauterization (ETV + CPC) is associated with metrics of popularity. METHODS: This cross-sectional study used comprehensive search terms to identify videos pertaining to ETV and ETV + CPC presented on the first 3 pages of search results on YouTube. Two pediatric neurosurgeons, 1 neurosurgery resident, and 2 patient families independently reviewed the selected videos. Videos were assessed for overall informational quality by using a validated 5-point Global Quality Score (GQS) and compared to online metrics of popularity and engagement such as views, likes, likes/views ratio, comments/views ratio, and likes/dislikes ratio. Weighted kappa scores were used to measure agreement between video reviewers. RESULTS: A total of 58 videos (47 on ETV, 7 on ETV + CPC, 4 on both) of 120 videos assessed met the inclusion criteria. Video styles included "technical" (62%), "lecture" (24%), "patient testimonial" (4%), and "other" (10%). In terms of GQS, substantial agreement was seen between surgeons (kappa 0.67 [95% CI 0.55, 0.80]) and excellent agreement was found between each surgeon and the neurosurgical resident (0.77 [95% CI 0.66, 0.88] and 0.89 [95% CI 0.82, 0.97]). Only fair to moderate agreement was seen between professionals and patient families, with weighted kappa scores ranging from 0.07 to 0.56. Academic lectures were more likely to be rated good or excellent (64% vs 0%, p < 0.001) versus surgical procedure and testimonial video types. There were significant associations between a better GQS and more likes (p = 0.01), views (p = 0.02), and the likes/dislikes ratio (p = 0.016). The likes/views ratio (p = 0.31) and comments/views ratio (p = 0.35) were not associated with GQS. The number of likes (p = 0.02), views (p = 0.03), and the likes/dislikes ratio (p = 0.015) were significantly associated with video style (highest for lecture-style videos). CONCLUSIONS: Medical professionals tended to agree when assessing the overall quality of YouTube videos, but this agreement was not as strongly seen when compared to parental ratings. The online metrics of likes, views, and likes/dislikes ratio appear to predict quality. Neurosurgeons seeking to increase their online footprint via YouTube would be well advised to focus more on the academic lecture style because these were universally better rated.

Development and validation of a synthetic 3D-printed simulator for training in neuroendoscopic ventricular lesion removal.

OBJECTIVE: Neuroendoscopic surgery using an ultrasonic aspirator represents a valid tool with which to perform the safe resection of deep-seated ventricular lesions, but the handling of neuroendoscopic instruments is technically challenging, requiring extensive training to achieve a steep learning curve. Simulation-based methods are increasingly used to improve surgical skills, allowing neurosurgical trainees to practice in a risk-free, reproducible environment. The authors introduce a synthetic, patient-specific simulator that enables trainees to develop skills for endoscopic ventricular tumor removal, and they evaluate the model's validity as a training instrument with regard to realism, mechanical proprieties, procedural content, and handling. METHODS: The authors developed a synthetic simulator based on a patient-specific CT data set. The anatomical features were segmented, and several realistic 1:1 skull models with all relevant ventricular structures were fabricated by a 3D printer. Vascular structures and the choroid plexus were included. A tumor model, composed of polyvinyl alcohol, mimicking a soft-consistency lesion, was secured in different spots of the frontal horn and within the third ventricle. Neurosurgical trainees participating in a neuroendoscopic workshop qualitatively assessed, by means of a feedback survey, the properties of the simulator as a training model that teaches neuroendoscopic ultrasonic ventricular tumor surgery; the trainees rated 10 items according to a 5-point Likert scale. RESULTS: Participants appreciated the model as a valid hands-on training tool for neuroendoscopic ultrasonic aspirator tumor removal, highly rating the procedural content. Furthermore, they mostly agreed on its comparably realistic anatomical and mechanical properties. By the model's first application, the authors were able to recognize possible improvement measures, such as the development of different tumor model textures and the possibility, for the user, of creating a realistic surgical skull approach and neuroendoscopic trajectory. CONCLUSIONS: A low-cost, patient-specific, reusable 3D-printed simulator for the training of neuroendoscopic ultrasonic aspirator tumor removal was successfully developed. The simulator is a useful tool for teaching neuroendoscopic techniques and provides support in the development of the required surgical skills.

A hospital-based analysis of pseudomeningoceles after elective craniotomy in children: what predicts need for intervention?

OBJECTIVE: In pediatric patients, the development of a postoperative pseudomeningocele after an elective craniotomy is not unusual. Most will resolve with time, but some may require intervention. In this study, the authors analyzed patients who required intervention for a postoperative pseudomeningocele following an elective craniotomy or craniectomy and identified factors associated with the need for intervention. METHODS: An institutional operative database of elective craniotomies and craniectomies was queried to identify all surgeries associated with development of a postoperative pseudomeningocele from January 1, 2010, to December 31, 2017. Demographic and surgical data were collected, as were details regarding postoperative events and interventions during either the initial admission or upon readmission. A bivariate analysis was performed to compare patients who underwent observation with those who required intervention. RESULTS: Following 1648 elective craniotomies or craniectomies, 84 (5.1%) clinically significant pseudomeningoceles were identified in 82 unique patients. Of these, 58 (69%) of the pseudomeningoceles were diagnosed during the index admission (8 of which persisted and resulted in readmission), and 26 (31%) were diagnosed upon readmission. Forty-nine patients (59.8% of those with a pseudomeningocele) required one or more interventions, such as lumbar puncture(s), lumbar drain placement, wound exploration, or shunt placement or revision. Only race (p < 0.01) and duraplasty (p = 0.03, OR 3.0) were associated with the need for pseudomeningocele treatment. CONCLUSIONS: Clinically relevant pseudomeningoceles developed in 5% of patients undergoing an elective craniotomy, with 60% of these pseudomeningoceles needing some form of intervention. The need for intervention was associated with race and whether a duraplasty was performed.

Does pre-resection endoscopic third ventriculostomy prevent the need for post-resection CSF diversion after pediatric posterior fossa tumor excision? A historical cohort study and review of the literature.

OBJECTIVE: Children with posterior fossa tumors (PFTs) may present with hydrocephalus. Persistent (or new) hydrocephalus is common after PFT resection. Endoscopic third ventriculostomy (ETV) is sometimes performed prior to resection to 1) temporize hydrocephalus prior to resection and 2) prophylactically treat post-resection hydrocephalus. The objective of this study was to establish, in a historical cohort study of pediatric patients who underwent primary craniotomy for PFT resection, whether or not pre-resection ETV prevents the need for post-resection CSF diversion to manage hydrocephalus. METHODS: The authors interrogated their prospectively maintained surgical neuro-oncology database to find all primary PFT resections from a single tertiary pediatric neurosurgery unit. These data were reviewed and supplemented with data from case notes and radiological review. The modified Canadian Preoperative Prediction Rule for Hydrocephalus (mCPPRH) score was retrospectively calculated for all patients. The primary outcome was the need for any form of postoperative CSF diversion within 6 months of PFT resection (including ventriculoperitoneal shunting, ETV, external ventricular drainage [EVD], and lumbar drainage [LD]). This was considered an ETV failure in the ETV group. The secondary outcomes were time to CSF diversion, shunt dependence at 6 months, and complications of ETV. Statistical analysis was done in RStudio, with significance defined as p < 0.05. RESULTS: A total of 95 patients were included in the study. There were 28 patients in the ETV group and 67 in the non-ETV group. Patients in the ETV group were younger (median age 5 vs 7 years, p = 0.04) and had more severe preoperative hydrocephalus (mean frontal-occipital horn ratio 0.45 vs 0.41 in the non-ETV group, p = 0.003) and higher mCPPRH scores (mean 4.42 vs 2.66, p < 0.001). The groups were similar in terms of sex and tumor histology. The overall rate of post-resection CSF diversion of any kind (shunt, repeat ETV, LD, or EVD) in the entire cohort was 25.26%. Post-resection CSF diversion was needed in 32% of patients in the ETV group and in 22% of the patients in the non-ETV group (p > 0.05). Shunt dependence at 6 months was seen in 21% of the ETV group and 16% of the non-ETV group (p > 0.05). The median time to ETV failure was 9 days. ETV failure correlated with patients with ependymoma (p = 0.02). Children who had ETV failure had higher mCPPRH scores than the ETV success group (5.67 vs 3.84, p = 0.04). CONCLUSIONS: Pre-resection ETV did not reliably prevent the need for post-resection CSF diversion. ETV was more likely to fail in children with ependymoma and those with higher mCPPRH scores. Based on the findings of this study, the authors will change the practice at their institution; pre-resection ETV will now be performed based on a newly defined protocol.

Endoscopic third ventriculostomy for pediatric tumor-associated hydrocephalus.

OBJECTIVE: Surgical options for managing hydrocephalus secondary to CNS tumors have traditionally included ventriculoperitoneal shunting (VPS) when tumor resection or medical management alone are ineffective. Endoscopic third ventriculostomy (ETV) has emerged as an attractive treatment strategy for tumor-associated hydrocephalus because it offers a lower risk of infection and hardware-related complications; however, relatively little has been written on the topic of ETV specifically for the treatment of tumor-associated hydrocephalus. Here, the authors reviewed the existing literature on the use of ETV in the treatment of tumor-associated hydrocephalus, focusing on the frequency of ETV use and the failure rates in patients with hydrocephalus secondary to CNS tumor. METHODS: The authors queried PubMed for the following terms: "endoscopic third ventriculostomy," "tumor," and "pediatric." Papers with only adult populations, case reports, and papers published before the year 2000 were excluded. The authors analyzed the etiology of hydrocephalus and failure rates after ETV, and they compared failure rates of ETV with those of VPS where reported. RESULTS: Thirty-two studies with data on pediatric patients undergoing ETV for tumor-related hydrocephalus were analyzed. Tumors, particularly in the posterior fossa, were reported as the etiology of hydrocephalus in 38.6% of all ETVs performed (984 of 2547 ETVs, range 29%-55%). The ETV failure rate in tumor-related hydrocephalus ranged from 6% to 38.6%, and in the largest studies analyzed (> 100 patients), the ETV failure rate ranged from 10% to 38.6%. The pooled ETV failure rate was 18.3% (199 failures after 1087 procedures). The mean or median follow-up for ETV failure assessment ranged from 6 months to 8 years in these studies. Only 5 studies directly compared ETV with VPS for tumor-associated hydrocephalus, and they reported mixed results in regard to failure rate and time to failure. Overall failure rates appear similar for ETV and VPS over time, and the risk of infection appears to be lower in those patients undergoing ETV. The literature is mixed regarding the need for routine ETV before resection for posterior fossa tumors with associated hydrocephalus. CONCLUSIONS: Treatment of tumor-related hydrocephalus with ETV is common and is warranted in select pediatric patient populations. Failure rates are overall similar to those of VPS for tumor-associated hydrocephalus.

Endoscopic third ventriculostomy with choroid plexus cauterization for the treatment of infantile hydrocephalus in Haiti.

OBJECTIVE: Untreated hydrocephalus poses a significant health risk to children in the developing world. In response to this risk, global neurosurgical efforts have increasingly focused on endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) in the management of infantile hydrocephalus in low- and middle-income countries (LMICs). Here, the authors report their experience with ETV/CPC at the Hospital Bernard-Mevs/Project Medishare (HBMPM) in Port-au-Prince, Haiti. METHODS: The authors conducted a retrospective review of a series of consecutive children who had undergone ETV/CPC for hydrocephalus over a 1-year period at HBMPM. The primary outcome of interest was time to ETV/CPC failure. Univariate and multivariate analyses using a Cox proportional hazards regression were performed to identify preoperative factors that were associated with outcomes. RESULTS: Of the 82 children who underwent ETV/CPC, 52.2% remained shunt free at the last follow-up (mean 6.4 months). On univariate analysis, the ETV success score (ETVSS; p = 0.002), success of the attempted ETV (p = 0.018), and bilateral CPC (p = 0.045) were associated with shunt freedom. In the multivariate models, a lower ETVSS was independently associated with a poor outcome (HR 0.072, 95% CI 0.016-0.32, p < 0.001). Two children (2.4%) died of postoperative seizures. CONCLUSIONS: As in other LMICs, ETV/CPC is an effective treatment for hydrocephalus in children in Haiti, with a low but significant risk profile. Larger multinational prospective databases may further elucidate the ideal candidate for ETV/CPC in resource-poor settings.

Rate-controlled intraventricular endoscopic irrigation via bipolar foot pedal activation: technical note.

Irrigation during intraventricular endoscopic surgery is critical for visualization, with normal intracranial pressure maintained by balancing fluid ingress and egress. Although irrigation is typically achieved through manual manipulation of inexact stopcocks, the authors have developed a rate-controlled, foot pedal-activated system for precise intraventricular irrigation by using a standard irrigating bipolar electrocautery machine.This study is a retrospective review of patients who underwent endoscopic intraventricular surgery between January 1, 2018, and September 25, 2019, in which this irrigation system was used. Important components of this system include a bipolar module irrigation regulator that is set to a desired rate, a secure connection of the bipolar irrigation tubing to the endoscope, and one or more open egress ports on the endoscope for passive fluid drainage. Nineteen consecutive patients were identified on review (average age ± SD, 4.3 ± 4.1 years). Procedures performed included third ventriculostomies (n = 10); arachnoid/choroid cyst fenestrations/resections (n = 3); biopsy/tumor resection (n = 1); and combined procedures (n = 5). Foot pedal-controlled irrigation provided visualization of all intraventricular structures. A single operator was able to control the endoscope, endoscopic instruments, and irrigation, with assistance as indicated for more complex maneuvers. There were no perioperative complications. Because this setup is easily constructed from a standard irrigating bipolar machine, delivers precise irrigation flow rates, and facilitates a single-surgeon bimanual technique, these data support the utility of foot-controlled irrigation for endoscopic intraventricular surgery.

Graft dural closure is associated with a reduction in CSF leak and hydrocephalus in pediatric patients undergoing posterior fossa brain tumor resection.

OBJECTIVE: The authors aimed to evaluate clinical, radiological, and surgical factors associated with posterior fossa tumor resection (PFTR)-related outcomes, including postoperative complications related to dural augmentation (CSF leak and wound infection), persistent hydrocephalus ultimately requiring permanent CSF diversion after PFTR, and 90-day readmission rate. METHODS: Pediatric patients (0-17 years old) undergoing PFTR between 2000 and 2016 at Monroe Carell Jr. Children's Hospital of Vanderbilt University were retrospectively reviewed. Descriptive statistics included the Wilcoxon signed-rank test to compare means that were nonnormally distributed and the chi-square test for categorical variables. Variables that were nominally associated (p < 0.05) with each outcome by univariate analysis were included as covariates in multivariate linear regression models. Statistical significance was set a priori at p < 0.05. RESULTS: The cohort consisted of 186 patients with a median age at surgery of 6.62 years (range 3.37-11.78 years), 55% male, 83% Caucasian, and average length of follow-up of 3.87 ± 0.25 years. By multivariate logistic regression, the variables primary dural closure (PDC; odds ratio [OR] 8.33, 95% confidence interval [CI] 1.07-100, p = 0.04), pseudomeningocele (OR 7.43, 95% CI 2.23-23.76, p = 0.0007), and hydrocephalus ultimately requiring permanent CSF diversion within 90 days of PFTR (OR 9.25, 95% CI 2.74-31.2, p = 0.0003) were independently associated with CSF leak. PDC versus graft dural closure (GDC; 35% vs 7%, OR 5.88, 95% CI 2.94-50.0, p = 0.03) and hydrocephalus ultimately requiring permanent CSF diversion (OR 3.30, 95% CI 1.07-10.19, p = 0.0007) were associated with wound infection requiring surgical debridement. By multivariate logistic regression, GDC versus PDC (23% vs 37%, OR 0.13, 95% CI 0.02-0.87, p = 0.04) was associated with persistent hydrocephalus ultimately requiring permanent CSF diversion, whereas pre- or post-PFTR ventricular size, placement of peri- or intraoperative extraventricular drain (EVD), and radiation therapy were not. Furthermore, the addition of perioperative EVD placement and dural closure method to a previously validated predictive model of post-PFTR hydrocephalus improved its performance from area under the receiver operating characteristic curve of 0.69 to 0.74. Lastly, the authors found that autologous (vs synthetic) grafts may be protective against persistent hydrocephalus (p = 0.02), but not CSF leak, pseudomeningocele, or wound infection. CONCLUSIONS: These results suggest that GDC, independent of potential confounding factors, may be protective against CSF leak, wound infection, and hydrocephalus in patients undergoing PFTR. Additional studies are warranted to further evaluate clinical and surgical factors impacting PFTR-associated complications.

Thirty-day medical and surgical readmission following prenatal versus postnatal myelomeningocele repair.

OBJECTIVE: Hospital readmission is an important quality metric that has not been evaluated in prenatal versus postnatal myelomeningocele (MMC) repair. This study compares hospital readmission outcomes between these two groups as well as their etiologies. METHODS: The medical records of patients who had undergone MMC repair in the period from 2011 to 2017 at a single academic medical center were retrospectively reviewed. Collected clinical data included surgery and defect details, neonatal intensive care unit (NICU) stay, and any readmissions or surgical procedures up to 1 year after surgery. Patient and defect characteristics, readmission outcomes at 30 and 60 days and 1 year after discharge from the NICU, and cerebrospinal fluid (CSF) diversion surgery rates were analyzed with the two-tailed t-test and/or k-sample test on the equality of medians. RESULTS: A total of 24 prenatal and 34 postnatal MMC repairs were completed during the study period. Prenatally repaired patients were born more prematurely (p < 0.001) and with lower birth weights (p < 0.001), although the NICU stay was similar between the two groups (p = 0.59). Fewer prenatally repaired patients were readmitted at 30 days (p = 0.005), 90 days (p = 0.004), and 1 year (p = 0.007) than the postnatal repair group. Hydrocephalus was the most common readmission etiology, and 29% of prenatal repair patients required CSF diversion at 1 year versus 81% of the postnatal repair group (p < 0.01). Prenatal patients who required CSF diversion had a higher body weight (p = 0.02) and an older age (p = 0.01) at the time of CSF diversion surgery than the postnatal group. CONCLUSIONS: Patients with prenatal MMC repair had fewer hospital readmissions at 30 days, 60 days, and 1 year than the postnatal repair group, despite similar NICU lengths of stay. The prenatal repair group had lower requirements for CSF diversion at 1 year and was older with greater body weights at the time of CSF diversion surgery, compared to those of the postnatal repair group. Future study of hospital quality metrics such as readmissions should be performed to better understand outcomes of these two procedures.

Cost of prenatal versus postnatal myelomeningocele closure for both mother and child at 1 year of life.

OBJECTIVE: Prenatal myelomeningocele (MMC) closure has been performed in the United States for 2 decades. While prior work has focused on clinical outcomes of prenatal MMC closure, the cost of this procedure in comparison with that of postnatal MMC closure is unclear. The authors' aim was to compare the cost of prenatal versus postnatal MMC closure for both the child and mother at 1 year. METHODS: A prospective database of patients undergoing prenatal and postnatal MMC closure between 2011 and 2018 with 1-year follow-up was retrospectively reviewed. Charge data for relevant admissions were converted to a cost estimate using the authors' institution's Medicare hospital-specific cost-to-charge ratio. Children, mothers, and mother/child pairs were considered separately. The primary outcome was cost. Secondary outcomes included the need for hydrocephalus treatment, length of stay (LOS), and readmissions. Other covariates included gestational age at birth, MMC lesion level, and obstetric complications. RESULTS: The median cost of care for children in the prenatal group was greater, although not significantly so, at $58,406.71 (IQR $16,900.24-$88,951.01) compared with $49,889.95 (IQR $38,425.18-$115,163.86) for children in the postnatal group (p = 0.204). The median cost for mothers in the prenatal group was significantly greater at $24,548.29 (IQR $20,231.55-$36,862.31) compared with $5087.30 (IQR $4430.72-$5362.56) (p < 0.001). The median cost for mother/child pairs in the prenatal group was $102,377.75 (IQR $37,384.30-$118,527.74) compared with $55,667.82 (IQR $42,840.78-$120,058.06) (p = 0.45). Children in the prenatal group had a lower gestational age at birth (235.81 days vs 265.77 days, p < 0.001) and fewer readmissions (33.3% vs 72.7%, p < 0.001), and hydrocephalus treatment was less common (33.3% vs 90.9%, p < 0.001). Index LOS did not differ between children in the prenatal and postnatal groups (26.8 days vs 23.5 days, p = 0.63). Mothers in the prenatal group had longer LOS (15.92 days vs 4.68 days, p < 0.001) and more readmissions (18.5% vs 0.0%, p = 0.06). CONCLUSIONS: The median cost of prenatal versus postnatal MMC closure did not significantly differ from a hospital perspective at 1 year, although variability in cost was high for both groups. When considering the mother alone, prenatal MMC closure was costlier. Future work is needed to assess cost from a patient and societal perspective both at 1 year and beyond.

Growing up with spina bifida: bridging the gaps in the transition of care from childhood to adulthood.

Spina bifida is the most common nonchromosomal birth defect, resulting in permanent disability of multiple organ systems, yet compatible with long-term survival. Important advances across various disciplines have now improved survival among the spina bifida population. Although the majority of individuals living with spina bifida are now adults, there are few publications in the neurosurgical literature regarding the care of adults with spina bifida, associated medical conditions, surgical interventions, and long-term complications. The major goals for transitioning adult patients with spina bifida are preservation of function and promotion of independence as well as general overall health. Nevertheless, many gaps exist in our knowledge and understanding of the complex needs of this aging patient population. The goal of this paper was to provide a comprehensive updated review of the literature regarding the challenges and considerations involved in the transitional care to adulthood for patients with spina bifida. Unique to this review, the authors provide a first-hand personal communication and interview with an adult patient with spina bifida that discusses many of these challenges with transition.

Reviewing the prognostic factors in myelomeningocele.

OBJECTIVE: The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC). METHODS: A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of children who underwent the primary repair of MMC at the Fernandes Figueira Institute-Oswaldo Cruz Foundation (IFF-Fiocruz) between January 1995 and January 2015, with a minimum follow-up of 1 year. The following variables were analyzed: demographic characteristics (gestational age, sex, and birth weight); clinical features (head circumference at birth, anatomical and functional levels of MMC, hydrocephalus, symptomatic Chiari malformation type II, neurogenic bladder, and urinary tract infection [UTI]); and surgical details such as timing of repair of MMC, age at first shunt placement, shunt surgery modality (elective or emergency), concurrent surgery (correction of MMC and shunt insertion in the same surgical procedure), incidence and cause of shunt dysfunction, use of external ventricular drain, transfontanelle puncture, surgical wound complications prior to shunting, and endoscopic treatment of hydrocephalus. RESULTS: A total of 231 patients with MMC were included in the analysis. Patients were followed for periods ranging from 1 to 20 years, with a mean of 6.9 years. The frequency of shunt placement was observed mainly among patients with MMC at the highest spinal levels (p < 0.01). The main causes of morbidity and mortality in patients with MMC were shunt failures, diagnosed in 91 of 193 cases (47.2%) of hydrocephalus, and repeated UTIs, in 129 of 231 cases (55.8%) of MMC; these were the main causes of hospitalization and death. Head circumference ≥ 38 cm at birth was found to be a significant risk factor for shunt revision (p < 0.001; 95% CI 1.092-1.354). Also, the lumbar functional level of MMC was associated with less revision than upper levels (p < 0.014; 95% CI 0.143-0.805). There was a significant association between recurrent UTI and thoracic functional level. CONCLUSIONS: Macrocephaly at birth and higher levels of the defect have an impact on worse outcome and, therefore, are a challenge to the daily practice of pediatric neurosurgery.

The role of sacral laminoplasty in the management of spina bifida and sacral cystic lesions: case series.

OBJECTIVE: Although laminae are not viewed as essential structures for spinal integrity, in the sacrum the anatomical weakness and gravity makes it a vulnerable area for CSF accumulation and expansion. The congenital or postoperative defects of sacral laminae, such as in patients with spina bifida, make this area more susceptible to forming progressive dural ectasia, pseudomeningocele, or expansile arachnoid cyst (Tarlov cyst). In addition, adhesions between the dura and surrounding soft tissue after laminectomy can cause some local symptoms, which are difficult to relieve. The authors propose that sacral laminoplasty with titanium mesh can provide a rigid support and barrier to resolve these sacral lesions and local symptoms. METHODS: From January 2016 to December 2017, patients with progressive CSF-containing lesions in the sacral area and defective sacral laminae were included in the study. After repair of the lesion, the authors performed sacral laminoplasty with titanium mesh in each patient. Subsequently, the soft tissue and skin were closed primarily. RESULTS: A total of 6 patients were included. Four patients with repaired myelomeningocele had progressive dural ectasia. One patient with lipomyelomeningocele previously underwent detethering surgery and developed postoperative pseudomeningocele. One patient had a symptomatic Tarlov cyst. Four of these 6 cases presented with low-back pain and local tenderness. During follow-up, ranging from 13 to 37 months, all 6 patients experienced no recurrence of dural ectasia or pseudomeningocele and were free from local symptoms. CONCLUSIONS: Sacral laminoplasty with titanium mesh is a safe and effective procedure for treating progressive sacral dural ectasia and refractory pseudomeningocele, preventing CSF leakage as well as relieving local symptoms that may occur years after previous surgery for spina bifida.

Contemporary management and outcome of myelomeningocele: the Rotterdam experience.

OBJECTIVE: Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases. METHODS: This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence. RESULTS: A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence. CONCLUSIONS: This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review.

OBJECTIVE: Myelomeningocele (MMC), the most severe form of spina bifida, is characterized by protrusion of the meninges and spinal cord through a defect in the vertebral arches. The management and prevention of MMC-associated hydrocephalus has evolved since its initial introduction with regard to treatment of MMC defect, MMC-associated hydrocephalus treatment modality, and timing of hydrocephalus treatment. METHODS: The Nationwide Inpatient Sample (NIS) database from the years 1998-2014 was reviewed and neonates with spina bifida and hydrocephalus status were identified. Timing of hydrocephalus treatment, delayed treatment (DT) versus simultaneous MMC repair with hydrocephalus treatment (ST), and treatment modality (ETV vs ventriculoperitoneal shunt [VPS]) were analyzed. Yearly trends were assessed with univariable logarithmic regression. Multivariable logistic regression identified correlates of inpatient shunt failure. A PRISMA systematic literature review was conducted that analyzed data from studies that investigated 1) MMC closure technique and hydrocephalus rate, 2) hydrocephalus treatment modality, and 3) timing of hydrocephalus treatment. RESULTS: A weighted total of 10,627 inpatient MMC repairs were documented in the NIS, 8233 (77.5%) of which had documented hydrocephalus: 5876 (71.4%) were treated with VPS, 331 (4.0%) were treated with ETV, and 2026 (24.6%) remained untreated on initial inpatient stay. Treatment modality rates were stable over time; however, hydrocephalic patients in later years were less likely to receive hydrocephalus treatment during initial inpatient stay (odds ratio [OR] 0.974, p = 0.0331). The inpatient hydrocephalus treatment failure rate was higher for patients who received ETV treatment (17.5% ETV failure rate vs 7.9% VPS failure rate; p = 0.0028). Delayed hydrocephalus treatment was more prevalent in the later time period (77.9% vs 69.5%, p = 0.0287). Predictors of inpatient shunt failure included length of stay, shunt infection, jaundice, and delayed treatment. A longer time between operations increased the likelihood of inpatient shunt failure (OR 1.10, p < 0.0001). However, a meta-analysis of hydrocephalus timing studies revealed no difference between ST and DT with respect to shunt failure or infection rates. CONCLUSIONS: From 1998 to 2014, hydrocephalus treatment has become more delayed and the number of hydrocephalic MMC patients not treated on initial inpatient stay has increased. Meta-analysis demonstrated that shunt malfunction and infection rates do not differ between delayed and simultaneous hydrocephalus treatment.

Pediatric neurosurgeons' views regarding prenatal surgery for myelomeningocele and the management of hydrocephalus: a national survey.

OBJECTIVE: The Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons' risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial. METHODS: A cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons' risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression. RESULTS: Compared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either "very favorable" or "somewhat favorable," and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts. CONCLUSIONS: The majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

Outcomes of hydrocephalus secondary to congenital toxoplasmosis.

OBJECTIVE: Hydrocephalus occurs in children with congenital toxoplasmosis and can lead to severe disability. In these cases, the decision to intervene is often influenced by the expectation of neurological recovery. In this study, clinical responses to neurosurgical intervention in children with hydrocephalus secondary to congenital toxoplasmosis are characterized. METHODS: Sixty-five participants with hydrocephalus due to congenital Toxoplasma gondii infection were evaluated as part of the National Collaborative Chicago-based Congenital Toxoplasmosis Study, and their neuroradiographic findings were reviewed. Clinical outcomes were scored on the basis of cognition and motor skills through the use of IQ scores and Gross Motor Function Classification System (GMFCS) level. Outcomes were then analyzed in relation to approach to management, anatomy of hydrocephalus, and time from diagnosis of hydrocephalus to surgical intervention. RESULTS: There was considerable variation in the outcomes of patients whose hydrocephalus was treated in early life, ranging from normal cognitive and motor function to profound developmental delay and functional limitation. Of the 65 participants included in the study, IQ and GMFCS level were available for 46 (70.8%). IQ and motor score were highly correlated (r = -0.82, p < 0.001). There were people with differing patterns of hydrocephalus or thickness of cortical mantle on initial presentation who had favorable outcomes. Time to neurosurgical intervention data were available for 31 patients who underwent ventriculoperitoneal (VP) shunt placement. Delayed shunt placement beyond 25 days after diagnosis of hydrocephalus was associated with greater cognitive impairment (p = 0.02). Motor impairment also appeared to be associated with shunt placement beyond 25 days but the difference did not achieve statistical significance (p = 0.13). Among those with shunt placement within 25 days after diagnosis (n = 19), the mean GMFCS level was 1.9 ± 1.6 (range 1-5). Five (29.4%) of 17 of these patients were too disabled to participate in formal cognitive testing, after excluding 2 patients with visual difficulties or language barriers that precluded IQ testing. Of the patients who had VP shunt placement 25 or more days after diagnosis (n = 12), the mean GMFCS level was 2.7 ± 1.4 (range 1-4). Of these, 1 could not participate in IQ testing due to severe visual difficulties and 8 (72.7%) of the remaining 11 due to cognitive disability. CONCLUSIONS: VP shunt placement in patients with hydrocephalus caused by congenital toxoplasmosis can contribute to favorable clinical outcomes, even in cases with severe hydrocephalus on neuroimaging. Shunt placement within 25 days of diagnosis was statistically associated with more favorable cognitive outcomes. Motor function appeared to follow the same pattern although it did not achieve statistical significance.